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Neurology Self-Assessment

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Instructor in Neurology. TX. MAZZIOTTA. TX Foreword by JOHN C. Temple. Dean. Harvard Medical School. MD Assistant in Neurology. PHD Vice Chancellor. SOILEAU. Baylor Scott & White Health. David Geffen School of Medicine. Texas A&M Health Science Center. University of California. KS MICHAEL J.Neurology Self-Assessment: A Companion to Bradley’s Neurology in Clinical Practice JUSTIN T. Massachusetts General Hospital. CEO. UCLA Health Sciences. Neurology Residency. Clinical Assistant Professor and Assistant Program Director. Boston. MD. Los Angeles. JORDAN. Wichita. MAYANS. MD Neurology Consultants of Kansas. University of Kansas School of Medicine. CA For additional online content visit ExpertConsult. UCLA Health. MD Director. Temple. Clinical Assistant Edinburgh  London  New York  Oxford  Philadelphia  St Louis  Sydney  Toronto 2017 . Plummer Movement Disorder Center. MA DAVID R.

or from any use or operation of any methods. With respect to any drug or pharmaceutical products identified. or any information storage and retrieval system. recording. neither the Publisher nor the authors. Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using any information. All rights reserved. methods. As new research and experience broaden our understanding. or medical treatment may become necessary. negligence or otherwise. to determine dosages and the best treatment for each individual patient. changes in research methods. without permission in writing from the publisher. Details on how to seek permission. products. compounds. including photocopying. It is the responsibility of practitioners. electronic or mechanical. to make diagnoses. further information about the Publisher’s permissions policies and our arrangements with organizations such as the Copyright Clearance Center and the Copyright Licensing Agency.elsevier. Notices Knowledge and best practice in this field are constantly changing. professional practices. Elsevier Limited/Inc. or experiments described herein. In using such information or methods they should be mindful of their own safety and the safety of others. and to take all appropriate safety precautions. can be found at our website: www. ISBN:  978-0-323-37709-6 eISBN:  978-0-323-39221-1 Senior Content Strategist:  Lotta Kryhl Senior Content Development Specialist:  Ailsa Laing Project Manager:  Louisa Talbott Designer:  Miles Hitchen Marketing Manager:  Michele Milano Printed in China Last digit is the print number:  9  8  7  6  5  4  3  2  1 . or ideas contained in the material herein. to verify the recommended dose or formula. or editors. No part of this publication may be reproduced or transmitted in any form or by any means. the method and duration of administration. contributors. To the fullest extent of the law. and contraindications. assume any liability for any injury and/or damage to persons or property as a matter of products liability. relying on their own experience and knowledge of their patients.© 2017. including parties for whom they have a professional responsibility. readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered.

  137 Test Five Answers.  120 Test Five Questions.  86 Test Four Questions.  viii Preface.  53 Test Three Questions. Mazziotta.  1 Test One Answers.  169 Test Six Answers. Contents Dedications.  ix Test One Questions.  69 Test Three Answers.  201 v .  vii Foreword by John C.  152 Test Six Questions.  103 Test Four Answers.  35 Test Two Answers.  17 Test Two Questions.  185 Index.

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for his encouragement through this process. MJS . JTJ I want to thank Laura and Gabriella for their patience with me while writing this book. who inspired my career. I would like to thank my mentors. I want to thank JJ for giving me the opportunity to be part of this exceptional book. DRM I wish to thank my wife Janiece and son Mason for supporting me throughout my career and for their love each day. and my family for their endless support.To my father. Finally. particularly Nikhil Balakrishnan. I’d also like to thank the many mentors and colleagues along the way who have molded me into the physician I am today and for igniting excitement for the field of neurology.

page-by-page. I felt I knew which areas required more study and Drs. consider the answers. reinforces Bradley’s Neurology in Clinical Practice. are response exercise. particularly is. This text provides exactly that stimulus- Physicians. in particular. ethics. make a selection. The Scott L. This assessment text. After that Practice. That the answers are referenced to Bradley’s always motivated to test their knowledge. A multiple-choice Neurology in Clinical Practice further provides the user with the question is an opportunity to see how deep one’s knowledge opportunity to read in more depth about a topic. and Soileau accomplishes sistent with the long-known neuroscience principle that a these goals admirably and is the perfect companion for stimulus and its response. Mazziotta. I speak on behalf of the four of us in complimenting exercise. Foreword It is difficult to write self-assessment texts. and then quickly refer to the answer is con- Assessment by Drs. Daroff. MD Such an offering is vital to high-quality patient care and pro- fessional requirements such as in-service examinations. board Joseph Jankovic. PhD range of the questions’ difficulty will be valuable for everyone interested in the field of neurology. Its six 105-question examinations will test John C. This was a laborious but rewarding task. comprehensive in scope and practical in usage. and interpersonal skills. Seventh Edition. if that person has answered incorrectly. self-assessment text provides all of that for the readers with no effort on their part. Jordan. when quickly rewarded. I obtained a recent edition of one of The trio has done a masterful job in providing a compre- the most comprehensive texts in the field at that time. Mayans. and neurologists. Neurology Self. and Soileau on their very successful self- where I should be confident with my state of knowledge. Pomeroy. They need to be The ability to examine a question. MD. PhD the readers’ knowledge across a wide range of topics. viii . Mayans. from medical students to seasoned practitioners. the information. and figure-by. table-by-table. When I was preparing for my board examination in Psy. in general. As one of the co-editors of Bradley’s Neurology in Clinical content. and allow for a thorough self-assessment of their knowledge figure and made mental multiple-choice questions out of its base. Jordan. Not only for the editors of Bradley’s Neurology in Clinical Practice does the self-assessment include all aspects of clinical neurol- ogy but also basic science. chiatry and Neurology. I went hensive set of 630 questions that will challenge the readers through the book. MD examinations. and maintenance of certification testing. MD. Robert B.

images. fication. On the whole. Mayans. This translates to improved quality of care for patients nations. and maintenance of certi. Justin T. The depth of knowledge required to perform well on As our drive for scientific progress continues. board examinations. with in-depth Michael J. Questions are written in a style similar to full exami- behind. Jordan. Soileau. MD Not only are the six 105-question examinations a tool for David R. It was in this context that we three approached Neu- rology Self-Assessment. neurologic expertise for practitioners at all levels. effective and revolutionary therapies follow close Practice. Preface The field of neurology is more exciting and rapidly advancing explanations for both correct and incorrect answers. and as new light is shed on the etiology further reading suggestions from Bradley’s Neurology in Clinical of disease. neurologists these examinations is likely more than sufficient to pass pro- should make every effort to maintain a firm understanding of fessional examinations. MD assessing one’s strengths and weaknesses. patients. although should not be beyond the available data in order to provide the best care possible for grasp of medical students. This necessity is not only apparent in daily practice. MD ix . but they also provide a valuable resource for exam studying. with a strong representation of clinical vignettes and and improved satisfaction for neurologists. we believe that Neurology Self-Assessment will but also in professional requirements including in-service become a strong tool in the development and maintenance of examinations. as well as today than ever before.

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Tyrosine D. Convulsive seizures during pregnancy can have all of the following effects on the developing fetus except? A. Major histocompatibility failure E. Molecular mimicry E.Test One QUESTIONS 2. L-Dopa C. Magnesium Her neurological examination is remarkable for D.  D. Diagnoses D. which is worsening in intensity. Which of the following is not a means by which A. Dopamine beta hydroxylase converts dopamine to which of the following? A. Environmental factors 4. In reference to the patient in the previous question. Which of the E. Treatment plan E. The B. Diamox pregnant.1. Enoxaparin following is the most likely diagnosis? 3. Venous sinus thrombosis B. Acetaminophen and rest headache is worse at night and causes blurry vision. Family history C. Identification B. The sagittal T1 magnetic resonance image (MRI) is shown here. A 23-year-old female presents to the clinic for treatment? evaluation of a headache. Fetal bradycardia and asphyxia B. Genetic predisposition C. Norepinephrine 5. Which of the following is not a standard component of a patient handoff between care providers? A. She complains of a holocephalic headache for the last week. Large for gestational age 1 . Increased risk of lower verbal IQ Figure for Question 1. Warfarin bilateral papilledema. She is currently 14 weeks A. Blunt trauma to the fetus E. Optic neuritis D. Meningioma A. Fetal increased risk of epilepsy C. Preeclampsia C. Epinephrine B. Pseudotumor cerebri autoimmunity occurs? B. Failure of self-tolerance D. which of the following would be the most appropriate 1. Serotonin E. Code status 6. C.

Electromyography demonstrates small motor 14. Contusion B. a peripheral cause? C. Decreased hearing in one ear D. Entacapone 12. Autopsy artifact both breast and brain tumors in her mother. A 26-year-old female patient is diagnosed with an from a 60-year-old woman who died of sepsis. (SAH) and underwent a clipping procedure on What pattern of abnormalities would be expected on admission. Acute onset most neurons? 9. Intravenous (IV) tissue plasminogen activator (tPA) D. Amyloid plaque problem? C. Obtain an electroencephalogram. Memantine D. Tinnitus in one ear 13. astrocytoma. Intraarterial calcium channel blockers E.7. and right arm weakness. +60 μV the last several months. B. worsening right A. Obtain GQ1b antibody testing. as opposed to admitted. Viral infection A. Neurofibromatosis 1 E. Which of the following suggests that a patient’s vertigo B. D. IV fosphenytoin load D. Multiple endocrine neoplasia type 1 E. Infarct C. Labs return showing a blood alcohol content of zero and slight hyponatremia. She has since been recovering in the this patient’s muscle biopsy? intensive care unit. +60 mV complaint of weakness which came on gradually over B. Rivastigmine B. 0 mV demonstrates significant proximal arm and leg D. Donepezil E. She has difficulty raising her arms and walking up stairs. Given her daughter’s report of chronic alcoholism. Her family history is notable for photomicrograph? several cancers in her maternal lineage. She has a rash around her eyes and on her knuckles. intoxication is suspected. −60 μV is 2500. Which of the following is the best treatment C. A 63-year-old female presented to the emergency room units with ample fibrillations and positive sharp waves with an acute aneurysmal subarachnoid hemorrhage seen in proximal muscles and paraspinal muscles. Her examination C. Which of the following is the most likely underlying B. A 55-year-old female presents to your office with a A. Endomysial inflammatory infiltrates with tomography (CT) and four-vessel angiogram are cytoplasmic inclusions and rimmed vacuoles shown. Placement of extraventricular drain E. Nests of angular atrophic muscle fibers with fiber for her condition? type grouping A. IV mannitol . Which of the following is an N-methyl-D-aspartate (NMDA) receptor antagonist? A. Ear pain E. On the tenth postoperative day. C. Retinoblastoma D. Perimysial infiltrate with perifascicular atrophy B. Continue metabolic workup as the patient is is from a brainstem or cerebellar lesion. A 64-year-old woman presents to the emergency room with confusion and difficulty walking. Endomysial inflammatory infiltrate with CD8 facial droop. 8. What is the next best step? Figure for Question 1. Her examination shows nystagmus. Von Hippel-Lindau 11. Nystagmus worse with visual fixation status. what is the resting membrane potential of E. limited ocular movements on lateral gaze. and ataxia. she also suffered from What is the cause of the lesion seen in the osteosarcoma. she was noted to have slurred speech. Creatine kinase E.  A. On average. Ragged red fibers that stain COX− and SDH+ C. including A.2 Neurology Self-Assessment 7. Li-Fraumeni syndrome D. In childhood. −60 mV weakness with forearm flexors spared. Vigabatrin C. Rule out infectious causes of her altered mental A. She was more T-cells difficult to rouse. Give intravenous (IV) thiamine 500 mg tid × 3–5 days followed by maintenance thiamine thereafter. The following section is obtained at autopsy 10. Noncontrast head computed B.

8 55 Ulnar Wrist 2. A 17-year-old girl arrived to class immediately after cross-country running practice. Ulnar neuropathy at the elbow D. Radial neuropathy at the spiral groove E. Posterior interosseus neuropathy C. and she quickly began to rouse. Generalized seizure Forearm 7.15  B. She initially appeared confused but was able to answer 15. A 17-year-old male who suffered a broken humerus questions appropriately.0 5. An electroencephalogram (EEG) is normal in the awake and sleep states. Over this past school year. arm with preserved triceps strength ever since the cast She returned to her baseline mental status within a few was removed. Landau-Kleffner syndrome C.5 6.0 E. Developmental dyslexia D. Complex partial seizure TABLE FOR QUESTION 1. he had progressive difficulty with reading. His neurological examination and mental status examination are normal and show that he is able to write a simple paragraph in clinic. Infarct of the splenium of the corpus callosum and left occipital lobe B.5 9. Attention-deficit/hyperactivity disorder 17.6 52 elbow .5 25 52 Radial Forearm Not Not Not recordable recordable recordable Which of the following best explains the patient’s symptoms? A. Her teacher rushed over to lower her Figure for Question 1. Mild intellectual disability E. She had some urinary during a football game noticed a wrist drop in his right incontinence during the episode but no tongue trauma. jerklike movements of her arms and legs. While sitting at her desk. and nauseated.0 7. Convulsive syncope Motor Latency Amplitude Velocity C. C7 nerve root avulsion 16.14. she felt sweaty.0 Below 7. Absence seizure Median Wrist 3.0 40 55 Ulnar (D5) Wrist 2.0 14. Test One 3 Motor Latency Amplitude Velocity nerve Location (ms) (mV) (m/s) 1 Radial Elbow 2.0 Midarm 6. She then slumped forward with fast. Which of the following is the most likely diagnosis? A.9 8.0 30 Motor Latency Amplitude Velocity nerve Location (ms) (mV) (m/s) Sensory Latency Amplitude Velocity nerve Location (ms) (mV) (m/s) Median (D2) Wrist 3.5 8. He previously hit all milestones and was an average student in school. but did not display behavioral problems at school or at home. warm all over. Posterior cord brachial plexopathy B. What is most likely the diagnosis? A. A 9-year-old left-handed boy presents to the clinic with his parents due to concerns over school performance. room nonetheless. The following nerve conduction study minutes of the episode but was taken to the emergency was performed. Nonepileptic spell nerve Location (ms) (mV) (m/s) D.8 53 elbow Above 9.  to the floor.

yells. Ethosuximide 22. Recalling a word when a related word is presented A.19? D. Her husband releasing hormone (CRH) and thyrotropin-releasing states that she does not swing her left arm when she hormone (TRH) walks and has had difficulty using this hand to manipulate buttons. Valproic acid E. so she sleeps in the next room. Polysomnogram (PSG) shows rapid eye movement D. What is the most likely imaging finding seen with this clues diagnosis for the patient in Question 1. Cervical cord compression from disc protrusion B. At She denies history of tremor. Tying one’s shoe E. What would be the treatment of choice for the patient C. Recalling a birthday brain E. Pacinian corpuscles hypothalamus? B. disease for the correct diagnosis discussed in the D. Vascular parkinsonism (REM) sleep without atonia. Based on the C. Which sensory organ/receptor type is most closely 23. First-line treatment is amitriptyline. Which of the following is an example of declarative C. His wife has noticed that he is not sleeping well. Surgical decompression C. Cervical myelopathy A. An 83-year-old male with a history of Parkinson constipation and falling out of bed during vivid disease presents to clinic for a follow-up appointment. Paraventricular nucleus—secretes corticotropin- and gait difficulty for the last 1 year. Predicting the weather based on environmental 21. Rotator cuff injury occasions. Carbamazepine B. Genetic testing for mutations in CHRNA2 would be previous question? positive. Contrast enhancement of basal ganglia on MRI of electroencephalogram (EEG) shown. Ruffini endings D. Hypophonia A. Suprachiasmatic nucleus—appetite function C. dreams for 6 years before the onset of gait difficulty. Levetiracetam A. E. Arcuate nucleus—dopaminergic neurons that inhibit prolactin D. Normal magnetic resonance imaging (MRI) of the D. Which of the following combinations is not correctly linked with perception of temperature? paired with the corresponding nucleus in the A. Suboptimal response to levodopa memory? D. High-volume lumbar puncture E. Increased uptake of dopamine on a dopamine 26. Expecting food when the oven timer ends B. A. PSG shows an abrupt onset of motor activity during 20. Anterior nucleus—lesions produce hyperthermia 19. Free nerve endings C. An 8-year-old boy presents with frequent staring transporter (DAT) scan episodes while in class. First-line treatment is ropinirole. Symmetrical bradykinesia and rigidity B. Phenytoin in Question 1. Peripheral neuropathy C.4 Neurology Self-Assessment 18. What is the most likely night he appears restless. Arthroscopic surgical repair of shoulder D. Severe orthostatic hypotension E. B. Which examination finding is typical early in the slow wave sleep. which of the the brain following is the most appropriate medication for this D. Meissner corpuscles B. Posterior nucleus—lesions produce hypothermia E. Postural instability 25. Aggressive medical treatment of vascular risk factors E. Trial of carbidopa/levodopa . Bilateral T2 hyperintensities in the periventricular patient? white matter A. She has been kicked on several A. Which of the following is true of this condition? B. and seems to be diagnosis? running in his sleep. The patient also reports 24. Idiopathic Parkinson disease B. Merkel disks A. C. A 63-year-old woman presents with left shoulder pain E.19? C.

Intravenous tissue plasminogen activator therapy C. Which of the following would have been appropriate therapy had she sought emergency care? A.27. An image of her brain taken at autopsy is shown. she complained of headaches. She spent all weekend in her yard pulling weeds in the warm summer sun. taking hormone replacement as her only medication. Her headache worsened. Unfractionated heparin and intravenous fluids E.  . The next day. Test One 5 1 Figure for Question 1. Platelets and clotting factors D. A 72-year-old female smoker was active and independent. Normotension and observation Figure for Question 1. and she became confused then sleepy over the next 2 days.  27.26. but declined an appointment with her physician. She passed away at home alone and was later found by her daughter. Triple H therapy B.

Phenelzine C. and has even stolen from a A. Right oculomotor nerve B. Child protective services notification for abuse B. A. Nortriptyline the following is not true regarding the most likely B. Ependymoma C. Anterior olfactory nucleus 30. the vision 33. Which of the following is an example of negative improves. He doesn’t feel there is a problem. Genetics consult for Tay-Sachs disease Which of the following is the most likely diagnosis? D. Major depressive disorder seems worst when watching television from bed. Patient has the capacity to understand information A. Neuroblastoma presented. Detailed document outlining all potential complications of a procedure is provided. All of the following are true of informed consent 34. Taking away video games for cursing E. Hematology consult for coagulopathy A. his mother because of problems with his behavior. C. She withdrew all limbs to painful burning pain in both feet. Narcissistic personality disorder left eye. except? considered assault or battery. C. Autism On examination. Further. Treatments performed without consent can be all of the following structures. B. Amygdala increasingly somnolent. A 42-year-old man develops vertical diplopia. D. pin prick. shown never follows his mother’s rules and won’t complete here. She moaned. A 43-year-old female with a history of depression B. Promising a trip to a theme park for making honor C. A computed tomography (CT) on the head is his chores. Physician must disclose risks and benefits of C. Which is the most directly increased risk as a result of this therapy? E. Peripheral neuropathy B. His neurological examination reveals slightly decreased movements in the right arm 32. Aripiprazole B. Trazodone diagnosis? C. E. Patient decision is made freely without coercion. Renal consult for malignant hypertension babysitter. A 4-month-old baby is brought to the emergency room by his grandmother. Disorders of glucose dysregulation cause the majority of cases. Olfactory sensation travels from the olfactory bulb to E. A 63-year-old man presents to the neurology clinic for did not speak. Nerve conduction studies are usually normal. which C. but 36. A computed tomography scan of her head walking. Juvenile pilocytic astrocytoma potential treatment. and temperature sensation. If he tilts his head to the right. but merely thinks his parents’ requests are unreasonable. Which of the following tumors is most commonly except? associated with opsoclonus-myoclonus? A. 31. His deep tendon reflexes are normal. On examination she was E. B. This condition most often affects myelinated Aδ receiving erythropoietin with a target hemoglobin of and unmyelinated C-fibers. A basic metabolic panel shoe at times. 14 g/dL. Nagging a child for not cleaning his or her room B. Ischemic stroke 37. 35. Antisocial personality disorder . who is visiting for the weekend. He performed and also shown here. Which of the following is the most although he complains of tenderness to palpation of likely cause of her symptoms? his feet. E. Headaches otherwise unrevealing. Yelling at a child for running into the street 29. Medulloblastoma B. Over the past 3 days. Which of the following is the affected reinforcement? cranial nerve? A. which causes significant family stress. D. He also feels as if something is stuck in his without contrast is normal. but is E. D. A. such that her family could no longer wake her up at home. Posterior entorhinal cortex unresponsiveness. causing discomfort while stimulation. His examination is notable for normal reveals a sodium level of 115 mmol/L (normal vibration. Left abducens nerve C.6 Neurology Self-Assessment 28. Seizures He has been inconsolable for many hours. Superior colliculus difficult to arouse with sternal rub. Neurosurgery consult for tumor resection The patient denies a poor mood or sleep difficulties. Which of A. 135–145 mmol/L). Which of the argues with his parents constantly (which is very following is the next most appropriate action? different from his siblings). Prepyriform cortex presents to the emergency department with C. Paying money for completing chores A. Wilms tumor D. His general D. Examination usually shows impaired proprioception and light touch. Citalopram A. He ophthalmological examination is abnormal. Dementia medical examination reveals a crying child. which is exacerbated with downward gaze of an adducted E. An 8-year-old male is brought into the clinic by his and leg compared with the left. Right trochlear nerve D. he has left hypertropia. Left trochlear nerve roll D. A 68-year-old man with stage IV renal failure is D. she had been D. The diagnosis is often made on skin biopsy. Oppositional defiant disorder (ODD) E. Right abducens nerve E.

Treat the patient medically with anticoagulation C. Sumatriptan E. Rhodopsin D. A. Treat the patient medically with an antiplatelet agent B. Topiramate 42. trisomy 21? and holocephalic in nature. and a computed tomography angiogram that D. Volt-gated calcium channel progressive right hemiparesis. which of B. and expressive aphasia. Which of the following proteins is involved in the B. Food and Drug Administration (FDA) A. with associated nausea. Increased tone at birth the following would be appropriate first-line daily C. Centers for Medicare and Medicaid Services (CMS) initial step in light absorption in the vision pathway? C.  38. Central nervous system (CNS) lymphoma echo. Abnormally low alpha fetoprotein (AFP) and The headaches occur at least twice per week and tend abnormally high beta–human chorionic to last about half a day. She refused a screening during pregnancy trial of oral birth control pills due to religious beliefs. Increased risk of leukemia B. Retinal Organizations (JACHO) D.37. Ependymoma shows 80% stenosis of the left common carotid artery E. American Hospital Association (AHA) B. Perform a right carotid artery stent (CAS) . A 24-year-old woman with asthma has several years’ 41. Which of the following organizations accredits C. Glioblastoma no evidence of atrial fibrillation. Perform a left carotid endarterectomy (CEA) D. visual field deficit on the right. Cyclic guanosine monophosphate E. Which of the following is the next best step? A. A 64-year-old woman with a history of poorly the emergency room and is shown here. after which she feels gonadotropin (HCG) levels in maternal blood “worthless” for the remainder of the day. Valproic acid payments from Medicaid and Medicare? E. Small white/brown spots on the outer iris therapy? D. Metastasis and 60% stenosis of the right common carotid artery. Atrioventricular (AV) septal defect A. Workup for stroke etiology reveals B. normal transthoracic C. Perform a right CEA E. A 55-year-old man presents with a 3-month history of E. Which of the following is not associated with history of recurrent headaches that are severe. Beta-carotene 43. American Medical Association (AMA) 39. hypertension. What is the controlled diabetes. A contrast-enhanced magnetic resonance image of the brain was obtained in 40. Joint Commission on Accreditation of Health Care C. Amitriptyline healthcare facilities. Based on the suspected headache diagnosis. Propranolol A. a process that is linked to D. Meningioma hemiparesis. and tobacco use is most likely diagnosis for the lesion shown? admitted 6 hours after acutely developing right A. pulsatile. Test One 7 1 Figure for Question 1.

and she often finds him responding to “the voices. She worries that he is becoming depressed like C. Which of the following is the mainstay of therapy for one of his friends. Whole-brain radiotherapy work. as he insists that she is cheating on him with 44. Which of the following is the most likely test to demonstrate the etiology of the patient’s symptoms? A. C.  48. Lateral medulla C. leaving him unable to B. Medial medulla . Stereotactic radiotherapy personal hygiene has declined. Delusional disorder right side. SEPT9 46. During resection of a right vestibular schwannoma.8 Neurology Self-Assessment his neck the day before. there was loss D. as well as weakness of the A. A patient presents to the emergency department with protein PrPc associated with sporadic Creutzfeldt-Jakob acute horizontal binocular diplopia and left disease and the normal prion protein PrPSc? hemiparesis. Magnetic resonance imaging (MRI) of the brain B. Edrophonium test E. They will frequently argue. the emergency room physician additionally notes the abnormality shown here. Magnetic resonance angiogram of the neck Figure for Question 1. What is 49.47. Transient right-sided hearing loss B. inferior rectus. Schizophreniform disorder of wave III and wave V. Three-dimensional structure inferior oblique muscles on the right. Which of the C. Brief psychotic disorder E. In completing a full neurological examination. She feels he is often not logical as meningioma? he moves from one topic to the next.” Lately. but wave I remained on the E. Observation A. He notes that he fell off a ladder and hurt E. Schizophrenia 45. Assuming no drug or substance use. No difference B. Chemotherapy his father. Amyloid precursor protein B. Which of the following is different between the prion 50. Electroencephalogram D. Displacement of acoustic stimulator D. Presenilin 2 D. Angular gyrus heaviness. No significant neurological injury E. Right pontine infarct C. Near the end of the surgery. N-terminal charge following is the most likely location of this stroke? D. ApoE4 E. Amino acid sequence medial rectus. Midbrain tegmentum E. These waves did not recover by the end of the surgery. Bipolar disorder brainstem auditory evoked potentials (BAEPs) were monitored.  spouse who complains that her husband’s behavior has changed in the last 8 months. Examination reveals mild ptosis and mydriasis on the right. his A. Cerebral peduncle 47. Presenilin 1 C. superior rectus. Chest x-ray C. A 45-year-old man presents to the emergency room with 1 day of headache and several hours of left arm D. Significant right-sided hearing loss A. Splice variants A. Which of the following is the most common cause of the clinical significance of this change? early-onset autosomal-dominant Alzheimer disease? A. BAEP on the left remained normal. Resection B. and B. A 26-year-old man is brought to your office by his Figure for Question 1. which is most likely the diagnosis? D.43.

Chiari malformation 53. Test One 9 51. A 23-year-old woman enjoys playing golf on the E. Microcephaly is often present. Thoracodorsal nerve dominant. Rivastigmine E. Vitamin D deficiency C. On month. (serum glucose 102 mg/dL). Maternal malnutrition presented to the emergency department because he could no longer void on his own. and a glucose level of 22 mg/dL C. His neurological 55. Seizure E. nerves? C. C. Entorhinal cortex are absent. her appetite has decreased. Alcohol exposure in utero 60. A general medical workup includes a lumbar B. Which of the following is the most likely D.5 mL/hr A. Which of the following is not true sinus syndrome. and head nodding. Vitamin E deficiency B. Long thoracic nerve E. A 6-month-old was brought to the emergency room is frequently tearful. Which of the following medications slows the B. Which of the which is primarily horizontal. disorder. They are used to treat major depressive disorder. as is the remainder of the patient’s neurological examination. 20 mL/hr 59. Valproic acid is not commonly used as a mood puncture that shows no white or red blood cells. She 58. Maternal rhinovirus infection months. normal protein. Fornix diagnosis? E. They are not used in treating schizoaffective A. creating various excuses. Lithium is contraindicated in patients with sick imaging is normal. The SLC2A1 gene is defective. Vitamin A toxicity E. 50 mL/hr metabolism of dopamine in the central nervous C. Anterior thalamic nucleus brisk reflexes at the knees. Entacapone A. He seems to have maintained strength and sensation in his arms. A. Vitamin B6 toxicity D. Paroxysmal exertional dyskinesia can occur with 57. 100 mL/hr B. Vitamin B12 deficiency . 0. Musculocutaneous nerve 52. Dysthymic disorder A. and she feels as if examination. Congenital nystagmus D. Which portion of the circuit of Papez is injured in examination shows prominent loss of sensation to Korsakoff syndrome? vibration and proprioception in his lower extremities A. Her boyfriend states that she had a and in no apparent distress. He has B. Spasmus nutans cerebrospinal fluid (CSF) production in an adult? E. B. 2 mL/hr system? D. but his reflexes at his ankles C. Hippocampus along with mild weakness in his legs. A ketogenic diet is an absolute contraindication. Which of the following is true of mood-stabilizing with abnormal movements. Seasonal affective disorder C. and her boyfriend often finds her with eye movement abnormalities and head bobbing. following diagnoses best applies in this case? Additionally. Suprascapular nerve weekends with her friends. Neurology is consulted for an infant in the nursery 56. for the last month she has not wanted to go. regarding this condition? E. Which of the following is the approximate rate of D. Major depressive disorder Brain imaging is normal. Lithium can cause hyponatremia. D. Carbidopa the pediatric population except? D. Three weeks ago he started having falls. Major depressive episode following is the most likely diagnosis? C. Bipolar 2 disorder B. Amantadine B. Trihexyphenidyl 54. Radiation exposure in utero surgery developed numbness in his legs over the last 2 D. Continuous agents? 1 electroencephalograph (EEG) monitoring shows A. A 36-year-old male with a history of gastric bypass C. crying in bed wishing she were dead. similar episode about 6 months ago. However. A. Central nervous system D. Toxoplasmosis E. the patient appears to be comfortable she has no energy. Mammillary bodies A. The posterior cord gives rise to which of the following this condition. Which of the B. Medial pectoral nerve D. Also in the last which were thought to represent seizure. stabilizer. Benign paroxysmal torticollis of infancy A. All of the following are causes of mental retardation in C. his neck intermittently turns to the left. He E. He has nystagmus. The most common inheritance is autosomal B. frequent seizures associated with a decreased level of alertness.

A. including gambling and inappropriate sexual comments at work. and he scores 28/30 on the mini mental state examination. Recent missed diagnosis of amyotrophic lateral D. Histopathological hallmarks include neurofibrillary tangles. Lacosamide and a biopsy of the largest lesion C. This is caused by buildup of amyloid protein. Treating a patient with a prior diagnosis of ALS the emergency room. Which of the following is true of this condition? A. vancomycin. metabolic panel. Ticlopidine 66. Magnetic resonance imaging (MRI) of his brain is also unremarkable. Which of the following is an example of availability bias? C. 9 months E. Apixaban C. Nonconvulsive status epilepticus C. Assuming a patient has ALS and not changing diagnoses despite contrary information 63. 2 months B. grunts. His wife has also noticed that he mixes up the names of medications. His D. urine drug screen. Fosphenytoin and methylprednisolone E. Obsessions and hoarding are common behaviors. 64. Finding fasciculations on electromyogram He suffers a focal seizure followed by secondary confirming suspicion of ALS generalization. He is a 56-year-old male cardiologist with no significant medical history whose wife is concerned about recent changes in his behavior. sclerosis (ALS) leads to ALS diagnosis in a second patient E. including a complete blood count. He was able to name seven animals in 1 minute. Lithium is commonly used for behavioral control. Dabigatran D. Which of the following is a direct thrombin inhibitor? A. A colleague presents to the office with her husband for an evaluation. Figure for Question 1. thyroid- stimulating hormone. Lacosamide. A 45-year-old man has recently moved from Mexico. Levetiracetam and acyclovir B. Levetiracetam and albendazole A. and cefepime D. Cilostazol E.  B. B. and moves all of his extremities equally. Which of the following is the most likely diagnosis? A. Ischemic stroke E. Fluorodeoxyglucose–positron emission tomography (FDG-PET) scan shows hypometabolism of the posterior temporoparietal region. A 57-year-old alcoholic was found unresponsive behind a gas station. liver enzymes. but he insists there is nothing wrong.10 Neurology Self-Assessment 61. and no nuchal rigidity is noted. 12 months 62. A head computed tomography was negative for hemorrhage. Complete blood count and basic metabolic profile were normal. Meningitis . His neurological examination is unremarkable. Laboratory work is normal. Rivaroxaban B. A magnetic resonance image without confirming the diagnosis (MRI) of the brain is shown. His examination demonstrates a somnolent male who wakes up briefly to painful stimulation. Hepatic encephalopathy D. At what age does the Moro reflex disappear in normal children? A. 6 months D. 4 months C.63. Concluding a diagnosis of ALS without any testing neurological examination has returned to normal in E. The electroencephalogram (EEG) is shown here. He denies symptoms of active infection and has not suffered any recent trauma. He is afebrile. vitamin B12. Herpes encephalitis B. Which of the following is the next best step in management? 65. and rapid plasma reagin. C.

Which of the following is the most likely source of her 68. Low serum cortisol movements. Preganglionic sympathetic fibers and epinephrine C. Preganglionic parasympathetic fibers and A. Manifestations of his Tourette syndrome acetylcholine B. . There is a high risk of recurrence with future E. Obsessive-compulsive disorder (OCD) 70. Treatment with clonidine is helpful. Postganglionic parasympathetic fibers and the wall four times before walking through a doorway. There is D. They do not interrupt her ability to reach for of cognitive function? objects or keep her from walking. High serum T4 no family history of movement disorders. A. He is often late for of sweat glands? work because he feels the need to check the lock on his A. Which of the following is C. After a prolonged course of steroids for reactive airway D. The Wisconsin Card Sorting Test is helpful in of her arms that seem to flow from body part to body determining functional abnormalities in which domain part. A 32-year-old female who is 27 weeks pregnant symptoms? presents to your office for evaluation of abnormal A. E. Postganglionic sympathetic fibers and front door five times each morning. Her child has a 50% chance of inheriting this disorder. were normal.  67. A 26-year-old man with a history of Tourette syndrome 69. Executive function A. She has no urge to move and C. Test One 11 79–1019 61 M 1 F3–C3 F4–C4 C3–P3 C4–P4 P3–O1 P4–O2 50 µV 1 sec Figure for Question 1. Visuospatial function ceruloplasmin. Panic disorder with agoraphobia E. If he forgets or is norepinephrine distracted. Slit lamp examination would demonstrate Kayser- Fleischer rings. Intelligence pregnancies. Postganglionic sympathetic fibers and acetylcholine includes stepping up on curbs three times and tapping C. Language including a complete blood count. Another example he provides B. C. D. She cannot sit still and is always fidgeting. Her husband says her arms always move but this stops B. Treatment with haloperidol is necessary. Low serum glucose not taking any medications. liver function tests. E. Laboratory work. a 30-year-old woman develops fatigue. High serum cortisol when she goes to sleep. Low serum T4 does not get a sense of relief with movements. metabolic panel. Obsessive-compulsive personality disorder (OCPD) disease. Her neurological examination is normal except for abnormal movements 71. B. he feels the need to start over and recheck the door five times. Paranoid personality disorder generalized muscle weakness. and B. Which part of the autonomic nervous system and presents for new symptoms that are causing great which neurotransmitter are responsible for activation distress with maintaining a job. and weight loss.66. norepinephrine What is the most likely diagnosis? D. and she is E. Declarative memory true of this condition? D. thyroid-stimulating hormone.

but D. Standard treatment involves 1 gram of intravenous retardation. In the following image. despite amputation. Valproic acid 1 73. Neural plate C. Somites B. Uhthoff phenomenon after strenuous labor D. Side effect of epidural anesthesia E. Lumbosacral plexopathy resulting from pelvic trauma C. Capillary telangiectasia Which is the most likely mechanism of her problem? A. An axial magnetic resonance image (MRI) is shown. knee flexion.12 Neurology Self-Assessment 72. 78. Which of the following structures is injury to his right lower extremity. Developmental venous anomaly birth to her first child. Which of the incontinence. and knee extension. Norepinephrine reuptake inhibitor D. methylprednisolone over 5 days. which ultimately represented by the number 1? required amputation. A 62-year-old female with a history of poorly controlled diabetes presents with left leg weakness. Mu-opioid receptor agonist 4 B. He has mild mental retardation. Rhizotomy 3 E. Standard treatment involves physical therapy. she had weakness in her left leg and loss of muscle mass in the left thigh. Gabapentin 2 D. but has noted significant weight 77. Sural nerve biopsy is necessary for the diagnosis. He has the least common inherited cause of mental A. After her epidural wears off. After an 18-hour delivery. he has had A. Cerebral aneurysm is tingling on the lateral aspect of both lower legs. Left with protuberant ears and enlarged testicles. E. There was no has a long history of mental retardation. A 43-year-old man recently returned from military 76. he was involved in developing fetal nervous system is shown at around an improvised explosive device explosion resulting in day 25. and B. Genetic testing for SEPT9 gene should be arm automatisms and lip smacking. Compressive neuropathy of bilateral common peroneal (fibular) nerves . A. followed by a performed. There C. Increases risk of serotonin syndrome 74. generalized convulsion. He has severe mental retardation. Neural tube treatment for his condition? C. C-reactive protein (CRP) were normal. He has moderate mental retardation. Which of the following is not a possible B. All of the following are true about tramadol except? A. Magnetic resonance imaging of her following is true regarding his condition? lumbar spine and lumbosacral plexus were normal. the patient goes for a walk in the hall and notices that B. Side effect of oxytocin B. sedimentation rate. This initially began with severe left hip pain. Notochord A. Laboratory work. An 8-year-old boy has a history of developmental delay loss. N-methyl-D-aspartate (NMDA) receptor antagonists D. Her examination demonstrates weakness of left with an intelligence quotient (IQ) of 45. Arteriovenous malformation otherwise intact strength and sensation throughout. A 25-year-old woman presents with a history of left D. migraines. a coronal cross-section of a deployment overseas. His hip flexion. Long-term immunosuppression is needed to for recurring headaches previously attributed as prevent recurrence. C. complete metabolic panel. a 26-year-old woman with a likely diagnosis? history of relapsing remitting multiple sclerosis gives A. Which of the C. She has minimal numbness. following is true of this condition? D. Her history is also remarkable E. Neural crest increased pain in his lower extremity.76. His family patellar and ankle reflexes were absent. Cavernous malformation both feet slap the floor more loudly than before.  course of several weeks. Over the Figure for Question 1. but pediatrician also notes that he has an elongated face normal foot plantar flexion and dorsiflexion. including complete blood count. E. condition. Decreases risk of seizures 5 E. Since his surgery. Serotonin reuptake inhibitor C. Tricyclic antidepressants E. While there. Which of the following is considered the most 75. Women do not manifest symptoms of this B.

Mal de debarquement syndrome 79. Stenting Trial (CREST). Benign paroxysmal positional vertigo C. Clopidogrel load upper extremities. A. Sydenham chorea C. Neurosyphilis still and difficulty sleeping. Follicle-stimulating hormone (FSH) C. She is adopted.78. Which of the following hormones is released from the posterior pituitary (neurohypophysis)? A. Human immunodeficiency virus dementia Figure for Question 1. Her magnetic resonance image (MRI) is shown. Huntington disease E. Carotid stenting diarrhea. Wilson disease 80. Catheter angiography Which of the following is the most likely diagnosis? E. Cardiac stenting A. He notes antecedent arthralgia. skin hyperpigmentation. Cocaine intoxication 81.80. Anti–N-methyl-D-aspartate (anti-NMDA) encephalitis D. Stroke B. Thyroid-stimulating hormone (TSH) E. Oxytocin B. A 42-year-old male presents with memory loss over the last several months. as well as tongue impersistence. Prolactin 82. A 33-year-old woman presents with an inability to sit B. D. and frequent B. On examination. Carotid endarterectomy low-grade fevers. but knows that her mother committed 1 suicide after years of abusing drugs.  B. On examination he has mild rigidity in his C. Adrenocorticotropic hormone (ACTH) D.  . According to the Carotid Revascularization D. Meniere disease Figure for Question 1. which is E. there are subtle choreiform movements involving both the trunk D. which have developed C. Whipple disease gradually over 3 years. Opsoclonus-myoclonus Endarterectomy vs. Test One 13 initiation of saccades. She also has delayed E. What is the most likely diagnosis? A. Labyrinthitis associated with a periprocedural increased risk of stroke? 83. Early-onset Alzheimer disease and the distal upper extremities. and concurrent slow pendular movements of the eyes and contraction of the masseter. Which of the following results from excess endolymphatic fluid? A.

Memory . Her speech has been get out of bed. Low cerebrospinal fluid hypocretin level E. Electroencephalogram reveals left in this condition? hemispheric spikes. Day 60 E. Abstraction D. Levetiracetam D. Carbamazepine B. In posterior tibial somatosensory evoked potentials (SSEPs). Cranial magnetic resonance imaging (MRI) E. Which day during embryogenesis does the distal neural 91. Spelling “world” backward in a mental status tube pore close? examination most closely tests which of the following? A. Executive function E. Phenytoin E. Intravenous (IV) immunoglobulin 85. Retinal pigment epithelium 87. In the last 2 weeks. He falls asleep movements of her right arm. A 50-year-old man experiences frequent recurrent pain on the left side of his throat. stabbing pain across his entire head each time he coughs.” He will frequently wake in the morning but be episodes and may have had shaking in her right leg unable to move or speak for 3 minutes before he can and right side of her face. Day 40 D. Cerebral cortex Figure for Question 1. near his tonsils. Which of the following would be seen regressing. Day 13 B. Photoreceptor layer E. Carbamazepine C. which of the following is the appropriate first-line therapy? A. Frequently associated with hyperphagia and C. A 17-year-old male presents to your office for 90.90. Apnea-Hypopnea Index (AHI) of 15 of her brain is shown. Which of the following layers of the retina does light first encounter after passing through the lens? A. His mother notes that when he is spontaneously. Assuming a radiographic evaluation is unrevealing. Retinal ganglion cell layer B. Sleep latency of 20 minutes B. Lumbar roots C. Lumbar plexus B.14 Neurology Self-Assessment 84.  D. Daily antihistamine B. Which of the following is the best treatment for this condition? B. the pain is too brief for therapy 86. A magnetic resonance image (MRI) A. Which of the following should be done? A. Optic nerve layer C. Day 7 A. he will drop objects or become “weak in the has noted that she seems unaware during parts of these knees. the patient’s mother angry. Nothing. Attention B. He says that this feels like a hot poker. Pulmonary function tests D. A 6-year-old girl presents with involuntary jerking evaluation of excessive sleepiness. Concentration C. Amitriptyline C. She had several episodes frequently in class and even fell asleep while giving a of this that would last for hours before stopping speech in class. Dorsal columns E. Hemispherectomy D. The pain is severe. Vagal nerve stimulator hypersexuality D. Medial lemniscus 89. Oxycodone 88. Day 27 C. Valproic acid E. but resolves quickly after coughing. the P37 peak corresponds with which anatomical location? A. A 16-year-old male suffers sharp. Inner plexiform layer D. Amitriptyline C. Electroencephalogram (EEG) with continuous slow wave and spikes during sleep A.

Neurofilaments weakness and confusion and is dead within 1 month.94. A. The nature of the act/intervention must not be intrinsically wrong. C. Test One 15 92. Type I malformations do not have neural tube defects such as a myelomeningocele. Microtubules The following image was taken from autopsy. E. Over the following days he develops diffuse D. B. which of the following is the best first treatment? A. Cowdry type B B. Listeria monocytogenes A. Embolization D. Treatment involves surgical decompression if D. Which of the following is not true regarding the clinical by the arrows? syndrome depicted? A. E. C. Diagnosis is made radiographically when one or B. Howell-Jolly bodies findings are present on magnetic resonance imaging E. Which of the following is not structurally responsible D. The action must be approved by the ethics for movement of cargo along the axon? committee. Pseudomonas aeruginosa are probable. Neisseria meningitidis B. Figure for Question 1. Intravenous antibiotics B. Group B streptococci following must be present except? B. D. 2 weeks of increasing ataxia. Based on the most likely diagnosis. Rosenthal fiber (MRI). Craniospinal radiation Figure for Question 1. Surgical resection C. Intravenous chemotherapy E. and postcontrast T1 sagittal imaging is shown. Neurolemma 96. Kinesin E. Negri bodies A. a rural farmer suffers from flu-like C. Select the most common bacterial cause of meningitis 95. What is the name of the pathognomonic findings indicated 94. with the principle of nonmaleficence. The bad effects are not a means to the good effects. Cowdry type A both cerebellar tonsils are below the foramen magnum by ≥5 mm. Staphylococcus aureus C.  . all of the 1 A. The intention is for good effect even if side effects D. 93. Dynein symptoms. For an action to be considered ethical in accordance for neonates (<1 year old). Syringomyelia is a common association with type 1 malformation. C. Syringomyelia is the most common presentation of type II malformations. and 2 days of headache and vomiting. A magnetic resonance image is obtained. After a bat bite.  97. A 6-year-old boy is brought to the emergency department by his parents for 3 weeks of headache. regardless of symptoms.96. The good effects must outweigh the bad effects. E.

Two weeks later he presents to Figure for Question 1. lipase are both elevated. Primary progressive aphasia B. and draw a clock accurately.  the emergency department with severe abdominal pain. Pituitary adenoma which of the following? D. 105. Four days later. His neurological examination is normal. Biceps and adductor pollicis 103. A voltage-gated potassium channel presentation at emergency room. Topiramate ophthalmoplegia. and retroorbital pain. The gamma aminobutyric acid B (GABAB) receptor is C. and bilateral B. In D. Dementia with Lewy bodies A. she is noted to have a dark mass in each nostril. He frequently calls objects D. He is C. Examination reveals normal strength and normal cranial nerves. but no sensory loss in other parts of the hand. Which of the following is the diplopia. Flexor pollicis longus and flexor carpi ulnaris C. A 53-year-old female presents to your outpatient clinic with 3 months of pain in the left arm. Triceps and extensor indicis proprius E. Propranolol following is the most likely etiology for her symptoms? D. On mental status Administration (FDA) approved for secondary examination he is alert and oriented. A 33-year-old male recently started a migraine preventive medication. Fingolimod either nonsensical words or the wrong words. including liver enzymes. A complete metabolic 98. There is no proptosis. Valproic acid numbness in V1 and V2 distributions. and agitation are common problems. A ligand-gated sodium channel B. Headache is the most common side effect on E. Alzheimer disease multiple sclerosis–related fatigue? B. A voltage-gated sodium channel E. but he has difficulty with A. Further. Brachioradialis and pronator teres D. by the wrong name or cannot come up with the words E. All of the following are true about cocaine use except? C. A ligand-gated chloride channel 99. and magnetic resonance imaging (MRI) of his 100. Mild cognitive impairment C. Dalfampridine 102. She describes a burning and tingling pain in the lateral aspect of her forearm and her left thumb. nausea. Idiopathic intracranial hypertension 104. Memantine E. First dorsal interosseous and abductor pollicis brevis B. and vomiting. Which of the C. A 42-year-old diabetic woman develops maxillary pain panel is normal. hallucinations. she notes epistaxis. Pemoline C. A 63-year-old male presents to the clinic with memory C. Amitriptyline A. Tolosa-Hunt syndrome E. she has bilateral ptosis. draw intersecting pentagons. problems with his speech. On presentation to the most likely cause of his condition? emergency room. he wants to say.97. Which of the following is Food and Drug brain shows temporal lobe atrophy. Modafinil D. His complete blood count has normal white blood cells and hemoglobin. It blocks synaptic reuptake of dopamine. A metabotropic receptor A. It causes pupillary constriction. Mitoxantrone Which of the following is the most likely diagnosis? 101. Over the last several months he developed side effects. Dimethyl fumarate able to remember three words at 3 minutes. Vascular dementia . Amantadine E. Which of the following muscles would most likely show electromyography (EMG) abnormalities? A. He frequently replaces words with B. Amylase and and rhinorrhea. Carotid-cavernous fistula B. Which of the following is least likely to improve A. D. His speech is progressive multiple sclerosis? clear and he can repeat. Her biceps and brachioradialis reflexes are reduced. She has reduced sensation over the lateral aspect of her forearm and her entire left thumb. and a mildly low level of platelets. Verapamil B. E. A. Teriflunomide naming objects. It can lead to development of chorea. Glatiramer acetate 1 minute he was only able to name four animals. Mucormycosis infection A. Paranoia.16 Neurology Self-Assessment D.

vital to normal neurological function. Epinephrine although heparin could be used. Enoxaparin is the preferred agent in pregnancy. which is metabolized placental abruption. E. 112. Figure from NH2 Yildiz OK.) for tension-type headaches and other pain during pregnancy. Elshatory category X and is not recommended. 1973–1991). diagnosis. outside YM. Cevik S. and environmental factors (see consideration in an initial diagnostic consultation with Bradley’s NiCP. Optic neuritis would show hyperintense optic nerves on T2 imaging Dopamine along with contrast enhancement. Diamox would be a treatment for pseudotumor neuronal ceroid lipofuscinosis. They can also cause maternal (through the action of tyrosine hydroxylase). O Topaktas S 2012 Cerebral venous sinus thrombosis presenting as transient ischemic attacks in a case with Norepinephrine homozygous mutations of MTHFR A1298C and Norepinephrine N–Methyltransferase (Pnmt) CG677T. stillbirth. 1973–1991).  This patient presents with worsening headaches and O features of increased intracranial pressure with O worsening headaches at night and papilledema. The fetus can have bradycardia to epinephrine (through the action of and asphyxia during and after a seizure.  Patient handoffs are an important component of 3. or seizures can cause the action of beta hydroxylase). which is consistent with an acute VST. but is not generally of high importance for 4. Brain Research 1162: 98–112. failure of self-tolerance. however. Papilledema could be seen additionally in O preeclampsia and optic neuritis. D.  Dopamine is a monoamine neurotransmitter that is passing off the care of a patient. lack of activity would lead to immune suppression.4 (from Weimer JM. Meningiomas are not β–Hydroxylase (Dbh) hyperintense on T1 imaging without contrast. which can even lead to death. Oztoprak I. E. epinephrine. Acetaminophen can be used permission. and an increased risk of . and code status as a minimum amount genetic predisposition. Magnesium is used to treat eclampsia (see Bradley’s NiCP. O Anticoagulation is recommended. 51. In Tyrosine pregnant patients. of information. Ch. there should be immediate concern Tyrosine for venous sinus thrombosis (VST). The fetus can be Dopamine is metabolized to norepinephrine (through injured with blunt trauma. This demonstrates a hyperintense DOPA T1 signal in the sagittal sinus and the great vein of Decarboxylase (Ddc) Galen. 5. Dopamine is 6. 7th edn. Bolayir E. O 2. The image provided NH2 does not show signs of optic neuritis because the optic O nerves are not in the imaging plane. Although there are many verbal and/ Although overactive MHC may cause autoimmunity. even in the case of N– intracranial hemorrhage. Journal of Stroke and Cerebrovascular Diseases 21(1): 75–77. Family history is an important molecular mimicry. The provided image is a noncontrast DOPA T1-weighted image.  Major histocompatibility (MHC) factors are key transitioning care between hospital shifts or between antigen-presenting components of the immune system. Ch. smallness for gestational age. a or written handoff systems in place across institutions. including tryptophan hydroxylase and compared with children born to epileptic mothers well aromatic L-amino acid decarboxylase. E. MRI and magnetic resonance NH2 venography are the first-line tests for determining O O whether there is a VST because both can be done O without contrast. pp. Ch. Pregnant patients are at higher risk of VST because pregnancy creates a hypercoagulable state often O worsened by dehydration. Repeated convulsive demonstrates the pathway from tyrosine to seizures can lead to miscarriage. Pregnancy-induced Hydroxylase (Th) pseudotumor is possible. C. 676–695). Preeclampsia has no Dopamine radiographic findings. Cil G. but VST must be ruled out first. trauma. 112. with permission. The figure controlled on medications. pp. However. Other common pathways to autoimmunity include treatment plan. most include patient identification. et al. to prevent propagation of the O H clot. O they do homogeneously enhance with contrast (see O Bradley’s NiCP. 2007 Alterations in striatal dopamine catabolism precede of pregnancy warfarin is the standard anticoagulant loss of substantia nigra neurons in a mouse model of juvenile used. prematurity.  Convulsive seizures can have significant effects on formed from L-dopa (through the action of dopa the fetus and are felt to be of higher risk than of decarboxylase). Serotonin is with epileptic mothers not on medications during also a monoamine created from tyramine through two pregnancy have twice the risk of developing epilepsy enzymatic steps. with and is pregnancy class C. Test One 17 ANSWERS O 1 NH2 1. pp. Children phenylethanolamine N-methyltransferase). and L-dopa is formed from tyrosine antiepileptic medications. medical facilities. patients. Benedict JW. B.  The patient has a cerebral venous sinus thrombosis. Warfarin is pregnancy Figure for Answer 1.

Amyloid plaques are within the parenchyma. 7th edn. DM involves perimysial considered in cases of ataxia and ophthalmoparesis. vertigo is an important. pp. Infections of the Nervous System. and ataxia is Wernicke 8. 13. so the resting potential of Ragged red fibers in combination with a COX− and neurons resides near the Nernst potential of SDH+ stain is highly suggestive of a mitochondrial chloride (see Bradley’s NiCP. chronic alcoholism who presents with encephalopathy. pp. IBM has inclusions and rimmed vacuoles. 7th edn.  Memantine is an NMDA receptor antagonist used such as “Swiss cheese” artifact from gaseous buildup or in the treatment of moderate-to-severe Alzheimer flattening artifact from resting on the bottom of a bucket disease. 71 and 75. few classic artifacts in autopsy evaluation of the brain. which neuroendocrine tumors (see Bradley’s NiCP. pp. Contusion does not generally cause Entacapone is a catechol-O-methyl transferase inhibitor pallor. 65. 920–967). tumors. 1018–1025 and 1065–1083). Figure disease (see Bradley’s NiCP. D. Rivastigmine and donepezil are during fixation. new focal nerve sheath tumors (neurofibromas). because of her recent pathways. pp. including RAS/RAF/MEK/ERK. with permission. 7. 34–35. Wernicke encephalopathy can develop to caring for the vertiginous patient. so the retina that is associated with autosomal-dominant an extraventricular drain is not necessary. but of Alzheimer disease. PM involves but is not the best answer in this case because there is endomysial inflammation with CD8 cells. but tends to improve with to this patient. Hedley-White ET 2010. so patients. 7th edn. as blood extravasates into adjacent tissues (see used as an adjunct in the treatment of Parkinson Bradley’s NiCP. Multiple endocrine Ch. early breast even death if not quickly recognized and treated. formulation. but it is important to recognize the flexors and quads before other weakness developing. manifested by anterograde associated vertigo may have other signs indicating ear amnesia. medial thalami. cancers. from Camelo-Piragua S. Inclusion no mention of peripheral neuropathy. Ch. A. 349–391). Obtaining GQ1b antibodies (often although in darker-skinned patients the rash may be helpful in Miller-Fisher syndrome) should be difficult to see. even if this leads to constitutive activation of cellular growth patient had an ischemic stroke. Nystagmus can be present in either periaqueductal gray matter. and worsen with first and foremost.  The image demonstrates a sharply outlined region of clear cell carcinomas of the kidneys. Ch. E. Ch. pp. 459–485 and Ch. disorder (see Bradley’s NiCP. Although seizures are with NF1 are at increased risk of developing multiple a possible cause of reduced level of alertness. not myopathic. are classic for cerebral infarction. and can lead to further neurological deficits or increased risk of childhood sarcomas. Peripherally into Korsakoff syndrome. symptomatic from thiamine deficiency. 1422–1461).) 12. Ch. dominant syndrome associated with mutations in the 1973–1991). On biopsy. and pancreas associated with uncomplicated seizure during the first trimester does autosomal-dominant mutation in the tumor suppressor not seem to have an increased risk for fetal gene MEN1. pp. 583–604). 483–518. and breast cancer. or ear shows petechial hemorrhages in the mammillary pain.  Dermatomyositis (DM) is differentiated from should be administered preferably with the IV polymyositis (PM) clinically by the presence of a rash. parathyroid. Viral aminobutyric acid (GABA) metabolism inhibitor used infection generally requires a much higher power to to increase GABA activity in the central nervous system.18 Neurology Self-Assessment major malformations. 110. D. Ch.  Li-Fraumeni syndrome is an autosomal-dominant hemorrhage vasospasm. 1915–1956). Before any glucose is given central or peripheral lesions. 112. The other body myositis (IBM) has a different pattern of choices mentioned are also reasonable in anyone with weakness than PM or DM. permeable to chloride. A. which the angiogram shows. Vigabatrin is a gamma do not represent a change in the parenchyma itself. Elsevier. albeit challenging. On biopsy. nystagmus. retinoblastoma spontaneously. A. This is a common disorder caused by a loss of function mutation in the complication that can occur in the first 14 days after p53 tumor suppressor gene. as glucose metabolism further visual fixation in brainstem or cerebellar lesions (see depletes thiamine. 11.  Differentiating peripheral from central causes of encephalopathy secondary to thiamine deficiency. C. 14. pp. syndrome and treat with thiamine early and first. and brain tumors. Magnetic resonance imaging most often involvement. 7th edn. pp. Given that this patient is Bradley’s NiCP. a single pituitary. involving the forearm altered mentation. ophthalmoparesis. and pancreatic pallor with scalloped edges and “spongy” center. 67. Although there are a Chs.  −60 mV is the average resting membrane potential Angular atrophic fibers with fiber type grouping would of most neurons. 981–995). gastrointestinal stromal evaluation for vasospasm. There is no inactivation of the Rb tumor suppressor gene in 40% of indication of severe increased intracranial pressure.  The most likely diagnosis in this woman with Diagnostic Pathology of Infectious Disease. hypothalamus. Figure neoplasia type 1 is a syndrome of cancers in the from Rabinstein AA. parenchymal pallor is not a common acetylcholinesterase inhibitors used in the treatment artifact. pp. Individuals hemorrhage and cranial surgery. Ch.  The patient is suffering from postsubarachnoid 10. Von Hippel-Lindau is an autosomal- malformations (see Bradley’s NiCP. Vertigo can be acute onset with either peripheral bodies. Resnick SJ 2009 Practical . At rest the membrane is most be seen in a neuropathic. The other 60% of patients develop mannitol is not necessary (see Bradley’s NiCP. Retinoblastoma is a cancer of The CT shown does not demonstrate hydrocephalus. malignant findings in the setting of an SAH should prompt an peripheral nerve sheath tumors. 95. and or central lesions. including tinnitus. condition. the loss of which angioplasty. 40. treatment doses 9. hearing loss. 7th edn. identify inclusions. Neurofibromatosis 1 (NF1) The main emergent therapy remains intraarterial is an autosomal-dominant disorder caused by a loss of administration of calcium channel blockers and function mutation in the NF1 gene. pp. IV tPA would not be indicated. infiltrates with perifascicular atrophy. 7th edn. component Chronically. Fortunately. In: Kradkin RL (ed. 46. B. IV thiamine should be supplemented visual fixation in peripheral lesions. 7th edn. Patients are at significantly SAH. VHL gene that leads to hemangioblastomas of the brain.

pp. 1st edn. It is not uncommon for patients and is without structural abnormalities on imaging. a relatively common occurrence with abdominal binders. Ch. 7th constellation of other symptoms presented (see edn. midodrine. Severe orthostatic hypotension is a presentation that suggest a syncopal etiology rather common symptom of a Parkinson-plus syndrome such than seizure include the brevity of her loss of as multiple system atrophy (MSA). parkinsonism would also not have these nonmotor deficit/hyperactivity disorder can lead to school features and would present predominately with lower problems in this age group. reading. Constipation and falling out of bed corpus callosum and left occipital lobe could cause during vivid dreams (rapid eye movement [REM] sleep alexia without agraphia. Ch. symptomatic parkinsonism may stem from are recurrent. DAT scan measures the presynaptic activity of Treatment for the case presented would likely include dopamine in the striatum (caudate and putamen) and volume resuscitation. A considered in case of an atypical seizure presentation. prodrome of nausea and diaphoresis. 7th question stem that would indicate a nonepileptic spell. 8–16). Test One 19 Neuroimaging in Stroke. but this should come on behavior disorder) are two nonmotor features of PD acutely. Ruffini amplitude of 50% with proximal stimulation compared endings are sensitive to stretch and mechanical with distal stimulation and reduction in velocity to the changes. than asymmetric upper extremity involvement. an electroencephalogram may also be structural lesions in the contralateral basal ganglia. 223–249). D. Ch.e. The diagnostic workup for syncope would generally 21. Note that there was no mention of associated with late postural instability. confirm these associated with a normal neurological examination. not a and loss of dexterity in the left hand. 16.  This patient suffered a radial neuropathy at the conservative measures (i. second digit and loss of strength in radial-innervated 18. examination findings. some of which persist after operations for history of dyslexia.  Developmental dyslexia. Remember that control during the episode. 35. liberalization of salt intake. C. If events case. skills. spiral groove. Pacinian corpuscles are spared). humeral fractures because the nerve passes through the moving legs before standing) and pharmacological spiral groove on the posterior portion of the humerus. and executive function. It to complain of joint pains likely from dystonia or is more common in left-handed males with a family rigidity. edn. demyelinating range.  Idiopathic Parkinson disease typically has no include orthostatic vital signs. 23). of course. 1301–1323). with no other definitive therapy would demonstrate decreased uptake with idiopathic . Vascular intelligence quotient between 50 and 70. and loss of The other choices are red flags that the diagnosis is not consciousness. including no evidence Holter monitor. two of the Bradley’s NiCP. early in the course is often a sign of progressive and the fast. posterior portion of the hand between the thumb and 7th edn. 20. also known as specific Note that tremor is not part of the diagnostic criteria of reading disorder. It is usually physical examination would. 23. a micrographia in idiopathic Parkinson disease (PD). 19. the brevity of the postictal confusion. but this can occur with any idiopathic PD typically presents with asymmetric onset alteration in consciousness. compression stockings. The to read despite normal intelligence. The radial motor response has a drop in sensitive to vibration and are rapidly adaptive. measures (i. but typically these patients extremity involvement and gait difficulty much more present with complaints of inattention. pp.  The case is consistent with convulsive syncope. more proximal (radicular injury or plexopathy). An infarct of the splenium of the orthopedic issues. 90. There is nothing in the of rigidity and/or bradykinesia (see Bradley’s NiCP. 7th edn. as one can have the akinetic/rigid subtype. Mild intellectual disability swing. If the patient suffers recurrent syncope. pp. Elsevier.  Free nerve endings are associated with sensation of muscles distal to the spiral groove (the triceps is temperature and pain. Associated symptoms include numbness on the pyridostigmine) may be considered (see Bradley’s NiCP. with indicated unless there are abnormalities on evaluation. and is associated with an The same is also true for cervical myelopathy.  The patient has shoulder pain and gait difficulty for likely at the spiral groove. A. cardinal features of idiopathic Parkinson disease (PD). A or impulsivity. indicating a conduction block. Bradley’s NiCP. including PSP and MSA.e. An absent radial sensory the last 1 year with decreased arm swing when walking response indicates this is a neuropathic lesion. fludrocortisone. Both a repetitive or stereotyped movement before losing suboptimal response to levodopa and symmetrical consciousness that would suggest automatisms or bradykinesia and rigidity are typical of Parkinson-plus tonic/clonic activity. Additional components of her idiopathic PD. Ch. Although shoulder injuries such as a language and can have electrographic status epilepticus rotator cuff injury could certainly cause decreased arm of sleep seen on EEG. droxidopa. C. Attention. which can result in disciplinary peripheral neuropathy is not likely given the problems at school or home (see Bradley’s NiCP. D. If the triceps is also weak. with little or no adaptation. that often precede the diagnosis by up to 10 years. Landau-Kleffner syndrome is an acquired epileptic pointing further toward a neurodegenerative alpha- aphasia in which children slowly lose previous verbal synucleinopathy. pp. 17. and/or transthoracic echocardiogram if of contrast enhancement. Meissner corpuscles are the most sensory studies. social and REM sleep behavior disorder make this less likely. Ch. unlike the progressive course in this case. an electrocardiogram. B. idiopathic PD is usually myoclonus. 2. is an unexpected difficulty in learning PD. the injury must be responsible for the sensation of pressure and vibration. other historical points with the constipation involves deficits in language. The Merkel disks sense light touch and pressure and are tables show normal median and ulnar motor and slowly adaptive. D. hyperactivity. abnormalities on MRI imaging. Postural instability consciousness. jerklike movements that were likely supranuclear palsy (PSP). memory.  Hypophonia is often appreciated along with based on the history of recent vigorous exercise. both 1 15. These historical radicular or isolated posterior interosseus lesion (see clues point toward bradykinesia and rigidity. permission. Although not relevant to this there is a high suspicion of a cardiac etiology. 7th edn. There was a loss of bladder syndromes. 366–390).

It can result indicating vascular parkinsonism. The lateral nuclei carbamazepine have the potential to worsen contain the “feeding center. Bilateral T2 hyperintensities are the loss of REM atonia. associated word is presented is called priming. This disorder involves vertical diplopia that is worsened by looking down the . which acts Triple H therapy (hypertension. CHRNA2 mutations are common in the brain. 101. but is not the best ventromedial nuclei serve as the “satiety center. The woman was prothrombotic due to the release of prolactin. and intort the progressive supranuclear palsy. 65. Finally. and dopamine. 7th edn. which stimulates adrenocorticotropic hormone. 7th edn. Ch. The option for generalized epilepsy. and is such as would occur with a bulging disc. it would demonstrate increased rather than parkinsonism (see Bradley’s NiCP. in the question are examples of nondeclarative (or volume lumbar puncture would be a treatment option implicit) memories.  The trochlear nerve innervates the superior oblique neurodegenerative conditions like Parkinson disease. Normotension and observation would not be 7th edn. would lead to diabetes insipidus (see Bradley’s NiCP. gonadotropin-releasing hormone. with permission. kicking such as hypertension.  The suprachiasmatic nucleus of the hypothalamus Valproic acid is also used in absence epilepsy. and weakness although if performed. Platelets and clotting factors are Not mentioned is the supraoptic nucleus. The hypovolemic in the sun. D. Cervical cord in injury to the patient or the bed partner. and cognitive decline. pp. Predicting the weather based on cues is incontinence. patients generally have atrophy. in men.” where choice listed (see Bradley’s NiCP. An arthroscopic cerebellar and amygdala function (see Bradley’s NiCP.  The case and image are classic for cerebral venous releasing hormone. or walking. 7th edn. usually with unfractionated heparin. With trochlear nerve palsy. including spasticity. the hypothalamus has addition to staring episodes. Ch. Levetiracetam is also a good A lesion here would lead to decreased oral intake. C. First. In this particular age group. 25. hormone. levodopa is considered the gold standard for treatment Ch. In contrast. The arcuate nucleus produces a number of Mazziotta JC. which later inhibits thrombosis. temporal lobe and hippocampi. a lesion here would lead to obesity and savage 1563–1614). Ch. 102. it releases somatostatin. 3rd edn. It is typically seen in association with 28. D. Elsevier. eventually becomes dopamine once it passes the and anticholinergics can cause or exacerbate this blood–brain barrier and presents itself for storage into disorder. 23. The other items listed or catechol-O-methyl transferase inhibitors. This involves talking. Figure from Daroff RB. multiple system eye. urinary frontal lobes. 96. Love S. The pathology image paraventricular nucleus releases three major hormones demonstrates thrombosed cortical veins as well as that control the pituitary function.20 Neurology Self-Assessment Parkinson disease. muscle. movements. outside of the blood–brain confused with frontal seizures due to the occurrence at barrier. monoamine oxidase-B inhibitors. hyperreflexia. 696–712). 57–65). depress. arm movements. receiving input from the basal ganglia and which typically presents with gait apraxia. PSG is not necessary for the diagnosis. Levodopa RBD. as well as compression from a disc protrusion would cause a result in very vivid dreams often confused with compressive myelopathy with upper motor neuron hallucinations. medication for absence epilepsy is ethosuximide. The best treatment for cerebral CRH. Expecting an event with a medical treatment of vascular risk factors would be certain stimulus is called classical conditioning. Figure disorder (RBD). including Clinical Practice. which sits appropriate for intracerebral hemorrhage if there is above the optic chiasm and helps to produce history of antiplatelet or anticoagulant use or vasopressin (antidiuretic hormone). features of parkinsonism and the decreased arm swing 26. Jankovic J. A. carbidopa/ night and odd behaviors (see Bradley’s NiCP. findings of an abrupt onset of motor activity during 22. This is a disorder that generally starts from Ellison D. et al. sedative-hypnotics. electromyogram activity during REM sleep. PSG Ch. RBD can be levodopa in the periphery. which is localized decompression would be indicated if a patient had to the basal ganglia. pp. Amitriptyline. would include explicit) memory. pp. The best (see Bradley’s NiCP. behavior. which releases thyroid stimulation anticoagulation. Tying one’s shoe is a procedural for patients with normal pressure hydrocephalus. 52. which is localized to the medial dopamine agonists. B. Both the anterior and posterior smoking and estrogen use and likely became nuclei serve to regulate temperature. of Parkinson disease. Other options (not listed). whose job is to abduct. and various dementias. it releases superficial hemorrhage. surgical repair could be considered if there were no 7th edn. hemorrhage. appropriate for cerebral venous thrombosis (see 24. and is a indicated for vascular parkinsonism. but receives input from the retina and helps to control typically if there are generalized tonic-clonic seizures in circadian rhythm. hypervolemia. Pomeroy SL 2016 Bradley’s Neurology in hormones that affect pituitary function. 920–967). 1422–1460). 7. Surgical an example of probabilistic learning. growth hormone– 27. other tricyclics. and tobacco use. pp. A high. Recalling a word when an weakness and pain from a compressive myelopathy. not treatment of choice for this patient. Phenytoin and two nuclei that control appetite. 7th edn. memory. with permission. venous thrombosis is volume expansion and It also releases TRH. resulting in patients acting out typical findings in patients with vascular risk factors their dreams. A lesion here laboratory evidence of clotting cascade insufficiency. Chimelli L. 7th edn. 7th edn.  A trial of carbidopa/levodopa would be the slow wave sleep are consistent with night terrors. generalized epilepsy.  Recalling a birthday is an example of declarative (or depending on disease severity. pp. signs. Ch.  This patient presents with REM sleep behavior Bradley’s NiCP. 23. Elsevier. 223–249).  The EEG demonstrates the typical 3-Hz spike and and pain were found to be due to orthopedic injury wave pattern seen in absence epilepsy. hyperlipidemia. 2013 in middle age or in the elderly and is more common Neuropathology. Aggressive localized to the neocortex. yelling. E. Ch. to inhibit release of growth hormone (useful in the hemodilution) is appropriate for subarachnoid treatment of acromegaly from growth hormone excess).” which stimulates appetite. pp. 1615–1685). pp. Carbidopa prevents the breakdown of autosomal-dominant frontal lobe epilepsy. B.

pp. pp. Resting gaze tends to have hypertropia ipsilateral impact on social functioning and can include changes 1 to the affected eye. or undue influence.  A negative reinforcement is an aversive event that is does not require a signed legal document outlining removed when a desired behavior transpires. the patient. 7th edn.  Citalopram is a selective serotonin reuptake chores or taking a child to Disneyland. pp. proprioception and light touch sadness. with a significant stairs. treatments given cleaning the room is the desired outcome. 1187–1195). although these are helpful in question. 104. NiCP. it would not cause hyponatremia. which has extensive uremia. but not by ESA administration. 19. and may be increased with electrolyte disarray or severe posterior entorhinal cortex. The Bielschowsky head tilt test may in sleep. also help localize the affected nerve. B.  Olfaction involves transmission of sensory Peripheral neuropathy risk is increased with chronic information through the olfactory bulb to the anterior uremia. recent evidence from large randomized trials has pediatric tumors listed (see Bradley’s NiCP. The superior polycythemia. but they are not decision should be made free of coercion. E. patients above the age of 15 and is usually comorbid 29. The discussion in social functioning. fast eye movements paired with diffuse myoclonus.  Opsoclonus-myoclonus is a rare. Informed consent 33. Impaired social interaction understand those risks and benefits. his A-alpha and A-beta fibers are likely Depression in younger children usually presents with unaffected. Test One 21 nose—watching television from bed or descending or anhedonia for at least 2 weeks. but components and even have smells associated with not directly from ESA. either as autoimmune or as an anti-Ri 31. Tricyclic for running into the street. and often decision makers about risk. particularly orienting to ESA administration (see Bradley’s NiCP. major depressive disorder involves a depressed mood They also may report coldlike pain. A positive against consent or without consent can be considered reinforcement is a desired event that occurs as a result of assault or even battery. akathisia. Autism is a disorder with impairments results of a medical intervention. 7th edn. Ch. Ch. Many patients report the feeling of a it involves anger. gynecological under scrutiny in recent years.  ODD is marked by consistent hostility and defiance 36. conjugate sedation. and hopelessness. Individuals with chronic authority. such as taking away video games. interest in activities. Ch. or withdrawal.  Administration of erythropoiesis-stimulating agents (also known as anti-ANNA2)–mediated paraneoplastic (ESA) to patients with renal impairment has come disorder related to small cell lung cancer. such as paying money for completing 30. but are otherwise not directly related colliculus is involved in vision. A. aggressiveness. Seizure risk olfactory nucleus. Dementia risk is increased by both elements of smell to be broken down into different chronic renal failure and cerebrovascular events. preoccupation with between the physician. or societal stress. He has a normal generally accepted. with inhibitor that can precipitate a hypertensive crisis if about 50% paraneoplastic presentations related to used in the setting of other serotonin-enhancing neuroblastoma. The patient’s can lead to oppositional behaviors. Whereas historical cancers. In the potential complications. appetite. energy levels. C. Trazodone overdose would cause behavior. E.  Informed consent is the process by which medical in patients with ODD. pp. or substance abuse. and should be in a manner which they can understand. or if it leads to he has normal vibration and pin prick on his potentially dangerous or criminal behaviors. In most states. imagination. amygdala. and any medical success or power. because his small peripheral nerve fibers are affected ODD) when the defiant behavior causes significant (myelinated Aδ and unmyelinated C-fibers). A negative punishment is antidepressant overdoses can cause fatal cardiac when a desired stimulus is removed due to unwanted arrhythmias. 7th edn. Often these patients display stereotypes or and the patient should have the capability to other repetitive behaviors. and lack of unconscious compensatory movement (see Bradley’s remorse. There are no paraneoplastic treatment goals were to obtain normal hemoglobin disorders commonly associated with the other levels. risk in patients treated to normal hemoglobin values. D. arrogance. although he is symptomatic Oppositional defiance is considered disordered (e. A. 920–967). examination. 35. Notably. manipulation.  This case discusses the most common scenario of an of authority figures out of proportion to what is early peripheral polyneuropathy. and extrapyramidal symptoms. 7th demonstrated an increased cardiovascular and stroke edn. which is known for causing hyponatremia. and about 50% autoimmune (often medications or if the diet is high in tyramine (see parainfectious). Ch. 1209–1225). trochlear nerve palsy may have a chronic head tilt as an deception. tingling. Headaches may relate to event memory and emotional memory. This allows for administration. Antisocial away from the affected eye leads to globe extorsion and personality disorder involves a pattern of disregard for correction of the diplopia. anxiety. Opsoclonus-myoclonus may present in Bradley’s NiCP. children with ODD do not neurological examination with no evidence of large- have associated depression. 81. usually devious. communication. Phenelzine is a nonselective monoamine oxidase This condition is most common in children. anxiety. or breast cancers. prepyriform cortex. 34. the eyes and head to different stimuli (see Bradley’s Ch. a desired behavior. worry. fiber nerve dysfunction. punishment is when an unwanted behavior is answered Although a nortriptyline overdose would cause by an unwanted consequence. 65.g. the nagging is the negative reinforcer. benefits. Narcissistic personality disorder decisions are made. adults as well. 32. Given that personal. as tilting the head and feelings of guilt or hopelessness. somnolence. Typically. family. 1720–1735). pp. but is generally not increased with ESA interactions with the hippocampus. A diagnosis of wrinkle in their sock or small pebbles in their shoe. This personality disorder is diagnosed in NiCP. 7th edn. impulsivity. such as yelling at a child somnolence. failure to conform to social norms. envy of others. and malpractice cases. or pins . 84. whereas in teenagers are also normal. involuntary. 190–196). disabling disorder Aripiprazole is a partial dopamine agonist and causes in which a patient has rapid. Positive inhibitor. This involves a conversation involves a grandiose self-opinion. B. but would not cause hyponatremia. and expected lack of empathy.

and Brushfield advances in understanding. rim enhancing. is most particularly acetylcholine in the neuromuscular commonly intraaxial. There is approximately a 9% ipsilateral stroke intraventricular tumors (either lateral ventricles or. Both valproic acid and topiramate may be the federal Department of Health and Human Services. 920–967). of physicians and their patients through many avenues. but are associated with and administers Medicare and Medicaid coverage. <70% stenosed. devices. estriol. risk in the next 2 years with CEA versus a 26% risk if more commonly. apnea. lupus. The abnormalities. Other commonly seen abnormalities include Figures from (A) Levin AV 2009 Retinal hemorrhages: atlantoaxial instability. daily preventive the safe delivery of healthcare. 50.  The patient has a symptomatic left carotid artery cellularity. 7th edn. 7th edn. Cyclic guanosine monophosphate characteristics of the image include the lightbulb– is activated in this pathway of vision.  Trisomy 21.  The image most likely demonstrates a meningioma activation of a G-protein–coupled complex and arising from the convexity. Pediatric Clinics of North spots on the iris. but diabetes or mechanical heart valve. CMS is a component of of asthma. but component of the federal Department of Health and frequent use can result in rebound headaches (see Human Services. given the patient’s refusal to use birth well as the Children’s Health Insurance Program and control. circulating fetal cells. these would be less favored. Emergency Clinics of North Alzheimer disease (see Bradley’s NiCP. approval and monitoring of medical therapies. often presenting with multiple lesions. Ch. 30. pp. Churchill Livingstone. Ch. Given that the right carotid is asymptomatic and Surgical Neuropathology: A Diagnostic Approach. The most telling eventually vision. and junction (see Bradley’s NiCP. Glioblastoma would generally appear which leads to a conformation change in the protein as an intraaxial. Given this information. The 39. A. efficacious for migraine. demonstrates hypointensity on an apparent diffusion pp. diagnosis of small-fiber neuropathy is frequently made Screening is often done for trisomy 21 during with a skin biopsy. abuts the ventricular system. NiCP. Sjögren syndrome. 17. Sumatriptan is parts of the Affordable Care Act. Guillain-Barré syndrome. flattened nose.  The images demonstrate flame and blot retinal with physical abnormalities including slanting eyes. preferably within 2 weeks from symptom watershed distributions. Light isomerizes retinal. 37. coefficient map related to restricted diffusion from high 40. which shows a reduction in the pregnancy. 314–323). 103. 1686–1719). and often in left CEA. the initial step. enhancement. and the dural tail at the used by photoreceptor cells. Down syndrome is Ch.  JACHO is a nonprofit organization that focuses on Given the frequency of her headaches. A current JACHO therapy is indicated. Classically. The FDA is a useful for abortive therapy in case of migraine. with various physical and developmental amyloidosis.  The patient suffers from migraine without aura. Figure from Perry A Practical onset. and mass effect. The AMA is a pregnancy and with a history of asthma. it Beta-carotene is a lipid that can be broken down into often extends to the corpus callosum. pp. Ch. A. pp. vaccines. When activated by light there is a promotes healthcare quality and safety. but is contraindicated with a history including legislative advocacy. causing Down syndrome. Patients with Down syndrome are at America 56(2): 333–344. is associated paraproteinemias. either using the “quad screen. (B) Leetch greatly increased risk of acute myelogenous leukemia AN. superior margin. pattern among immunocompetent patients. Carotid indicated by an abnormal 2-hour glucose tolerance test artery stenting is often used for patients who are poor or abnormal fasting glucose level. complex regional pain syndrome type 1. hepatitis C. 38. There or CAS at this time. 65. and the CT shows a subdural hematoma. conformational change in rhodopsin leading to 43. as these tests detect more recently by directly evaluating the karyotype of large-fiber pathology (see Bradley’s NiCP. with permission. hemorrhages. with medically treated alone. and a single This is a classic pattern of findings for shaken baby palmar crease. or conduction studies are normal. Amitriptyline is considered a accreditation is one condition of licensure and the first-line migraine therapy. 648–675). 41. retinal. and is safe both in case of receipt of federal insurance payments. which starts the vision-signaling cascade. homogeneous enhancement pattern. Propranolol is physician organization that aims to advance the issues also a preferred medication in migraineurs of child. and plays important roles in the Bradley’s NiCP. which is extraparenchymal location. Retinal is a form of vitamin A. 163–179). E. third or fourth ventricles). Metastasis is generally Symptomatic Carotid Endarterectomy Trial study. Volt-gated calcium channels are activated to although generally homogeneous in enhancement increase synaptic release of neurotransmitters. there is no indication for a right CEA 185–217. substantial edema. In the absence of atrial fibrillation are many causes of small-fiber neuropathy. low-set ears. C. 7th edn. and inhibin-A. as teratogenicity. associated with various levels of intellectual disability. and many other health-related concerns. Other conditions operative candidates for CEA or in cases such as that can lead to small-fiber neuropathy include human stenosis induced from radiation therapy (see Bradley’s immunodeficiency virus. as well as early-onset evaluation of child abuse. Woolridge D 2013 Emergency department or acute lymphoblastic leukemia. centrally necrotic mass with rim rhodopsin. America 31(3): 853–873. Intracranial ependymomas are generally stenosis. Ch. as well as long-term disease risks. bearing potential.22 Neurology Self-Assessment and needles sensation with burning or allodynia.” which nerve fiber density. CNS lymphoma. At birth. as medical management with anticoagulation. based on the North American heterogeneous enhancement. Child protective services should be are generally hypotonic and may have obstructive sleep contacted immediately upon suspicion of child abuse. B. 7th edn. the patient should undergo a with substantial edema and mass effect. AV septal defects. Electromyography and nerve includes AFP. .  Rhodopsin is a light-sensitive protein present in AHA is a professional advocacy organization that rod receptor cells. but is not the bright. C. and paraneoplastic syndromes. pp. children with Down syndrome syndrome. with permission. 7th edn. pp. 42. there is no indication for and insulin resistance are the most frequent causes. E. macroglossia. beta-HCG. with permission.

an environmental exposure to 50. Given that the vast majority of 18. of wave V would indicate a brainstem stroke (see 49. Finally. event that the brachial plexus were also affected by a This is generally related to occlusion of distal branches Pancoast tumor. decreased hygiene). contralateral hemisensory dissection. because the tumors are focal and the pathophysiology is thought to be excess dopamine global treatment would carry unnecessary toxicity. which is the symptoms is between 1 and 6 months. 1049–1064). from the vertebral arteries. pp. or a stochastic peduncle where the oculomotor fascicles and misfolding of a normal prion protein that prompts corticospinal tracts are adjacent to each other. and presenilin prion protein PrPSc (Sc stands for scrapie. Figure from Bazari F. SEPT9 is involved in hereditary source of controversy. disease is caused by mutations in three main genes: 46. with the presenilin 1 mutation being the most sheep form of prion infection) from normal PrPC (C common mutation in early-onset disease. mood stabilizers. disorganized speech. However. there could be transient hearing loss or no deficit Because the patient in this question has auditory at all. except the duration of lesion at the next location after the cochlea. Ch. Hind M. 94. and is usually preceded by a pattern of a normal wave I and loss of wave III and V on precipitating event. contralateral cause of this patient’s left arm weakness and right ataxia) results from infarction in the midbrain Horner syndrome is right carotid artery dissection. the abnormal protein that prompts refolding of contralateral hemiparesis) localizes to the cerebral previously normal prion proteins. in the case of cervical carotid dissection. 407–410). pp. It affects 1% not be employed before pathological diagnosis or safe of the population and affects male and females surgical resection. that postoperative radiographic and clinical 48. demonstrate evidence of ischemia related to the carotid ipsilateral facial numbness. nucleus ambiguous. The tegmentum where the oculomotor fascicles and red sympathetic chain runs inside the carotid sheath in the nucleus are adjacent. 179–189). The occlusion of the middle cerebral artery. Whole-brain radiation is not used equally (although men have earlier onset). decreased Whereas stereotactic radiation may be used in cases of concentration. E. A brief during brainstem surgeries to monitor the cochlear psychotic disorder is characterized by psychosis that nerve and brainstem for acute ischemia or injury. Gerstmann syndrome Further. Electroencephalogram would not confusion) localizes to the dominant angular gyrus on be a useful test in this patient. nystagmus. due to injury to the cochlear nerve. B. by at least 6 months of positive symptoms Observation alone in this case would not be advised (hallucinations. Ch. E. in the spinothalamic tract. 1365–1379).  Weber syndrome (ipsilateral oculomotor palsy. cause an ipsilateral Horner syndrome with contralateral Wallenberg syndrome (vertigo. If waves III and V Persecutory delusions are the most common type. The grade I. Similarly. dysphagia. as well as for ptosis without miosis (see Bradley’s NiCP. because this is the normal cellular allele is common in autosomal-dominant late-onset prion form) is the three-dimensional folding. there is sufficiently low chance of recurrence Lancet 375(9716): 776. symptoms of anterior ischemia. With quick identification during surgery. remains after surgery or recurs. which is the 2. Test One 23 44. 7th edn. if waves III and V do not disorder.74. and sympathetic tract are adjacent. B. Ong meningiomas are World Health Organization (WHO) YE 2010 Horner’s syndrome: not to be sneezed at. Chest x-ray would be a medullary lesions where the trigeminal nucleus. 1380–1421). vestibular nucleus. pp. or residual. MRI of the brain may ipsilateral ataxia. symptom relief. Ch. Claude 47. reasonable test to look for a Pancoast tumor. 7th edn. (atypical > typical). because seizure would be the parietal lobe.  Brainstem auditory evoked potentials are useful electroconvulsive therapy. Preservation of waves I and III with bilateral loss hallucinations. poverty of speech. This may be caused by an neuralgic amyotrophy. undifferentiated. results from occlusion of branches from the posterior Ch. which then multiplies. the recent trauma in this patient reduces the (agraphia. Schizophreniform disorder is the right and a normal BAEP on the left indicates a similar to schizophrenia. (D2 receptors) in the limbic area and left amygdala. and psychotherapy. The ApoE4 stands for cellular. schizophrenia is a better answer. Delusional disorder is characterized by return. However. with permission. Different subtypes WHO grade II or III meningioma where residual tumor include paranoid (best prognosis). return. 38. in this patient that demonstrates reoccurring episodes of the surgeon can quickly adjust the procedure to have mania or periods of depression that are seen in bipolar the responses return. A. pp. which may restiform body. acalculia. disorganized.  In sporadic prion disease. Dejerine-Roussy . B.  Early-onset autosomal-dominant Alzheimer Bradley’s NiCP. although that would present with 1 pathological diagnosis and grading. this results from neck and.  The mainstay of treatment for meningioma is edrophonium test would be useful to diagnose maximal safe resection. This syndrome is often related to quite unlikely to cause Horner syndrome. presenilin 1.  Schizophrenia is a psychiatric disorder characterized observations are sufficient for management thereafter. The for meningiomas. affect. finger agnosia. C. 7th edn. 7th edn. the only change in the amyloid precursor protein. the weakness would also be ipsilateral. but would not be the most direct means of loss to pain and temperature) results from lateral diagnosing the problem. which is useful both for myasthenia gravis. This a cascade of the same (see Bradley’s NiCP. there is likely significant permanent hearing loss nonbizarre delusions that last more than 1 month. this would generally catatonic. 7th edn. the most likely syndrome (ipsilateral oculomotor palsy. cerebral artery (PCA) or the top of the basilar. may occlusion of branches of the PCA or top of the basilar. disorganized given the progressive neurological deficits and the mass behavior or delusions) or negative symptoms (blunted effect demonstrated on the magnetic resonance image. anhedonia. not Alzheimer disease (see environmental exposure to the abnormal protein Bradley’s NiCP. chemotherapy has little role in meningiomas Treatment is characterized by antipsychotics (see Bradley’s NiCP.  Given the history of recent trauma. How Alzheimer disease but does not generally play a role in abnormally folded PrPSc proteins come to be is still a early-onset disease. The lasts for <1 month. Ch. and left–right likelihood of a tumor. There is nothing cochlear nerve. pp. ipsilateral Horner syndrome. 95. antidepressants. 45. cause ipsilateral Horner syndrome.

vomiting. Frohman EM 2013 Isolated Depression affects 10%–25% of women and 5%–12% mammillary body involvement on MRI in Wernicke’s encephalopathy. Imaging is often Cingulate normal. near-drowning). such as agranulocytosis and broad. 57–65). The commissure presence of symptoms for at least 2 weeks. nearly every Olfactory day. oxcarbazepine. and has 50–70). However. including infections (congenital toxoplasmosis. and suicidal ideation. along with a change from her previous state. pregnant. the the mammillary bodies. Frohman TC. which facilitates thalamic nucleus. Oxcarbazepine and carbamazepine both Ch. 7th edn. Ch. and metabolic (although not the most common) are life-threatening (hypothyroidism).  Maternal rhinovirus infection has not been anticonvulsants. Valproic acid is an anticonvulsant with mood- 53. moderate (IQ = 35–49). Oxcarbazepine has a slightly different pediatric patient. E. Finally. MR can be classified as mild (IQ = chemical structure than carbamazepine. 104. D. and the entorhinal cortex. loss of interest. Ch. environmental (alcohol drug levels. 1720–1735). she Amygdala Subiculum meets criteria for major depressive disorder.24 Neurology Self-Assessment syndrome (ipsilateral tongue weakness. produced by the choroid plexus. 111. loss of concentration. worthlessness.55 The components of the Papez circuit are agitation or retardation. which interrupts this pathway. and side effects include weight the onset was more acute. volume of CSF in an adult is replaced approximately hair loss. The circuit includes the hippocampus. evaluation of lesion. Diagnosing the underlying cause rashes such as Stevens-Johnson syndrome or toxic . cingulum. 55. and EEG typically shows epileptiform activity gyrus that is improved with the ketogenic diet (see Bradley’s Fornix NiCP. in bold font. guilt. The most well-known side effects (hemorrhage.  The circuit of Papez is a major pathway involved in pp. C. issues.  CSF. A.  Mood stabilizers consist of lithium. Serum drug levels should be disruption in her social and occupational function. contralateral of MR is performed by neurocognitive testing. valproic acid. in part. mammillothalamic tract. sadness. acquired serum levels. This means that the entire action. where the hypoglossal nucleus. fragile X syndrome). pp. by inhibiting exposure leading to fetal alcohol syndrome). dysmorphic features. and evaluation of growth curves. epileptic encephalopathy with infantile-onset seizures. fornix. Evaluation shows a remarkably low cerebrospinal fluid glucose level. cause signal pathways. recent stressor. In this some atypical antipsychotics. or poor social support. 1956–1972). including a combination of selective serotonin 56. as well as psychotherapy. 7th edn. pp. loss of energy. are sodium channel inhibitors that are used as 54. There is no indication that gain. same gene that is mutated in paroxysmal exertional disrupting the ability to learn new information or dyskinesia. pp. 7th edn. The potential etiologies are fewer side effects. Lithium works by case dysthymic disorder is the incorrect answer because inhibiting inositol and glycogen synthase kinase 3 the patient’s symptoms are more severe. A. evaluation proprioception) is related to a medial medullary for speech and language delays. tremor. and neural tube defects if taken while four times per day (see Bradley’s NiCP. reuptake inhibitors or serotonin-norepinephrine lamotrigine. hyponatremia. 7th edn. developmental delay. decreased appetite. carbamazepine. Ch. from Beh SC. substance abuse. severe (IQ = 20–34). of men and is more likely to occur when there is a Journal of the Neurological Sciences 334(1–2): 172–175. the patient has any symptoms of hypomania. anterior glucose transporter type I (GLUT1). Lamotrigine acts. This generally Depending on the level of severity. and even reuptake inhibitors. nephrogenic diabetes insipidus (leads to there is no indication that the patient’s symptoms had hypernatremia). is produced at stabilizing properties through enhancing GABAergic approximately 20 mL/hr. as well weakness and sensory loss to vibration and as a good prenatal and perinatal history. nausea. The In Korsakoff syndrome frequently there is damage to GLUT1 protein is encoded by the SLC2A1 gene. Fatigue and ataxia can occur with higher cytomegalovirus. but also have mood-stabilizing associated with mental retardation (MR) in the properties. as well as and profound (IQ ≤ 20). medical permission. Anterior thalamic Corpus nucleus 52. glucose transport across the blood–brain barrier. nausea. 51. given Mammillary bodies recurrent episodes more than 2 months apart. remitted at a different time of year. 1261–1278). Side effects include tremor. and medial lemniscus are adjacent. thyroid dysfunction. 88. Other Entorhinal Hippocampus common symptoms of depression include SIG-E-CAPS: cortex sleep disturbance (insomnia or hypersomnia). genetic serotonin reuptake and does not require monitoring (trisomy 21. 7. Treatment involves both medicinal and nonmedicinal modalities. complex movement disorders. GLUT1 deficiency often leads to an retrieve recent memories (see Bradley’s NiCP. psychomotor Figure for Answer 1. perforators (see Bradley’s NiCP. rubella). is bulb classified as major depressive episode. and frequently monitored. pyramidal tract. and microcephaly. pregnancy.  The case describes an infant with deficiency of mammillary bodies.  The described symptoms of anhedonia (loss of callosum Mamillothalamic interest). appetite change (increased or decreased). theoretically less metabolism by the liver. D. thrombocytopenia. with family history. memory. and negative chronotropic effect on a temporal onset with a season change or that it the heart (contraindicated in sick sinus syndrome). patients may be results from occlusion of the anteromedial artery able to fully care for themselves or require maximal (off the vertebral artery) or from basilar artery assistance. Anterior tract and decreased energy all point toward depression.

6) a 3 a post ill C6 Ax 3 rry Ca ds erio r r Sup C7 co 3 rm Lateral pectoral A r s dle Mid epe al nerve (C5. 7) r rio ste Po Contribution 1st rib from T2 Axillary To longus colli and scalene nerve (C5. pp. Test One 25 epidermal necrolysis. Further. 106. Zager EL. with permission. Magnetic Resonance Imaging Clinics of North America 20(4): 791–826. 6) al Medi muscles Radial nerve (C5. B. 6. The such as tumors in the optic pathway. 8. T1) 1st intercostal nerve Median nerve Long thoracic (C5. The nystagmus terminal branches of the posterior cord are the radial can be horizontal. Ch. 7. 6. which is a triad of topiramate and gabapentin. the lower spasmus nutans may be found to have structural causes subscapular nerve. T1) Ulnar nerve (C7. The medial pectoral nerve branches pronounced more in one eye than the other. and cervical 1 dystonia. 6. but occurs in up to 30% of the patients e calen Inters e ria ngl Some or T ts teri 5 roo mi of Pos icia ns (ve n t r a l ra s) ve Contribution h n l ner from C4 Tec spina s Dorsal scapular unk Dorsal l e 3 tr nerve (C5) ic ramus av To phrenic Cl s Suprascapular n’t on s isi sion nerve Do i v d iv i nerve (C5. 7th edn. The long thoracic nerve the nystagmus is almost never synchronized between is derived from C5. 7. T1) Upper subscapular nerve (C5. The cervical dystonia is not NiCP. D. 7) C8 in s Be r m che Te ran b l ior T1 ra er at e Inf Musculocutaneous L nerve (C5. 8. The head motion associated with a terminal branch of the lateral cord. and C7 nerve roots before the the two eyes. Most cases begin between 4 and 12 months 57. Loevner LA 2012 MR Imaging of the brachial plexus. 1766–1790). T1) Medial brachial cutaneous nerve (T1) Medial antebrachial cutaneous nerve (C8. or rotatory and is usually and axillary nerves. directly from the medial cord. 8. Other mood stabilizers include 58. 8) (C5. 8) Lower subscapular nerve (C5. T1) nerve (C5. pendular nystagmus.57 (from Mikityansky I. 6. head nodding. 6) Inconstant contribution Thoracodorsal (middle subscapular) nerve (C6. always present.  The patient has spasmus nutans. Yousem DM. and the thoracodorsal nerve.  Nerves branching directly from the posterior cord of age and are sporadic. The suprascapular spasmus nutans is usually oblique with both nodding nerve comes directly off of the superior trunk. 7) Medial pectoral nerve (C8. The musculocutaneous nerve is dysconjugate gaze. although rarely patients with include the upper subscapular nerve.) . C6. See the and shaking and is usually a compensatory measure representation of the brachial plexus (see Bradley’s that suppresses nystagmus. 7. 6) C5 ior r d To subclavius n ter erio nerve (C5. 6) Figure for Answer 1. 6. 7. vertical. and most often is associated with a brachial plexus forms. 6.

  Availability bias is a common diagnostic error in myopathy with proximal leg weakness. whereas posterior temporoparietal of head nodding or cervical dystonia. Benign hypometabolism is seen in Alzheimer disease. Diagnosis momentum simultaneous neck extension. E.  The clinical presentation and MRI findings are anticholinergic medication used in the treatment classic for neurocysticercosis. neither a biopsy nor antibiotics would be deficiency is common in patients who have pernicious warranted in this patient currently. and Pediatric Neurology (48)6: 432–435. usually edema or inflammatory disease (see Bradley’s NiCP. Ch. Other the diagnosis. Carbidopa reduces the peripheral with Taenia solium. in the treatment of Alzheimer disease and dementia with Parkinson disease. Trihexyphenidyl is an 63. 60. The other main enzymes are he has some impulsivity and hypersexuality. pp. 1956–1972). 1147–1158).  Entacapone is a catechol-O-methyl transferase trazodone. can cause a edn. usually resolve. then the hands are involves accepting a prior diagnosis without confirming brought back together and the child cries.  The patient presents with behavioral variant nystagmus almost always begins at less than 6 months frontotemporal dementia (FTD).  Dabigatran is a direct thrombin inhibitor approved head ipsilaterally. Rivaroxaban and Galant reflex) is elicited by turning the head to one apixaban are factor Xa inhibitors also used for stroke side. a tapeworm infection of dystonia. this usually disappears after 1 month for the prevention of stroke in the setting of of age. 7th edn. the cysts on this patient’s MRI appear active with a scolex. Vitamin A toxicity is report of seven cases and review of published patients. which causes the ipsilateral arm to extend while prevention in the setting of nonvalvular atrial the contralateral arm bends and the patient appears to fibrillation. which can be seen of cervical dystonia. B. MRI may show forced gaze deviation or head deviation rather than anterior temporal and frontal atrophy. Ch. Whereas treatment with an antihelminth may be safely pp. 1380–1421).  The Moro reflex is a primitive reflex seen in the second patient. As such. 85. 7th edn. sensory ganglionopathy. 7th edn. primitive reflexes include the rooting reflex in which stroking the cheek will cause the newborn to rotate the 65. which is one of the main enzymes antipsychotics are used for behavioral control.  This patient presents with signs and symptoms of suggesting that treatment may be beneficial in reducing subacute combined degeneration of the spinal cord. Ch. pseudotumor cerebri.26 Neurology Self-Assessment with spasmus nutans. In addition. 7th doses of more than 200 mg/day. Vitamin B12 seizures. with permission. 79. points to FTD rather than a manic episode (see Rivastigmine is an acetylcholinesterase inhibitor used Bradley’s NiCP. as there is lateral columns of the spinal cord. Frequently 59. 1422–1460). 528–572). pp. 1237–1253). but does not have nystagmus (see histologically as Pick bodies. A. avoided in cases of completely calcified cysts. including impulsivity. pp. This causes a loss of no evidence of viral infection in the history or on MRI. Vitamin B6 toxicity. and is rarely used in months of age. Ch. Idiopathic spasmus nutans is until 1 year old or until the child begins walking (see considered self-limiting and transient. joint pain. FTD is paroxysmal torticollis may have intermittent episodes caused by a buildup of tau protein. FDG-PET nystagmus or nodding. which is marked by old and is not associated with head nodding or cervical early behavioral changes. Other larger amplitude. treating with both which is a severe consequence of vitamin B12 levetiracetam and albendazole is warranted. The most common neurological presentation of vitamin D deficiency is a mild 64. ALS. of undercooked pork. 95. Finally. The extensor plantar reflex is present thrombotic stroke prevention. dystonia. in severe OJ 2013 Neurocysticercosis in infants and toddlers: cases. which affects the posterior columns and Levetiracetam and acyclovir is not suggested. This disease is commonly seen but does not affect central nervous system dopamine in individuals from Mexico and South America. Ch. congenital nystagmus has a hypersexuality. are vegan. and apathy. 7th edn. deficiency. the monoamine oxidase B and dopamine beta hydroxylase presence of memory loss and language dysfunction (which converts dopamine to norepinephrine). The tonic neck reflex (previously known as the nonvalvular atrial fibrillation. and its presence diagnosis is assumed and not changed despite later could indicate developmental delay. anemia. using a test to prove a diagnosis rather than using tests which causes the hands to extend upwards with to rule out alternative diagnoses. obsessions. subsequent seizure risk. Confirmation bias is elicited by a quick movement of the head or a startle. Anchoring bias occurs when a newborn until the age of 4 months. The reflex is identifying contrary evidence. 96. this may cause him or her to be hypersensitive and quick to diagnose it (even if incorrectly) in a 61. 111. and does not common features include difficulty with planning. selective serotonin reuptake inhibitors. Lithium responsible for the breakdown of dopamine in the is used in the treatment of bipolar disorder. which is often related to ingestion destruction of L-dopa (a precursor to dopamine). Seizures are typically associated with early loss of speech. tangles are seen in Alzheimer disease. and inhibitor. Ch. sensation to vibration and proprioception and can Although lacosamide may also be used for focal result in weakness in severe cases. metabolism (see Bradley’s NiCP. diagnosis. lasting only a Bradley’s NiCP. few months to years. 7th edn. methylprednisolone would not necessarily provide any Vitamin E deficiency leads to a clinical picture of a therapeutic benefit for this patient in the absence of spinocerebellar ataxia. or have had gastric bypass surgery. A Chiari malformation may scans show anterior temporal and frontal lobe have downward nystagmus but without the association hypometabolism. It is also which a diagnosis is made by referring to a recent associated with diffuse bone pain and muscle experience (or what easily comes to mind) to make a pain (see Bradley’s NiCP. pp. Although central nervous system. whereas neurofibrillary Bradley’s NiCP. This should disappear by 6 that blocks platelet aggregation. Cilostazol is a phosphodiesterase inhibitor be in a “fencing” posture. Figure from Del Brutto predominantly sensory polyneuropathy and. Alternatively. and compulsions. congenital 62. Ticlopidine is an . slower frequency. D. If a provider recently missed a diagnosis of pp. D. 44. A. C. associated with dry pruritic skin.

Without a family history and given her pregnancy status. as the with the cards. or cause weight gain. steroid taper is generally preferred and various levels of encephalopathy can be seen (see if exposure has been sufficiently long to warrant it. Ch. There is a high risk of recurrence with future pregnancies. D. diarrhea. with Cushing syndrome. tremor. There is no indication of paranoia such as distrust or suspiciousness to suggest paranoid personality disorder. discharges with spikes and slow waves. but these are rarely required and not necessary in most patients. 67. lipodystrophy. which are receptors. with movements. pp. insulin Obsessions are recurring ideas or thoughts that are insensitivity. A.  The EEG demonstrates triphasic waves. Elsevier.) . Ch. but have a “lurching” gait. Panic disorder is less likely. there is no indication that these are motor including the Wisconsin Card Sorting Test and Trail tics. often excessive sweating. C. The EEG in acute cortisol production by the adrenal glands. constipation. Compulsions serum T4 is associated with hypothyroidism. Low serum glucose is generally affecting school or workplace performance. which are evaluated with a variety of tests. as seen 7th edn. tricyclic intake. checking. Test One 27 adenosine diphosphate receptor inhibitor that acts helpful with tics but not chorea (see Bradley’s NiCP. Pomeroy SL 2016 Bradley’s Neurology in Clinical Practice. Wilson disease would show Kayser- Fleischer rings on slit lamp examination. decreased caudate size and hypermetabolism in the planning. as there is no indication of preceding urge or sense Making Test. 1867–1895). but is rarely used in thrombotic Ch. The adrenal be seen in other metabolic or toxic encephalopathies. syndrome. but seizures availability. Most patients realize that the High serum T4 is associated with hyperthyroidism and obsessions or compulsions are unreasonable and may cause weight loss. A. or excessive. The EEG in herpes norepinephrine. palpitations. 68. patient’s ceruloplasmin and liver function tests are Aldenkamp AP 2008 Performance of children with epilepsy on the normal. weight loss. The Wisconsin Card Sorting Test involves of relief after. Patients are able to walk. but they can system that releases acetylcholine. medulla is innervated by preganglionic sympathetic The patient’s history of alcoholism should also point fibers resulting in the release of epinephrine and to hepatic encephalopathy. 108. muscle weakness. Low intrusive. The parasympathetic system only encephalitis would show temporal periodic lateralized releases acetylcholine—it does not innervate epileptiform discharges (PLEDs). Although OCD can coexist with Tourette inhibition. which can be 70. and frontal cortex.  Prolonged corticosteroid use may suppress natural confused with triphasic waves. and agitation. and low blood pressure. Low doses of haloperidol or similar dopamine antagonists can be useful if treatment is needed. 84. causing great anxiety and stress. 7th edn. palpitations. In OCPD. organization. as there is no a patient given a set of cards and told to make matches mention of panic attacks. 65. Mazziotta JC. an autosomal-dominant disorder like Huntington disease is less likely. Hendriksen JGM. no sense of relief with the or lateralization? Epilepsy Research 83(2–3): 184–189. sympathetic fibers releasing acetylcholine to muscarinic 66. repeating actions. and edema. 112. Clonidine is permission. forms of behavioral therapy. nonconvulsive status epilepticus would show rhythmic pp. and cannot be suppressed. Sudden strokes is frequently normal. anxiety. Jankovic J. 1 stroke prevention (see Bradley’s NiCP. The EEG in sweat glands (see Bradley’s NiCP. Imaging usually shows 71. leave a patient with acutely low cortisol not have any characteristic EEG findings. similar to clopidogrel. B. but focal slowing or even discontinuation of exogenous corticosteroids may. and may are repetitive behaviors such as counting.  Executive function includes problem solving. Chorea gravidarum is not uncommon and typically starts in the second to fifth month of pregnancy and typically resolves within a few months of delivery without needing treatment. This is the only part of the sympathetic classically seen in hepatic encephalopathy. generalized Figure from Daroff RB. Her movements are not tics because there is Rey-Osterrieth complex figure test: is there an effect of localization no urge to move. As such. antidepressants. or loss of consciousness. They can reach for objects. focal PLEDs can be seen in some cases. The patient is not told what are obsessions and compulsions in this case are seen as considered matches but only told when the match is unhealthy and unwanted. Bradley’s NiCP. Treatment related to exogenous insulin use with inadequate food involves selective serotonin reuptake inhibitors. and psychotherapy involving various confusion. OCPD is incorrect. and may acutely cause sweating. immunosuppression. Symptoms of low cortisol include fatigue. 7th edn. but this Figure for Answer 1. 1209–1225).  This patient presents with movements concerning for chorea. fatigue. Meningitis does therefore. 69. thinning of the skin. 7th edn. 1973–1991). and a score is based off of the rather than OCPD. Ch. but may have extra movements on the way to that object. selective attention. they are often rational and desirable.  Sweat glands are activated by postganglionic pp. 920–967).71 (from Schouten D. High serum cortisol. C. pp. with permission. OCD can be very time consuming. which tend to go with OCD correct or incorrect. These can be described as movements that flow from one part of the body to the next without interrupting overall movements. may cause weight gain. vomiting.  OCD often coexists with Tourette syndrome.

and NMDA receptor (neural folds) into a tube. and enlarged testicles.  The common peroneal (fibular) nerve passes over birth injuries or infections. but many factors may contribute to 107. This prosthesis. immunosuppression (see Bradley’s NiCP. toxic 106 and 107. 54. Pain is described as sharp. chromosome). Tramadol increases the risk of (temporarily becoming the neural folds). hereditary neuralgic amyotrophy. represent pairs of mesodermal tissue that develop Ch. dorsal root entry zone lesions. mild MR has an condition usually occurring in patients over the age of IQ range of 55–70. or squeezing. Elsevier. Angelman syndrome. vasculitis is is often a strong family history of MR. 7th edn. 78. prior use of 76. Chs. represented by beta-blockers. Generally the symptoms of weakness and Visuospatial function is evaluated simply with the numbness will resolve spontaneously within weeks. fusion of 73. Neither Wechsler Memory Scale is a typical tool used. often including the Boston Naming Test. it presents with severe hip. although is often early. pp. 720–741). The neural tube develops between the pairs of somites. but the orthotic to prevent falls as her strength returns. This can be a Common physical signs include elongated face. but would present with 7th edn. The onset of pain Uhthoff phenomenon after delivery. Treatment will later become the nucleus pulposus of the spinal can include opiates. protuberant ears. pp. or The somites are labeled 4 in this drawing. This is a based on his IQ of 45. 54. but may be delayed for months or years. Figure from seizures at high doses due to gamma aminobutyric Moore KL. usually during times of uncontrolled diabetes. shooting. The neural crest. along the axis of the developing embryo. result from pelvic trauma. 720–741). B. metabolic disturbances. lower limb amputation. 50. high similar presentation to a vasculitic neuropathy.  The patient has moderate mental retardation (MR) formerly known as diabetic amyotrophy. Figure 17-1. foot drop may be seen with either a multiple sclerosis It is most common in patients who suffered pain flare or Uhthoff phenomenon (assuming prior before their amputation. 7th edn.  The patient has phantom limb pain. or leg pain included description of his habitus along with a strong for a few days and is followed by weight loss and family history of MR. a condition the first few postpartum months. 511–527). pp. such as vascular etiology for amputation. findings are more widespread. rhizotomies. as well as brain and spinal cord. Ch. The neural tube. the fibular head superficially. 74. and pre/perinatal 75. C. more widespread symptoms. she may compress able to do so. D. have improvement in their strength. surgical treatments may provide multiple mesenchymal cells. with permission. smooth muscle cells. D. Ch. 7th edn. B. which typically affects though it often includes premature ovarian failure. is unknown. fragile X syndrome is most likely. Prader-Willi syndrome.  This patient has diabetic radiculoplexopathy. Clock Draw Test or the Rey-Osterrieth complex (see the although the patient may benefit from an ankle–foot figure). and severe MR has a range of 25–40. oxytocin nor epidural anesthesia causes Intelligence is tested using an IQ test. This increases the risk of serotonin the fourth somite. 1766–1867). but forehead.  The most likely diagnosis is a complex partial if a woman holds back her legs around the knees or seizure with secondary generalization from an Neurology Books . moderate MR has a range of 40–55. Subsequently. neuropathy. and bone. Nonmedicinal treatments can also be of the number 3. These sympathectomies (see Bradley’s NiCP. In prolonged childbirth. A lumbosacral plexopathy may Wechsler Adult Intelligence Scale (see Bradley’s NiCP. 1791–1866).  The notochord is represented by the number 1. biofeedback. It will later develop into the antagonists such as memantine or ketamine. SEPT9 mutations are seen in normal X copy. cord. lidocaine. pp. 7th edn. so syndrome due to X inactivation (lyonization) of the biopsies can be helpful. phantom limb pain. and the also block reuptake of both serotonin and ultimate division between brain and spinal cord is at norepinephrine. muscle atrophy in the proximal leg that eventually This is the most commonly inherited form of MR and is stabilizes. pp. initially forms the roof of the neural benefit. Based on the Initially. 9th edn. Although the risk of multiple sclerosis relapse is thought to increase during 72.  Tramadol is a mu-opioid receptor agonist that can the neural tube begins at the fifth somite. The neural plate becomes the neural tube reuptake inhibitors or serotonin and norepinephrine and neural crest cells after folding in on itself reuptake inhibitors. these cells differentiate into massage. E. and patients may also central nervous system lesion and therefore would not have vivid feelings of the previously amputated limb have concurrent sensory loss in the same peripheral (phantom limb sensation). represented by the number 2. Bradley’s NiCP. E. pp. The exact pathophysiology nerve distribution (see Bradley’s NiCP. this is most likely to result from a stabbing. Language is evaluated with a variety of these nerves and lead to temporary peroneal tests. such as acupuncture. and prior history of stump pain. back. disturbances. tricyclic antidepressants. A sural biopsy would be normal because the often affected (due to the dysfunction of a single X pathological abnormality is in the lumbosacral plexus. Further. benefit in refractory cases. In: The Developing Human. there is little study of that commonly follows amputation. and Torchia MG 2013 acid A inhibition and delta opioid antagonism (see Nervous system. is antiepileptics such as carbamazepine (not calcium formed by the neural plate bending and folding channel blockers). There with normal sedimentation rate and CRP. Diabetic radiculoplexopathy is causes of MR include chromosomal abnormalities such generally a single event and does not require long-term as Down syndrome (trisomy 21). Their phenotype is generally less severe. including glia. and tube. Persaud TVN. 389–427. Other the upper extremities. Memory is assessed with multiple tests. Finally. 77. and men are most unlikely. Over the course of several months. most due to a CGG expansion in FMR1 on the X chromosome.28 Neurology Self-Assessment ability to make a match and how quickly the patient is malpositions her legs in the stirrups. Ch. The neural plate is not present in syndrome when combined with selective serotonin this drawing. including surgical melanocytes. Normal IQ is >70. 43. commonly the mononeuropathies. Females may also be affected by fragile X In cases of vasculitis. symptom thereof). Rett syndrome. cartilage.

neuroblastoma or ovarian cancer as a paraneoplastic and tends to be earlier onset with greater numbers of syndrome. Miller DC. irritability. known as genetic anticipation. From a with a viral infection or less frequently with psychiatric perspective. Neurosyphilis causes psychosis. and growth hormone (see Bradley’s creates either a saccular aneurysm or a fusiform NiCP. Benign outcomes of stereotactic radiotherapy for cerebral paroxysmal positional vertigo is associated with arteriovenous malformations. 40. 459–485 and 56. patients can have depression. Other examination findings include arthralgia. Anti-NMDA receptor encephalitis headaches. This a periprocedural increased risk of stroke among syndrome often resolves spontaneously with time. mutism. seen in the MRI). They often appear in the inner ear. pp. is associated with vertigo. Treatment options include would not have absent caudate nuclei (see Bradley’s surgical resection. muscle wasting. or seizures. severe bursts of vertigo with head movement.  Oxytocin and vasopressin are produced in congenital malformation of veins that drain normal the supraoptic and paraventricular nuclei of the brain and is considered one of the most common hypothalamus. NiCP. but would not show unruptured. unknown or reportedly negative. surgical risk. D. An aneurysm is a weakening of a vessel wall that TSH. even suicide. usually lasting seconds. Neurological symptoms include extremities. prolactin. pp. and may be associated with Generally. 7th edn. Imaging will often show extrapyramidal symptoms. A developmental venous anomaly is a 81. as well as jerking (see Bradley’s NiCP. Patel MC. 7th edn. can intervene: age. 65. Chs.  Although the outcome of the CREST trial with otolith repositioning maneuvers such as the Epley demonstrated no difference in the composite risk of maneuver. luteinizing hormone (LH). also known as endolymphatic hydrops. There is AVMs are arteriovenous connections without capillary nothing in the question stem indicating a recent 1 networks. Ch. Opsoclonus-myoclonus is felt to be CAG repeats of the HD gene on chromosome 4. pp. Hochberg >3 cm). The Spetzler-Martin grading system is a scale FH 2010 Atlas of Clinical Neurology. and developmental dominant condition resulting from an expansion of delay. 583–604).  Meniere disease is caused by excess endolymph and are typically found in the pons. Typically. Anderson JFI 2014 Neuropsychological This disease may uncommonly run in families. Labyrinthitis. C. including the “letdown on contrast sequences or susceptibility weighted reflex” involved with breastfeeding and uterine imaging. and tinnitus (see Bradley’s NiCP. among patients who underwent carotid endarterectomy random movements of the eyes. most commonly associated repeats. 46. AVMs. it did demonstrate movement felt after a cruise or turbulent flight. Neurology Books . Other 80. It may improve 79. 1422–1460). myocardial infarction. tinnitus. limitations of vertical gaze. ACTH. Mal de débarquement syndrome is a vertigo stroke. location. Symptoms include episodic vertigo. Sydenham chorea.” In this case. Wilson setting of family history of suicide and drug use makes disease is an autosomal-recessive disorder resulting in HD high on the differential. but the onset Wilson disease. progressive hearing 758–783). pp. psychosis. autoimmune. HD appears with more than 40 CAG repeats. Figure from Murray AL. PiG including FSH. obsessive-compulsive disorder. genetic testing should movement disorders. or generalized). Figure from. conjugate. Jeffreys A. pp. 7th edn. endovascular NiCP. such as cocaine intoxication. Fever and still be completed. Ch. 920–967). These often present with hemorrhages. resulting in “box-car and characteristic oculomasticatory myorhythmia ventricles. the gradual onset (rather than (pendular oscillations of the eyes with rhythmic acute) of both motor and psychiatric symptoms in the contractions of the masticatory muscles). but usually has an acute onset and lifetime hemorrhage risk. Ch. or death between carotid syndrome of a persistent sense of rocking or swaying stenting or carotid endarterectomy. A. 52. usually with gliotic brain tissue within the streptococcal infection that would point toward AVM. gender. and is often thought to be associated with otolith malpositioning. truncal. Many times. 7th edn. AVMs are observed if has been associated with chorea. 696–712). it posterior pituitary gland (neurohypophysis). Dally M. Substance abuse resulting consideration when determining whether or not to in dopamine release. 7th edn. aneurysm. A stroke of the subthalamic nucleus oculomasticatory myorhythmia are not present in can also lead to choreiform movements. A cavernous malformation is a collection of contractions during labor. Neuroscience 21(4): 601–606. with permission. medications or trauma. and also lead to chorea. atrophy or absence of the caudate. especially in young. On imaging. Hwang P. Test One 29 arteriovenous malformation (AVM. Capillary telangiectasias are capillary-sized vessels that have normal brain tissue in between them 82. and aural pressure. patients will complain of Ch. diarrhea. leading to low-grade fevers. The examination 83. radiosurgery. movements of the axial skeletal muscles. and dystonia and chorea (either facial. is usually acute and leads to unilateral chorea. pp. Lane RJM. then stored in and secreted from the cerebral vascular malformations. Even if family history is accumulation of copper resulting in liver disease. but several factors are taken into an absence of caudate nuclei.  Huntington disease (HD) is an autosomal. loss in one or both ears. and a Opsoclonus-myoclonus is a rare syndrome in which periprocedural increased risk of myocardial infarction patients have continuous. The other hormones listed vessels without elastic tissue and has no normal brain in the question are released from the anterior pituitary parenchyma between the hyalinized vessels. and dementia. It drives appears as a curvilinear enhancing structure best seen many physiological functions. Tropheryma whippelii. involuntary. patients who underwent carotid stenting. Saunders. A. as ill-defined areas of focal enhancement (see Bradley’s The exact cause of the fluid buildup is not known. onset HD. embolization. symptoms include ataxia. of 1–5 that helps estimate the risk of surgical repair of with permission. distal hyperpigmentation. difficulty with sleep initiation as well. or a multimodal approach (especially if Perkin DG.  Whipple disease is caused by an infection with will often show parkinsonism. 96. B. They gland and can be remembered by the mnemonic FLAT typically appear as a “popcorn kernel” on T2 imaging. 3rd edn. or hearing loss. Journal of Clinical sudden.

Both is not generally considered in the treatment algorithm carbamazepine and valproic acid are good medications for this disorder. Although or N22 peak. 348–366). C. pp. 7th edn. Ch. failure of closure of this pore results in anencephaly (see Bradley’s NiCP. and with Rasmussen encephalitis.  On average. The P37 associated with an underlying structural cause. cataplexy. Although for trigeminal neuralgia. 7th edn.  SSEPs are used to evaluate the sensory system from surgical procedure to provide seizure relief in patients the lumbar roots to the somatosensory cortex. 1563–1614). spina bifida. 7th edn. retinal ganglion cell layer. The layers of the retina from most 90. Sleep apnea can occur in narcolepsy. where Erb’s point detects the brachial plexus illness. inner which can last for extended periods. Imaging can show atrophy of the affected hemisphere. hypnogogic External limiting membrane hallucinations. unlike the upper pulmonary function test would identify any respiratory extremity. but will not control the disorder overall. This response is analogous to the a cause. but as salvage options. 814–834). 89. sleep attacks. outer plexiform layer. and associated retinopathy: current concepts and emerging therapy. which are country based on a group of medically and surgically Neurology Books . B. Ch. Along the Rasmussen’s encephalitis: experience from a developing path various waveforms can be recorded. Levels <200 pg/mL suggest narcolepsy. The N34 is a far-field Patients may also have similar pain with other Valsalva subcortical waveform that represents the brainstem and maneuvers. 103. but no associated cataplexy. This commonly presents with focal motor seizures. Ch. Doing nothing would not be prudent.  Primary cough headache is most commonly corresponds to the typical latency expected. E. B. and a number that 85. MRI should be performed to identify such medial lemniscus. dementia. inner plexiform layer. it is not the preferred 88. with of hypersomnolence associated with hyperphagia and permission. Kleine-Levin syndrome is a syndrome and Metabolism Clinics of North America 42(4): 721–745. Failure of closure of the distal pore results in syndrome (see Bradley’s NiCP. Ch. which move up the dorsal columns of the Ramesha KN. pp. but an AHI of 15 would only prove there is sleep apnea. 7th edn. Amacrine 58. Rajesh B. 2009 spinal cord to the somatosensory cortex. Ganglion Early-onset Alzheimer disease presents with dementia Nerve fiber layer cells but without systemic symptoms or movement disorders. Ashalatha R. C. and 1686–1719). Ch.  This patient presents with signs and symptoms Horizontal Outer nuclear layer concerning for narcolepsy with cataplexy. Ch. whereas levels <110 pg/mL are Retinal pigment epithelium diagnostic. peak in posterior tibial SSEPs corresponds with the including a Chiari I malformation or aneurysm. A vagal nerve stimulator can be used (see Bradley’s NiCP. 1254–1260). aggression. 17. not Bruch’s membrane narcolepsy. 163–179). Peripheral nerves are stimulated with repeated electric 7th edn. photoreceptor seizures progress to cause complex partial and layer. Human immunodeficiency virus dementia could present with many of the systemic symptoms Bipolar Inner plexiform layer described. either positive (P) or negative (N). Levetiracetam definitive treatment is hemispherectomy. The EEG in narcolepsy is normal and shows normal rapid eye movement (REM) Sclera during sleep attacks. The lumbar point. Typically. This can photoreceptor be tested with a lumbar puncture. neither would address the underlying (see Bradley’s NiCP. et al. This is a cell nuclei of photoreceptor disorder marked by sleep attacks. EEG showing continuous slow wave and Choriocapillaris spikes during sleep is seen with Landau-Kleffner syndrome. Eventually these nuclear layer. Endocrinology sleep-onset REM. pp. Narcolepsy is associated with a Figure for Answer 1. represents the lumbar roots. not narcolepsy. The anterior pore closes on day 24.30 Neurology Self-Assessment agitation. 1279–1300). as is shown in the MRI. 34. near-field cortical response. Figure from shocks.) hypersexuality. in refractory epilepsy.  This patient presents with Rasmussen encephalitis. A. Ch. 7th edn. and retinal pigment epithelium (see Bradley’s generalized convulsion. Ch. or sleep paralysis (see Bradley’s NiCP. but will not adequately so is only used for emergency or last-resort scenarios control seizures. and sleep paralysis. There is no an antihistamine may reduce coughing and a waveform for the lumbar plexus. as the N13 peak of the median nerve SSEP. who have Rasmussen encephalitis (see Bradley’s NiCP.86 (from Rosberger DF 2013 Diabetic short sleep latency. 87. however. D. the distal neural tube pore closes on appropriate first-line therapy for a secondary headache day 27. superficial to deepest are as follows: optic nerve layer. 103. multiple cranial Internal limiting membrane neuropathies. usually <5 minutes. 1615–1685). pp. Patients with this Müller cell disorder typically have a hypocretin (also known as Outer segments of orexin) deficiency in the lateral hypothalamus. pp. cells Outer plexiform layer 84. pp. Oxycodone may provide temporary for partial-onset seizures and may be used in patients benefit. 101.  The optic nerve layer is the most superficial layer of the retina. Phenytoin and amitriptyline some patients respond to IV immunoglobulin. oculomasticatory myorhythmia cells Inner nuclear layer would not be present (see Bradley’s NiCP. and multifocal vasculitic infarcts. 7th edn. Amitriptyline would not be an 89. 102. as well as hypometabolism of the affected hemisphere. pp.  Carbamazepine is the first-line therapy for Positron emission tomography will show glossopharyngeal neuralgia (as in this case). come from one hemisphere. the may also be tried. intracranial problem. epileptic discharges NiCP. pp. however. 86. 7th edn. patient may have a serious structural defect.

Microtubules and actin Purkinje cells of the cerebellum and are associated with neurofilaments are involved in both anterograde and rabies virus infection. Depending on the etiology of the syrinx. leading to loss of clock-drawing tests or trail-making tests. Multiple coexisting symptoms can occur. monocytogenes intervention is not intrinsically wrong. E. Rosenthal fibers cerebellar tonsils by ≥5 mm below the foramen are extracellular. Decompressive surgery is indicated in of pilocytic astrocytoma. Newborn Group B streptococci. stridor. bony abnormalities.  Group B streptococci. pilocytic astrocytoma. Syringomyelia is a fluid-filled space in 1 91. pneumonia. coli. A. Saunders. shoulders and upper torso. and/or progressive 18(8): 567–572. As such. syringomyelia. some low-grade tumors. evidence on cine phase MRI that cerebrospinal fluid Neither chemotherapy nor radiation is necessary for flow is restricted. pneumoniae. 1121–1146). 79. H. E. even intentional inaction) must be weighed carefully to L. nuchal pain that is paroxysmal and exacerbated by 97. object-specific tracks are involved. not side effects. N. the bad effects are not a means to the good effects. pp. Most patients with Chiari malformation who onset of symptoms in this patient argues against are asymptomatic and do not have an associated syrinx abscess. Ch.  A cerebellar mass in a pediatric patient may physical activity or coughing. pp. 1236–1237. and headaches. performing procedures. 23–33). pneumoniae. 105. Ch. H. the most notably planning. Cowdry type A inclusions are retrograde transport. including laminectomy. This may be tested by asking patients about symptoms are loss of pain. 6th Age or comorbidity Bacteria edn. and the 93. with permission. However. with permission. disorders (see Bradley’s NiCP. and are associated with adenovirus or poliovirus 94. The syrinx can also often tested in the clinic as both immediate and recent involve the anterior corticospinal tracks. B. and is rarely have associated neural tube defects such as required at all if total resection is obtained.  The picture shows a Chiari type I malformation with infection.  Negri bodies (eosinophilic intracytoplasmic away from the cell body and retrograde movement inclusions) are found in the pyramidal cells and in the toward the cell body. 7th syringotomy. 78. comorbidities is shown here (see Bradley’s NiCP. most commonly from a Chiari malformation as noted Attention is the ability to maintain focus. as well as other bacterial causes of meningitis based on age range or surgical procedures.  The principle of nonmaleficence is a guiding Toddler S. with herpes simplex virus. A. S. 7th edn. Figure from Quinones-Hinojosa A 2012 Schmidek and Sweet: TABLE FOR ANSWER 1. cranial neuropathies. pneumoniae. trauma. laughing.  Attention is tested in the mini mental state the spinal cord caused by any number of etiologies. proximally. tumors. concentration is the ability to attend while ignoring or membranous abnormalities can also contribute. other stimuli. As the syrinx progresses. B. an associated syrinx. 1147–1158). pp. myelopathy. These patients may have dysphagia. myelomeningocele and are usually detected prenatally Embolization may be used for arteriovenous or at birth. are also eosinophilic nuclear inclusions associated with which includes the myelin sheath and is not involved margination of chromatin on the nuclear membrane directly in axoplasmic transportation. Cowdry type B inclusions The neurolemma is the outer covering of an axon. with permission. or medulloblastoma. Executive dysesthesias in a shawl-like distribution across the function involves many higher-order cognitive abilities. pp. round. hydrocephalus. or some metabolic hydrocephalus. A chart with common is present or shunting of the syrinx). The slow imaging.92  Operative Neurosurgical Techniques 2-Volume Set. Figure from Ferri FF 2009 Ferri’s Color the latter of which is the most common presentation. Abstraction is the consideration of Because the decussating fibers of the spinothalamic general ideas rather than concrete. 7th edn. Many Chiari malformations are found incidentally on hemangioblastoma. H. The appearance of an avidly enhancing mural can be managed conservatively with clinical and MRI nodule with large cystic component is most suggestive surveillance. and similarities and differences between objects. D. which is often curative. Howell-Jolly bodies are small. N. S. aureus patient. examination by spelling the word world backward. B. Most often. the intention is Sinusitis or otitis media H. An action is considered ethical if the Immunosuppressed L. This is often tested with posterior columns may be affected. Chiari type II malformations can newly diagnosed pilocytic astrocytoma. Atlas and Text of Clinical Medicine. or sneezing. treatment can include shunt (cerebral if hydrocephalus 92. coli 95. the most common presenting thoughts. pneumoniae to bring about positive results. 1736–1765). Test One 31 treated patients. or cervical decompression (see Bradley’s edn. meningitidis prescribing medications. but is not routinely Neurology Books . A Chiari type I malformation is basophilic nuclear inclusions found in erythrocytes associated with downward displacement of the associated with splenic dysfunction. which usually memory through multiple-item recall (see Bradley’s causes distal hand weakness and atrophy. anterograde (also known as orthograde) movement 96. influenzae. NiCP. but kinesins are specifically eosinophilic nuclear inclusions seen in cells infected anterograde. ependymoma. 4. or Elderly S.  Axoplasmic movement is categorized into benefits outweigh the side effects. meningitidis. spreading NiCP. Ch. influenzae. the headaches present with occipital or Elsevier. apneic spells. monocytogenes determine whether any ill effects may occur to the Neurosurgical patients S.” Physician action (whether Adolescent through adult S. and earlier. temperature. principle in medicine that states that the physicians influenzae must “do no harm. 7th edn. cerebellar dysfunction. eosinophilic bundles that often appear magnum. Memory is proprioception and vibration. surgical resection is those who are symptomatic or in those who have the best choice of therapy. influenzae. A. Seizure: European Journal of Epilepsy weakness. pp. and dyneins are specifically retrograde. represent abscess. arm malformations or aneurysms. Ch. like a corkscrew and may be seen with long-standing including elevated intracranial pressure from obstructive gliosis.

risk of pancreatitis. seek medical attention after using cocaine. cardiac 100. but many brachioradialis. chorea. paranoia. Pituitary adenomas may invade the however. recurrent. Propranolol has an increased risk of worsening depression. Dimethyl fumarate is an oral hyperpolarization. With acute use. superficial radial sensory.  Valproic acid has been shown to have a slight. and nasal mass noted in the question stem and dalfampridine all have been shown to improve make this less likely. nephrolithiasis. pp. Amitriptyline has classified as an antineoplastic therapy.  This patient has a C6 radiculopathy on the left auscultation (see Bradley’s NiCP. The area of sensory loss is innervated by the lateral antebrachial cutaneous 99. It is FDA approved for relapsing forms of Figure from Grant LE. pp. and cognitive slowing. although the mechanism of its efficacy is poorly involving invasion of the cavernous sinuses. Bolded muscles indicate major root contribution. inhibitor. sclerosis (see Bradley’s NiCP. accumbens. proliferation. and hallucinations. like the biceps. but hallucinations. EMG should show abnormalities in muscles Headaches are the most common reason for patients to with significant C6 innervation. 98. 1720–1735). amantadine. C. QT prolongation. C. 7th edn. root. Fingolimod is an oral sphingosine 104. Ch. 1159–1186). symptoms. risk of cardiac arrhythmias. 1563–1614). B-cells. anhydria. medication whose mechanism of action is unclear. metabolic acidosis. ocular injection. Ch. 80. with permission. progressive vision loss. a placebo-controlled trial of memantine did cavernous sinuses and cause compressive cranial not show improvement of cognitive impairment related neuropathies. epistaxis. E. cocaine typically causes only area in which these nerves all meet is at the C6 pupillary dilation. but it is not used to treat fatigue. E. Tolosa. suppressing an increased risk of depression. and agitation are a result of significant. Verapamil has an increased approved for secondary progressive multiple sclerosis. and median nerve. modafinil. pp. 1254–1260). pulsatile proptosis. and an ocular bruit on careful 102. A. Idiopathic intracranial 101. It is believed that 103. among many other side cocaine blocking dopamine reuptake in the nucleus effects. Ch. 87. It is FDA approved for relapsing multiple Hunt syndrome is an idiopathic. pronator teres. 101. and hypertension.102  C5 C6 C7 C8 T1 Rhomboids Biceps Triceps Extensor indicis proprius Abductor pollicis brevis Infraspinatus Brachialis Pronator teres Flexor pollicus longus First dorsal interosseus Supraspinatus Pronator teres Extensor carpi radialis Extensor digitorium communis Pronator quadratus Deltoid Triceps Flexor carpi radialis Pronator quadratus Adductor pollicis Biceps Brachioradialis Anconeus First dorsal interosseus Adductor digiti minimi Brachialis Anconeus Adductor digiti minimi Deltoid Adductor pollicis Supra/infraspinatus Flexor digitorium profundus Extensor carpi radialis Flexor carpi ulnaris Adapted from AANEM Minimonograph #32. tachycardia. pp. Finally.  Mitoxantrone is an infused drug that is technically arrhythmias. 79. and T-cells. The stimulant. 7th edn. sinus syndrome. She has dermatomal sensory loss in a pattern 1147–1158 and 104. consistent with a C6 radiculopathy.102).  GABAB receptors are metabotropic receptors that 1-phosphage receptor modulator that keeps cause a downstream efflux of potassium leading to lymphocytes in lymph nodes (and therefore out of the hyperpolarization. but is not associated with cavernous conditions. and brachialis other neurological side effects are possible. intravenous antibiotics would only be necessary if the other than the fact that it inhibits immune cells and lesion were an abscess. paranoia. along with surgical draining may have protective antioxidant properties in the brain (see Bradley’s NiCP.  Mucormycosis classically affects diabetic patients. responsive granulomatous disease that usually affects 1159–1186). to multiple sclerosis. Notably. Italicized muscles indicate minor contribution from the root. thrombocytopenia. hemorrhagic or ischemic strokes. This drug is FDA other cardiac arrhythmias. Churchill synthetic protein that is thought to simulate myelin Livingstone. Chs. However. basic protein and block myelin-damaging T-cells. D. 1065–1083). Pemoline. the cavernous sinuses. carotid-cavernous fistulae more commonly 7th edn. side. present with pain. teratogenicity. including (see Table 1. 7th edn. steroid. an enzyme required by lymphocytes for pp. and spine. pp.  Memantine is an N-methyl-D-aspartate antagonist hypertension may cause abducens palsy due to tension used to treat cognitive impairment related to various on the nerve. and milder effects like tremor and promoting addiction through stimulation of reward weight gain. 7th edn. TABLE FOR ANSWER 1. triceps. tics. and macrophages. hepatotoxicity. 75. Ch. understood. Glatiramer acetate is an injectable Allison’s Diagnostic Radiology Essentials. Ch. It is FDA approved for relapsing chloride channels leading to an influx of chloride and multiple sclerosis. the rapidity of symptom onset. seizures. Neurology Books .  Cocaine is the most commonly abused illegal nerve. 7th edn. including hepatoxicity. causing psychiatric symptoms and teratogenicity. and worsening asthma. 80. Griffin N 2013 Grainger and multiple sclerosis. C. pp. It is FDA approved for relapsing multiple sclerosis. GABAA receptors are ligand-gated blood or brain).32 Neurology Self-Assessment necessary for pilocytic astrocytomas. Topiramate has an increased risk of centers (see Bradley’s NiCP. Rather than cavernous sinus multiple sclerosis–related fatigue (see Bradley’s NiCP. and Teriflunomide is an oral pyrimidine synthetase paralytic ileus (see Bradley’s NiCP.

Mild cognitive impairment is a disorder frontotemporal dementia that involves progressive loss of mild memory impairment that is isolated and not 1 of speech to the point of mutism. Although this with normal memory and orientation but has patient’s language dysfunction is isolated. Dementia with Lewy with stroke risk reduction measures. This patient presents significant enough to affect daily life. 7th edn. Vascular dementia is the second may not be appreciated unless testing of verbal fluency most common type of dementia and results from is performed. related to either large or small infarcts. lesions. memory loss. pp. the underlying bodies presents with well-formed visual hallucinations. Test One 33 105. Memory and visuospatial function abnormalities are significant and abnormalities are should be normal. including categorical fluency (naming as cerebral ischemia. Early in the disease this disorder noted on imaging. or naming as fashion. the speech paraphasic errors. Ch. 95. Alzheimer disease would present with disease except the MRI shows multiple ischemic short-term memory loss and visuospatial dysfunction. and Bradley’s NiCP. This many words starting with the letter “S” as possible in 1 often results in poor short-term memory and slow minute) or more formal testing like the Boston processing. Neurology Books . process of this type of dementia can be slowed (see fluctuating levels of alertness. 1422–1461). usually progressing in a stepwise many animals as possible in 1 minute.  Primary progressive aphasia is a subclass of parkinsonism. This disorder should be recognized because in addition to anomic aphasia. and can appear similar to Alzheimer Naming Test. C.

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Primary progressive 6. Intermittent progressive B. Lisinopril patient’s right arm will seemingly rise on its own accord. Debranching enzyme deficiency 3. Which of the following is the primary source of energy production in the human body? D. A 12-year-old boy presents to the office for evaluation A. The patient’s most likely diagnosis is caused by which of the following? A. hypertension. Serum creatine kinase (CK) level is elevated at 650 IU/L (normal is usually 50–200 IU/L). Relapsing-remitting B. along with stiffness. Progressive relapsing A. Myophosphorylase deficiency C. An exercise forearm test is performed and shows a normal rise in ammonia. Lamotrigine B. Which of the following is typical of this diagnosis? 2. Secondary progressive risk of fetal malformation? D. The patient’s MRI is shown in the figure below. Carbamazepine E. but no rise in the lactate.5. Phenobarbital A. Malate-aspartate shuttle E. On examination. A 62-year-old woman presents with gradually of progressive pain in her shoulders and hips. There is also intermittent myoclonus when the right arm is held up. His neurological examination is normal. Tau deposition of weakness. Atorvastatin and she will use the left arm to pull it back down. Niacin body. he gets cramps and fatigue in his legs to the extent that he is unable to keep up B. slurred. Her D. Which of the following medications is and slow. PMP22 duplication D. and he seems to get D. Beta-oxidation Neurology Books 35 . and intermittent jerks of her right upper and has noticed the need to use her arms when rising extremity. from a chair. Her speech has become monotone. she has dystonic posturing not associated with this condition? of the right arm with increased rigidity and A. Prominent hallucinations after the cramps begin. When speaking.Test Two QUESTIONS D. Labetalol family feels that she often neglects the right side of her E.  A. the B. Levodopa-responsive rigidity within minutes of starting exercise. Which of the following medications has the highest C. Urea cycle E. Anaerobic metabolism 1. Colchicine bradykinesia. Cramps usually occur C. C. With exercise. Alpha-glucosidase deficiency B. A 45-year-old female with a history of hyperlipidemia. Absence of muscular dystrophin E. Phenytoin 4. Autonomic dysfunction a “second wind” if he is able to rest for a few minutes E. Alpha-synuclein deposition with his peers during gym class. difficulty reaching objects in her tall kitchen cabinets slowness. Which of the following is not a type of multiple sclerosis? Figure for Question 2. She has progressive cognitive decline. and gout presents with a 2-week history 5. Valproic acid C. Krebs cycle C. He denies dark urine.

Intracranial hypertension C. and ceftriaxone B. GAA triplet repeat on chromosome 9 B. Gamma secretase 13. Vancomycin and ceftriaxone C. Macroglia C. and glucose of 10. Presenilin-1 contraindication to ECT. C. PMP22 duplication on chromosome 17 A. Recent myocardial infarction is a relative B. and severe headache. E. A 53-year-old female presents to the emergency E. Motion artifact D. absent reflexes. loss of sensation to the midshin in both legs. Subdural hemorrhage E. Loss of balance 9. Chloride A. A. adjunct therapy with A. Nerve conduction studies demonstrate motor conduction velocities at 20 m/s in all nerves Figure for Question 2. His examination demonstrates bilateral foot drop. Potassium B. MFN2 mutation on chromosome 3 regarding ECT except? C. Direction of nystagmus B. Magnesium D. Presenilin-2 E. and ceftriaxone D. confusion. Neutrophils B. Electromyography shows neurogenic units in 12. Lymphocytes D. metronidazole. Hearing loss D. papilledema. and ethambutol E. Eosinophils E. rifampin. 10. Sensation of rotating vs. undulating ground C.36 Neurology Self-Assessment 7.6°F. At rest. and high arched feet with atrophy in his feet. neuronal membranes are most permeable to 11.  without conduction blocks and absent sensory responses. Vancomycin and metronidazole Neurology Books . Electroconvulsive therapy has been shown to be a safe but effective treatment for depression. Which is the most prevalent bloodborne inflammatory cell type within infarcted tissue at 1 hour poststroke? A. Mutation in which of the following is not associated with an increased risk of Alzheimer disease (AD)? C. ampicillin. Anterograde amnesia is a possible side effect of ECT. Electroconvulsive therapy (ECT) is considered. Which genetic mutation is most likely major depressive disorder that is refractory to treatment responsible for this patient’s symptoms? with multiple serotonergic agents. and a temperature of 103. SOD1 mutation on chromosome 21 catatonia. Wegener granulomatosis 14. Amyloid precursor protein D. Which of the following is the most appropriate empiric treatment? A.11. A 45-year-old woman presents for evaluation of severe the distal leg. protein of 90. Calcium C. Isoniazid. E. Vancomycin. Fever E. Sodium demonstrated here? B. A lumbar puncture is performed and demonstrates a white blood cell count of 400 (with a predominance of neutrophils). Vancomycin. Glioblastoma department with fevers. and antipsychotics. PMP22 deletion on chromosome 17 mood stabilizers. Electroconvulsive therapy is for the treatment of D. A 15-year-old male presents to your office with a complaint of falls. All of the following is true B. On neurological examination she has a stiff neck. Computed tomography scan of her head is unremarkable. Which of the following is the most likely cause of the which ion? magnetic resonance imaging (MRI) finding A. A space-occupying brain lesion is a relative D. Apolipoprotein E4 contraindication to ECT. This has been occurring over the last several months. Which is the key distinction between vestibular neuritis and labyrinthitis? A. Dendritic cells 8.

school he progressed with the other children. and the perception of middle cerebral artery (MCA) is shown. Suprachiasmatic nucleus 16. Progressive multifocal leukoencephalopathy (PML) D. Intraarterial calcium channel blocker considered part of the workup for this condition? A. A 63-year-old female who is on her seventh day of a 17. Mild intellectual disability C. IV tissue plasminogen activator (tPA) without change in blood pressure. A 17-year-old girl with a history of anxiety and hospital stay for a subarachnoid hemorrhage (SAH) migraines is evaluated for dizzy spells. Metachromatic leukodystrophy Figure for Question 2. and electroencephalogram (EEG) in both the awake and sleep state is normal. An otherwise healthy 68-year-old woman presents with developmental milestones. Neurological examination is following is the most likely cause of her symptoms? normal. Supine plasma norepinephrine levels Ultrasound Doppler instrument B. palpitations. seems to occur upon standing. Which of the A. following is the best treatment for this condition? Increased fluid intake has not improved the symptoms. Test Two 37 15. Which of the falling. Plasma metanephrines 100 18. Her brain magnetic resonance image is with his math class to the extent that he is now well shown. Each episode 2 acutely develops right arm weakness and slurred speech. A 7-year-old left-handed boy presents for evaluation E. although she has never lost consciousness. Lateral nucleus Sec 0 1 2 3 4 C. he maintains average grades diffusion coefficient (ADC) images. Standing plasma norepinephrine levels 300 C. Meningioma A. Dyscalculia D. Arcuate nucleus of poor grades.  D. A. last 4 months he has developed significant difficulty was normal. including T1 postcontrast and the apparent behind his peers. Anterior nucleus Figure for Question 2.  Neurology Books . Oral nimodipine and electroencephalogram is normal. Magnetic resonance image (MRI) of the brain 200 E. Holter monitor D. IV mannitol tachycardic on the tilt-table. A tilt-table test reveals an increase of heart rate by 40 beats per minute B. and is associated with The following transcranial Doppler (TCD) of her left lightheadedness. Attention-deficit/hyperactivity disorder (ADHD) E. Supraoptic nucleus B. and in his first 2 years of 1 week of difficulty reading and right-sided clumsiness. Glioblastoma following is the most likely diagnosis? B. However.19. Although C. Central nervous system (CNS) lymphoma C. The patient previously hit all 19. Gerstmann syndrome E. Metastatic lung cancer B. Which of the following hypothalamic nuclei controls the circadian rhythm? cm/s 0 A. Which of the in his other classes. including complete blood count. Extraventricular drain placement spell of dizziness. she experiences a typical D. Which of the following would not be E. In the Routine blood work.15.

Leptomeningeal metastases from unknown primary tumor C.38 Neurology Self-Assessment 20. Preserved immediate memory E. Which of the following is a correct statement scanning regarding the condition? C. Ionizing radiation exposure A. He has a history of the somatotrophic type. Glioblastoma E. juvenile cataracts.  24. The diagnosis is made on imaging alone. Over the last 6 months he has had worsening insomnia. The most common cell origin in this condition is and progressively impaired balance. gradual bilateral hearing loss. When he is asleep he seems to act out his dreams. A 36-year-old man presents with a 1-year history of detect corticotropic lesions. This condition is an autosomal-dominant disorder. Magnetic resonance imaging (MRI) of brain and migraines but has had some recent lactation along spine with and without contrast with a late menstrual cycle. Medulloblastoma B. Impaired procedural memory D. This condition is associated with sympathetic activation. a sense of aural fullness. A brain magnetic resonance image (MRI) with contrast is shown. His neurological examination demonstrates impairments of attention and short-term memory. A magnetic B. Meningioma Figure for Question 2. particularly the dorsomedial nuclei. First-line treatment is intravenous immunoglobulin (IVIG) at a dose of 2 g/kg. There are no contrast-enhancing lesions or restricted diffusion. His wife mentions that her father-in-law had similar memory loss. Which of the following most likely represents the D. He has intermittent myoclonus present in his arms and an ataxic gait. She has no history of A. This condition usually causes a binasal D. and flow cytometry evaluations B. A 45-year-old male presents to the clinic with his wife for evaluation of memory loss. Which of the following is not necessary in the 23. Which of the following is not a feature of a classic B. Sporadic disease amnestic syndrome? C. Figure for Question 2. A. Disorientation to time or place Neurology Books . This condition is a prion disorder. Imaging of entire body.21. He sleeps only 1–3 hours each night. Rapid eye movement (REM) sleep without atonia is present. Cunningham virus DNA C. All of the following are true of this condition except? A.23. etiology of this man’s tumor(s)? E. Cerebrospinal fluid evaluation. A pregnancy test by the primary care physician was negative. similar difficulties with their hearing. B. 22. Tuberous sclerosis B. Preserved remote memory D. and his brother and father have E. Neurofibromatosis type 1 25. A 33-year-old woman presents to the neurology clinic evaluation of the aforementioned patient? for headaches and blurry vision.  C. Ophthalmological evaluation A. Impaired recent memory E. Bilateral vestibular schwannomas D. Cerebrospinal fluid evaluation for John of hormones. either with computerized resonance imaging (MRI) of the brain was ordered and tomography or positron emission tomography is shown. including cytology hemianopsia. Magnetic resonance imaging (MRI) of the brain demonstrates atrophy of the thalamus. This condition can lead to an excess or deficiency E. D. Neurofibromatosis type 2 C. Insulin-like growth factor 1 (IGF-1) can be used to 21. Which of the following is the most likely cause of his symptoms? A.

He suffers an injury to his lumbar spine. Which of the following best describes the EEG? A. Hyperreflexia Neurology Books . Left temporal lobe epilepsy D.  E.27. Which of the following is not a likely L2 component of his clinical examination upon arrival to the hospital? A. The electroencephalogram (EEG) shown here was taken from a 15-month-old boy.  26. Saddle anesthesia C. the magnetic resonance image for which is shown here. Flaccid paraplegia D. Bladder and bowel dysfunction B. Slow spike and wave B. Sympathetic surges Figure for Question 2. Burst suppression E. Test Two 39 2 Figure for Question 2. A 30-year-old man has a severe motorcycle accident while driving in the rain. He presented at 6 months of age with spells of truncal flexion and arm abduction lasting 2–3 seconds and recurring multiple times a day. Hypsarrhythmia 27.26. Normal quiet sleep C.

which waxes which of the following is an important component to and wanes throughout the day. Cerebral angiography E. Conversion disorder rehabilitation B. Over the last year. IX D. V B. Hyperventilation condition. her right eye is deviated inferiorly and A. E. E.40 Neurology Self-Assessment 28. An immunocompromised state is a risk factor for developing this condition. The location of the preceding virus does not affect D. C. condition? expiratory pressure [PEEP]). After a night without her continuous positive airway Which of the following is true regarding this pressure (CPAP. A. she has experienced 37. a morbidly obese woman A. Ziprasidone eruption. On examination. receptors. 31. she has mild right ptosis. and normal blood work looking for metabolic. Generalized anxiety disorder Neurology Books . Which of the B. numbness in both hands and forearms. Clozapine present pain is much more severe than the original D. The C. Taste receptors on the fungiform papillae are appropriate response? innervated by which cranial nerve (CN)? A. 6 months laterally. Which of the following antipsychotics should be 36. Which of the following is the most 35. Assuming a nonfatal injury and medical stability. 32. She is preoccupied C. She was previously seen patient care after a gunshot wound to the right parietal by three other neurologists and had normal magnetic lobe? resonance images (MRIs) of her neck and head. 12 months following is the most important next step in her care? C. Refuse to perform any further procedures. This condition always occurs after a vesicular rash. Despite return of circulation and respiratory function. Hypoxia D. Urgent removal of bullet fragments electromyography and nerve conduction studies. Brain computed tomography (CT) voiding state? A. Sympathetic outflow 29. Approximately 4 months before his visit. Magnetic resonance imaging (MRI) of the brain to She has even joined a local MS support group. Antiplatelet therapy D. Pontine micturition center after suffering an anoxic injury from cardiac arrest. Factitious disorder E. Young age is a risk factor for developing this A. B. Consult the ethics committee. Malingering C. Thalamic nuclei patient’s family is requesting that the patient have a D. Aripiprazole the same dermatome. normal A. C. 24 months B. which has now resolved. Motor cortex tracheostomy placed for long-term nutrition and ventilation. Comply with family requests. Local spinal reflex percutaneous endoscopic gastrostomy and E. Hypercarbia the risk of developing this condition. Early transcranial magnetic stimulation for A. Olanzapine dermatome. Contact organ donation services. A 67-year-old woman with a long history of smoking 33. Hemoglobin A1C 34. Broad-spectrum antibiotics to prevent abscess with the idea that she may have multiple sclerosis formation (MS). By what age should a child be able to produce presents with new horizontal diplopia and headache. X E. Ophthalmology consult switching from a urinary storage state to a urinary E. despite reassurances of multiple normal MRIs. usually set at 20 cm positive end. On two-word sentences? examination. D. Which of the following is the likely etiology? B. Hyperammonemia C. and toxic exposures. VII C. 18 months A. he was diagnosed with acute herpes zoster infection in B. an 82-year-old patient has been declared brain dead B. Haloperidol rash. Which identify the extent of tissue damage of the following is the most likely diagnosis? E. there is scarring from his prior E. Illness anxiety disorder D. along with a decrease in sensation to temperature and pin prick in the left T4 dermatome. A 43-year-old man who recently received chemotherapy avoided in patients with obesity? presents with sharp and stabbing pain in the left T4 A. Transfer the patient to a different hospital. 36 months C. B. XII 30. B. The C. D. and she has marked right mydriasis with no reaction to light. Angiography to rule out pseudoaneurysms inflammatory. Which of the following is primarily responsible for D. The condition affects only peripheral pain is noticeably more drowsy and confused than usual. A 22-year-old female presents for evaluation of arm numbness. Uremia E.

Which of the following correctly identifies lateral sclerosis? this condition? A. Conceptual apraxia D. Dermatomyositis resonance imaging confirms the suspected etiology of C. Atrial fibrillation D. Which of the “cocktail party” personality? following would be the best choice for a first-line A. Fragile X syndrome B. Ideomotor apraxia B. Asperger syndrome therapeutic trial? B. Medicaid A. Polymyositis her symptoms. Medicare B. A patient is asked to show how to use a hammer. Perimenstrual magnesium oxide and riboflavin 42. Arterial dissection Figure for Question 2. The Medicare tax. Her examination A. Tricare E. Williams syndrome C. Which syndrome is associated with a surprisingly good lateralized.  Figure for Question 2.40. among other symptoms. a right homonymous hemianopsia. Conversion disorder image is shown. Which of the following insurance programs is available to Americans over the age of 65 who have paid 39. 41.  Neurology Books . or to a person of any age with a patient acts out cutting with a saw instead of using a diagnosis of end-stage renal disease or amyotrophic hammer. These tend to occur during her menses and intelligence quotient (IQ). Hypercoagulable state from neoplasm C. Perimenstrual scheduled propranolol E. A 40-year-old woman underwent muscle biopsy for 40. Emery-Dreifuss muscular dystrophy shows nonfluent aphasia. and decreased vision on of the following is her most likely diagnosis based on the right field. Angelman syndrome D. Affordable Care Act C. Test Two 41 38. Down syndrome A. A 33-year-old previously healthy woman has an acute progressive weakness. often referred to as having a may last for up to 3 days at a time. Limb-kinetic apraxia E. pulsatile headaches with light and sound verbal and social capacity despite a mildly diminished 2 sensitivity. A 16-year-old female suffers with recurrent severe. Magnetic B. Aetna D. Dissociation apraxia 43. Inclusion body myositis the most likely etiology of her condition? A. Oral contraception pills E. workup is negative. Which onset of neck pain. Valproic acid as needed E. Ideational apraxia C. She also complains of heaviness in her the image shown? right arm and difficulty speaking. Venous sinus thrombosis B.43. from which a key T1 fat-saturated D. Perimenstrual scheduled naproxen D. and right hemiparesis. headache. Otherwise. Hydrocodone as needed C. What is E.

Positive waves and fibrillations in the first dorsal interosseous D. Vitamin E magnetic resonance image (MRI) is obtained with contrast and reveals no mass lesion. Rasagiline coordination. She disease is a reversible acetylcholinesterase inhibitor? underwent surgery and chemotherapy with A. Paraneoplastic syndrome emergency room? B. He C. Which of the 50. Which of implicated in the positive symptoms of schizophrenia? the following is not an ideal treatment for this A. Levodopa C. which of the following is not an indicator of this disease? A. which worsens in the evenings or after reading for prolonged periods. According to various diagnostic criteria for probable sporadic Creutzfeldt-Jakob disease (sCJD). Rivastigmine She now presents to the clinic with 1 month of C. and paclitaxel. he has full strength throughout. A 55-year-old woman was recently diagnosed with 49. He also has multiple 48. A brain E. Myoclonus C. Which of the following describes the most likely cause of his neuroophthalmological complaint? 51. Allopurinol D. Purine-restricted diet E. A 65-year-old man with an 80-pack-year smoking history complains of recurrent horizontal diplopia. D1 condition? B. D2 A. Levetiracetam Neurology Books . Seizure B. cyclophosphamide. as well as radiation therapy to the breast and affected axilla. Hemiparesis E.  shoulder abductors with repeated testing. He has a lifelong history of cognitive and motor delay with refractory epilepsy. Which of the following treatments of Alzheimer stage IV breast cancer with bone metastases. Tetrabenazine E. Complication of chemotherapy B. cervical E. Retinal-directed antibodies dystonia. and spasticity with hyperreflexia. Akinetic mutism D. type 1 ulnar neuropathy at the wrist? A. D. Which of the following abnormalities would not be expected in a severe. Ataxia C.50. B. Visual disturbance E. Monocular blindness E. Leg weakness 45. although he loses power in his Figure for Question 2. Which of the following receptors is most commonly excoriations and teeth marks on his hands. Calcium channel–directed antibodies neurological symptoms. A 4-year-old boy presents to the neurology clinic for a A. Complication of radiation C. Absent sensory nerve conduction response at digit 5 B. and antibodies may chew on his own hands if not monitored closely.42 Neurology Self-Assessment 44. facial grimacing. Aquaporin-4–directed antibodies On examination he has minimal verbal output. and a change in her speech. Microscopic brain metastases A. difficulty with eye–hand D. D5 D. D4 C. On examination. Memantine progressive gait ataxia. Thyroid-stimulating hormone receptor–directed also has abnormal posturing of his arms and neck. Diplopia D. Acetylcholine receptor–directed antibodies second opinion regarding his complex constellation of B. Edrophonium doxorubicin. Prolonged motor distal latency at adductor digiti minimi C. A patient with the following angiogram findings would following is the most likely cause of her symptoms? most likely have had which acute symptoms in the A. D3 B. Cerebellar disturbance 47. Positive Froment sign E. Spine metastasis D. upper limb dystonia. Absent dorsal ulnar cutaneous response 46.

  D. A young African-American woman who has strong A. A young woman who is going through a divorce B. D. Choroid plexus likelihood of smoking cessation. The child will likely need antiepileptics to help attenuate these episodes. Which of the following is true of the mixed- D. C. Arachnoid villi hand. Test Two 43 52. Mirtazapine can be used to improve sleep and but is close to her family and not impulsive stimulate appetite. C6 Which of the following signs is not associated with this condition? C. An 8-month-old is noted to have global developmental following nerve roots is involved in this birth injury? delay. B. A magnetic resonance image (MRI) is shown. Trazodone often leads to insomnia. Serotonin borderline personality disorder with numerous suicide attempts 56. B. 53. Which of the following is the primary inhibitory suicide the greatest? neurotransmitter of the spinal cord? 2 A. Pericyte D. Dopamine use and access to weapons at home D. Which of the 54. Hypotonia 58. Which of the following is the most likely causative agent? A. What should the advice be to the parents at this time? A. A middle-aged Caucasian woman with history of E. C8 B. The fetus is noted to have a flaccid right E. A consult to neuroophthalmology should be made to evaluate for nystagmus. C. Low-set ears D. In which of the following scenarios is the risk of 55. C5 panting. B. the event lasts for approximately 6 hours E. A married man with newly diagnosed cancer with a mechanism antidepressants? high cure rate and with children at home A. Bupropion is safe to use in those with seizures. Which of the following is the chief component of C. Glycine B. T1 C. His blood pressure remains above 220 systolic despite two doses of intravenous labetalol. 59. An emergent computed tomography scan of his brain demonstrates a small. Amantadine Figure for Question 2. L-dopa E. Ependyma 57. Glutamate C. Oculomotor apraxia E. right-sided intracerebral hemorrhage in the internal capsule. Entacapone B. There are no abnormal movements of the eyes or limbs. E. Chorionic villi E. The child needs a trial of ranitidine to decrease gastroesophageal reflux. and appears to have episodes of A. C7 A. E.54. An 82-year-old male with a history of Parkinson disease presents to the emergency department with headaches and mild left-sided weakness. A 2-month-old was evaluated by his pediatrician for D. An older Caucasian man with history of cocaine C. nystagmus. as well as right ptosis and miosis. Ropinirole Neurology Books . A term fetus is delivered breech and suffers a shoulder dystocia. Gamma aminobutyric acid (GABA) religious support B. Bupropion has not been shown to increase the A. This is part of normal development for the child. His wife mentions he was recently started on a new medication for his Parkinson disease. Each time. Neither duloxetine nor venlafaxine are typically used for centralized or peripheral pain disorders. Rasagiline C. The child will likely outgrow these episodes. cerebrospinal fluid (CSF) reabsorption? D. Ataxia intermittent episodes of forced head turns to the left. Hemiplegia but is not associated with any distress to the infant.

which of the following is the best course of action after removing a large volume of cerebrospinal fluid (CSF)? A. Creutzfeldt-Jakob disease 65. Which cognitive bias is shown by this example? E. Initiate antituberculous therapy D. Separate profiles for private and professional use episode of left arm weakness. Initiate acetazolamide E. Herpes simplex virus (HSV) encephalitis D. Oligodendroglioma A. Congenital spinal stenosis Figure for Question 2. A computed tomography scan of the brain demonstrates diffuse atrophy. Based on your suspected diagnosis and while awaiting further studies. Sensitive to low levels of illumination C. Confirmation bias which condition? C. followed by a decline in level of consciousness.62. normal red blood cells. The magnetic resonance image (MRI) of the lumbar spine shown here demonstrates which abnormality? A. Diastematomyelia E. Subacute combined degeneration D. A 50-year-old man with acquired immunodeficiency syndrome (AIDS) presents to the hospital with several days of confusion. Anchoring bias A. protein of 140 mg/dL (normal is 15–45 mg/dL). Treatment for MS is conditions started.60. but no acute findings. Using high privacy settings to protect personal a magnetic resonance imaging (MRI) of the brain to information help confirm that suspicion. Alzheimer disease retina except? E. Initiate vancomycin and rifampin therapy 61. Contain more photopigment than cones Neurology Books . Contains chromatic photopigments D. Syringomyelia B. and the only test ordered is B. A lumbar puncture is performed. More easily activated by light E. Availability bias 62. Reporting unprofessional content by colleagues subcortical white matter hyperintense fluid attenuated D. Emergent ventriculoperitoneal shunt B. A 22-year-old female presents to the office with an A.  C. Messaging a patient to start a romantic relationship A. The brain photomicrograph shown here is suggestive of B. Premature closure B. The suspected diagnosis is multiple sclerosis (MS).  63. All of the following are true about rod cells in the D. Initiate amphotericin B and flucytosine Figure for Question 2. All of the following are appropriate uses of social media by physicians except? 64.44 Neurology Self-Assessment 60. Transverse myelitis C. Communication with patients about their medical inversion recovery (FLAIR) lesion. It shows a single small C. Dementia with Lewy bodies E. and opening pressure is 60 cm (normal is <20 cm). and 9 white blood cells/mL (normal is <5 cells/mL) with a lymphocytic predominance. Predominantly in the peripheral portion of the retina B. The spinal fluid further demonstrates a glucose of 10 mg/dL (normal is 45–80 mg/dL). Diagnosis momentum C.

macrolides. Nonconvulsive status epilepticus left eye and cannot look upward for more than a few C. This disorder is transmitted to the child in an D. She recently started an oral magnesium fourth day of admission his vital signs had normalized. Panic disorder in the child for 6 weeks after delivery. Herpes encephalitis demonstrates left-sided ptosis. the patient could not Which of the following is the most likely diagnosis? open her left eye. and to save his brother. The started 1 week ago and has been intermittent during electroencephalogram (EEG) shown here was obtained. Bacterial meningitis nasal speech. Double vision but he would not wake up off of sedation. He has also avoided vacations to the B. Now each time he closes his eyes. Posttraumatic stress disorder (PTSD) D. Prednisone is the first-line treatment during beach with friends due to a fear of swimming. A 27-year-old female who is currently 25 weeks sepsis from pneumonia. She has limited movements of the B. Rapid eye movement (REM) sleep behavior disorder E. What is pregnancy. Her neurological examination A. Since witnessing his younger brother drown in a pool. Poor feeding and respiratory weakness can persist A. B. Intravenous immunoglobulin (IVIG) is a treatment option during pregnancy. D. flashbacks are vivid and are often hard to distinguish from reality. C. E. Today on awakening. and her left eye is adducted at rest. fluoroquinolones. A 61-year-old male was admitted to the hospital with 68. Medication-induced encephalopathy She has no pupillary abnormalities. Bereavement Neurology Books .66. These certain anesthetics. Generalized anxiety disorder autosomal-dominant manner. Hepatic encephalopathy seconds with the right eye before her right eye closes. Test Two 45 Fp1 F3 2 F3 C3 C3 P3 P3 O1 Fp1 F7 F7 T3 T3 T5 T5 O1 Fp2 F4 F4 C4 C4 P4 P4 O2 Fp2 F8 F8 T4 T4 T6 T6 O2 Fz Cz ECG1+ECG1– No Presente Figure for Question 2. this time. and she has mildly E.  66. which results in poor sleep. normal. supplement because of headaches. A. Exacerbations can be precipitated by the use of he has flashbacks of attempting to jump into the pool magnesium. the most likely diagnosis? C. All of the following are true of this condition a 20-year-old man has panic attacks and recurring during pregnancy except? dreams of drowning. Magnetic resonance imaging and magnetic resonance angiography of the head were both 67. On his double vision. He was intubated in the pregnant presents to the emergency department with intensive care unit (ICU) while being stabilized.

The diagnosis is consistent with somatization questions and insists on interrupting his peers. Mu receptor she is tachycardic with labile blood pressures. Left parasylvian cortex C. Mesencephalon There is no rash. but about 10 minutes lesion to which circuit? later became drowsy and lost consciousness again. B. She has had several sympathetic and parasympathetic systems? days of nausea. While in the ER. She is E. Change of classroom environment with altered A. He A. a pathological lack of football and lost consciousness for about 1 minute. B. Which of the following receptors is involved in 74. D. A computed other 6-year-olds. and absent patellar reflexes with severe headache and blurred vision after spending all preserved ankle jerk reflexes. The teacher states that he often has a difficult processing that are responsible for many of the time sitting still and is unable to stay on task while features of this condition. for a parent–teacher conference due to her son’s erratic D. After many years of caring for children. After segmentation of the neural tube during Her examination reveals tenderness to palpation over development. abdominal pain. Urine porphobilinogen and δ-aminolevulinic acid C. Prosencephalon B. von Willebrand deficiency study (NCS) B. Verapamil Neurology Books . Telencephalon into the 1000s. decreased strength and a strong autoimmune family history presents with throughout the legs. Psychotherapy E. which of the following structures will the sternocleidomastoid muscle. muscle aches. Psychostimulant D. The remainder of the neurological examination is normal. She also has a history of irritable bowel syndrome and depression. Which of the following is the most likely B. and several other locations. He motivation not from a mood disorder results from a awoke and walked off the field. In neuropsychological testing. Total creatine kinase is usually elevated markedly E. There are alterations in central nervous system pain behavior. 77. Which of the A. Papez circuit B. She complains of fatigue. She was dragging her feet while walking and even had a fall. Serum niacin level E. Electromyography (EMG) and nerve conduction A. Tearing of middle meningeal artery E. A 19-year-old male with a history of chronic migraine organization.46 Neurology Self-Assessment 69. Her neurological examination reveals intact cranial nerves. Which of A. A 32-year-old woman presents to the neurologist for D. Muscarinic receptor before her arrival at the emergency room (ER). and constipation A. Concussion multiple joints. She disorder. Lupus anticoagulant antibodies D. Discipline from the parents the following medications is the likely causative agent? B. A. 70. There is an association with giant cell arteritis. Lateral orbitofrontal cortex source of his symptoms? C. On examination in all her extremities. D. N-methyl-D-aspartate (NMDA) receptor and confusion during this same time. or loss of reflexes. headaches presents to the emergency department with the teacher states that his behavior is not typical of right-sided back pain and bloody urine. Lumbar puncture for cytoalbuminological following abnormalities is most likely to appear on her dissociation blood work? B. and anxiety. Alpha 2 receptor Her husband mentioned she had visual hallucinations C. JAK2 mutation C. A 26-year-old woman with chronic renal insufficiency normal strength in the arms. Anterior cingulate gyrus A. The mother of a 6-year-old boy is called to the school C. Metencephalon A. Vitamin K deficiency 75. Topiramate C. Valproic acid curriculum B. Dorsolateral prefrontal cortex was rushed to the emergency room for further evaluation. aches in E. Which of the following is the tomography scan of his abdomen and pelvis treatment of choice for the patient’s condition? demonstrates a large right-sided renal calculus. would be most helpful in making the diagnosis? including no nuchal rigidity or weakness. Which of the following tests she has bilateral papilledema but is otherwise normal. Tearing of bridging veins D. He does not raise his hand for E. doing assignments. Amitriptyline D. weakness. Rhombencephalon Which of the following is true regarding her condition? C. Elevated vitamin A levels E. She has normal sensation weekend hiking in the summer heat. Cerebral contusion possible myopathy. ultimately give rise to the thalamus? medial fat pad of the knee. Shearing of axons 72. A 16-year-old boy was hit in the head while playing 71. A 35-year-old female presents to the emergency autonomic preganglionic transmission for both department with leg weakness. Antiepileptics C. Propranolol E. also informs the mother that much of the boy’s homework is stuffed into his backpack without any 73. 76. Nicotinic receptor somnolent but arousable and disoriented. greater trochanter. Stool test for botulinum toxin D. There is an increased risk of malignancies.

On examination. Which is most likely to explain his hyponatremia? A. hallucinations. Chronic hypertension 81. Puffer fish C. Traumatic brain injury course of 2 days to the level of his hips. Peyote Neurology Books . Syndrome of inappropriate antidiuretic hormone secretion (SIADH) E. and a diagnosis of Guillain-Barré syndrome (GBS) is made. Which of the following is C. Idiopathic intracranial hypertension may commonly cause which of the following secondary symptoms? A. Dopamine depleters His laboratory tests (including coagulation profiles) are largely unremarkable and show no electrolyte B. Conductive hearing loss B. An 83-year-old man with cognitive decline presents 80. Intravenous immunoglobulin (IVIG) D. Benzodiazepines A. Phonagnosia A. Selective serotonin reuptake inhibitors (SSRIs) abnormalities or infection. Carbidopa/levodopa the most likely cause of the finding shown? D.78. His hands are also now symptomatic. Which of the following medications would not be with altered mental status. tachycardia. Simultagnosia 79. Shellfish E. Psychogenic polydipsia 82. he is also noted to have no reflexes in his arms or legs. mydriasis.  A. Sensorineural hearing loss C. Cerebral amyloid angiopathy E. Visual agnosia C. Amusia be found in which of the following? E. Palatal myoclonus 83. Test Two 47 78. Subarachnoid hemorrhage distal lower extremities that have ascended over the E. The neurotoxin psilocybin. Arteriovenous malformation (AVM) with weakness. Laboratory evaluation performed on presentation was also notable for hyponatremia. Mushrooms B. Persistent tinnitus E. Spinal fluid analysis reveals cytoalbuminologic disassociation. Home-distilled ethanol D. and paresthesias in his D. There was no preceding infection. which causes euphoria. Which of the following describes a patient with intact vision who is unable to perceive more than one object at a time? Figure for Question 2. The patient’s vital signs are indicated in the treatment of Huntington disease? 2 normal and there has been no recent illness or fever. Pulsatile tinnitus D. Dehydration C. A. Antipsychotics B. and seizures. can D. numbness. Prosopagnosia B. A 32-year-old man presents to the emergency room C. Cerebral salt wasting B.

Central apnea D. the 85. Cerebral cortex A. Which of the following may be problematic after overnight polysomnogram. Cheyne-Stokes breathing 88. Which of the following is the next best step? B. Mixed apnea B. Nimodipine therapy E. The figure below shows a 120-second segment from an 87. Lumbar puncture C. Thalamus B. Ocular alignment B. Angiogram D. Venography 86. Paradoxical breathing E. Heart rate D. In median nerve somatosensory evoked potentials. Anaerobic respiration Neurology Books . Medial meniscus scan of the brain reveals no evidence of hemorrhage.  84. Hearing A. A computed tomography (CT) A. Higher amount of mitochondria E. type I muscle fibers include which of the following qualities? A. Fast-twitch fibers B.84. Cervical spinal cord C. Which of the following is blunt-force neck injury? demonstrated in the picture? A.48 Neurology Self-Assessment Figure for Question 2. Sense of smell C. Easy fatigability C. Articulation E. Higher contractile strength D. A 48-year-old woman awakens with a severe N13 peak corresponds with which anatomical “thunderclap” headache and a stiff neck and is taken to location? the emergency room. In comparison to type II muscle fibers. Obstructive apnea C. Ergotamine therapy E. Erb’s point D.

Landau-Kleffner syndrome Figure for Question 2. Juvenile myoclonic epilepsy D. Schizencephaly 90. Lissencephaly B. Porencephaly E. Test Two 49 89.89.90. The electroencephalogram (EEG) of a 7-year-old boy during wakefulness and sleep is shown in the figure below. Childhood absence epilepsy B.  Neurology Books .  E. Encephalocele D. Lennox-Gastaut syndrome C. Autosomal-dominant nocturnal frontal lobe epilepsy Figure for Question 2. Which of the following is the most likely diagnosis? A. A magnetic resonance image (MRI) of a newborn infant is shown here. Which of the following defects is 2 demonstrated? A. Pachygyria C. He presented with speech delay.

Pain perception lost weight since her last visit. B. The most likely etiology given the presentation and 93. Listeria monocytogenes C. B. Language this condition? A. The depicted image shows noncommunicating C. Add donepezil and recommend dietary supplements.94. Findings are compatible with normal-pressure 92. of those listed here for college-aged patients. Additionally. Substance P is a peptide neurotransmitter involved in imaging is benign familial macrocephaly. Report suspected abuse to local authorities. You suspect the patient is being neglected by her son. Her vital signs show she has C. She E. Ignore signs and schedule follow-up in 6 months. Digit span is a test of which of the following? 94. Immediate memory macrocephaly. Neisseria meningitidis hydrocephalus. Which of the following is true regarding E. Confront the son about his poor treatment of his mother. which of the following? A. A 3-month-old infant is evaluated by neurology for A. E. E. Pseudomonas aeruginosa D. (VHL) Figure for Question 2. Muscle contraction 95. Circadian rhythm no specific complaints. D. She reports B. Remote memory of 50 cm. B. The depicted image shows communicating A. Endocrine regulation lives with her son. but the patient has a head circumference B. Call police immediately to detain the son.  Neurology Books . Select the most common bacterial cause of meningitis hydrocephalus.50 Neurology Self-Assessment 91. and her body mass D. Calculation are shown. and he is unsure of her medications or whether she is taking them. Sweat production index is 16. Implicit memory restricted upgaze. C. She has several unexplained bruises. the infant is found to have C. D.7. Which of the following is the most appropriate response? A. Group B streptococci hydrocephalus. An 83-year-old female with a history of dementia presents to your clinic for a follow-up visit. Neither his mother nor father has macrocephaly. Staphylococcus aureus term treatment. Magnetic resonance images (MRIs) D. A course of acetazolamide is indicated for long- E.

Vestibular schwannoma related to B. He was very aggressive. Which was the most likely cause of the neurological scan of his head. A 22-year-old woman comes to the neurology clinic with 3 months of daily headaches that occasionally 2 wake her at night (PCP). Assuming findings of a lumbar puncture align with her other findings. Risk of seizure B. Liver impairment D. An 18-year-old man presents with 1 month of C. On examination. Risk of headache neurofibromatosis 2 C. he became unresponsive. Risk of progressive multifocal leukoencephalopathy D. On examination the patient A. A computed tomography 96. Urinary analysis is normal except for myoglobinuria. Hemangioblastoma related to Von Hippel-Lindau syndrome A. Acetazolamide D. Viral encephalitis is somnolent but arousable with painful stimulation. History of which of the following is a contraindication E. B. is febrile. Further.” E. Which of the following is the most serious potential adverse effect of mitoxantrone? A. Shortly after his arrest. Transverse myelitis Neurology Books . Hypotension B. Risk of allergic reaction the Rb gene E. Glatiramer B. Test Two 51 98. Cerebellar hamartoma related to Cowden syndrome for use of dalfampridine (4-aminopyridine) for multiple sclerosis? A. and pattern of disease noted in the image? electrolytes are normal. Pyridostigmine 99. A 19-year-old male is brought into the emergency department by the police. Paraneoplastic syndrome He is hypertensive. Phencyclidine E. you note no focal neurological deficits. Intoxication with which of the following is the most likely cause of his symptoms? D. Plasma exchange C. but otherwise are normal. Cocaine 97. Retinoblastoma related to familial genetic defect in D. and has both vertical and C. He has a past medical history of renal cysts. Neuritic plaque accumulation A.96. Seizure horizontal nystagmus. Magnetic resonance imaging (MRI) and venography of the brain reveal flattened optic discs bilaterally. and it took multiple officers to restrain and arrest the patient. Seizure B. Risk of cardiomyopathy C.  earlier in the evening after initiating a fight at a party. Opioid Which of the following is his most likely underlying diagnosis? 100. There is a strong D. Marijuana family history of renal cancer and “brain tumors. complete blood count. she complains of new horizontal diplopia when looking to the right. which of the following is the best treatment to address her underlying disease? A. The patient was arrested Figure for Question 2. Optic glioma related to neurofibromatosis 1 101. Visual acuity is normal and visual fields are full. Alcohol headaches and 10 days of increasing ataxia. except bilateral papilledema. Warfarin E. Complicated migraine C. Tuberculosis E. as well as a right abducens palsy.

Opening of the activation gate leads to an influx of E. Repetition is severely impaired. Phenytoin E. Broca aphasia B. D. Valproic acid of Ranvier. He can understand and follow commands. Levetiracetam examination he has mild right arm and face weakness. A 75-year-old male presents with an acute stroke. Propranolol C. Which statement about voltage-gated sodium channels myasthenic crisis except? is false? A. Lisinopril A. A. Carbamazepine 105. The activation gate is closed at resting membrane B. Conduction aphasia E. Ciprofloxacin sodium and resting membrane hyperpolarization. Prednisone potential and rapidly opens upon depolarization. Transcortical aphasia C. Which of the following best explains his speech pattern? A. Anomic aphasia D. Voltage-gated sodium channels cluster in the nodes B. The inactivation gate is open at resting membrane potential and closes slowly upon depolarization. All of the following medications can precipitate a 104. Magnesium B. C. Which of the following antiepileptic medications is a D. Zonisamide He has spontaneous speech with occasional paraphasic errors. On D. 103. The inactivation gate remains closed until strong hepatic enzyme inhibitor? repolarization has occurred. Wernicke aphasia Neurology Books . E.52 Neurology Self-Assessment 102. C.

A Other common substances that can lead to toxic Disability myopathy include zidovudine. In: Waldman SA. leaving a juvenile form presents with progressive proximal waste product of lactic acid. This is a polymyositis. Occasionally patients males. Jong FJ. with permission. Glycogen metabolism disorders show a oxidation refers to the breakdown of fatty acid normal elevation of ammonia. which can appear similar to Duchenne muscular dystrophy (DMD). The image Prominent hallucinations are found in dementia with shows the typical pattern of progressive disability with Lewy bodies (see Bradley’s NiCP. and corticosteroids. and labetalol are all known for causing toxic myopathies. E. Anaerobic metabolism occurs when form of this condition presents in childhood with no oxygen is available.) strength and reduction in pain after a short rest due to mobilization of blood glucose as a muscle energy 4. 2. 7th edn. Patients typically present D in childhood with exercise intolerance. followed by intense exercise of the produce 38 ATP per glucose molecule. is an autosomal. In this test. progressive supranuclear palsy or frontotemporal often mimicking amyotrophic lateral sclerosis. Debranching enzyme deficiency is a rare mind of its own. 96. (A) relapsing-remitting (the most pp. An adult form presents in the 5. and pyruvate is broken down in hypotonia. produces 2 ATP per molecule of glucose. Alzheimer’s & Dementia: The Journal of the Alzheimer’s Association 9(4. Alpha-glucosidase deficiency mitochondrion for use during oxidative is also known as Pompe disease. lipids. C recessive disorder resulting in a deficiency of myophosphorylase. pp. The urea cycle hand with levels of ammonia and lactate drawn at is a pathway for turning ammonia into urea. with permission. CBGD is usually unresponsive to progressive weakness.  The Krebs cycle (also known as the citric acid cycle source rather than relying on breakdown of glycogen. idiopathic PD.  This case is a typical clinical scenario for 40s.S): 23. and dystonia. 1915–1955). autonomic dysfunction is found in MSA. in the range of 9%–10%. CK may be Figure for Answer 2. B. 1915–1955). phenomenon (see Bradley’s NiCP. sensory Charcot-Marie-Tooth disease type 1A.  McArdle disease. or the tricarboxylic acid cycle) produces the majority of An exercise forearm test can identify a disorder of energy in the human body in the form of adenosine glycogen metabolism by measuring levels of ammonia triphosphate (ATP). 110. with proximal muscle weakness similar to corticobasal ganglionic degeneration (CBGD). niacin. (C) primary Swieten J 2013 An unusual case of logopenic progressive. Alpha-synuclein deposits are commonly associated pp. The most common phosphorylation. and Years pain induced by brief intense activity. Figure from Lize J. (B) secondary progressive. lumbar lordosis. However. whereas other antiepileptics are in the range of Neurology Books . 7th edn. cardiomegaly. but there is no “second wind” differentiate with asymmetric parietal lobe atrophy. alcohol. This is the most common disorder of carbohydrate metabolism. which presents with (PD). Often a phenomenon known as second wind is sclerosis. van common). myoclonus.) Pharmacology and seen with McArdle disease. also known as myophosphorylase deficiency or type V glycogenosis. but a lack of rise in molecules for the production of acetate (which later lactate. Anaerobic metabolism muscle weakness. 1422–1460). 110. and is associated with more cortical findings gastrocnemii. pp. magnetic resonance imaging will often failure can also occur. acetate from and lactate. syndrome. Absence of dystrophin on muscle biopsy look clinically similar to idiopathic Parkinson disease makes a diagnosis of DMD. cramps. and (D) progressive relapsing (see Bradley’s progressive aphasia associated with corticobasal NiCP. and a Gower’s sign typically in young than is typical for idiopathic PD. 51. with multiple system atrophy (MSA).  Valproic acid is the anticonvulsant with the highest risk of fetal malformations. PMP22 duplication is the genetic defect seen in including motor apraxia. autosomal-dominant hereditary demyelinating Typically. Ch. B. cortical sensory loss. B. Liver dementia. glycogenosis that presents in the third to fourth decade Although CBGD may have some clinical overlap with and causes severe weakness and atrophy of muscles.  The four types of multiple sclerosis are named for Typically. and a waddling gait. A. B. B 7th edn.” known as alien limb syndrome.3 (from Greenberg BM 2009 Multiple elevated. whereas colchicine. and dementia with Lewy bodies. which is an neglect. Test Two 53 ANSWERS 2 1. 6. but not with CBGD. Elsevier. Pompe disease is diagnosed with an progressive neurodegenerative disease characterized by assay for alpha glucosidase activity or muscle biopsy. tremor. 7th edn. and hepatomegaly. The the cytoplasm (rather than mitochondrion). propofol. to name a few (see Bradley’s NiCP. tau deposition and a number of clinical findings. 676–695). Terzic A (eds. which involves a return of Therapeutics: Principles to Practice. The diagnosis is confirmed with a muscle enters the Krebs cycle). baseline ammonia and lactate carbohydrates. Ch.  Lisinopril does not cause a toxic myopathy. There is no “second wind” phenomenon in will have one limb that will slowly rise and “have a DMD. and proteins is oxidized to levels are drawn. statin medications. In the Krebs cycle. pseudohypertrophy of the levodopa. Beta- serial intervals. The malate shuttle is a shunt of biopsy that shows a lack of uptake to a electrons across the inner membrane of the myophosphorylase stain. Ch. rigidity. these disease types. Ch. 3. the onset begins asymmetrically and may neuropathy. the pattern of their clinical presentation.

nausea. rheumatoid arthritis. including Lyme disease. 11. 7th edn. 95. GAA triplet cause of meningitis anaerobes metronidazole repeat on chromosome 9 is involved in Friedrich ataxia Brain abscess S. Ch. Sjögren disease. Although age unless there are no other options to control motion artifact is present on this scan. 65. though there is no causative Bacteroides spp. leading to activation of effective treatment (in up to 80% of patients) of many second-messenger systems expressing proinflammatory conditions. E. platelet-activating factor (PAF). 7th negative bacilli cefepime edn.. C. The key distinction between the two is that predominance. pp. Spinal epidural Staphylococcus spp. and macrophages follow thereafter. acid may lower verbal IQ and memory function (see Figure from Mayans D. Recommendations are shown in the following syndromes can lead to fever or loss of balance. with Bradley’s NiCP. demonstrating diffuse enhancement of the meninges pyrazinamide) called pachymeningitis. ECT must be repeated for seven to eight alpha (TNF-α). which typically causes an factors for Listeria L. the affected nerves in these conditions (see Bradley’s NiCP. catatonia.. TABLE FOR ANSWER 2. Ch.54 Neurology Self-Assessment 3%–5%. ceftriaxone or increased risk of AD development. though it has not enteric gram. astrocytes become hypertrophic memory impairment such as retrograde amnesia for and microglia activate. 107. 349–391). rifampin. sulfamethoxazole and presenilin-2 are all causative of early-onset AD. Macrophages become the predominant cell type by 13. pp. pp. usually caused by viral elevated white cell count with a neutrophil infection. the most epilepsy. Ch.14  Empiric Antibiotics for Bacterial Infections of the CNS 9. and various fungal infections. Ch. 1147–1158). B. inducible factor 1 (HIF-1). 7th edn. streptococci. Vancomycin + Apolipoprotein E4 is an isoform associated with an abscess Streptococcus spp. Either of these viral factors. As noted earlier As noted earlier axonal neuropathy more than a demyelinating or otitis (depending on age (depending on age neuropathy. Injured brain cells also secrete severely depressed patients who are also pregnant. By space-occupying lesions. mania.  Localized hypoxia prompts the initial step in 12. including medically unresponsive genes such as nuclear factor kappaB (NF-κB). causes hearing loss in addition to relatively normal glucose indicates a viral infection. Lymphocytes. PMP22 deletion cefotaxime or causes the syndrome of hereditary neuropathy with cefepime liability to pressure palsies. Lamotrigine has a slightly higher-than-average temporal arteritis. The Bacterial meningitis most common hereditary demyelinating neuropathy is Age <50 and no risk S.) genetic mutation known for this. 1973–1991). Absolute contraindications are unclear. The table. A. SOD1 on chromosome 21 is involved in predisposing and risk factors) + and risk factors) + autosomal dominant motor neuron disease. increased risk of malformations at 5%–6%. meningitidis ceftriaxone or a PMP22 duplication on chromosome 17. Any of the above M. 7th edn. 7th edn. pneumoniae. which 8. and to a few hours after stroke and peak by 1–3 days. 920–967). highly suicidal patients. Valproic and several infections. personal collection. carcinomatosis. and signal transducer and activator of schizophrenia. Ch. tumor necrosis factor Many times. syphilis. and anaerobic ceftriaxone or 10. (see Bradley’s NiCP. 583–604). cefotaxime or been proven to be causative (see Bradley’s NiCP. congestive heart failure. Ch. autonomic monocytes.  This patient presents with bacterial meningitis. tuberculosis (high Four-drug therapy suspicion) (isoniazid. the time surrounding the hospitalization. 105. cells to invade the infarcted tissue within 30 minutes poorly controlled hypertension. interferon regulatory factor neuroleptic malignant syndrome. hypotension. 1380–1421). and low glucose.  Gamma secretase is an amyloid-cleaving enzyme. mastoiditis. Neurology Books . In this patient. therefore. pp. 7th edn.  This patient has a hereditary. 46. A. C.2. Electroconvulsive therapy is also used in transcription 3 (STAT3). Autosomal-dominant Nocardia Trimethoprim- mutations in amyloid precursor protein. but reduce the stability of the blood–brain barrier and relative contraindications include unstable coronary allow for cellular infiltration. and acute 1 (IRF1). aureus. 7. Vancomycin + Charcot-Marie-Tooth (CMT) type 1a.  Electroconvulsive therapy is considered a safe and poststroke inflammation. as evidenced by slow nerve Disease entity Organisms Empiric antibiotics conduction velocities without conduction block.  Labyrinthitis and vestibular neuritis are both is diagnosed by her lumbar puncture showing an peripheral vertigo syndromes. 79. permission. depression. acid is not recommended in women of childbearing tuberculosis. 7th edn. not acquired. demyelinating neuropathy. headache. elevated protein. A labyrinthitis affects both the cochlear and vestibular lymphocytic pleocytosis in the cerebrospinal fluid with nerves and. seizure disorder.  At rest the membrane is most permeable to chloride 5–7 days postinfarction (see Bradley’s NiCP. Valproic acid may have longer-lasting effects. whereas vestibular neuritis affects the vestibular Empiric treatment depends on age and predisposing nerve and only causes vertigo. B. D. pp. 112. Ch. pp.  The MRI shown is a postcontrast T1 image 6ethambutol. MFN2 is a common Age >50 and/or risk As noted earlier + As noted earlier + abnormality in CMT2a. and confusion after ECT. aerobic Vancomycin + (see Bradley’s NiCP. Table 53C. monocytogenes ampicillin Sinusitis. and interleukin 1-beta (IL-1β). 14. oral cefotaxime or and gastrointestinal cefepime + Excessive gamma secretase activity has been associated flora (including metronidazole with AD development. 7th edn. 1736–1765). significant finding is pachymeningeal enhancement (see Some studies suggest that fetal exposure to valproic Bradley’s NiCP. 34–35. pp. 1791–1867). Neutrophils are the first disease with recent infarction. which is seen in intracranial From Bradley’s NiCP. Wegener granulomatosis. vertigo. because she is over the age of 50. These sessions. Possible side effects include 4–6 hours postinfarct. which is caused by factors for Listeria N. dendritic cells. type of perceived movement and the direction of ampicillin must be added for empiric coverage of Listeria nystagmus are not specifically helpful in determining (see Bradley’s NiCP. Ch. instability. pp. hypoxia. presenilin-1.

vasovagal syncope). 7th edn. distractibility. are extraparenchymal tumors that cognitive domains. blurred or tunneled vision. Castillo M. D. Meningiomas. 90. Test Two 55 15. with permission. Figure from Winn H 19. 486–503).  The imaging appearance of an avidly and 2011 Youmans Neurological Surgery. 2. is common with CNS information. although brightly and intellectual disability involves deficits in multiple solidly enhancing. Figure from leukodystrophy is a disorder caused by a deficiency in Naidich TP. seizures. system (CNS) lymphoma. tremors. bacterial cultures. or with a positron emission There is also a higher incidence of anxiety and chronic tomography scan of the whole body. to standing (5–30 minutes). pp. which inhibits appetite). develop normally but later have significant regression 20. may have normal blood work. Further. Smirniotopoulos JG the enzyme arylsulfatase A resulting in destruction of (eds. consideration. abdomen. abuts a cerebrospinal fluid space is classical for CNS lymphoma. and low diastolic flow. IV tPA would controls circadian rhythm. shortness of tomography. with contrast of the brain and entire spine. which may include neurological examination and normal cognitive affected sites in the brain. it is important to identify the postural orthostatic tachycardiac syndrome (POTS). extent of disease involvement within the CNS before This is a syndrome defined by having a heart rate beginning therapy in order to accurately monitor the increase of more than 30 beats per minute from supine efficacy of therapy with subsequent imaging. a fatigue in patients with POTS. velocity with elevated diastolic flow (see Bradley’s NiCP. 7th edn. representing the increased cellularity of the arithmetic procedures in the setting of a normal mass. D. The supraoptic nucleus not be indicated because of the recent SAH and synthesizes antidiuretic hormone and oxytocin. Importantly. E. protein. or hyperactivity. The because the mechanism for ischemia is vasospasm lateral nucleus controls appetite (as opposed to rather than thrombosis. although impaired attention. and flow cytometry. sympathetic tone). including language. cellularity. spinal fluid. Ch. Ch. but this patient does not show impulsivity. In older men. Saunders. normal or parasympathetic nervous system when necessary. Cha S. with homogeneously enhancing intraparenchymal mass that permission. 41. D. Dyscalculia is common in patients with vitreous. or medications clinically has symptoms of ischemia that correspond to such as fludrocortisone. a lesion in the dominant inferior parietal lobe (angular which was not mentioned in this patient (see Bradley’s gyrus or supramarginal gyrus). or nausea. but would not be useful in 7th edn. Metastatic lung cancer would function. Ch. it is important to first identify rigidity. and dementia (see the presence of systemic lymphoma involvement (if Bradley’s NiCP. Ensuring there is no systemic POTS-associated symptoms may include mental involvement of the cancer is done with either computed clouding. and longer. Primary CNS lymphoma. pp. finger this infection with the John Cunningham (JC) virus agnosia. Treatment includes an increase of dietary 215 m/s). E. In the juvenile form. Increased intracranial pressure the ventromedial nucleus. palpitations. 8–16). dizziness.  TED should be used on patients with subarachnoid POTS include pheochromocytoma. intraparenchymal disease. an enhancing. whereas the arcuate nucleus produces dopamine (see Bradley’s waveform in the figure demonstrates an elevated NiCP. 696–712). A Holter monitor would blockers and angioplasty should be performed to ensure that only sinus tachycardia is present. 52. Ch. Mild central necrosis. with edema disproportionate to the size of the patients do not develop normally. the acute setting. Generally. learn arithmetic facts. Although the patient appears to have 18. with and without contrast of the chest.  Dyscalculia is the inability to manipulate numeric shown on the ADC map. although generally affecting the white usually known before starting school. Diagnosis of POTS is typically made with a flow velocities reach 200 m/s (and our case is tilt-table test. The low velocities. Metachromatic NiCP. and constructional apraxia due to tends to be associated with severe immunosuppression. spine. is usually a nonenhancing disease. When mean activation. 459–485). midodrine. restricted diffusion of water. propranolol. exaggeration of symptoms extent of disease evaluation for the CNS includes MRI when standing. patients must have symptoms for 6 months or ophthalmological examination with a slit lamp. usually do not lose consciousness with this condition. because prevent further ischemia. cytology. is common in patients with SAH and should be a The anterior nucleus controls body temperature. or execute lymphoma. and impairments are tumor(s). patients initially Series). and is often be associated with multiple ring-enhancing associated with an IQ between 50 and 70. eventually leading to muscle weakness. syndrome involves left–right dissociation. which requires separate treatment by medical 17. executive arise from the meninges.  The suprachiasmatic nucleus of the hypothalamus cerebral ischemia based on clinical exam. 7th edn. exercise. The patient salt. pp. present). does not affect other parts of the body and ADHD. Emergent treatment with intraarterial calcium channel pyridostigmine. PML. Additionally.  Upon suspicion or diagnosis of central nervous in school. B. pp. Whereas John Cunningham (JC) virus Neurology Books . Saunders. or function. and must have standing plasma norepinephrine lumbar puncture with cerebrospinal fluid analysis for levels of 600 pg/mL or more (both standing and glucose. supine plasma norepinephrine levels are collected). neutrally neurally- hemorrhage (SAH) for at least the first 14 days after mediated syncope (e. and social skills. as this is the most common site of lymphoma in this and blood pressure is generally normal. SAH to prevent vasospasm. Further. Second. Ch. dyscalculia. as 16. clonidine (which decreases the artery that has velocities indicating vasospasm. is generally Generally patients with ADHD have problems in all of peripherally or heterogeneously enhancing due to their classes rather than a specific subject. and improvement with recumbence. Glioblastoma.) 2013 Imaging of the Brain (Expert Radiology myelin sheaths. or mast cell 2 the hemorrhage to assess for vasospasm. memory. compression stockings. 40. or modafinil. vision loss. Mimickers of population.  There is no indication for an MRI of the brain in oncologists. Oral nimodipine is used in other arrhythmias can mimic POTS (see Bradley’s NiCP. this indicates severe vasospasm. breath. These lesions. and pelvis. 1301–1323). patients testicular ultrasound may additionally be considered.g. TCD in increased intracranial pressure preventing overheating through activation of the typically shows a high-resistance waveform. 7th edn. reading. pp. Gerstmann matter.

1538–1562). Ch. A. as well as consistent with Lennox-Gastaut syndrome (LGS). C. Ionizing Frequently. These are noted to have bright. waves. 1538–1562). Ch. Plotkin SR and memory loss. This disease is marked away from this diagnosis. which can lead to very high levels of serum evidence-based treatment.  Taking into account the patient’s medical and fluid rhinorrhea and pituitary apoplexy.  The patient is suffering from fatal familial Meningiomas are a reasonable consideration given the insomnia. which normally encodes a protein are not consistent with limbic encephalitis (see called merlin. with the “cone” represented adenomas). 696–712). C. such as the bromocriptine or transsphenoidal surgery with or acoustic nerves. The MRI findings chromosome 22q11. and internal auditory canal. and would not explain the internal auditory canal with permission. Also the positive family by dramatic insomnia and disruption of the typical history suggests only vestibular schwannomas out of sleep cycles. Figure from Rennert J. this is less likely (see in the midportion of the recording. IVIG could be used in cases of an cataracts. one can proceed with therapy such as metastasis does often affect cranial nerves.  The pattern demonstrates typical hypsarrhythmia. Positron emission tomography tumor predisposition syndrome that most classically scans show hypometabolism of the thalamus and presents with bilateral vestibular schwannomas. neurofibromas. E. B.5 Hz is angiofibromas. as well as the classical ice serological studies such as prolactin (lactotrophic cream cone shape on MRI. autoimmune encephalitis. and inguinal regions. pp. pp. both gonadotrophic adenomas. 102. not solely left temporal silent. Tuberous sclerosis is an autosomal. Otolaryngologic Clinics of catecholamines. Ch. dominated by suppression with brief bursts of activity which is likely a pituitary microadenoma (<10 mm). myoclonus. REM is almost nonexistent. MRI can show atrophy of the North America 45(2): 471–486. involvement or the enhancement homogeneity. freckling of the axilla NiCP. but cingulate cortex. 57–65). 100. This is an autosomal-dominant prion radiographic appearance. with the remainder being clinically demonstrates multifocal spikes. Patients with such caused by mutations in the NF1 gene on chromosome syndromes generally have impaired recent memory and 17 encoding a protein called neurofibromin. The seizure discussed in the question stem is dominant disorder that affects multiple organ systems. arrest of development. and remote memory (see Bradley’s associated with café-au-lait spots. Ch. pp. is more common than neurofibromatosis type 2 and is immediate memory. with permission. arising from the suppressed background. and kidneys. infantile spasms. Doerfler A 2007 Imaging of sellar and parasellar lesions. 7. The disorder leads to coma and death may also involve juvenile posterior subcapsular in a short period. amenorrhea) and MRI imaging. particularly the anterior ventral and 22. 7th or with such large tumor burden on MRI. which may be either as related to the TSC1 (encoding hamartin) and TSC2 described in the question or with truncal extension and (encoding tuberin) genes. Over time. given their bilateral nature and the positive family 25. These adenomas can but is not bilateral or homogeneously enhancing. Further. Slow spike and wave at 1–2. E.56 Neurology Self-Assessment DNA levels are usually detectable in cases of progressive Of those that are silent. quiet sleep is a pattern seen in neonates. the available choices (see Bradley’s NiCP. an abnormal gait. This meningiomas.  Neurofibromatosis type 2 is an autosomal-dominant dorsomedial nuclei. thalamus. is found. This EEG also hormone secreting. 7th edn. but this patient’s family The inactive gene leading to this syndrome is NF2 on history suggests an inherited disorder. West syndrome involves the triad of demonstrate subependymal giant cell astrocytomas. There is also increased sympathetic 2012 Chemotherapy in vestibular schwannoma: activation. Other symptoms can include cerebrospinal 21. considered a burst suppression pattern. generally involving the cerebellum. there is no reason to bitemporal hemianopsia or headache that the neoplasm routinely evaluate for this infection in CNS lymphoma. it is usually not until there is a multifocal leukoencephalopathy. The attenuation Approximately 60%–70% of pituitary adenomas are shown is when the epileptic spasms occur. neurofibromas. the posterior fossa.  A classic amnestic syndrome refers to loss of recent history. children with a hypsarrhythmia pattern will radiation may increase the risk of gliomas and transition into an LGS pattern later in childhood. but would be rare to present so slowly without adjuvant radiotherapy (see Bradley’s NiCP. 24. as well as homogeneous enhancement. subependymal nodules. Affected individuals may arm extension. Lisch nodules. It common type of pituitary macroadenoma (>10 mm). decreased libido. Glioblastoma is exceedingly rare in the posterior fossa. which would be 23. and Shagreen patches.  The MRI shows a small lesion in the pituitary gland. patients develop ataxia. lungs. Figure from Terry AR. This disease orientation. IGF-1 (somatotrophic adenomas). facial hypsarrhythmia. Medulloblastoma is a tumor of luteinizing hormone or follicle-stimulating hormone. or gliomas. but without mention of exposure and brief clip from the EEG does show an area of attenuation with positive family history. is the precursor to slow wave sleep and is marked by a Neurology Books . which generally relates autosomal-dominant tumor predisposition syndrome to bilateral hippocampal damage. meningiomas. 1615–1685). erectile schwannomas. but preserved procedural memory. is no leptomeningeal enhancement on MRI. but this is not Bradley’s NiCP. Bradley’s NiCP. scoliosis. pp. also cause pituitary hypofunction when nonsecreting Therefore involvement of the internal auditory canals is adenomas compress the normal pituitary cells. 100. and cortical tubers. and learning involves a chaotic pattern with multifocal spike and disabilities. Neurofibromatosis type 1 is a separate (short-term) episodic memory. Ch. 7th edn. Diagnosis is family history. This optic pathway gliomas. Leptomeningeal diagnosed. but the specific internal disease that can be transmitted to others who come in auditory canal involvement and lack of dural tail point contact with the prion protein. The tumors are unlikely to be sporadic. the most likely cause usually made using a combination of history (visual of his hearing difficulties is bilateral vestibular complaints. Once not easily explained by this diagnosis. consistent with infantile spasm. fairly dysfunction. Clinical Neurology and Neurosurgery 109(2): 111–124. difficulties with the heart. fatigue. The gonadotrophic adenomas are the most spikes. 26. pp. 7th edn. as well as the MRI. 24-hour by the enhancing portion of the tumor entering the urine-free cortisol (corticotrophic adenomas). 52. there edn. 7th edn. Finally.

Hypoxia is generally associated with Permanent areflexic bladder Permanent areflexic bladder intrinsic lung disease rather than obstructive disease. with permission. Hyperventilation. Ch. and simple consultation is not necessary. Ch. 7th edn. often causing a respiratory acidosis. The spinal shock is usually associated with low tone and main side effects include akathisia. and abnormal neurological function to explain the therefore is legally dead in most states. dyspnea. 101. Figure may lead to encephalopathy with confusion and from Daroff RB. and leads to muscle tension. Acute and respiratory systems can keep working for some stressors are not required at the onset for a diagnosis of time after all cerebral activity has ceased. making the conversion disorder. but can only concentration. A. B. Haloperidol and ziprasidone can cause QT 27. A 30. Mazziotta JC. Ch. 7th 28. Other causes of encephalopathy and altered level of consciousness. one might and Statistical Manual of Mental Disorders and captures a consider an evaluation for ischemic cause of the large portion of patients formerly classified as neuropathy. olanzapine is notorious for causing weight years of age. children generally have about a 10-word vocabulary. especially given her smoking history (see hypochondriacs. Ethics 33. 7th edn. but at higher levels it is an antagonist. and should speak in complete sentences that gain.  The patient suffered a spinal column fracture at prolongation. 7th edn. 2016 Bradley’s Neurology in Clinical Practice. 7th edn. young man. C. or other causes Conus Medullaris Syndrome and Cauda Equina Syndrome of reduced ventilation. communicating artery. 881–902). Babbling usually occurs by 6 months. or loss of consciousness in severe cases (see Bradley’s NiCP. and diabetes. and sudden loss of Absent deep tendon reflexes Absent deep tendon reflexes consciousness. Test Two 57 pattern of tracé alternans (see Bradley’s NiCP. 10. or in response to Symmetrical motor impairment Asymmetrical motor impairment metabolic acidosis (as with ketoacidosis). All of the symptoms listed are of action because it is a partial dopamine agonist at D2 commonly seen with severe spinal cord injury. confusion. Physicians are not obligated to perform futile intentional production of symptoms without a clear procedures.  The patient has been declared brain dead.” and “bye-bye” by 9 months. D. and the lack of severe anxiety concept of brain death hard to comprehend. and absent reflexes. metabolic syndrome. 7th edn. Pomeroy SL altered level of consciousness.  Hypercarbia. E. Jankovic J. 92–114). paresthesias. Ch. the discussion with family. pp. Neither transferring the patient to a secondary gain. This is an can be understood by strangers by 3 years of age (see important consideration because patients usually Bradley’s NiCP. The patient described in the Reassurance and negative workups do nothing to question stem has pupil involvement in her alleviate the anxiety. Ch. Aripiprazole is unique in its mechanism of the conus medullaris. Ch.  The patient presents with illness anxiety disorder. sleep apnea or obesity-related hypoventilation (both of which may be contributing to the current case). and are delineated in the table (see 31. Conversion disorder involves a loss or Bradley’s NiCP. 66–72). with permission. One of the more typical manifestations of confusing situation for families because the circulatory conversion disorder is a nonepileptic seizure. In the immediate postinjury setting. Jankovic J. Clinical Practice. entity that is conceptually divided by whether or not 32. Given that the pupillary which is a somatic symptom disorder (formerly parasympathetic fibers are located superomedially on somatoform disorder) in which patients are the oculomotor nerve. pp. require long-term treatment with antipsychotics.27  Similarities and Differences between medication side effect as with opiates. and ultimately the illness becomes oculomotor neuropathy. as the patient is dead and no further about daily activities that makes one easily fatigued. Illness anxiety disorder is evaluated for an enlarging aneurysm as the cause of her a new diagnosis in the fifth edition of the Diagnostic symptoms. interventions will benefit her care. By 1 year. Contacting organ irritable. pp. 63. and periodic electrocardiograms are T12–L1 that severed the spinal cord just above the level recommended. may lead to Vertebral column injuries Vertebral column injuries distal respiratory alkalosis and is commonly associated with between T12 and L2 to L2 lightheadedness. 1563–1614). situation the physician is acting ethically by refusing Malingering involves the intentional feigning of disease further procedures and should have a discussion with for personal gain. If her pupil were not involved. change in motor and/or sensory function without 29. involvement often caused by panic. Factitious disorder involves the withheld. 104. the pupil is involved.  The patient presented with an oculomotor palsy. Pomeroy SL 2016 Bradley’s Neurology in frequent complete blood count monitoring. A. pp. and therefore should be part of the patient’s identity. Neurology Books . agonist. causes sleep disturbance. Figure from Daroff RB. and there is nothing in the question the family about why these procedures are being to suggest this. especially when acute. Generalized anxiety disorder is a different hospital nor complying with family requests persistent and excessive feeling of worry or tension is ethical. including Conus medullaris syndrome Cauda equina syndrome hyperammonemia and uremia. it acts as an for hyperreflexia. At low dopaminergic activity. an edn. are generally not Upper and lower motor neuron Lower motor neuron involvement related to respiratory dysfunction.” “dada. pp. they are particularly at risk of preoccupied with the idea that they are ill or that they compression by an aneurysm of the adjacent posterior may acquire an undiagnosed medical condition. D. except receptors. 8. 7th edn. 814–834). Hypercarbia may result from untreated obstructive Elsevier. Absent bulbocavernosus reflex Absent bulbocavernosus reflex and may cause lightheadedness. Symptoms of cauda equina syndrome worsening of extrapyramidal symptoms (see Bradley’s and conus medullaris syndrome are also likely in this NiCP. 1720–1735). be performed at the request of the family. Clozapine can cause agranulocytosis and requires 2 Mazziotta JC. pp. This can be a symptoms. Elsevier. Bradley’s NiCP. but could be helpful in “mama. In this distinguishes it from illness anxiety disorder. TABLE FOR ANSWER 2. 58. impairs donation services may be reasonable. somnolence.  Although all antipsychotics have a mild risk of child should be able to use two-word sentences by 2 weight gain.

virus. CN X innervates the taste sensation present in the 39. patients cannot In fact. as it resides Dissociation apraxia occurs when a certain stimulus in the dorsal root ganglion of the affected dermatome. 7th edn. It is thought that both central handle instead of the head. (especially in the immunocompromised). so neither antibiotics nor stroke where vessel imaging and echocardiogram are Neurology Books . which are located on the anterior headache therapy.  The etiology of her acute stroke is most likely an distribution. due to tip and lateral portions of the tongue. pp. The axial those with sacral or lumbar involvement have the image shows an intramural hematoma along the left lowest risk. but know what it cutaneous infections and PHN are most common in should be used for. fall). with or without cutaneous eruption. 47). making multistep actions quite as burning. motor vehicle collision. roller coaster ride. B. Antivirals may shorten or aggressive coughing. or topical analgesics such as capsaicin as injury. These patients do not have problems 19. 7th edn. pp. single agents or in combination. 78. but they demonstrate errors in mechanical infection in the left T4 dermatome distribution. which is completed with additional after menses for migraine prevention. In the storage state. Patients with penetrating injuries a young person. There surgery for bullet fragment removal outweighs the is no indication of atrial fibrillation in the question benefits. input prevents detrusor activity. local pudendal control are primarily responsible for 38. whereas arterial dissection. 1686–1719). the heat associated with a bullet stem. as well as those who are a hammer is. 12. 7th edn. as dissections present with pain followed by acute there may not be a preceding rash. and their working in transcranial magnetic stimulation should be avoided concert requires input from various levels of the due to the potential ferromagnetism of remaining nervous system. reflex. even after the initial infection. but certainly this should be considered in a leaves the fragments sterile. E. and a lack of parasympathetic menses but not at other times during the month. sympathetic outflow and Ch. pp. but does not discussed or they are unable to discuss actions of innervate taste buds (see Bradley’s NiCP. CN VII through the chorda prevention when taken daily. Usually. diagnosis. anticoagulation) for at least 3–6 months followed by a pp.  The detrusor and urethral sphincter muscles fragment removal are generally required.  Vascular injury is common with penetrating traumas tomography angiogram or magnetic resonance to the brain (25%–36% incidence). and are unable to point out a tool when its function is touch sensation on the oral mucosa. 62. C. they often have more global findings to the brain should undergo early angiogram to screen and can be associated with strokes of the deep for pseudoaneurysm. Most often. Ch. Treatment often neurological deficits (in this case. There is no parasympathetic input causing the detrusor muscle to evidence of efficacy with short-term prophylaxis using contract (see Bradley’s NiCP. 7th edn. but a high index of suspicion is required. the history makes the internal carotid artery (crescent sign). migraine prevention. CN V is involved in pain.  Conceptual apraxia is a disorder in which patients pharynx. Ideomotor apraxia results in impaired severe pain in the same region. For example. pp. with coordinating their movements or following 36. recent trauma (football nortriptyline. making movements like tying shoes difficult. The pain can occur after reactivation of the weakness. B. either abortive or preventive. Magnesium oxide and 35. a patient knows what patients over 60 years old. Ch. Management for extracranial the course. as well as a follow-up angiogram structures. 103. B. Ch. MRI and function in opposite tasks. CN IX innervates rebound headaches (see Bradley’s NiCP. The pontine short-term prophylactic therapy may be efficacious for micturition center (with input from the hypothalamus. 7th edn. which is consistent control of skilled movements. but attempts to hammer a nail with the immunocompromised.  The patient has a history of a recent herpes zoster commands. the risk of flow on imaging rather than arterial disruption. pseudoaneurysms can form pseudoaneurysms develop when all layers of the vessel after dissections. tricyclic antidepressants such as chiropractic manipulation. 1686–1719). circumvallate taste buds across the posterior tongue. 1121–1146 and 103. and medial prefrontal antiinflammatory drugs or triptans may be started 2–3 cortex) is responsible for switching from the storage to days before menses and continued for up to 2 weeks voiding state. Generally. but continue to have flare-ups of PHN without a rash they can mimic the action (see Bradley’s NiCP. and stabbing. Both herpes zoster incorrect posture when using a tool. 122–127). beta-blockers or antiepileptics. Traumatic angiogram. have trigeminal nerve involvement. Those who Ch.58 Neurology Self-Assessment 34. with knowledge. have the highest risk of PHN.  Innervation of taste and the tongue involves riboflavin both have limited evidence for migraine multiple cranial nerves. Limb-kinetic apraxia results in a loss of with allodynia (pain evoked by normally nonpainful fine motor movements and dexterity not related to stimuli). B. so these patients have with postherpetic neuralgia (PHN). One would expect to find a lack of venous within the first several months. 7th edn. Occasionally. Further. including the brain and local spinal bullet fragments (see Bradley’s NiCP. 867–880). Opioids are generally avoided for fungiform papillae. especially in the V1 40. as shown in the image. E. cannot evoke the proper reaction. and can be associated confusing. particular tools. repeat vessel imaging study such as computed 37.  For women who suffer migraine headaches during urethral contraction. scheduled nonsteroidal periaqueductal gray matter. and vaccines may prevent the outbreak of dissections includes antithrombotics (antiplatelets or herpes zoster (see Bradley’s NiCP. temperature. Generally. as well as innervates the soft palate. Chs. pp. Ideational apraxia is the and peripheral pain receptors are involved in the inability to correctly order a series of movements to pathophysiology of PHN. but not only tympani branch innervates the taste buds on the perimenstrually. sharp. Common history clues include pregabalin. The pain is often described perform an action. Although venous sinus thrombosis wall are damaged and surrounding brain tissue forms can also present with headaches and lead to strokes in an aneurysmal wall. 190–196). patients may perform an action when verbally asked to do so. referable to the left involves the use of antiepileptics such as gabapentin or frontal region). CN VII also risk of medication overuse headache.

42. Emery communication is generally poor (see Bradley’s NiCP. Tricare is an insurance program administered aortic stenosis. a clinician should be suspicious of cancer recurrence or progression when a treated cancer patient develops a paraneoplastic syndrome. pp. that tends to affect boys between the ages of 5 and 15. increasing the age until which a small talk. including wide variation in fiber size and increased fibrous and adipose tissues. pp. AC.  Ulnar neuropathy at the wrist is uncommon. military retirees. 97. facial appearance described as “elfin. hypotonia. The severity and broad distribution of symptoms in this patient makes it unlikely that a microscopic brain metastasis could be causative. Additional immunosuppressive therapy may be required for the treatment of paraneoplastic disorders. although it from Mauro MA. where both the federal Department of Health and Human Services. Venbrux receives a large proportion of its funding from CMS. income or limited financial resources. Type 1 entrapment and Medicaid Services (CMS). et al.) would not affect speech (see Bradley’s NiCP. Table 97. Department of Defense to provide civilian poor social interaction. 2 with an otherwise negative workup. Ch. Test Two 59 normal. and is on the autism spectrum.  The image demonstrates endomysial lymphocyte known for particularly good verbal abilities. Morgan RA 2014 Image-Guided Interventions. dialysis) or any patient with amyotrophic lateral especially in women over 35 years old who smoke (see sclerosis. 2012 speech change. Chs. E. 1324–1341). Elsevier. Down syndrome and fragile X syndrome are associated and their families. but that would generally not cause Figure for Answer 2. severity.” as shown in the establishing a federal insurance marketplace and following image. including supravalvular provider. but are not 43. Figure from Uchino M. Angelman infiltration indicative of polymyositis. Oral include any patient with end-stage renal disease (on contraception pills may also increase the risk of stroke. 1915–1955). Yamashita S.  Paraneoplastic syndromes are generally immune- mediated constellations of symptoms indirectly related to the presence of a tumor through molecular mimicry.41 (from Haldeman-Englert CR. This can also Additional special populations covered by Medicare predispose patients to venous thrombosis. A hypercoagulable state from a neoplasm is Medicare is available to individuals over the age of 65 another cause of stroke. other things. 1461–1483). with permission. and conditions.  Williams syndrome is a rare neurodevelopmental Care Act is a multifaceted federal law that. Physically. which is a component of is the most common and most proximal. Thomson KR. Whereas the treatment of underlying cancers most often improves paraneoplastic syndromes. B. Clinical Neurology and Neurosurgery 115(5): 603–606. resulting in Neurology Books . individuals with Williams child may be on their parent’s insurance (26 years). Paclitaxel causes peripheral neuropathy in many patients. 50. 65. This pattern is syndrome is associated with intellectual disability and a in contrast to dermatomyositis. 7th edn. with pathology showing vacuoles with basophilic granules (see Bradley’s NiCP. et al. health benefits for military personnel. Although SE (eds. among disorder caused by a multigene deletion on 7q.) Avery’s Diseases of the Newborn. and developmental delay with mildly providing monetary disincentives for remaining decreased IQ. Dreifuss muscular dystrophy is an X-linked disease 7th edn. C. with no inflammatory pathological findings. 2013 Muscle biopsy findings predictive of malignancy in rare infiltrative dermatomyositis. Medicaid is an insurance program Bradley’s NiCP. Asperger syndrome is associated with by the U. Inclusion body myositis is an inflammatory myopathy. where biopsy shows characteristic happy personality. In: Gleason CA. 7th edn. Aetna is a private medical insurance various heart malformations. pp. increased the number of insured Characteristic findings in Williams syndrome include a Americans by expanding Medicaid coverage. Radiation to areas outside of the brain Specific chromosome disorders in newborns. Juul would not account for the symptoms listed. pp. 9th edn. D. 44. but program. widely spaced teeth. Figure administered by each individual state. Saunders.  Medicare is a federally administered insurance 45. but strong language skills and knack for uninsured by choice. Ch. but with dystrophic features. Ch. Murphy KPJ. and response to antineoplastic therapy. syndrome may also have difficulty gaining weight (with and preventing insurance denial for preexisting failure to thrive). motor and sensory fibers are affected. 7th edn. 7th edn. depending on the type. with spine metastases may affect sensory function. managed through the Centers for Medicare has four main types of entrapment. 110. The Affordable 41. 920–967). they permission. Uchino K. A.1). pp. Paraneoplastic cerebellar degeneration is associated with anti-Yo (PCA1) antibodies in many cases (see Bradley’s NiCP. with various levels of intellectual disability.S. especially recurring strokes who have paid Medicare tax from earned wages. 648–675 and 90. but verbal perifascicular inflammatory infiltrate only. 2nd Medicaid is generally reserved for individuals with low edn. with permission.

B. bradycardia. 7th edn. seizures. women attempt suicide more schizophrenics and increased D2 receptor occupancy on frequently than men. rivastigmine.  The correct answer is the older Caucasian man schizophrenia is the D2 receptor. Common side NiCP. as well as pyramidal gravis to assist in making the diagnosis. Middle with exercise or fatigue or in the evenings. and nerve conduction receptors are in the thalamus. abnormal spontaneous activity and neurogenic 49. numbness. diplopia is hypotonia. 1380–1421). magnetic is a monoamine oxidase B inhibitor used in the resonance imaging often shows hyperintensity in treatment of Parkinson disease. Ch. Although patients may have of visual acuity. LNS is associated with motor and cognitive with Grave disease. and visual and cerebellar intravenously to reverse acute symptoms of myasthenia disturbance are all common. including exophthalmos. acetylcholinesterase inhibitor that can be injected Myoclonus. hormone receptor–directed antibodies are associated Clinically. nonfluent) aphasia if in the dominant hemisphere (see leading to motor weakness in the affected muscle. cases.  The angiogram demonstrates a left common carotid of the patients. B. Caucasian. To test for Froment sign. headaches. A purine-restricted diet in cancer-associated retinopathy. one would not may also have movement disorders. whereas D1 suicide include previous suicide attempts. elevation of uric acid due to hypoxanthine-guanine- bulbar symptoms are rare in LEMS. 7th edn. Further. 7th edn. Memantine is a finger. A. 7th edn. pp. hold a piece of paper between the thumb and index or gastrointestinal hemorrhage. 1791–1866). with an abrupt cutoff at the Patients commonly complain of symptoms worsening left middle cerebral artery branch point (M1). D.  The patient in this case has Lesch-Nyhan syndrome proximal muscle weakness and constipation. akinetic mutism. 91. Further. and D5 chronic illness. given that the ocular and extraocular behavior. cerebral artery strokes generally cause hemiparesis and Acetylcholine receptor–directed antibodies block sensory loss. substance abuse. although a patient’s electroencephalogram is often Edrophonium is a short-acting. pp. pp. hemianopsia. a patient should atrioventricular block. Both levodopa and disorder commonly seen with melanoma and other tetrabenazine may be used for the movement disorders cancers.60 Neurology Self-Assessment weakness. and galantamine are all motor units with reduced recruitment can be seen. socially isolated. as well as aggressive and self-harming ophthalmopathy. Those with an increased risk of receptors are located in the striatum. LEMS patients commonly complain of 51. and Froment sign is indicative of adductor pollicis depression. 107. which later becomes spasticity. 1365–1379). pp. Pomeroy SL associated with Lambert-Eaton myasthenic syndrome 2017 Bradley’s Neurology in Clinical Practice. treatment of Alzheimer disease. confusion. with early ophthalmopathy. whereas diplopia is not common (see seizures. determined (see Bradley’s NiCP. Allopurinol is a xanthine oxidase optic neuritis (frequently bilateral). Bradley’s NiCP. this fact is a red herring. and an expressive (or acetylcholine receptors at the neuromuscular junction. but men complete suicide more positron emission tomography imaging. if the patient bends the distal portion of the glutamate receptor antagonist used in the treatment of thumb (using the flexor pollicis longus) to hold the moderate to severe dementia or as an add-on therapy paper in place. such as chorea or expect fluctuation if diplopia were due to thyroid athetosis. a paraneoplastic also helps reduce uric acid. 95.  The most common receptor implicated in 52. include nausea. More serious side effects include weakness. Vitamin E was studied the caudate and putamen. (LEMS). Serum uric acid levels are often elevated. Cerebrospinal fluid 14-3-3 to mild Alzheimer disease. levetiracetam would not be the best choice of Bradley’s NiCP. The reversible acetylcholinesterase inhibitors used in the dorsal ulnar cutaneous nerve branches before the wrist. pp. Chs. pp. Rasagiline and/or extrapyramidal signs. headaches. 1896–1914). patient does have a long history of smoking. Ch. Patients generally complain of progressive loss associated with LNS. The patient fairly rare among patients. Bradley’s NiCP. D3 and D4 are in the limbic system. B. but not mild cognitive impairment temporoparietal cortex. Common side effects so it is not injured in ulnar neuropathy at the wrist. 7th edn. fluctuating diplopia inhibitor and benefits patients with LNS due to is not common. with (LNS). pp. 44. Although this Elsevier.  Myasthenia gravis first affects the eyes in about half 50. Most D2 frequently than women. 65. Although this is associated with regression after 3–6 months of age. Aquaporin-4 antibodies diagnosis of LNS can be made by clinical examination are associated with neuromyelitis optica (Devic and by mutational analysis of the HPRT gene on the X disease). artery contrast injection.  Donepezil. Although patients commonly present with chromosome. 7th edn. The muscular changes are static. Additionally. Those with a decreased risk of suicide Neurology Books . The role of vitamin assay is often used for diagnostic confirmation in the E in Alzheimer disease treatment is yet to be absence of a brain biopsy (see Bradley’s NiCP. 528–572 and antiepileptic because it may worsen aggression (see 109. and abnormalities in sensory and motor fibers. 1324–1341). older age. diarrhea. with symptoms of diplopia or ptosis. 7th edn. Jankovic J. an X-linked recessive metabolic disease causing improvement in the former after exercise. and ganglia. and there may be fluid in Alzheimer disease and has had mixed findings of attenuated inversion recovery (FLAIR) or diffusion possibly slowing the progression from moderate to weighted imaging (DWI) hyperintensity in the severe disease. In general. In severe hypothalamus. Froment sign is present (see Bradley’s to an acetylcholinesterase inhibitor. effects include dizziness. A. hippocampus. Ch. E. 46. and rarely Stevens-Johnson syndrome. Ch. Figure Calcium channel–directed antibodies (P/Q) are from Daroff RB. Thyroid-stimulating phosphoribosyl transferase (HPRT) deficiency. 47. reversible abnormal with periodic sharp wave discharges. Retinal-directed antibodies are found reduction of uric acid levels. a paraneoplastic disorder.  Seizure is not a common symptom of sCJD. 920–967). syncope. receptors are located in the cerebral cortex and basal men. with permission. There is increased who has a history of substance abuse and access to dopamine receptor density in the limbic regions of weapons. Mazziotta JC. Ch. 48. somnolence. anorexia. 94.

Taylor D 2013 Pediatric Ophthalmology these are paroxysmal episodes typically induced after and Strabismus. and good health. A. Sandifer syndrome is abnormalities. of the lower trunk. Ependyma refers to affect the upper trunk. women. but ropinirole have similar side effect profiles. or ataxia. Although she is at an 57. all depending on the type of PCH (see also a possibility and typically presents with extraneous Bradley’s NiCP. GABA is a major inhibitory edn. which antagonizes because of her strong support system. lack of histamine and alpha2-adrenergic receptors. Amantadine is an antiviral agent also used of serotonin and norepinephrine. pp. Ch. ptosis. order to attract attention. Saunders.  Rasagiline is a monoamine oxidase B inhibitor. The woman dermatome. There is no indication that the child is causes breathing abnormalities.  The MRI shows the classic “molar tooth sign” often injury is fairly rare due to the anatomical location of the seen in Joubert syndrome. B. 528–572). and is used for in Parkinson disease that has possible side effects of depression. Ch. This injury and central canal of the spinal cord. include Dandy-Walker syndrome and pontocerebellar Some consider paroxysmal torticollis in infancy to be a hypoplasia (PCH). Ch. also The woman in choice C has a history of borderline helps relieve insomnia and depression. 103. 7th edn. distinct facial features (widely this time. the child is usually alert and responsive spaced eyes. including kidney suddenly lasting for a few hours to a few days. pp. The cause of Klumpke paralysis is dysfunction family network to lower her risk of suicide. 7th edn. and corticospinal always normal in this condition. 96. so it has a potentially serious side for their dual mechanism of action. 1766–1790). The fact that this child throughout the ventricular system and is reabsorbed by also has evidence of Horner syndrome suggests that the the arachnoid villi. Serious complications and is generally thought to be contraindicated in include orthostasis. Pericytes surround the endothelial T1 root is affected. E. and glutamate are excitatory transmitters. The used for depression. pp. Additionally. 1422–1460). feedings and are shorter in duration. usually inherited in an autosomal-recessive fashion. pallor. and has been shown to improve woman in choice A has a decreased risk of suicide appetite and sleep. younger age. and infancy. and/or combined with some selective serotonin reuptake noradrenergic syndrome. having seizures. and vision abnormalities including retinal legs. dopaminergic. However. 59. cystic CACN1A gene. which is involved in the breakdown of dopamine and 56. Dopamine. excessive reward-seeking behavior. The man in choice D has muscles. age. doses and norepinephrine and dopamine at higher psychosis. seizures. This is an idiopathic condition characterized by hypotonia. although this may be challenging to test in a in choice E also has a lack of impulsivity and a strong newborn. Injury to the C5 or C6 nerve root can cells of blood vessels in the brain. and arrhythmias (see Bradley’s doses. hallucinations. L-dopa and Venlafaxine also is used for the same indications. including works by blocking the reuptake of serotonin at low orthostasis. is accompanied by breathing abnormalities. Chorionic villi are may lead to Erb–Duchenne paralysis (“waiter’s tip” a part of the placenta (see Bradley’s NiCP. including internal rotation of the arm with an Ch. lack of norepinephrine and serotonin and antagonizes impulsivity. Neurology Books . dyskinesias. and occasionally weak triceps. This posterior fossa with elevation of the tentorium. Mirtazapine increases the release of NiCP. intellectual disability. serotonin receptors but inhibits synaptic uptake. Other cerebellar hypoplastic syndromes accompanied by vomiting. and is Entacapone is a catecholamine methyltransferase used for smoking cessation and depression. 88. PCH condition is self-limiting and almost always disappears involves atrophy of the pons and cerebellum that by 5 years of age. Priapism is a personality disorder and performs these attempts in unique side effect of trazodone. 106. During and liver abnormalities. irritability. her risk is slightly weak finger and wrist flexors. particularly when the serotonin system. low-set ears. African-American. oculomotor apraxia with nystagmus. including hyperpnea alternating with periods of apnea. 7th edn. psychosis. Test Two 61 include no prior attempts. usually involving effect of malignant hypertension. married. B. limb and 58. 44. Dandy-Walker syndrome is migraine equivalent.  The child likely has benign paroxysmal torticollis in truncal ataxia. pp. from Hoyt CS. Many other signs and symptoms may also periods of cervical dystonia that begin and end be associated with Joubert syndrome. 1261–1278). Bupropion works by blocking inhibitors. 1686–1719). and arrhythmias. Occasionally these movements can be dystrophy. E. and a relationship with the characterized by agenesis of the cerebellar vermis. but should also have 55. It is mostly 2 plan or exposure of weapons. and patients with epilepsy. has been discovered in some families. palsy).  The mixed-mechanism antidepressants are known norepinephrine. and neuropathic pain. giving patients often have numbness in the C8 and/or T1 him likely reasons to not commit suicide. serotonin.  Glycine is the main inhibitory neurotransmitter of nystagmus and head nodding (see Bradley’s NiCP. 7th edn.  CSF is continually produced by the choroid plexus to either the C8 or T1 nerve root. Figure movements caused by gastroesophageal reflux. B. and an enlarged migraine. Duloxetine inhibits the reuptake psychosis. often resulting from stretching injury 53. broad forehead). seizures. pp. with permission. Isolated C7 54. without other adventitious movements of the arms or polydactyly. 7th the spinal cord. This is a condition root. and electroencephalograms are almost motor neuron degeneration. fibromyalgia. neurotransmitter in the brain. syncope. extended elbow and pronated forearm due to weakness of shoulder abduction and elbow flexion. both a family at home and hope of a cancer cure. the reuptake of norepinephrine and dopamine. which is linked to familial hemiplegic dilatation of the fourth ventricle. It Ch. Trazodone. and often result from stretch the glial-cell inner lining of the ventricles in the brain injury of the neck away from the affected side. It can inhibitor (catechol-O-methyl transferase) that slows the increase the risk of seizures at doses above 450 mg/day degradation of dopamine. dermatomal sensory loss (see Bradley’s NiCP. resulting in serotonin syndrome.  The presenting symptoms of Klumpke paralysis are increased risk of suicide attempt. Spasmus nutans is associated with cervical dystonia. weak intrinsic hand lower for actual completion. It generally results in triceps weakness and associated with hypoplasia of the cerebellar vermis. and ethnicity. E.

pp. recollections of the event.  PTSD occurs when a person experiences or witnesses 63. Ch.  With an underlying diagnosis of AIDS. 163–179). In a type 2 lesion. Congenital spinal stenosis passed on from another physician without reconfirming demonstrates a small spinal canal. Elsevier. in critically ill help protect personal information. This condition is considered equally colleagues is important if the content violates medical emergent and critical as convulsive status epilepticus ethics or is below the standard expected of a physician. Figure from Fernández- Alzheimer disease shows neurofibrillary tangles. tachycardia.  Rods contain the photopigment rhodopsin. with permission. the spinal cord divides in half and 64. The pathological hallmark of HSV vital signs had stabilized. involves closing the diagnostic process early because of Subacute combined degeneration shows T2 a characteristic presentation leading to a diagnosis. not ruling out alternative diagnoses. Chs. 62. Dementia with suffering from bacterial meningitis (see Bradley’s NiCP. Treatment involves surgery to remove the are many reasons a patient could have arm weakness fibrous septum that tethers the cord in place. pp. dreams. There childhood. Torre JL. Oligodendroglioma shows multiple cells with a “fried Hernández-Hernández MA 2012 Nonconvulsive status egg” appearance. Symptoms must be present for longer chronic obstructive hydrocephalus may be treated with than 1 month to be diagnosed with PTSD (shorter a ventriculoperitoneal (VP) shunt. Rods perceive light and are NiCP. D. Each time the patient constellation of significantly elevated intracranial reexperiences a trigger or cue that reminds him or her pressure with CSF lymphocytosis. and inciting event. with permission. shown in the MRI. The EEG in herpes Social media and e-mail are not preferred to encephalitis shows temporal periodic lateralized communicate with patients about their medical epileptiform discharges. Although lumbar drain activities. but may be used as long as patients are encephalopathy classically shows triphasic waves. that diagnosis. 55. Pathology. Meningitis can cause seizures. E. proper and comatose forms. The stabilizers. and requires immediate treatment. Gutiérrez A. 3rd edn. 7th edn. hyperintense lesions in the posterior columns and Diagnosis momentum involves accepting a diagnosis lateral corticospinal tracts. romantic relationship with a patient or a former 66. B.  The EEG demonstrates diffuse rhythmic. the spinal cord splits into two spinal canals patient (see Bradley’s NiCP.  Social media presents new ethical challenges in than cones and contain more pigment. and enhanced Treatment with amphotericin B and flucytosine should physiological reactivity such as startle. López-Espadas F. Psychotherapy and Neurology Books . the patient may have recurrent high protein is classic for cryptococcal meningitis. C. unless 65. Using social media to start a pp. B. low glucose. This ultimately leads to avoidance of reduce intracranial pressure. separated by a bony septum. 105. Premature closure bias spinal cord and generally enhances with gadolinium. 1147–1158). the patient is an actual or threatened traumatic event that invokes at risk for multiple opportunistic infections. or be initiated. Transverse myelitis shows hyperintense lesions in involves continuing with a diagnosis despite evidence the spinal cord. On T2 imaging this would be seen as a bias involves making a diagnosis because of a recent hyperintense lesion in the central portion of the spinal experience with a similar diagnosis. 7th edn. Clinically this can present as a myelopathy in a diagnosis. or horror. which is media. helplessness. places. The EEG in hepatic conditions. pp.  Spongiform changes are consistent with Creutzfeldt. mood due to risk of fungal seeding into the abdomen. These receptors are Fenton D 2011 Imaging Painful Spine Disorders. as well as daily lumbar punctures to hypervigilance. C. including varying degrees of face-to-face conversations. Ch. whereas cones are in the fovea. cerebrospinal fluid space would be greatly reduced and the spinal cord should appear normal. 1736–1765). Reporting unprofessional behavior by mentation. so it is unlikely that he is encephalitis is Cowdry type A inclusions. 67. generalized slowing to a burst suppression pattern. as is 1102–1120 and 79. or in patients who have suffered convulsive identifying information about patients should be status epilepticus but have yet to return to normal posted. pp. pp. Each more easily activated. which may involve part of or the entire contrary to that diagnosis. which significant compression has occurred (see Bradley’s is an achromatic pigment. Lewy bodies shows eosinophilic cytoplasmic inclusions. benzodiazepines. Anchoring bias cord. 742–758). B. 17. 94.62 Neurology Self-Assessment 60. Rods help with night or low- facility has its own policy on the use of social media light vision (see Bradley’s NiCP. 7th edn. he or she consistent with nonconvulsive status epilepticus. 7th edn. predominantly in the peripheral portion of the retina. Rods are larger cells 61. Figure from activated by very low levels of light. or people that arouse memories of the may be used in place of daily lumbar punctures.  Confirmation bias results when a diagnostic error is is separated by a fibrous septum in a single spinal made by seeking out only information that will confirm canal. B. by physicians and staff. with a white halo around the nucleus epilepticus in adults: electroclinical differences between (see Bradley’s NiCP. or antipsychotics. Rebollo M. with permission. and of the initial event. Treatment course of the infection a VP shunt should be avoided includes antidepressants. Ch. but in this patient his Jakob disease. In type 1 insufficient (in the case of acetazolamide) for this lesions. 77. which has two different types. 1365–1379). 7th edn. The fear. Clinical Neurophysiology Figure from Klatt EC 2014 Robbins and Cotran Atlas of 123(2): 244–251. Saunders. high- patient is considered an inappropriate use of social amplitude slow waves with occasional spikes.  Diastematomyelia is a congenital disorder of the other treatments mentioned are inappropriate and/or spinal cord. aware that the conversations and e-mails may not be as Medication-induced encephalopathy can have many secure as patient portals. making them communication with patients and their families. or patterns on EEG. Availability spinal cord. phone conversations. No personal patients. On T2 imaging. during the acute than 1 month is acute stress disorder). that would not be apparent on a brain MRI. This should separate professional and personal accounts to condition is more common in the ICU. so other Syringomyelia involves an enlarged central canal in the potential etiologies should be considered. If a physician uses social media. Ch.

Huntington disease. Test Two 63 desensitization are also very helpful. connective tissue Ch. choice. unnamed cause and her strong autoimmune family and lead to depression. Given her renal insufficiency of Propranolol can cause arrhythmias. NMDA receptors are reuptake inhibitors. They may also develop encephalopathy. but does not generally cause Neurology Books . result in analgesia (see Bradley’s NiCP. and cognitive behavioral therapy (see Bradley’s are excitatory. and hypothyroidism. Breastfeeding does not typically transmit the disease to and thyroid-stimulating hormone. 73. and comorbid psychiatric conditions.  The patient likely has fibromyalgia as evidenced by by eye movements that do not follow specific cranial a normal physical and neurological examination. Mu receptors are targets of opioids and NiCP. physical glutamate receptors in the central nervous system that therapy. option in pregnancy and has been found to be and peripheral pain receptor alterations. give a specific diagnosis. pp. cortex is involved in generation of speech (see Bradley’s 68. In addition she recently started magnesium. 1973–1991). aching. Ch. even in pregnancy.  The anterior cingulate gyrus is the anatomical symptoms. neurohormonal first-line treatment. C-reactive protein.  This patient presents with typical features seen in would also help guide the differential diagnosis. Ch.  The symptoms of headache and blurred vision with nephrolithiasis. Symptoms can last up to 6 weeks after birth. There is no even depression. and hepatic toxicity type of antiphospholipid antibody) are the most likely (see Bradley’s NiCP. 7th edn. sun exposure. including evidence suggests that there are alterations of central antibiotics and anesthetics. There is no mendelian pattern of inheritance tests are often done to exclude other conditions. B. and orthostasis). started during pregnancy. Muscarinic fatigue syndrome). von acute porphyria. and a history of fatigue. Patients are relatively safe. glaucoma. C. erythrocyte baby. which was significant side effects of worsening depression. pp. 7th edn. 1563–1614). diseases. Asking about prior spontaneous abortions 74. polymyalgia rheumatica. Niacin deficiency can cause a mild substrate for motivation. coagulation. The which can precipitate a myasthenic crisis. As such. 1736–1765). encephalopathy. Lesions to the anterior sensorimotor neuropathy. C. and presence of at least 11 of 18 specific areas of point a nasal voice. neither is specifically implicated by the (GBS). Porphyria is marked by autonomic Willebrand deficiency is a genetic cause of impaired dysfunction (constipation. 69. 43. pressure. planning ability. and teratogenicity. anterior thalamic situations. Alzheimer disease. Transient neonatal myasthenia can picture mentioned earlier along with normal laboratory occur by transplacental transfer of antibodies to the testing. JAK2 mutation is associated with tachycardia. although only studied in a small diagnosed with fibromyalgia when they fit the clinical number of patients. and cardiac arrhythmias. which is common in neuromuscular tenderness. creatine kinase. The left parasylvian such as in generalized anxiety disorder. there is no indication of excessive worry. including inflammatory myopathies. nor that controls executive functions like organization and they coexist with another neurodegenerative process. labile blood pressures. controls impulsivity. migraine. irritable bowel syndrome. and event are better explained by PTSD. This patient’s history is not 7th edn. E. interstitial cystitis. including hepatotoxicity. pp.  Topiramate has significant side effects of acute-angle 108. pp. causing hallucinations. worsen asthma. Prednisone is considered pain processing. Ch. This is clinically suspected 72. Ch. mammillary bodies. The dorsolateral prefrontal cortex indication that these symptoms occur in sleep. Amitriptyline has from a cerebral venous sinus thrombosis. Many other pathophysiology of fibromyalgia is unknown. pp. 1867–1895). fatigable eye weakness with ptosis. and glands as well as postganglionic parasympathetic vulvodynia. typical for botulism because of the significant cognitive 71. metabolic acidosis. the initiation. polycythemia vera and essential thrombocytosis. teratogenicity. abdominal pain. receptors are cholinergic receptors involved in sweat temporomandibular disorder. Treatment includes tricyclic activation. paralytic ileus. nuclei. neuropathies. or even seizures. the woman is most likely suffering pancreatitis. Ch. precipitated by intravascular volume depletion from anticholinergic side effects. Verapamil can cause history. pp. lupus anticoagulant antibodies (which are a arrhythmias. autonomic nervous system changes. C. Lumbar puncture for cytoalbuminological Vitamin K deficiency may be seen with prolonged dissociation would rule out GBS. The Papez circuit involves the Although the patient avoids symptom-triggering hippocampus. E. 920–967). and 70. although and δ-aminolevulinic acid are increased in most types this has no bearing on venous sinus thrombosis. nausea. including complete blood count. systemic exertion intolerance disease (formally chronic which binds to nicotinic receptors. it would not therefore increased risk of bleeding (see Bradley’s NiCP. Urine porphobilinogen correlate with classic pseudotumor cerebri. associated with myasthenia gravis (see Bradley’s NiCP. of porphyria. exercise programs. Later in the course patients can develop an acute motor Although either of these may increase the risk of neuropathy that looks like Guillain-Barré syndrome thrombosis. The lateral orbitofrontal cortex thus making REM sleep behavior disorder unlikely. These laboratory the child. Alpha 2 receptors are adrenergic receptors antidepressants. sedimentation rate. This is common in frontotemporal 2 recurring flashbacks sparked by an initial traumatic dementia. serotonin and norepinephrine not involved in the ANS. 511–527). 105. Although EMG/NCS would be helpful prolonged international normalization ratio and for determining pure motor involvement. 7th edn. clinical history given. 65. sleep abnormalities. 101.  The autonomic nervous system (ANS) uses Conditions coexisting with fibromyalgia often include acetylcholine as its preganglionic neurotransmitter. musculoskeletal disorders. and the cingulate gyrus. IVIG is an axis abnormalities. Although panic cingulate gyrus can result in a lack of motivation and disorder (and attacks) can occur with PTSD.  The patient presents with myasthenia gravis that NiCP. Valproic acid has many significant side a finding of papilledema imply increased intracranial effects. 112. but new medications can precipitate a crisis. D. 7th edn. Elevated vitamin A levels may hallucinations. which is less likely antibiotic use (depleting vitamin K–producing gut because of her encephalopathic state and bacteria) or more commonly with warfarin use.

The triad of niacin population. whose result from coup–contrecoup injuries and first appear actions resemble lysergic acid diethylamide. and magnetic resonance imaging 2039–2048. loss of consciousness. 86. A. voltage-gated sodium channels and causes perioral and confusion. abnormalities (see Bradley’s NiCP. but usually shows a consciousness. Although etiologies of hyponatremia include excessive water microhemorrhages are often seen on MRI. As the leptomeningeal space. CAA is a serum sodium concentration with elevated urine condition in which amyloid deposits in the media and osmolality and urine sodium concentration. known. 968–980 and 95. Ch. and insomnia (see Bradley’s NiCP. which becomes the midbrain. In SIADH.  Psilocybin is a structural analog of serotonin. requirement of ventilator support. deficiency is dermatitis. leading to decreased serum osmolality and with cerebral amyloid angiopathy (CAA). A. and paralysis. dysphagia. thalamus. Although structure and tendencies. At tetrabenazine. amnesia) but no radiographic acral paresthesias. Alpha-agonists such as clonidine or manifestations of obsessive-compulsive disorder guanfacine may also be used.  The case is one of attention-deficit/hyperactivity disease (and other hyperkinetic movement disorders) is disorder (ADHD). the temporal bone is fractured and usually follows the cortical convexity in the the underlying middle meningeal artery is torn. A traumatic brain injury may hematoma expands. and forgetfulness. 867–880). (hypovolemic hyponatremia). brevetoxin activates the channel) and cause subthalamic nucleus) and the telencephalon (cerebral perioral and acral paresthesias. SIADH is an although psychotherapy is important for comorbidities independent risk factor for increased mortality.64 Neurology Self-Assessment significant motor weakness. D. work by inhibiting the vesicular least some of the symptoms must occur before the age of monoamine transporter (VMAT) protein. Imaging reveals a classical convex usually is not associated with microhemorrhages (see hyperdensity. and some cases of dehydration antiplatelet agents is increased in this population. Silva V. Figure from. Axonal shearing is seen in traumatic brain injury with Journal of Stroke and Cerebrovascular Diseases 24(9): diffuse axonal injury. 80. the prosencephalon brevetoxin. losing objects needed of Huntington disease. cerebellum) and the Bradley’s NiCP. most notably saxitoxin and the rhombencephalon. Shellfish are associated with rise to the prosencephalon. 62. There is nothing in the history (SIADH) secretion has been reported in Guillain-Barré that suggests a learning disability or cognitive decline for syndrome (GBS). pp. Finally. it is 7th edn. The puffer hours. or cerebellum. 1422–1460). Silva C. due to a slightly higher risk of bleeding. and various neurotoxins. functional often at increased risk of lobar hemorrhages. anxiety. inflammation: report of 3 cases and systematic review. 96. From there. microhemorrhages. caudate. Both SSRIs and choice is a psychostimulant such as amphetamine or benzodiazepines are aimed at treating the nonmotor methylphenidate. this would age >50. Ch. not be the first treatment of choice. 7th edn. Home-distilled ethanol pp. 107. patients are intake (psychogenic polydipsia). substantia nigra. Among patients with GBS. C. B. and leads to Albuquerque L. Other more months of inattention. which breaks down to formic acid and causes permanent optic nerve 76. channels in different ways (saxitoxin inhibits the hypothalamus. Chs. Concussion refers to traumatic brain injury with fish has been associated with tetrodotoxin. 7th edn. depression). structures of the brain such as the pons. pp. important to recognize medical and psychological causes 81. globus pallidus. This is characterized by at least 6 or typically aimed at reducing dopaminergic action. diarrhea. Ferro JM 2015 development of subdural hematoma. the correct treatment of action at the D2 receptor. as are that can occur with ADHD (i. though the pathophysiology is not which a change in the curriculum is needed. which affect the voltage-gated sodium divides into the diencephalon (thalamus. difficulty listening. hyponatremia. putamen).  The initial segmentation of the neural tube gives damage and blindness. discipline can help organize behavior in children. A subarachnoid hemorrhage Upon direct impact. D. and dementia (see Chronic hypertension usually leads to bleeding in deep Bradley’s NiCP. Imaging shows a Cerebral amyloid angiopathy associated with concave hyperdensity that may cross skull suture lines. progressive ascending weakness. An AVM can be identified on 75. such as to complete tasks. may show microhemorrhages along white matter tracts and in subcortical gray matter. which blocks associated symptoms (e. The mesencephalon Peyote is a small cactus that contains mescaline. myelencephalon (medulla). and hypoventilation. excess 78. It is found as multiple microhemorrhages that convalesce and in various species of mushroom. cortex. pp.g.e. Cerebral contusions 79. Typically. It is notable that patients Caution must also be taken when using statins in this with SIADH generally appear euvolemic. 7th edn. avoidance of tasks. 7th edn. Antipsychotics block dopamine’s subtypes.  After head injury. Caldas AC. then brief lucidity. Bradley’s NiCP. 66. basal ganglia. as opposed to Neurology Books .  The findings shown on this gradient echo sequence antidiuretic hormone (ADH) prevents water loss in the on magnetic resonance imaging (MRI) are consistent kidneys. with permission. Dopamine depleters. 1237–1253). although accounts for “blossom” into a larger hematoma within the first 24 only one of several mushroom neurotoxins. may contain methanol as a byproduct. the mesencephalon. then loss of conglomeration of arteries and veins rather than small consciousness again is classic for epidural hematoma. channel. pp. Pimentel J. 1380–1421). the level of consciousness result in contusion from a coup/contrecoup injury but decreases. In this particular case. thereby 7. Other adventitia of small and medium vessels.  The treatment of the motor features of Huntington 77. dizziness. ataxia. Ch. the pattern of initial loss of this sequence on MRI. 1791–1867). acceleration–deceleration injury.  Syndrome of inappropriate antidiuretic hormone of behavioral dysfunction. Ch. Bridging vein tearing tends to occur after rotational or pp. treatments are aimed at treating the nonmotor features disorganization. and bulbar weakness. which does not cross skull suture lines. there are hyperactive and inattentive decreasing dopamine. D. The rhombencephalon divides has hallucinogenic action similar to psilocybin (see into the metencephalon (pons. The risk hypovolemia (congestive heart failure [CHF] and of cerebral hemorrhage with either anticoagulation or cirrhosis). distractibility.

have higher contractile strength. Schizencephaly is Neurology Books . Sensorineural hearing loss thrombosis. poorly organized cerebral cortex. Figure from Daroff RB. 7th between the ependyma and the subarachnoid space. Type II fibers (chronic exposure to loud noises). Palatal myoclonus consists of rhythmic for the remainder of the listed symptoms would not be myoclonic activity of the soft palate with associated present in the neck (see Bradley’s NiCP. which is therapy would be important if subarachnoid related to a pressure gradient between the cranial and hemorrhage were identified on CT or lumbar puncture cervical vascular structures.  This demonstrates obstructive sleep apnea. This may be related to cerumen. tympanic risk of symptomatic vasoconstriction. which is a far-field potential. This may relate to Whipple disease. given the ear. pseudohyponatremia. B. restriction. Associated causes vary 7th edn. 7th edn. P14. fluid in the middle would be helpful to identify cerebral venous ear. 7th edn. Ch. Ch. tonal type of tinnitus. 7th edn. 1720–1735). The N13 peak represents a to recognize faces. This may be 86. Test Two 65 cerebral salt wasting. C. and the cyst is lined by white matter. pp. 696–712 and 55. Along the path various portions due to visual inattention (not visual field waveforms can be recorded. or (see Bradley’s NiCP. hearing loss. and involves a thoracic effort and negative abdominal effort. have neurological dysfunction (as in cerebellopontine angle lower numbers of mitochondria. and have a mix of masses). to the somatosensory cortex. tumors. Porencephaly is a cyst or implies inspiration is caused by thoracic muscles rather cleft in the brain in which there is a connection than diaphragmatic excursion (see Bradley’s NiCP. pp. 122–127). (indicated by the flat line). but near the end a disorder of neuronal migration that involves a effort begins without any airflow. or ossicle dysfunction. but would not yet be necessary in Conductive hearing loss occurs when vibrations are not this case. Ch. or age-related. 742-757). pp. gluten sensitivity. 348–366). 52. which central apnea occurs when there is no airflow and no results from failure of skull closure and allows cranial thoracic or abdominal effort. 7th edn. breathing occurs when airflow is positive with positive Pachygyria can be seen in lissencephaly. 1686–1719).  Carotid body injury may affect vagal output and pathophysiology of persistent tinnitus is not well lead to heart rate irregularities. widely. Amusia is the loss of musical abilities. but this patient has not received Although cranial CT is very sensitive for subarachnoid any treatment yet (see Bradley’s NiCP. Patients with breathing involves bursts of progressively faster and lissencephaly demonstrate severe developmental delay deeper breaths with a gradual decrease and stop of and seizures. see evaluate the sensory system from the lumbar roots Bradley’s NiCP. 1615–1685). Pomeroy SL 2016 Bradley’s hypovolemic. cerebral processing of auditory input. C. of delayed vasospasm. Cheyne-Stokes smooth. In the cortical response and is usually best recorded over the oronasal channel. clicking sound. A mixed apnea starts contents to herniate outside the skull. and a number that corresponds to objects by sight. utilize mostly aerobic including cytomegalovirus or rubella). Lissencephaly is initially with no flow and no effort. pp. Treatment for which is subarachnoid hemorrhage. The nerves responsible understood. head injury. 103. 7th edn. Ergotamine therapy should be avoided in appropriately transmitted through the outer or middle suspected cases of subarachnoid hemorrhage. and have long endurance. are used in quick (aminoglycosides. D.  Pulsatile tinnitus is a common complaint of patients not confirmed by CT. and ARX. DCX. dysfunction of the vestibulocochlear nerve. Mazziotta JC. and others. Persistent tinnitus is a more aerobic and anaerobic respiration (see Bradley’s NiCP. and is associated with large urinary output due 85. Angiography and nimodipine with idiopathic intracranial hypertension. Erb’s point is represented by the N9 peak. constant. Ch. whereas treatment for SIADH is fluid cerebral venous thrombosis. Visual agnosia is the inability to recognize (P) or negative (N). causing a bruit. 103. recognize voices.  Simultagnosia is the inability to visually perceive stimulated with repeated electric shocks. Chs. Paradoxical certain genes. Meniere disease. toxic involve fewer mitochondria. Phonagnosia is the inability to near-field potential recorded over the cervical spine. Patients to identify the source of the bleed and to reduce the risk generally report a whooshing sound without tone. thorax and abdominal channels have continued effort. D. edn. salicylates). pp. and intracranial hypotension. cervical artery dissection. which are either positive loss). including ototoxic medications. 1915–1956). with 2 in brain injuries or central nervous system (CNS) permission. a lumbar puncture should always be pp. 88. 1686–1719). which move multiple objects at one time. pp. traumatic respiration.  The MRI is an example of an encephalocele.  Somatosensory evoked potentials are used to Mollaret triangle (dentato-rubro-olivary network. D. Other less cerebral salt wasting is replacement of fluid and frequently reported causes include pituitary apoplexy. including LIS1. where patients appear Jankovic J. Elsevier. The 87. loop diuretics. 12. It may also result from mutations in respiration. Venography membrane dysfunction or rupture. Ch. 104.  Type I muscle fibers are slow-twitch fibers with congenital (as in the case of perinatal infections lower contractile strength. the most threatening of high urinary excretion of sodium. A. 34. pp. This cortex with relatively few gyri. pp. 7th edn. The excess fluid given with IVIG can cause hypertensive crisis. although this is lower on the differential results from dysfunction of inner ear structures such as and on the acuity scale than subarachnoid hemorrhage hair cells. C. The including both production and appreciation of music brainstem and medial meniscus are involved in the (see Bradley’s NiCP. flow of air stops four different times contralateral hemisphere (see Bradley’s NiCP. and therefore several underlying pathologies. 102. or structural disruption of the Guillain. Ch. Cerebral salt wasting is most often seen Neurology in Clinical Practice. Peripheral nerves are 83. performed if subarachnoid hemorrhage is suspected but 82.  Thunderclap headaches may result from one of to inability to retain sodium in the body. 7th edn. 110. focal movements. This is a type of central apnea. hemorrhage. sodium. A 89. The N20 is the 84. During this same time the Ch. Patients with this up the dorsal columns of the spinal cord to the condition may see parts of a picture but miss large somatosensory cortex. whereas prosopagnosia is the inability the typical latency expected.

7th edn. Ignoring the possible signs of abuse could Newborn Group B streptococci. SL 2016 Bradley’s Neurology in Clinical Practice. E. 39. Figure from Winn H 2011 is cerebral aqueductal stenosis. Mazziotta JC.  Older adults. influenzae is not the best situation because the patient is not in Adolescent through adult S. Ch. Parinaud syndrome can occur. should be considered in patients who have 92. L. A chart with common unexplained weight loss and unexplained injuries. Elsevier. Neurology Books . the eyelids has a pattern of generalized 3-Hz spike and wave. seizure. influenzae. with hydrocephalus in children. ventricular system is enlarged. but in most states. repeating. 1279–1300). C. pp. The patient’s areas of speech. accumulation (see Bradley’s NiCP. and following commands (see Bradley’s NiCP. a common cause of Youmans Neurological Surgery. The EEG hallmark is continuous spike examination reveals restricted vertical gaze. Low P. naming. meningitidis immediate danger of death or serious injury. it is almost never indicated for long-term management of hydrocephalus. 52. 7th edn. are at great risk of being abused. 696–712). or vascular of poor hearing) and can progress to involve other causes such as venous sinus thrombosis. situation for the patient. monocytogenes medically helpful. short term. Endocrine function (as with severe atherosclerosis or dissection). coli. 4. particularly those suffering from writing. 1563–1614). aureus and recommending dietary supplements may be Immunosuppressed L. suspected abuse must be reported to local authorities. also cause great animosity between the patient’s family E. C. This differs extends from the ependyma to the pia mater and is from communicating hydrocephalus. Mazziotta JC.  The image demonstrates infarcts in bilateral 93. Circadian rhythm is case of sepsis) or to reduced arterial luminal diameter regulated. Ch.  Landau-Kleffner syndrome presents between the posterior fossa abnormalities such as Dandy-Walker ages of 2 and 8 years old. pp. not generalized discharges unilateral or bilateral papilledema.  Substance P is closely related to neurokinin A and watershed territories between the anterior cerebral is an important messenger in pain perception. Saunders. Autosomal. Of note. Elsevier. paraneoplastic syndrome. 411–458). pp. The treatment of edn. reading. which 7th edn. pneumoniae and neglect. 7th edn. 7th edn. or differentiate different sounds or words not because infectious causes such as toxoplasmosis. Taking Toddler S.92 the issue. influenzae. arterial density is least in watershed locations. D. Because his parents 91. benign familial tests remote memory. Pomeroy SL 2016 Bradley’s placement of a ventriculoperitoneal shunt.  Neisseria meningitides. Childhood absence epilepsy intracranial pressure (ICP). bacterial causes of meningitis based on age range or Local regulations may differ. Jankovic J. 7th nerve VI palsies. would not resolve TABLE FOR ANSWER 2. Sometimes. Figure from Daroff RB. hydrocephalus in infants and children can include 90. C. Although adding donepezil S. manifested by Juvenile myoclonic epilepsy demonstrates a pattern of light-near dissociation. H. Figure priming wherein subjects are shown a list of words from Daroff RB. 7th edn. or macrocephaly. pneumoniae. comorbidities is shown here. Language is tested through many means. including assessment of fluency. Other findings can include frontal spikes during sleep. hydrocephalus.66 Neurology Self-Assessment an abnormality that involves a cleft in the brain that contrasting with enlarged lateral ventricles. 95. in part. S. E. E. these ignore the concern for abuse Sinusitis or otitis media H.  Immediate memory (sometimes known as working do not have macrocephaly and because of his memory) is tested by digit span. 94. pneumoniae. pp. Ch. mass lesions such with verbal auditory agnosia (an inability to recognize as pilocytic astrocytoma or medulloblastoma. by melatonin. on somatostatin as a peptide no indications in the image of viral encephalitis. N. with permission. coli result in more serious injuries to the patient. pneumoniae. Voluntary muscle contraction and sweat production perfusion pressure may relate to hypotension (as in the both rely on acetylcholine. identifiable by a small fourth ventricle 2nd edn. or neuritic plaque pp. monocytogenes and the physician. where the entire lined with gray matter (see Bradley’s NiCP. Ch. dementia. Figure from Yachnis AT 2012 Neuropathology. with permission. If the pressure continues to with frequent slow spike and wave abnormalities. neurotransmitter (see Bradley’s NiCP. Ch. 96. The etiology in this case 89. It most commonly presents syndrome or Chiari malformation. immediate action by calling the police to arrest the son H. Elsevier. Asking historical facts abnormal imaging of the brain. increase. 7th edn. Implicit memory is tested in macrocephaly is not the correct diagnosis (see many ways. often Lennox-Gastaut demonstrates an abnormal background called the setting-sun sign. N. There are relies. as well arteries and the middle cerebral arteries. Calculation is tested through mathematical problems. pp. related to and slow waves seen during slow wave sleep with a midbrain tectal dysfunction in the setting of elevated normal EEG when awake. 23–33).  The image depicts noncommunicating 920–967). Confronting the son about possible neglect will erode the patient–physician relationship. noncommunicating hydrocephalus usually includes Jankovic J. retract and there can be a forced downgaze. including through studies of memory Bradley’s NiCP. H. low aprepitant is a novel antiemetic that inhibits substance perfusion pressure may lead to localized infarcts. This will Elderly S. A. pp. Other causes of permission. meningitidis. and eyelid retraction (eyelid retraction is dominant nocturnal frontal lobe epilepsy demonstrates also known as Collier sign). nystagmus. Pomeroy and then asked later if they have seen specific words. and could result in a disastrous Neurosurgical patients S. 101. bilateral cranial as is seen during this segment (see Bradley’s NiCP. pneumoniae. 40–74. 65. Ch. with acetazolamide may be used for increased ICP in the permission. convergence-retraction polyspike and wave at a rate of 4–6 Hz. in part. Given that as in the vomiting center of the medulla. influenzae. and could even result in more injuries to the Age or comorbidity Bacteria patient. Although Neurology in Clinical Practice.

and angiomatosis of the eye. Oftentimes patients pheochromocytoma. and corticosteroids. but later in the Patients with this syndrome are predisposed to intoxication it can lead to severe aggression. Individuals with neurofibromatosis 1 have echocardiograms or multigated acquisition scans is multiple associated signs and symptoms. 109. Treatment is supportive. Ch. C. but not for IIH (see Bradley’s lamotrigine and valproic acid together because NiCP. and may be an indication for more consistent with IIH. 80. Further. Ch. Lumbar puncture should not a contraindication for its use. are seen with interferons. dysphoria. Cocaine intoxication the NF2 tumor suppressor gene. fever. and may occur in up to 4% of patients. Warfarin would be an appropriate aminoglycosides). Glatiramer would be an dalfampridine use to improve motor function (see appropriate immunomodulatory therapy for multiple Bradley’s NiCP. pp. Ch. cutaneous mitoxantrone due to cumulative risk. 87. 7th edn.  Phencyclidine (PCP) is a relatively uncommon drug autosomal-dominant disorder associated with loss of of abuse. and melanoma. renal cell carcinoma. optic gliomas. however. nearly rarely strokes or seizures. bizarre hemangioblastomas in the brain and spine. macrolides. 100. so history of tuberculosis is not a with a demyelinating lesion of the pons. Therefore regular monitoring with gene. complicated migraine is hypertension (IIH). Test Two 67 97. refractory flares that do not improve with antibiotics (particularly quinolones. including recommended. 1538– If a patient has a seizure while on dalfampridine. Alcohol intoxication usually results in a ependymomas. risk of other types of tumors. malignant peripheral nerve sheath with dalfampridine. Liver impairment does demonstrate increased opening pressure (>25 cm). but is not including schwannomas (often bilateral vestibular associated with aggression. resulting in Neurology Books . Although retinoblastoma is the most euphoria. Allergic reactions. hallucination. renal and behaviors. and commonly associated tumor with Rb1 mutation. myoglobinuria. however. as does the absence of fluctuating other medications by slowing their metabolism. 1254–1260). Ch. endometrial pp. although 102. 7th edn. Ch. the 1562). meningiomas. sclerosis after resolution of an acute flare. which is used to improve walking tumors. Other acute symptoms. B. as well as a lifetime maximum dose of café-au-lait patches. Myasthenia gravis often causes diplopia. history of seizure is a contraindication to starting dalfampridine. pp. and coma (see germline Rb1 mutation. paranoia. hallucinations. pancreatic cysts. hamartomas. 1895–1914). This symptoms. suppressor gene PTEN. 7th edn. or nerve schwannomas). there is an increased Bradley’s NiCP. catatonia. When a dysplastic gangliocytoma is 101. or hallucinations. and gabapentin (see Bradley’s NiCP. pp. Bradley’s NiCP.  Valproic acid is a strong hepatic enzyme inhibitor. 7th edn. skinfold freckling. 98. Initially it can lead to euphoria. 7th edn. hypertension.  Among other adverse reactions. and nystagmus. Plasma exchange may also be used to medications to be used with caution include any treat multiple sclerosis. and dimethyl most commonly skin and mucosal surface fumarate (see Bradley’s NiCP. that is associated with a loss of function mutation in and somnolence. somnolence. in patients with multiple sclerosis. 103. Neurofibromatosis 1 is an gastrointestinal symptoms and immunosuppression. including abductor weakness. lidocaine. Abducens palsy is commonly associated with increased although renal impairment may increase the likelihood intracranial pressure from this or other entities (such of seizure and should be considered before starting as trauma). predisposed to several types of benign tumors. hypertension. A. 1686–1719).  All of the listed medications can precipitate a generally corticosteroids would be used to treat the myasthenic crisis except for lisinopril. this patient’s contraindication. pp. intoxicated with PCP will be quite difficult to restrain Neurofibromatosis 2 is an autosomal-dominant and seemingly feel no pain even with injurious disorder associated with loss of function mutations in behavior. Affected patients are can cause euphoria. Pyridostigmine would be an appropriate interaction is particularly important when prescribing treatment for myasthenia.  Von Hippel-Lindau (VHL) syndrome is an 99. myoglobinuria. 1159–1186). autosomal-dominant cancer predisposition syndrome mitoxantrone may cause an irreversible associated with mutation in the NF1 tumor suppressor cardiomyopathy. not preclude a patient from receiving dalfampridine. Marijuana intoxication can result in the Rb1 gene. and seizures. most for severe. Acetazolamide is first-line therapy for IIH. hypotension. 2 function mutations in the VHL tumor suppressor gene. Seizure is a risk neurofibromas. no such 7th edn. therapy. nystagmus. A. Transverse myelitis is a symptom of overall picture and lack of demyelination on MRI is multiple sclerosis. and dysplastic gangliocytoma (cerebellar hamartoma). the findings of papilledema argue against which means it can greatly increase the availability of myasthenia. associated with natalizumab. 103. 1159–1186). the term Lhermitte-Duclos is used (see dalfampridine. cimetidine. Opioid intoxication results in half of the cases are sporadic and the mutation is euphoria or dysphoria. paranoia. lamotrigine levels can be greatly increased. including sarcoma. A. breast cancer. However. Retinoblastoma is a cancer of the retina sense of euphoria or dysphoria. fingolimod. 80. tachycardia. A. pp. including pinealoma. strokes.  The patient’s symptoms and examination findings Although patients may have headache as a general side are most consistent with idiopathic intracranial effect to dalfampridine therapy. dysphoria. fevers. drug should be discontinued. cancer. cerebral venous sinus thrombosis. evidence was found on venography. hallucinations. mostly associated with loss of function of the tumor injection site specific. phenytoin. and nystagmus. Patients with this syndrome Progressive multifocal leukoencephalopathy has been have an increased risk of multiple types of tumors. respiratory depression. thyroid cancer. Headache is a risk Cowden syndrome is an autosomal-dominant disorder of beta-interferon therapy. although is generally reserved neuromuscular junction–blocking anesthetics. Ch.  Seizure is a dose-dependent side effect of present. C. and developmental delay. In patients who inherit a hypothermia. localized to the tumor. treatment if the cause of intracranial hypertension was carbamazepine. There is no immune suppression that results Although multiple sclerosis may cause abducens palsy from dalfampridine. 100.

remains closed until the membrane potential returns to Anomic aphasia involves fluent speech with word- −60 mV. structures. returning it to its original state. D.  Conduction aphasia comes from lesions of the and repetition. so they may increase the metabolism paraphasic errors) and preserved speech of hepatically cleared medications. C. 7th edn. reaches +60 mV. Transcortical aphasia has several patterns of speech impairment. Due to the surrounding anatomical zonisamide do not affect hepatic enzyme function. Ch. Phenytoin and carbamazepine are hepatic preserved speech production (aside from mild enzyme inducers. sodium channels cluster in the nodes of Ranvier to but can be caused by lesions to the left angular gyrus. but repetition is intact.  The voltage-gated sodium channel has an activation share in common preserved repetition. with relatively syndrome. the activation gate is closed and the impaired comprehension. Broca aphasia comes from lesions in the metabolized and affected by both inducers and posterior part of the inferior frontal gyrus and results inhibitors (see Bradley’s NiCP. 1563–1614). or right-sided being metabolized. This hyperpolarization closes the activation finding pauses. and intact repetition. the inactivation gate closes and impaired comprehension. 128–144). circumlocution. pp. and comprehension is intact. With slight depolarization. 13. or even mutism at an extreme. intact comprehension. Ch. NiCP. right sensory loss.68 Neurology Self-Assessment a serious risk of rash. in nonfluent speech. pp. Zonisamide is hepatically hemianopsia. The voltage-gated This can be seen in disorders like Alzheimer disease. Neurology Books . and further depolarization. including Stevens-Johnson and is marked by inability to repeat. 101. At the resting membrane transcortical aphasia results in nonfluent speech with potential. all of which 104. inactivation gate is open. symptoms that often accompany conduction Levetiracetam is not affected by inducers or inhibitors aphasia include apraxia of the left side. allowing an influx of sodium speech. Mixed gate and an inactivation gate. right because it is primarily excreted in the urine without hemiparesis. Transcortical motor aphasia results in nonfluent the activation gate opens. facilitate saltatory conduction in myelinated fibers. Wernicke aphasia involves impaired comprehension 105. As the membrane potential Transcortical sensory aphasia involves fluent speech. 7th edn. Levetiracetam and comprehension. but intact fluent speech (see Bradley’s superior temporal lobe or the inferior parietal region. gate. and intact repetition. and impaired naming.

Test Three

QUESTIONS A. Anticoagulation
B. Corticosteroids
1. A 6-year-old obese girl is evaluated for intellectual
disability and marked hyperphagia. She requires special C. Antibiotics
education for all classes at school, and her parents have D. Observation
recently put padlocks on the refrigerator and cabinets E. Hemicraniectomy
to keep the patient from unsupervised eating. Which of
the following is true of this condition? 3. Which of the following is not a hereditary disorder of
A. Paternally inherited mutation on 15q myelin metabolism?
B. Maternally inherited mutation on 15q A. Alexander disease
C. Sporadic development of XXY B. Adrenoleukodystrophy
D. Sporadic development of trisomy 21 C. Marchiafava-Bignami syndrome
E. Sporadic development of trisomy 13 D. Canavan disease
E. Metachromatic leukodystrophy
2. A 6-year-old boy suffered Mycoplasma pneumonia 2
weeks ago, from which he fully recovered. He now 4. Which of the following is not an important component
presents to the emergency room with headache, of the blood–brain barrier (BBB)?
confusion, and generalized weakness. A computed A. Astrocytes
tomography (CT) scan is shown here. Based on the
B. Pericytes
most likely diagnosis, which of the following is the
best course of therapy? C. Tight junctions
D. Oligodendroglia
E. Basement membrane
5. A 53-year-old man with a history of depression,
hypertension, and asthma presents to clinic for tremor
that began gradually. His tremor will often worsen with
stress or anxiety, and will improve slightly with a glass
of wine. His wife also notes an occasional head tremor,
mostly at the end of the day. On examination, there is
an action tremor bilaterally, occurring mostly in the
distal extremities. Further, there is slight imbalance
with tandem gait and mild hearing loss. There is no
evidence of a resting component to his tremor, nor loss
of dexterity or rigidity with activating maneuvers.
Which of the following is considered the best first-line
agent to treat this patient?
A. Carbidopa/levodopa
B. Clonazepam
C. Propranolol
D. Primidone
E. Topiramate

Figure for Question 3.2. 

Neurology Books 69

70 Neurology Self-Assessment

6. A 33-year-old female with a history of prior deep C. Dextrocardia
venous thrombosis (DVT), ischemic stroke, and D. Late-onset ataxia
antiphospholipid antibody syndrome presents to the
office for an evaluation. She has concerns about her E. Mental retardation
medical history because she would like to become 11. A 53-year-old male with no significant medical history
pregnant. Which of the following medications would presents for evaluation of confusion. In the past
be the safest for stroke prevention? month, he developed intermittent jerking movements
A. Warfarin in his right arm and leg occurring multiple times per
B. Dabigatran day. Additionally, his wife states that he recently lost
his keys, couldn’t find his car in a parking lot, and even
C. Low molecular weight heparin forgot close friends’ names. In the emergency
D. Aspirin department he is afebrile with an elevated white blood
E. Rivaroxaban cell count. His examination is nonfocal with periodic
jerking movements in the right arm. He is noted to
7. The patient whose autopsy photo is shown here most have hyponatremia, but otherwise a metabolic panel
likely died of what? and hepatic enzymes are normal. Lumbar puncture
A. Epidural hemorrhage shows no white blood cells, no red blood cells, protein
B. Subarachnoid hemorrhage of 95 mg/dL (normal is <45 mg/dL), and glucose of
63 mg/dL (normal is 45–80 mg/dL). Herpes simplex
C. Subdural hemorrhage virus polymerase chain reaction is negative. His
D. Bacterial meningitis magnetic resonance image (MRI) is shown here. Which
E. Intraparenchymal hemorrhage of the following is the most likely diagnosis?
A. Cerebral autosomal-dominant arteriopathy with
subcortical infarcts and leukoencephalopathy
B. Corticobasal degeneration (CBD)
C. Limbic encephalitis
D. Early-onset Alzheimer’s disease
E. Streptococcal meningitis

Figure for Question 3.7. 

8. Which of the following is not a potential source of
A. Salicylates
B. Gentamycin
C. Cisplatin
D. Levetiracetam
E. Vancomycin
9. Which of the following is the most common treatable
cause of neuropathy in the world?
A. Hypothyroidism
B. Diabetes
C. Paraproteinemia
D. Lead toxicity
E. Leprosy
Figure for Question 3.11. 
10. Which of the following is not commonly seen in fragile
X syndrome?
A. Large ears
B. Large testes

Neurology Books

Test Three 71

12. In reference to the patient in Question 3.11, which of A. Anterior spinal infarct
the following abnormal findings would be expected in B. Hypotension 3
this patient?
C. Displacement of stimulating electrodes
A. Anti-voltage-gated calcium channel antibodies
D. Posterior spinal infarct
B. NOTCH3 gene mutation
E. Bolus of rocuronium
C. Neurofibrillary tangles
D. Anti-voltage-gated potassium channel antibodies 16. A 7-year-old boy presents for evaluation of side-to-side
head movements and frequent throat clearing in the
E. Cowdry A inclusions last 3 months. Each movement is preceded by the urge
13. Myelin increases the speed of an action potential by to move and a sense of relief after. His parents have
doing which of the following? noted frequent hand washing and the need to flip the
light switch multiple times before going to sleep. All of
A. Reducing membrane capacitance and increasing the following could be used to treat this patient except?
membrane resistance
A. Pimozide
B. Reducing membrane capacitance and reducing
membrane resistance B. Clonidine
C. Increasing membrane capacitance and increasing C. Tetrabenazine
membrane resistance D. Fluoxetine
D. Increasing membrane capacitance and reducing E. Methylphenidate
membrane resistance
17. A 6-year-old boy who had hypotonia at birth is
E. No effect on membrane capacitance and reduces brought to the neurologist for developmental delay.
membrane resistance His mother notes that his eyes move abnormally, his
14. A 21-year-old female presented to the emergency head bobs, and he has weakness and incoordination in
department after a new-onset seizure. Before this, she his legs. He has never walked like other children, and
was complaining of a severe headache with blurred is not performing well in school. There are two male
vision for the last 2 days. Physical examination cousins with similar symptoms, but no affected female
demonstrates a somnolent female with normal vitial family members. On neurological examination, he has
signs and bilateral papilledema. She has mild weakness nystagmus, truncal titubation, and spasticity in the
of her right face, arm, and leg. Computed tomographic bilateral lower extremities. His magnetic resonance
(CT) imaging of the head without contrast and CT image is shown. What is the most likely genetic
angiography of the brain with contrast are shown. abnormality in this patient?
Which of the following is the most appropriate A. GFAP mutation
treatment? B. Deletion of PMP22
A. Intravenous (IV) antihypertensive C. HTT mutation
B. IV unfractionated heparin D. PLP1 mutation
C. Oral aspirin E. Mutation in FXN gene
D. Hematoma evacuation
E. IV corticosteroids

Figure for Question 3.14. 

15. Transcranial motor evoked potentials (tcMEPs) and
somatosensory evoked potentials (SSEPs) are
monitored during a cervical spinal surgery. Halfway
through the surgery, the tcMEP responses disappear,
but the SSEPs are still present. All of the following can
cause this pattern except? Figure for Question 3.17. 

Neurology Books

72 Neurology Self-Assessment

18. After a stroke, a 67-year-old woman is left with 21. A 36-year-old female presents to the emergency
Dejerine-Roussy syndrome. Which of the following is department with several falls and a tightness around
the most likely location of her initial infarct? her upper abdomen over the last 3 days. Her
A. Ventral posterior lateral nucleus neurological examination demonstrates intact cranial
nerves. She has normal strength, sensation, and reflexes
B. Pulvinar in her upper extremities. In her lower extremities she
C. Lateral geniculate nucleus has weakness of hip flexors, knee flexors, and ankle
D. Ventral posterior medial nucleus dorsiflexors. Numbness is noted to all modalities
E. Anterior nuclear group below her upper abdomen. Magnetic resonance
imaging (MRI) is performed, from which a T2 sagittal
19. A 32-year-old, previously healthy woman presents with image and a T1 sagittal image with contrast are shown
left leg weakness and urinary incontinence that here. Which of the following is the most appropriate
developed over the course of 1 day. She has never had treatment?
this or other neurological symptoms previously. Her A. Intravenous immunoglobulin (IVIG) at 0.4 g/kg for
thoracic spine magnetic resonance image (MRI) reveals 5 days
an enhancing lesion at T4, and her brain MRI is
shown. Based on this information, which of the B. Intravenous (IV) methylprednisolone at 1000 mg
following best describes her diagnosis? daily for 5 days
A. Multiple sclerosis C. Five treatments of plasmapheresis
B. Neuromyelitis optica D. Oral prednisone at 60 mg followed by a taper over
2 weeks
C. Clinically isolated syndrome
E. Anticoagulation with a goal international
D. Radiographically isolated syndrome normalized ratio of 2–3
E. Cerebral autosomal-dominant arteriopathy with
subcortical infarcts and leukoencephalopathy


Figure for Question 3.21. 

22. A 59-year-old male with a long history of human
immunodeficiency virus (HIV) and medical
noncompliance presents with a 3-month history of
progressive painless gait disturbance and incontinence.
His examination demonstrates diffuse weakness in his
legs, spasticity, brisk reflexes, and bilateral Babinski
responses. No sensory level is present. Magnetic
resonance images of his brain and spinal cord are
Figure for Question 3.19.  unremarkable. Which of the following is the most
likely diagnosis?
A. Vacuolar myelopathy (VM)
20. For the woman in Question 3.19, after acute treatment B. Cytomegalovirus (CMV) myelitis
for her illness, which of the following is the best choice C. Varicella zoster virus (VZV) myelitis
of therapy thereafter?
D. Hereditary spastic paraparesis
A. Observation
E. Cryptococcal meningitis
B. Mitoxantrone
C. Monthly steroids
D. Interferon beta-1a
E. Rituximab

Neurology Books

she had a single seizure associated she is demented and has chorea. Disseminated infection D. His symptoms were slowly he has not been himself and that his appearance has progressive over a couple years. E. Given the most likely which are improved with nonsteroidal diagnosis from the pathological photo shown here. Next week you are electroencephalography (EEG) at the time were giving a lecture to medical students and feel this unremarkable. Myelin proteolipid protein (PLP) (GnRH) C. Cognitive behavioral therapy D. demonstrating two episodes of sleep-onset rapid eye movement (REM) and an average sleep-onset latency of 5 minutes. oily skin. remember. Continuing anticonvulsant drugs until attacks have risk of experiencing? stopped A. All of the following conditions could cause this except? A. Under no circumstances film the patient. though central and medial incontinence. when she seemed to have regression. D. Panayiotopoulos syndrome D. antiinflammatory drugs. Recent heavy alcohol use Figure for Question 3. which of the following is true of the B. Narcolepsy D. Ask the nursing facility’s medical assistant for C. Lennox-Gastaut syndrome E. Obtaining multiple neurological opinions nose. He additionally suffered from dizziness vision has been normal bilaterally. A man was seemingly healthy until the age of 25 years. Ocular complication C. he has a which of the following blood tests would have been bitemporal hemianopsia. Which of the following is the most likely diagnosis? B.27. Magnetic resonance imaging and Huntington’s disease as the cause. Normal magnetic resonance imaging (MRI) of the brain E. hypogonadism. A 72-year-old female presents to the clinic alone for months ago. you suspect with a fever. His wife states that and syncope upon standing. A 15-month-old girl is brought to the emergency E. Urinary N-acetylaspartate (NAA) level A. as well as coarse facial abnormal if analyzed premortem? features. He notes an A. and right temporal visual fields.  E. He also complains of mild headaches. Somnambulism 25. Which of the has noted quick jerking movements of both arms over following is the most appropriate response? the last several weeks. Behaviorally induced sleep deprivation B. Film the patient and ask her family for consent before use. vision changes. followed by the eruption of a vesicular lesion on the side of his A. A 60-year-old man notes increasingly severe pain on nonepileptic attacks? the left side of his midface for about 3 days. An autopsy was performed. Which of the following may he be at increased B. lower 3 progressive worsening of vision out of the left and extremity neuropathy. Test Three 73 23. Infectious meningitis 26. A multiple sleep latency test (MSLT) was performed the next morning. Neurological examination A. evaluation of abnormal movements. Sulfatide level B. Which of the following has been shown to benefit 28. even changed. Shift-work sleep disorder C. Benign familial neonatal epilepsy C. he has had at which point he slowly developed paraparesis. B. A 54-year-old man is seen in the emergency room for 27. Antidepressant medications B. Trigeminal neuralgia room by her mother because of concern for seizures. Normal x-ray of mandible 24. Low cortisol D. The mother you would like to film the patient. Ohtahara syndrome movements. For the last month. A 26-year-old male presented to the sleep laboratory for daytime hypersomnolence. the patient developed recurrent patient would be an excellent example of chorea and clonic episodes in the absence of a fever. Electroconvulsive therapy C. Very-long-chain fatty acids increase in his glove and shoe size recently. and a square jaw. Dravet syndrome permission to film her. Cavernous sinus invasion E. Today. Given the likely diagnosis. Decreased gonadotropin-releasing hormone E. The patient had been reaching milestones until 3 29. On examination. His overnight polysomnogram was normal. Film the patient without asking because she won’t revealed myoclonic jerks but was otherwise nonfocal. and he ultimately died. Elevated insulinlike growth factor 1 (IGF-1) D. On examination At 6 months old. Neurology Books . Ask the patient for permission to film her A. Glial fibrillary acid protein (GFAP) genetic testing condition? C.

he requires an symptoms. High-resolution computed tomography (CT) of the in a short period of time temporal bones D. his wedding of his left foot. First dorsal interosseous muscle the following is true of the underlying diagnosis? A. A 63-year-old male smoker presented to the hospital with fever and lower extremity pain. and he has a new gap in wound healing and numbness in both feet. Natalizumab 36. but he is except? otherwise asymptomatic. Binge eating at least once a week for a 3-month forehead. He is found to 31. The headache resolves within 6 hours. A 37-year-old female with multiple sclerosis recently 35. Given the most likely cause of his new period symptom. malodorous 37. All of the following are true of this condition has a large hematoma over his right temple. Indomethacin sensitive A. pulsatile B. C. leading to her seizure? B. He endorses a very long history of poor band no longer fits his finger. The E. Ejaculation pleocytosis C. she cannot maintain a normal D. Dalfampridine E.1% (normal is <6%). He is sent home without A. A 38-year-old man notes that his shoe size has have elevated inflammatory markers and osteomyelitis increased from 11 to 13 over the last year. Pronator teres headache. Extensor indicis proprius though the weakness persists for the next 24 hours. Magnetic resonance imaging (MRI) of the brain complication. Progressive supranuclear palsy C. Hepatic ultrasound A. Glatiramer C. This was likely the result of connective tissue loss. thinning hair. and scarring on the dorsal aspect of her right after a bar fight. Flexor pollicis longus patient’s sister has suffered similar episodes. Huntington disease D. Alzheimer disease likely added. Idiopathic Parkinson disease A. She now presents with new. Use of laxatives. Fingolimod D. Spike and slow wave on electroencephalogram 34. the next morning he awakes with severe right facial weakness. diuretics. Magnetic resonance imaging of the brain 33. Inability to control self when eating further evaluation. Despite performed and demonstrates the direct cause of his aggressive intravenous antibiotics. yet is still losing weight. Which of E. to avoid becoming obese. Which of the following is a parasympathetic activity? B. BMI must be <17 B. Acetylcholine receptor antibody serology interosseous nerve? A. Frequent skin checks and early medical care for E. Dimethyl fumarate E. After appropriate blood work is hemoglobin A1C of 13. except? onset seizures. Abductor pollicis brevis 38. Which of the following medications was A. The patient next test to find the source of the blood abnormality? wonders how this could occur in the first place and A. D. Eating more than what a normal person would eat A. including the B. Then. A 34-year-old man with history of migraine develops acute left hemiparesis along with a lateralized. Urinary storage D. or other agents to lower C. A 32-year-old man is brought to the emergency room breath. Perspiration C. Increased physical activity could have prevented this C. Her physical examination shows a cachectic female. Which of the following is innervated by the anterior E. Renal ultrasound what he should have done to prevent it. What is the best response to the patient? B. Associated with calcium channel mutation D. Computed tomography of the chest B.74 Neurology Self-Assessment 30. Valacyclovir and prednisone E. meals on a regular basis. He complains of severe headache and hand. Mydriasis Neurology Books . Dementia with Lewy bodies B. Associated with cerebrospinal fluid (CSF) B. The D. Anosmia is common in all of the following conditions adjusted her treatment. Human immunodeficiency virus serology weight D. This likely had to do with smoking rather than patient feels that she needs to monitor her weight daily poorly controlled diabetes. Bowel evacuation E. Her mother says that she even ate four slices of prevented this complication. which of the following should be done? C. physician’s office because of concerns about weight C. Whole-body positron emission tomography scan wounds or infections is critical in patients with 32. pizza last weekend. A 16-year-old female is brought to her primary care poorly controlled diabetes. Autosomal-recessive disorder E. He has a his lower teeth. which of the following is the most rational amputation of the left leg below the knee. Even aggressive glucose control could not have weight. The family has noticed that despite eating large disorder he inherited from his mother.

Autosomal-recessive inheritance pattern D. Maintain active medication and allergy lists D. and strokelike episodes (MELAS) C. Decreased conduction velocity hypertension. Associated with deficient hexosaminidase A activity C. Examination reveals normal arm strength and sensation.  Neurology Books . Myoclonic epilepsy with ragged red fibers (MERRF) B. His labs are notable for HbA1C of 7. and a sural nerve biopsy was undertaken and is shown below. his vitals show a pulse of 103. His transthoracic echo shows only a slightly dilated right atrium. lactic acidosis. Leigh disease D. Which of the following was the most likely abnormality on his nerve conduction testing in his arms? A. Decreased motor unit recruitment Figure for Question 3. E. He has a normal neurological examination except for numbness and tingling on the plantar aspects of both feet and an irregular pulse. Classically associated with cherry-red spot on ophthalmoscopy E. Copy-forward previous notes 43. Reversible with enzyme replacement 42. Test Three 75 39. Which of the following is the D. In addition to controlling lipids. but distal weakness and numbness in his legs. Leber hereditary optic neuropathy A. A magnetic resonance image (MRI) of the brain is shown. respirations of 12. Warfarin and elevated lactate. what is considered Figure for Question 3. Decreased motor unit action potential amplitude B. leaving him back to his baseline. He is admitted to the hospital. E-prescribing B. A 6-year-old boy has high arches and hammer toes. His symptoms last only for 1 hour and then resolve. and oxygen saturation of 98% on room air. Which of the following is not true of Tay-Sachs disease? B. and renal failure on dialysis presents with acute onset of left upper extremity weakness and numbness and left facial droop. His nerve conduction and electromyography were abnormal both in his arms and legs. On examination in the emergency room. He presents to the hospital in the setting of a prevention? 3 gastrointestinal illness and has now developed acute A. and glucose. Aspirin blindness. His strength is normal. blood pressure (BP) of 153/93. Automated drug-drug and drug-allergy checks C. His spouse makes him go to the emergency room 5 hours after symptom onset for further evaluation.  C.43. Rivaroxaban most likely explanation? E.2 and low-density lipoprotein (LDL) cholesterol of 164. A 63-year-old man with a history of diabetes. Aspirin/extended-release dipyridamole combination A. Which of the following is not considered a component of meaningful use for electronic medical records? A. A 9-year-old boy with short stature is developmentally the most appropriate regimen for secondary stroke delayed. and blood B. Mitochondrial encephalomyopathy. A noncontrast head computed tomography is performed and shows no hemorrhage or blurring of the gray/white margin. as well as a postural and action tremor in both hands. and further evaluation shows a normal magnetic resonance imaging (MRI) of the brain and magnetic resonance angiography of the head and neck.39. Decreased sensory nerve action potential amplitude 40. Clopidogrel work is only notable for an elevated leukocyte count C. Kearns-Sayre syndrome 41. Record smoking status for patients over 13 years old E. He begins to trip over his own feet. but is otherwise normal. blood pressure. Relatively high incidence among individuals from Cajun and French Canadian backgrounds E. Myotonia D.

which of the following is not true of genetic prion disease? A. Marcus Gunn pupil A. Argyll Robertson pupil course of action? E. Pick disease D. Longer survival 47. compressing the adjacent dorsal midbrain. Human immunodeficiency virus dementia C. Serotonin E.76 Neurology Self-Assessment Figure for Question 3. Which of the following neurodegenerative conditions in the same motor unit caused the pathological findings seen in the figure? B. Chemotherapy C. Variability in firing time between two motor units A. Focal radiation B.44. Number of single-fiber potentials firing D. Parinaud syndrome shown here. Initial parkinsonism or ataxia C. Whole-brain radiation A. His primary care physician refers him for a magnetic resonance image (MRI). Which of the following neurotransmitters is thought to B. Younger age of onset B. Which of the following describes increased jitter seen D. In comparing genetic prion disease with sporadic prion disease. Stereotactic biopsy 48.49. Norepinephrine on single-fiber electromyography (EMG)? E. is most likely to cause which of the following? Figure for Question 3. pretectum. Which of the following is the next best D.  44. Gamma aminobutyric acid (GABA) 45. Dopamine D. which is C. Adie pupil personality change. Variability in firing time between two muscle fibers 49. Maximal safe resection play a role in psychosis? C. A mass lesion in the pineal gland. Mobius syndrome weeks of progressive headaches and 2 days of B.  Neurology Books . Severe personality changes early on E. and superior colliculi. Slower disease course D. Alzheimer disease in the same muscle B. Intermittent transmission failure of one muscle fiber potential C. A 56-year-old otherwise healthy man presents with 2 A. Parkinson disease synchronously with the initial fiber E. All the above A. Huntington disease E. Measure of quanta of acetylcholine released from the presynaptic terminal 46.

An 11-year-old boy is admitted to the hospital because resonance imaging reveals well-defined restricted of respiratory distress related to respiratory syncytial 3 diffusion indicative of a stroke. Recurrent artery of Huebner not part of the possible treatment? E. he develops pathological processes or cerebral edema. Riboflavin C. Low-protein diet B. Supplementation of lysine and tryptophan E. His examination shows obtundation. elevated ammonia. L-carnitine D. Which of the following is D. What seizures. A 64-year-old smoker is found comatose.51. Left middle cerebral artery On laboratory evaluation. Artery of Percheron image of his brain is shown. but no other acute virus infection. Laboratory progressive deterioration of his mental status and tests and toxicology are relatively unremarkable. While in the hospital. Test Three 77 50. is the most likely artery involved in this stroke? generalized dystonia. Left posterior cerebral artery reveal elevated glutaric acid. Urine organic acids B.  Neurology Books . he has ketoacidosis. Artery of Adamkiewicz A. and hypoglycemia. A magnetic resonance C. A. and mild orofacial dyskinesias. Magnetic 51. Intravenous hydration Figure for Question 3.

Aicardi syndrome language. Extensor carpi ulnaris and global developmental delay. Levetiracetam E.54. Major depressive disorder 56. She has a history of mental retardation C.78 Neurology Self-Assessment 52. Dysthymic disorder B. They are active at the GABAA receptors. Autism milestones due to stiffness in the bilateral arms and E. He recently started a new antiepileptic medication. what is the most likely diagnosis? A. 60 57. Lamotrigine C. 15 E. confirms chorioretinal lacunae. 5 D. Adductor pollicis convulsions. 45 A. On examination. Norepinephrine is still able to hold his job as an administrative C. They are active at the gamma aminobutyric acid B D. He was nonverbal. Pectoralis major 54. and he is behind in meeting his motor D. she had D. Dopa-responsive dystonia (DYT-5) 59. A. but B. Which of the following is true regarding B. Felbamate B. Nonketotic hyperglycinemia E. Maple syrup urine disease (MSUD) D. She has poor vision. C. which of the D. and ophthalmology born at home without a physician or midwife present. Acetylcholine that can be done to help him. Which of the following neurotransmitters is most depression. His examination demonstrates reduced peripheral vision in both eyes. He states that he has been depressed for as associated with rapid eye movement (REM) generation? long as he can remember and feels there is very little A. A 35-year-old man presents to your office for 55. What is the D. Major depressive episode benzodiazepines? C. and poor concentration. Which of the following medications did he recently start? A. The child image (MRI) is shown. Angelman syndrome lethargy. Magnetic resonance imaging of his brain is unremarkable. decreased energy. with associated postictal confusion and B. she is E. Which of the following is the was finally brought in by his grandparents after they correct diagnosis? assumed custody. He has difficulty learning in school due to C. He denies prior suicide attempts. Phenylketonuria (PKU) C. The antidote is naloxone. A magnetic resonance and has never seen a physician before now. Deltoid infantile spasms as an infant. E. B. 30 following muscles will suffer weakness? E. Given his history and examination. and axial hypotonia with appendicular hypertonia. Vigabatrin D. He complains of sadness. A 14-year-old male with a history of Lennox-Gastaut syndrome presents with complaints of blurry vision. Schizoaffective disorder (GABAB) receptors. Fragile X syndrome legs. Homocystinuria B. He has mild spasticity in the bilateral arms and legs. Phenytoin Figure for Question 3. Serotonin most likely diagnosis? E. They are active on the ion channel coupled to an an adult (in cm H2O)? inhibitory G protein. A 2-year-old girl is hospitalized for generalized B. he has a fair complexion with light hair and a musty odor. They state that he had an episode of A. They are active on the same receptor as baclofen. Hypocretin assistant.  Neurology Books . A 5-year-old boy presents for possible seizure. and she is 58. What is the normal pressure of cerebrospinal fluid in C. Bipolar 1 disorder A. 53. Dopamine A. cleft lip. In the setting of a left C6 radiculopathy. On examination. Rett syndrome generalized tonic-clonic movement lasting for 2–3 minutes. Additionally. Rhomboids found to have microcephaly.

Symptoms will continue to progress B. Oral prednisone 60 mg daily his family insisted that he be evaluated. Boston Naming Test B. he was back to his normal self. Administer atropine 0. as are A. Ruling out a condition from a false-negative result E. Early Alzheimer disease and sweating on the head and neck. A 45-year-old male recently suffered a motor vehicle A. Wechsler Memory Scale 66. not rule out a diagnosis 65. His with severe headache. Administer 1 liter 0. A 32-year-old African American female with a history 62. Her not have dysarthria. No vital capacity on admission is 1 L. Wechsler Adult Intelligence Scale C. He denies pain or anxiety. Which of the following neuropsychological tests best examines executive function? A. including B. dysphagia.61. Which of the head is unrevealing. Diagnosing a condition from a false-positive test result D. IV immunoglobulin 0. or focal weakness. Most infectious in first 48 hours B. Accepting a prior diagnosis without confirming the diagnosis B. and the rest of motor neuron facial palsy. pulse is 50.  E. and a right lower blood pressure is 195/110. Administer lorazepam 1 mg IV simplex virus. Airborne precautions should be observed 61. Intravenous (IV) methylprednisolone 1000 mg daily remember anything from the previous day. yet his chest. Hypertension D. Transient global amnesia headache. Test Three 79 60. Cerebral vasculitis A. Cerebral amyloid angiopathy 64.9% NaCl intravenously (IV) polymerase chain reaction (PCR) tests for herpes B. E. His blood × 5 days count and blood metabolic profile are normal. Accepting a diagnosis despite evidence contrary to the diagnosis C. Which of the odd questions and repeated himself frequently. A negative diagnostic test will likely convert to positive A. A 58-year-old male presents to the emergency room of myasthenia gravis is admitted to the hospital for a (ER) after an episode of confusion. Perform fecal disimpaction condition? E. Trails B Test Figure for Question 3. Which of the following is true of this D. Seeking out information to confirm a suspicion. and legs have no color changes and appear 63. in high-risk sexual behavior. with diplopia and dysphagia that yesterday the patient was acting odd. fever. Electroencephalogram performed in the ER is D. His family reports myasthenic exacerbation. A computed tomography scan of the bowel movements have been absent. He is examined sitting up during an episode. but he did in association with an upper respiratory infection. vomiting. Azathioprine 100 mg daily Computed tomography of the head without contrast is normal. abdomen. All bacterial and fungal cultures of the cerebrospinal fluid are negative. though intravenous drugs.5 mg IV A. He has been suffering from frequent episodes of facial flushing C. varicella zoster virus. and spinal fluid evaluation reveals following is the next best step? a lymphocytic pleocytosis.4 g/kg × 5 days etiology? Neurology Books . Ciprofloxacin 500 mg twice daily also normal. Partial status epilepticus accident in which he had multiple fractures. but does not use A Foley catheter is in place and flowing freely. Plasma exchange or intravenous immunoglobulin C. He does not B. Transient ischemic attack 3 a T3 vertebral fracture with spinal cord injury. Have the patient lie flat in bed cytomegalovirus. elevated blood pressure with bradycardia. During these spells. C. he develops a D. A 26-year-old man presents to the emergency room dry. Which of the following is the most likely E. Metastasis E. but A. and negative abnormal movements were noted. He has never suffered his physical examination is consistent with a complete similar symptoms and denies sick contacts. and C. When following is the most appropriate treatment? he woke up today. Which of the following is an example of a type I error? E. He engages thoracic spinal cord injury. He asked his family inspiratory force (NIF) is −25 cm/H2O. Clock Draw Test D. Alcohol withdrawal over the last day. Which of the following is the most common cause of the pathological specimen shown here? C. Glioblastoma therapy should begin immediately D.

She has seen four other providers. he following is most commonly associated with this developed painful. Parasympathetic activation leads to which of the C. but normal for 3 weeks. Huntington disease concentration. Sweating 74. She is seeking medical disability and needs E. weakness. parietotemporal region and posterior cingulate gyrus. poor sleep. Other complaints include pain with intercourse. Which of the following is the mechanism of action for D. Alzheimer disease C. D. Sodium channel blocker B. and gastroenterological evaluation. C. CD20 monoclonal antibody 69. 73. along with comorbid depression. Pseudoathetosis is present. Opioids studies are necessary for the diagnosis. Electromyography (EMG) and nerve conduction C. 71. D. Physical therapy A. CD52 monoclonal antibody A. She has a history of tick bite followed by targetoid posterior left hip that radiates down his lateral leg and rash. blurred vision in the left eye about condition? 1 week before. Bronchiolar dilatation all modalities in his extremities. A. 72. B. Increased detrusor activity smoking. he has sensory burning pain on her right thigh that has been present loss in the same distribution of his pain. Which of the following is the most E. Additionally. A. B. This scan shows decreased A. A 21-year-old woman presents to the neurologist with a A. A 73-year-old man presents with shooting pain in his E. Sarcoidosis number of complaints that include headache. On magnetic resonance imaging (MRI) B. The pain began after a 68. which of the following fluorodeoxyglucose (FDG) positron emission is most likely to be found upon further questioning? tomography (PET) scan performed in the evaluation of memory loss. The patient has a history of physical and sexual fluorodeoxyglucose metabolism in the posterior abuse. Lumbar puncture is necessary for the diagnosis. A 24-year-old female who is 36 weeks pregnant comes fall. Lumbar laminectomy following is true of this condition? B. and nausea that precedes her A. and poor C. Which of the A. His neurological examination reveals normal cranial nerves. E. The pain is mostly on the lateral thigh and strength and reflexes. He has a history significant for D. Anti-antineuronal nuclear antibody 1 likely diagnosis? (anti-ANNA-1) Neurology Books . Dementia with Lewy bodies headaches. decreased sensation to E. A 35-year-old Pakistani man presents to the emergency He has a positive Romberg and demonstrates sensory room with 3 days of tremor. Multiple sclerosis and has undergone an exhaustive workup. including E. the dorsal aspect of his left foot.80 Neurology Self-Assessment 67. normal strength. He has a history of recurrent oral ulcers. Which of the following is the most likely diagnosis? photophobia. and absent reflexes. an enhancing lesion with no associated edema is identified in the left basal ganglia. She describes a upon leaning forward. Which of the following would not never goes below the knee. Pupil dilation E. 70. Temporary activity modification C. Nurses C. Treatment involves an injection of local steroid and anesthetic around the femoral nerve. The patient has a history of joint pain across many body parts along with fatigue. Lupus cerebritis nausea. nerve conduction studies. and whole-body numbness. Sphingosine-1-phosphatase inhibitor E. Behçet disease neurological imaging. Anti–N-methyl-D-aspartate (anti-NMDA) receptor although he has found no benefit from antiviral antibody medications. which were all normal. Frontotemporal dementia D. Nonsteroidal antiinflammatory drug (NSAID) B. On examination. D. The patient has a history of flashing lights. Which of the incoordination in his right arm. Alpha-4-integrin adhesion molecule antibody following physiological changes? D. Treatment involves avoidance of tight-fitting rituximab? clothing. Primary progressive aphasia someone to fill out her paperwork. Acute disseminated encephalomyelitis (ADEM) generalized weakness. It is worse with standing be considered a reasonable first option for treatment of and is better with sitting or lying down. Anti-Jo antibody evaluation. Anti-GQ1b antibody phlebotomy sites. B. Permanent weakness can occur if left untreated. Anti-GM1 antibody also tell you that he’s developed ulcerative lesions at all D. and ataxia. Her his condition? neurological examination is normal. B. A 53-year-old male presents to clinic with a several- C. A 78-year-old male presents after having a Given her suspected diagnosis. and is described as paroxysmal with improvement to the office for evaluation of pain. and anxiety. Increased cardiac contractility month history of falls.

  77. His mother has an acute onset of headache that stopped her states that these movements started gradually about 3 midsentence. or how she got in the basement. been increasingly irritable. Which of the following is C. On examination. Which of the following is the most likely B. Ciguatoxin C.78. His medical history is generally unremarkable. A 12-year-old boy presents with the inability to sit still. Stimulant medication Neurology Books . Monoplegia B. Absent reflexes Figure for Question 3. She is sodium channels except? also unable to give any details of what happened to A. Dissociative identity disorder D. Habit reversal therapy C. A. hyperactive. Carbidopa/levodopa E. Absent vibratory sensation C. All are true of severe. 79. and obsessed Her head computed tomography (CT) is shown. α-latrotoxin B. Test Three 81 75. Further. Tetrodotoxin her. the substantia gelatinosa appears to be damaged on magnetic resonance imaging. She is rushed to hospital via ambulance. Saxitoxin diagnosis? C. Often associated with significant autonomic instability D. A 23-year-old woman with a history of kidney disease he has a normal neurological examination. 1 month A. Posttraumatic stress disorder D. weakness. Penicillin G E. Brevetoxin A. Cord contusion may lead to worse arm weakness than leg E. he has choreiform movements. 1 day psychological features. Upon the arrival of authorities. worse E. the woman does not remember her name. and incontinence B. over the last 2 months he has and vomiting. Dissociative amnesia on the left upper extremity than the right. Absent noxious stimulus sensation D. Genetic counseling D. Presents with areflexia. Among the affected Rexed laminae. Depersonalization 80. 1 week indicated? D. 1 hour except for a sore throat approximately 4 months ago. A 23-year-old woman was abducted from her home and taken to a basement where she was sexually and physically abused for 1 week. A 45-year-old man suffers a partial spinal cord transection at T12 on the left. numbness. All of the following neurotoxins affect voltage-gated where she lives. 78. High-dose steroids may improve ambulation at 3 months C. Which of the following leg deficits correlates with this radiographic finding? A. Otherwise. There is no family history of similar neurological or B. 1 year B. acute traumatic spinal cord injury except which of the following? 3 A. Ataxia E. When with making sure that his shoelaces are double- is her peak risk of vasospasm? knotted. Requires immediate magnetic resonance imaging (MRI) in case of reversible compression 76. Concussion E. and was followed immediately by nausea months ago.

Sjögren syndrome Figure for Question 3. All of the following are necessary criteria for the headache 3 days after delivering her first baby. In muscle contraction.  D. Left posterior semicircular canal performed to help confirm the diagnosis. With his eyes closed. Right posterior semicircular canal B. Facial trauma E. Absent N22 D. Large-volume lumbar puncture 86. Right lateral semicircular canal C. Migraine 85. Diagnosed on overnight polysomnogram with B. A patient who suffered a stroke has intact sensation in E. papilledema. Troponin 87. which was easily controlled. She reports sensation in the legs inability to breastfeed since delivery and complains of polydipsia and polyuria. Postepidural spinal headache E. Myosin B. Which of the following abnormalities would be expected? B. Ventriculoperitoneal shunt B. Discomfort relieved by movement A. Postpartum angiopathy periodic electromyogram (EMG) activity occurring every 20–40 seconds C. For a woman with recurrent pressurelike headaches. Acetazolamide D. An urge to move the legs caused by uncomfortable hemorrhage. If vertigo and a right beating nystagmus are observed episode of suspected thoracic transverse myelitis. Conversion disorder C. 30 seconds after performing a Dix-Hallpike maneuver Unfortunately the magnetic resonance imaging with the right ear toward the ground. Cerebral venous sinus thrombosis D. What is this disorder called? A. Agraphesthesia C. which of the following is not a therapeutic consideration? A. Cerebellar vermis D. Prolonged N9–N13 interpeak latency E. Left lateral semicircular canal A. he cannot identify objects placed in his hand affected by the stroke. Tumor 88. Visual agnosia Neurology Books . what is the most machine is broken and will not be repaired for days. A 34-year-old woman presents with an excruciating 84. Sheehan syndrome E.82 Neurology Self-Assessment 81. and pulsatile tinnitus. An urge to move occurring during periods of rest Which of the following is the correct diagnosis? C.81. Titin E. Astereognosia B. Brain imaging is shown. Nebulin C. Optic nerve sheath fenestration C. Which of the following is highest on the differential diagnosis for an otherwise-healthy 24-year-old woman presenting with new trigeminal neuralgia? A. Prolonged P34–P37 interpeak latency his extremities. Absent N20 83. An urge to move that is worse at night D. Prosopagnosia D. Actin D. which of the following is the mobile part of the sarcomere resulting in a decrease in sarcomere length? A. Prolonged N22–P34 interpeak latency C. likely localization of the patient’s underlying problem? Somatosensory evoked potentials (SSEPs) are A. Her diagnosis of restless legs syndrome (RLS) except? only complication was mild postpartum vaginal A. Isotretinoin E. Simultagnosia E. Multiple sclerosis B. B. A 27-year-old female presents to the hospital with an 82.

A common etiology includes dehydration or D. Lysosomes E. Despite his best efforts. A. leading to progressive hemiparesis. The malformation is self-limiting and usually does B. Which of the following is not a part of the classic following picture? construct of grief? 3 A. Anger C. but they have been occurring more frequently A. Denial B. Acceptance E. Listeria monocytogenes C. Autism not require treatment. he cardiac failure in neonates. Chylomicrons 90. Test Three 83 89. Staphylococcus aureus 93.94. Projection 92. cannot control these episodes. Which of the following is demonstrated in the 91. Mitochondria B. Strokes or steal phenomena can occur. Hypothalamic hamartoma infection. C. neurological examination is unremarkable. Mesial temporal lobe sclerosis D. Lissencephaly D. Which of the following is the most likely cause of his symptoms? C. Bargaining D. leading to defective venous drainage. Parasagittal meningioma E. E. A 9-year-old boy presents with episodes of uncontrollable laughing for no reason and at 94. Agenesis of the corpus callosum A. Neisseria meningitidis D. This condition frequently leads to low-output over the last few months. Holoprosencephaly C. but should be considered in an 80 year old? A. Pachygyria B. Pseudomonas aeruginosa E. Which of the following is an unlikely cause of meningitis in a 30 year old. Use of levetiracetam Figure for Question 3. Endosomes Figure for Question 3. The malformation consists of arteries draining into complained of headaches. Polymicrogyria E. Peroxisomes C.  Neurology Books .89. Group B streptococci B. Which of the following is absent in individuals with Zellweger syndrome? A. His capillaries. Which of the following is true regarding the condition inappropriate times. These outbursts usually last <1 depicted in this image? minute.  D. mostly at night. In addition he has B.

Alcohol Left eye Right eye C. Living in northern latitude Neurology Books . Which of the following is the E. Vestibular schwannoma alleles E. However. Change the order as requested. The study was ordered A. With acute use. hallucinations. Meningioma A. but the center inadvertently gave the patient contrast anyway. Cyclophosphamide B. A local radiology center contacts your office about a 98. Carbon monoxide C. Left occipital lobe the expensive contrast. Prednisone 97. Rituximab D. Which of the following is the most common 101.84 Neurology Self-Assessment 95. Intravenous immunoglobulin C. and unsteadiness. Metastatic tumor D. Fire the MRI technician. Acrylamide E. Leave the order as intended. D. A 53-year-old male is admitted to the psychiatric unit for treatment of substance abuse. but was fired for inhaling paints and lacquers. Rapid increase in the dose of which of the following medications may exacerbate myasthenia gravis symptoms? A. Sella without contrast (as you intended). Certain major histocompatibility class (MHC) I E. Which of the following variables is associated with intracranial tumor in adults? worse prognosis for multiple sclerosis? A. Contact Medicare to report the center for fraudulent billing. Pituitary adenoma C. Which of the following is the most likely lesion magnetic resonance imaging (MRI) study performed localization that would result in this visual field deficit? on one of your patients. Left optic nerve most appropriate response? A. Aluminum 100. C. after long-term use he also developed a mild neuropathy with dysautonomia. 96. Glioblastoma B. Which of the following substances is the most likely cause of his symptom? A. he reported feelings of euphoria. First symptom sensory in nature D.96. Methanol Figure for Question 3. The most likely cause of death for this 45-year-old stay-at-home mother is exposure to which of the following? A. Carbon monoxide E. Organophosphates B. Manganese 99. B. Left parietal lobe and without contrast” so they can be reimbursed for D.  E. D. Contact a medical malpractice attorney. E. N-hexane D.98. Left temporal lobe radiologist asks that you change your order to “with C. Tobacco B. Older age at onset B. Pyridostigmine Figure for Question 3. Female gender C. He previously worked in a cabinet shop. The B.

sensory responses were normal. 104. stroke. Naming is impaired. D. Carbamazepine latencies and velocities. Lacosamide kinase antibodies are present. Spores in honey are responsible for this A. After warfarin therapy. On fibrillation. On nerve medication 2 weeks ago. How long after blood flow is cut off to cerebral C. Superior temporal lobe B. but cannot read what she has just written. Presynaptic voltage-gated calcium channel C. E. his strength previously. Gabapentin B.  E. 8–10 minutes 105. Splenium of the corpus callosum Figure for Question 3. 1–5 seconds condition. Although she was on levetiracetam repeated testing of the quadriceps. What is the most likely localization for her stroke? A. Valproic acid A.102. Which of the following is true B. Pyridostigmine is the initial treatment. B. Lamotrigine about this condition? C. Test Three 85 102. Angular gyrus 0.5 mV C. cerebral neurons to stop? D. On examination she has fluent speech with intact comprehension. Inferior parietal lobule Neurology Books . All most likely antiepileptic started 2 weeks ago? motor responses had low amplitudes but normal A. An 82-year-old female presents with an acute stroke. Postsynaptic anti–muscle-specific receptor tyrosine D. She can write. Posterior lateral frontal lobe 2 ms D. 2–4 minutes antibodies are present. 12–15 seconds E. and seizure presents with another 3 physical examination. she switched to a new antiepileptic improved and the patellar reflex appeared. he has bilateral proximal arm acute ischemic stroke despite compliance with her and leg weakness along with absent reflexes. A 68-year-old female with a history of atrial progressive weakness and a severe dry mouth. This disorder is commonly associated with colon circulation does it take for electrical activity of the cancer. Repetitive nerve stimulation at 50 Hz is shown here. Which of the following is the conduction testing. A 45-year-old male presents with a 2-year history of 103. 4–6 minutes E.

botulinum toxin or deep brain stimulation of hemorrhagic encephalopathy. this is a familial tremor characterized by a distal intellectual disability with seizures. At high doses. encoding the protein aspartoacylase. 1422–1460). Ch. erroneously suspect from this CT scan. the father leads to Prader-Willi syndrome. 112. salicylates may cause hereditary basis. includes primidone or propranolol.  Ototoxicity is a common concern with medications chronic alcoholism and nonspecific nutritional used in neurology. Warfarin is known to be be an appropriate therapy for any possible diagnosis. et al. 1261–1278). from Ellison D.  Acute hemorrhagic leukoencephalitis. 1226–1236 insufficiency. as it death in infancy (see Bradley’s NiCP.  The patient most likely has essential tremor. In such a She is at a high risk of recurrent stroke. 7th edn. or acute tremors. However. slanting eyes. especially scenario. Yanni D. teratogenic and is pregnancy category X. the thalamus (ventral intermediate nucleus) can be inflammatory illness associated with demyelination considered. Most syndrome. which is associated with First-line medical treatment for essential tremor microcephaly. cryptorchidism. 66. 80. which is associated with rigidity. This patient has antiphospholipid antibody associated infarct and hemorrhage. affect the hearing (with profound. Alexandru most easily differentiated from other hemorrhage types D. low-set ears. and loss a characteristic happy demeanor. and will occasionally improve with alcohol. and gabapentin. Although there is generally no bradykinesia.  Prader-Willi is a genetic syndrome characterized by components of the BBB include astrocytic end feet. diagnosis in this case (see Bradley’s NiCP. anxiety. such stroke. pp. pp. C. pericytes. and obesity. and sterility. 3rd edn. so than a postinfectious process. Alexander disease is associated with tinnitus or temporary hearing loss. hypotonia.  The image demonstrates a subdural hemorrhage. Trisomy 13 is or caffeine. C. 88. 1324–1341). 1159–1186). but not as much as unfractionated heparin. Cisplatin can cause tinnitus and profound Metachromatic leukodystrophy is associated with sensorineural hearing loss. B. as one might syndrome and a history of DVT and ischemic stroke. head. Cerebral venous sinus thrombosis may have be difficult. anticoagulation would be correct. tremors worsen with stress. Ch. encoding arylsulfatase A hearing loss. The 1. Elsevier. whereas amikacin tends to only encoding peroxisomal membrane transporter proteins. Trisomy 21 is seen with Down asymmetrically. 7th edn. 7th edn. Ch. Angelman syndrome is characterized by often. cogwheeling may still be decreased muscle mass and body hair. pp. genital defects. central desire pregnancy. Unless the physical examination suggests and hemorrhage. All of the other listed concurrent administration with other ototoxic disorders of myelin metabolism have a known medications. D. C. pp. Chs. and is often dependent on dose. Unfractionated There is evidence that hemicraniectomy may improve heparin or low-molecular-weight heparin are the long-term outcomes and prevent herniation in cases of recommended anticoagulants to use during pregnancy malignant infarcts. dextrocardia. Among mutations in the GFAP gene. Observation would not they are not recommended. although that is not the underlying (see Bradley’s NiCP. 1973–1991). Adrenoleukodystrophy is aminoglycosides. This particular variety of acute features of parkinsonism. tight junctions. Immunosuppressive therapy is the best therapy. Other pp. pp. ASPA gene. medications that can be tried include topiramate. D. but maternal inheritance of the same mutation leads to Angelman 5. Kim R.86 Neurology Self-Assessment ANSWERS 4. fatigue. and may even affect the voice. increased breast seen in this disorder. and basement membrane. A karyotype of XXY is of balance. seen with Patau syndrome. mild sensorineural hearing loss. with permission. There is no evidence of a hereditary duration of therapy. 7th edn. pp. 96. low-set ears. 3. This disorder demonstrates Oligodendroglia are not involved in the BBB. For medically refractory 2. although there is limited information nervous system spread of this infection is less likely on the safety of these medications during pregnancy. craniofacial abnormalities. but the rare genetic principle of parental imprinting. Ch. D. Love S. Commonly. 50. found in Klinefelter syndrome. short stature. hand flapping. and as hypercoagulable history or clear imaging evidence of aspirin is pregnancy class D. intellectual disability. The tremor will occasionally start tissue. instead myelinate neurons in the brain (see Bradley’s wherein receiving a particular mutation on 15q from NiCP. Aspirin would reduce the risk of without other details to specifically suggest CVST.  Marchiafava-Bignami syndrome is a toxic/metabolic disease of the myelin that is classically associated with 8. 7. 2013 Neuropathology. and action tremor. carbidopa/levodopa is not disseminated encephalomyelitis is almost always usually used (see Bradley’s NiCP. with permission. during pregnancy. Vancomycin may cause mutations in the ARSA gene. monophasic. deficiencies. hyperphagia. and a single palmar crease. and not be the most appropriate choice in this patient. flattened nose. Dabigatran and a delta sign. Figure from Kao HW. metabolic clearance. be associated with temporary vertigo (including Neurology Books . may worsen both his depression and asthma. macroglossia. is an acute. Hasso AN 2012 Value of because the falx cerebri remains visible and intact susceptibility-weighted imaging in acute hemorrhagic within the skull convexity in the examiner’s hands (see leukoencephalitis. 968–980). Propranolol would rocker-bottom feet. or postural instability.  Decisions on anticoagulation during pregnancy can success. or legs.  The BBB is a highly selective filter that keeps many circulating blood substances away from the brain. clonazepam. this is a less likely diagnosis. A. and component to this syndrome. balance and hearing. Figure 19(12): 1740–1741. Ch. 85. pp. permanent hearing Canavan disease is associated with mutations in the loss) and streptomycin tends to only affect balance. 7th edn. Journal of Clinical Neuroscience Bradley’s NiCP. 7th edn. but quickly spreads to the other side syndrome. gentamycin tends to affect both associated with mutations in the ABCD1 gene. especially in the setting of renal (see Bradley’s NiCP. although with variable 6. most commonly Mycoplasma. associated with a recent respiratory tract infection. with symptoms of intellectual disability. 648–675). Chimelli L. Although some antiepileptic drugs may and 91. Ch. Given the rivaroxaban could be considered if the patient did not 1-week interval from his Mycoplasma infection.

  The patient presents with subacute memory loss stimulation. 15. the anterior portion of the cord through stimulation of the upper motor neurons through transcranial 11. CADASIL electrodes). and brainstem. the X-linked pattern of heredity. NOTCH3 gene mutation is seen in disease. pp. and axons with nodes of Ranvier every 1 to 2 mm. hypotension. children frequently develop spastic quadriparesis. 7th edn. 1196–1200). 583–604). antipsychotics (risperidone. which spares the U-fibers. Clinically. Figure from Mayans D. et al. with permission. symptoms begin in the first 2–4 months of life. diagnostic MRI findings for Alzheimer disease. 107. A inclusions are the main pathological feature seen in spasticity. D. D. antibodies are seen in Lambert-Eaton myasthenic 17. and alien limb syndrome. There are detect injury in that pathway. 407–410). haloperidol. 34–35. which may be either guanfacine) or selective serotonin reuptake inhibitors. seizures. fragile X syndrome include mental retardation. E. Women are carriers of this syndrome.  This case is an example of Pelizaeus-Merzbacher syndrome. Ch. measuring connectivity by and myoclonus with a lumbar puncture showing electromyography activity in various muscles. Ch. pathological feature seen in Alzheimer disease. Given 82. not meningitis (see Bradley’s NiCP. 7th edn. Problem Solving in Emergency Radiology. 1422–1460). cerebellum. large 7th edn. pp. tremor. athetosis. this is a disease of 13. are auto-antibodies. with can occur (compared with unmyelinated fibers). and more-than-adequate depolarization to occur so that the lacosamide). and the CT angiography Asia. carbamazepine. 53–101. (displacement of either recording or stimulating which is common in limbic encephalitis. diabetes is the most common treatable cause of Cerebral venous sinus thrombosis is a neurological neuropathy (see Bradley’s NiCP. or anesthetics (particularly usually involves headaches. Streptococcal 16. lamotrigine. pimozide. 82. without fail (see Bradley’s NiCP. and neuromuscular blocking agents like rocuronium). pp. Brain MRI of Ranvier. Figure 4-13. Ch. Ch. Other agents used to treat the comorbidities of Tourette syndrome include 12. Ch. C.  Leprosy is the most common treatable cause of 14. There are no Bradley’s NiCP. E. The MRI demonstrates these signals can be due to technical failure hyperintensities in the bilateral mesial temporal lobes. Typically herpes encephalitis (see Bradley’s NiCP. patients with fragile X Kubal WS. 7th edn. and are associated with Methylphenidate would not be used in this patient limbic encephalitis and neuromyotonia (Isaac because of the lack of ADHD symptoms (see Bradley’s syndrome). Motor milestones are often delayed. pp. In this question. given the such an emergent scenario. Neurology Books . Symptoms of treatment of a venous thrombosis (see Bradley’s NiCP. 90. dementia.  Anti-voltage-gated potassium channel antibodies centrally acting alpha2-antagonists (clonidine. strokelike episodes. the coding region of the FMR-1 gene on chromosome hyperosmotic therapy is superior to corticosteroids in X. SSEPs are transmitted through the dorsal column and rigidity. anterotemporal lobes. 7th edn. and South America. Capacitance is reduced by the high concentration of On pathology. 7th motor evoked potentials roughly measure function of edn. C. 9. Africa. 38. 7th medications that block or deplete dopamine. Ch. emergency for which the primary treatment remains pp. 968–980). Elsevier. Transcranial relation to fragile X syndrome (see Bradley’s NiCP. 46. which begins in the fifth or sixth decade with tremor and ataxia. Further. These are also susceptible no diagnostic MRI findings for CBD. Test Three 87 phenytoin. 391–400 and Ch. D. giving the sample a “tigroid” appearance. pp. paraneoplastic or sporadic. Conduction of an action potential occurs at the node Cognition is also frequently impaired.  The patient’s CT scan demonstrates a left-sided neuropathy in the world. 7th edn. and the MRI shows can also indicate a pathological state such as anterior subcortical white matter lesions. Aspirin has no role in acute presence of a normal X chromosome. 96. 7th edn. 1196–1200). 1791–1867). Neurofibrillary tangles are a prominent often duplication) of the PLP1 gene. 349–391). Tics can be treated with a variety of encephalitis. 66. syndrome are at increased risk of a disorder known as pp. fluphenazine) or tetrabenazine. there can also be islands of white sodium channels in the nodes. ears. including edn. the increased signal intensity in bilateral hemispheres. and elevated white compulsive disorder (OCD) and/or attention-deficit/ blood cells and protein in the spinal fluid. levetiracetam is not associated with signal is transmitted to the next node of Ranvier 3 vertigo or ototoxicity (see Bradley’s NiCP. and ataxia. CBD involves myoclonus. A. 1301–1323). with permission. pp. Ch. Loss of inflammation without infection. or dementia later in the course. which is caused by a deleterious event (most CADASIL. and large testes. pp. anticoagulation even in the setting of a hemorrhagic stroke. By decreasing the area where conduction commonly reveals diffuse leukodystrophy. 36. personal collection. particularly in the spinal infarct or injury from the surgical procedure. enhancement of the meninges can be seen. patient has motor and vocal tics along with OCD but hyperintensities within the parenchyma signify behaviors. In the United States shows a filling defect in the left transverse sinus. fragile X tremor ataxia syndrome. encephalopathy.  tcMEPs and SSEPs are frequently used during Dextrocardia is not seen at an increased rate with intraoperative spinal surgery monitoring.  Myelin develops an insulating layer around the males. Further. the meningitis. This patient’s blood pressure is normal. In hyperactivity disorder (ADHD). so no 10. pp. Cowdry Pelizaeus-Merzbacher presents with nystagmus. Ch.  Tourette syndrome is marked by both motor and meningitis would present more acutely with high phonic tics and can be associated with obsessive- fevers. anesthetics. while the disease of cytosine-guanine-guanine (CGG) repeats in intracerebral hemorrhage is associated with swelling.  Fragile X syndrome is an autosomal-dominant antihypertensives are indicated. particularly in Southeast intracerebral hemorrhage. Ch. Figure from Mirvis SE. total resistance of the membrane is increased. although atrophy to technical failure. and hypotension (see of the parietal lobe can be present. Anti-voltage-gated calcium channel NiCP. which allows for matter. B. although global atrophy and atrophy of the temporoparietal junction are common.

chronically patients may be left with a isolated syndrome) measures detailed above thalamic pain syndrome (Dejerine-Roussy syndrome) in the same distribution. as well as taste from all portions of the studies are hopeful for rituximab in the use of RRMS.88 Neurology Self-Assessment Management for Pelizaeus-Merzbacher is largely TABLE FOR ANSWER 3. vascular dementia. strokes. Radiographically isolated syndrome is diagnosed Oral steroids are not an appropriate treatment. tongue and epiglottis. A. Elsevier. with normal females in the family suggesting Clinical presentation sclerosis an X-linked inheritance. Symptoms of transverse myelitis depend on Neuromyelitis optica is a demyelinating disorder. 50. modulation. ovoid. demyelinating conditions of the central nervous 7th edn. Friedreich’s ataxia (see Bradley’s NiCP. 7th edn. and migraines. In: Goldman L. This and weakness. Clinically isolated syndrome is diagnosed with contrast.g. however.19 (see Bradley’s NiCP. CADASIL is a hereditary no response to initial steroids. Mano T. 648–675). Also worth noting is remitting multiple sclerosis (RRMS) may include an the frequent presence of MRI fluid attenuation interferon (interferon beta-1a or interferon beta-1b).19. typical of Pelizaeus- Merzbacher disease. one clinical attack and objective clinical evidence of ≥2 21. evidence of ≥2 lesions demonstrated by simultaneous caused by a duplication-inverted triplication- presence of asymptomatic duplication in chromosomal segments including the enhancing and nonenhancing PLP1 region. A mutation in the FXN gene leads to juxtacortical. IVIG has no role in transverse affected areas) with no clinical history suggestive of myelitis. but an based on MRI evidence of prior demyelinating episodes oral steroid taper could be used after the initial IV (e. associated with this condition commonly present with a bandlike recurrent demyelinating events of the optic nerves sensation across the chest or abdomen. 80. 1159–1186).  First-line immunomodulatory therapy for relapsing attention and saccade initiation. The MRI scan of her spine demonstrates disorder is associated with antibodies against T2 hyperintense lesions within the cord that enhance aquaporin-4. the bilateral pulvinar associated with Creutzfeldt-Jakob Mitoxantrone is approved for progressive forms of MS. a sensory level. or a new 55(6-7): 400–403. myelitis. This patient’s is associated with defects in the NOTCH3 gene (see examination is most consistent with a complete spinal Bradley’s NiCP. Rituximab is an anti-CD20 primary visual cortex. or await a second clinical attack sensory information from the limbs to the postcentral One attack. inversion recovery (FLAIR) sequence hyperintensity in glatiramer. Saunders. lesion and not an anterior spinal artery syndrome. Alexander disease of Two or more attacks. 7th edn. 1159–1186). disease (pulvinar sign). deletion in the PMP22 gene leads to hereditary clinical evidence of one lesion demonstrated by ≥1 T2 lesion neuropathy with liability for pressure palsies. Neurology Books . In addition. objective None the infant/juvenile type usually has a frontal clinical evidence of two or more lesions. well-circumscribed foci in commonly steroids are completed. 30. objective clinical Dissemination in time. this is not syndrome associated with recurrent small-vessel used first line because of risks of hypotension. undue side effects and lack of efficacy in disease 7th edn. A in at least two of four typical trinucleotide expansion of HTT leads to Huntington regions for MS (periventricular. CADASIL metabolic disturbances. 7th edn. Although initial from the face. infratentorial. lesion developed on follow-up 18. A Two or more attacks. The medial geniculate nucleus there are no definitive data for this choice. disease. or fingolimod. 80. et al. methylprednisolone given over the course of 5 days. spinal cord). pp. Patients with previously known as Devic disease. sepsis. Schafer AI Goldman-Cecil Waldman SD. or one lesion predominance of white matter change rather than the with a prior attack confluent nature of Pelizaeus-Merzbacher disease. Initial treatment begins with IV with no radiographic evidence of prior attacks. Ch. attack in a different region Kashiwagi M. demonstrated by sensory loss. Medicine. A. objective clinical Dissemination in space and gyrus. dimethyl fumarate. Additional data needed for The question stem suggests involvement in a male diagnosis of multiple cousin. Ch. The ventral posterior medial monoclonal antibody that reduces many immune- nucleus is a sensory relay for pain and temperature mediated diseases and symptoms. See the table for more information. pp. pp. so Figure from Calabresi P 2016 Multiple sclerosis and anticoagulation is not indicated (see Bradley’s NiCP. Monthly relays auditory information from the inferior colliculi steroids are generally avoided as a monotherapy due to to the primary auditory nucleus (see Bradley’s NiCP. 80. and longitudinally extensive transverse myelitis. the section of the spinal cord involved. this 3.  The ventral posterior lateral (VPL) nucleus relays scan. Campbell RSD 2010 Imaging of Pain. objective Dissemination in space. A GFAP mutation leads to Alexander for Multiple Sclerosis disease and presents in an autosomal-recessive fashion. The pulvinar is the largest thalamic nucleus and is associated with visual 20. 314–323). Ch. Ch. patient meets the diagnostic criteria for MS based on 1159–1186). and bleeding. with permission.  Based on the 2010 revisions of the McDonald the definitive diagnosis of MS reached in Question Diagnostic Criteria for Multiple Sclerosis (MS). with permission. Whereas an acute lesion in the VPL may cause evidence of 1 lesion (clinically time. Observation is not the best choice given 19. B. The lateral geniculate nucleus but is generally used only for refractory disease due to is a relay of visual input from the optic tract to the the risk of cardiac toxicity. 2012 Pelizaeus-Merzbacher disease One attack. This is the typical appearance of acute in an individual with only one attack of demyelination transverse myelitis. European Journal of Medical Genetics lesions at any time. Figure from Shimojima K. D. Figure from system. pp. or await another pp.  This patient presents with an acute transverse lesions. Ch. with permission.  2010 McDonald Diagnostic Criteria supportive. Plasmapheresis can be considered if there is demyelinating attacks.

and poor sucking and feeding. other focal lesions. Clinically. A. Generally. 7th edn. Although lower extremity myelopathy in the setting of HIV. 113. or upper Dravet syndrome. atonic seizures. calls). seizures. which is inherited disorder with a mutation in voltage-gated apparent on a contrast-enhanced MRI of the sella. imaging.  The patient has a bitemporal hemianopsia. until they present with a febrile seizure under the age however. and if the average sleep-onset latency is <8 minutes and if progressive quadriparesis. 77. but rather a severe absence seizures. the SCN1A gene. progressive. 52. Ch. Pain. Alexander disease is associated with GFAP mutations and generally causes 24. macrocephaly. intentional sleep deprivation (like 30-hour NAA in the urine and central nervous system.  Cognitive behavioral therapy has the most autosomal-recessive lysosomal storage disorder with supportive data for nonepileptic attacks. Low cortisol is seen in adrenal whereas heterozygous female carriers may be insufficiency (see Bradley’s NiCP. but remits by 6 months of hemianopsia due to compression of the optic chiasm. Canavan disease is caused by deficiency of the positive MSLT. This test is positive delayed milestones. sensory level. cysts on magnetic resonance imaging. which is not an answer functional neurological illnesses. typically cause a myelopathy. or hypogonadism. as well as a distinct phenotypes. including ictal vomiting. it is presenting symptoms of late infantile cases are ataxia. myeloneuropathy later in life. like alcohol. There are also no data for electroconvulsive changes within the myelin sheaths in the thoracic cord. Ch. Sleep Pathologically. A. VM occurs in up to 50% of HIV patients at evidence for their use with nonepileptic attacks autopsy. p. syndrome is also known as early myoclonic headache. Canavan disease causes cerebral walking does not cause sleep deprivation or suppress vacuolization. Brain biopsy is generally two out of four (or five) naps have sleep-onset REM. Cryptococcal meningitis does not types. and narcolepsy. and complex partial seizures. and the history of HIV should milestone regression and develop multiple seizure be a red flag for VM. These seizures have prominent combination of history. Seizures headache with increased intracranial pressure and may be exacerbated by carbamazepine or lamotrigine. Decreased GnRH would lead very-long-chain fatty acids in peroxisomes and to diminished follicle-stimulating hormone or ultimately severe myelin loss. Most often. X-rays of bones such as the mandible also are Panayiotopoulos syndrome is a disorder that usually abnormal. potassium channels. can suppress REM. 696–712). Initially. noted to have abnormal accumulation of Rosenthal Disorders that reduce REM sleep times can result in a fibers. but would not suppress excess GH in (see Bradley’s NiCP. unlike other 26. The presenting symptoms of adult Neurology Books . therapy for nonepileptic attacks (see Bradley’s NiCP. The wider range of functional symptoms. or dopamine the ABCD1 gene. 1992). Test Three 89 22. in addition to 1615–1685). acromegaly. hypotonia. The child develops normally despite these seizures. visual changes. possible seizures.  Of the various options. Administration of develops between the ages of 3 and 5 and involves 100 grams of glucose would suppress GH secretion multiple seizure types along with cognitive dysfunction normally. a normal overnight begins in the first months of life and is associated with polysomnogram is necessary the evening before an macrocephaly. Once the disorder develops. poor visual fixation MSLT is performed in order to rule this out. A. or inherited leukodystrophy. shift-work enzyme aspartoacylase. pp. depending on age of onset.  Adrenoleukodystrophy is the most common therapy with somatostatin analogs (octreotide). pp. that anticonvulsant drugs be discontinued. This leads to seizures on the Enlarging masses may result in a bitemporal second or third day of life. and autonomic involvement. Over the course of months they continue to have suggest a family history. It is an X-linked disorder in GH-receptor antagonists (pegvisomant). patients may also develop neurological examination is abnormal at birth with gigantism (tall stature. and abnormal encephalopathy and involves focal myoclonus that shifts growth of the hands and feet (acromegaly). This comes from a mutation affecting VZV myelitis. hypotonia. 7th edn. or inflammation. seizures (see Bradley’s NiCP. the excessive GH is Benign familial neonatal epilepsy is a dominantly caused from a secreting pituitary adenoma. 7th edn. ultimately once a diagnosis of nonepileptic attacks is made and leading to death. recommended that additional referrals are not made dysarthria. epilepsy of infancy. this patient most likely has complications of HIV. examination. 102. Treatment often involves a transsphenoidal resection of the tumor. Ch. children develop normally Hereditary spastic paraparesis can present similarly. which can often be seen as multiple REM (see Bradley’s NiCP. Ohtahara 23. leading to accumulation of disorder. there is no good choice). This antidepressant medications (especially tricyclic 3 is most consistent with a VM or possibly human T-cell antidepressants) have been shown to help a variety of lymphotropic virus 1 (HTLV1. Various and tracking. including myoclonic seizures. luteinizing hormone. 1563–1614). coarse facial features). Ch. laboratory evidence of elevated IGF-1 and GH level along with occipital spikes. Lennox-Gastaut typically after an oral glucose tolerance test. This most from one region to the next in the first 3 months of likely relates to excessive growth hormone (GH) life with spasms developing later in the disorder. and cognitive decline. or even multifocal slow spike and wave. E. and pathological samples show vacuolar specifically.  The patient presents with a painless. C. which would result in infertility Males commonly present as in the case noted here. macrocephaly. The diagnosis is made through a develops at the age of 5. seizures. asymptomatic or develop mild myelopathy or pp. Metachromatic leukodystrophy is an 25.  This patient has a positive MSLT. pp. as well as pharmacological 27. 101. there is nothing in the question stem to of 1. substances. E. This can include sleep apnea. also known as severe myoclonic extremity symptoms would point toward CMV myelitis. age. The production. Because sleep apnea can cause Clinically. it does not improve with 7th edn. 7th edn. leading to impaired breakdown of agonists (bromocriptine). as is shown in the image. dysphagia. myelin loss. the most severe type of Canavan disease an MSLT to be positive. An initially normal EEG can change to hypsarrhythmia 1102–1120). and a very abnormal EEG. Ch. coarsening of facial features. highly active antiretroviral therapy.

it may cause walking. 34. 7th edn. 80. B. reducing the incidence of diabetes mellitus type 2. 1867–1895). 1324–1341). and nasociliary branch of the ophthalmic division of the is associated with tachycardia. pp. 7th edn. Whereas the most severe form of and purging must occur at least once a week for a Pelizaeus-Merzbacher disease associated with PLP1 3-month period. types of tumors may develop facial weakness late tend to have better secrete GH. pp. or taking diuretics. unethical because he or she cannot give proper and frontotemporal dementia may have mild levels of informed consent. dementia with which can be shown to others in the medical Lewy bodies. usually are cognitive and 32. A. on the other hand. fractures result in facial weakness.  Bulimia nervosa is an eating disorder that is psychiatric changes. D. Notably. pp. the film could be made control and frequent evaluations by healthcare but only shown in an educational setting if consent is providers in those who have diabetes. The nursing facility staff is not smell loss (see Bradley’s NiCP. mydriasis.  Patients can be filmed for educational purposes as (see Bradley’s NiCP. 108.  Dalfampridine is a potassium channel inhibitor an active infection or laceration to their foot. and idiopathic community who follow similar privacy-related policies. PLP). Patients should be educated that these complications The rest of the listed medications do not commonly are preventable and that frequent. The first dorsal interosseous nerve is 91. D. compromise patient information. Binge eating protein. Ch. appropriate to give consent because they are not the 190–196). the best test after finding elevated GH prognosis for facial strength recovery. with permission. Huntington disease. Simon JH 2012 Dysmyelinating and edn. A. and ataxia. medical decision maker for the patient. 19.” Sympathetic input originates in the spine. 25. corticobasal degeneration. As peripheral obtained from the healthcare decision maker before nerves are damaged with excessive blood glucose. depending on the weight gain.  Increasing ring and shoe size and growth of the jaw Ch. 7th edn. One of cellulitis or spread to the bone or bloodstream. The disease. D. there is a milder form less than 18. Ch. Ch. miosis. 1720–1735). in which patients eat more than is typical in a short leading to increased levels of sulfatide.) Neuropathology. This disorder 37. Ch. the main side effects is that it can precipitate seizures. Bradley’s NiCP. The strongly predicts subsequent ocular complications (see parasympathetic system is associated with bradycardia.  This case underscores the importance of glucose decision maker is not present. Parkinson disease. 7th edn. 7th edn. Alzheimer disease. it 1159–1186). The remainder of the Neurology Books . Ch. pp. 7th edn. pp. progressive supranuclear palsy. urinary and bowel evacuation. A CT of the levels in the blood would be an MRI of the brain to temporal bone should be performed to ensure that assess for a pituitary tumor (see Bradley’s NiCP. the night before. the 28. perspiration.  Anosmia is now recognized as an early symptom in filming children. for facial weakness alone). Elsevier. If an used in multiple sclerosis to help improve speed of infection persists without intervention. D. 183–239. motor developmental nervosa in which the patient must have a BMI delay. demyelinating disorders. 7th surgery is not required (as it would not necessarily be edn. There are no BMI criteria for a duplication events occurs in infancy with abnormal eye diagnosis of bulimia nervosa. pp. pp. Filming the patient idiopathic dystonia.  The patient likely has herpes zoster after a sympathetic system may be thought of as “fight or recrudescence of the virus at the trigeminal ganglion. E.90 Neurology Self-Assessment cases. The film should be considered part of several neurodegenerative conditions. and urinary and bowel storage. 52. Although exercise is important for themselves (see Bradley’s NiCP. 31. pp. 7th DeMasters BK. Figure from Kleinschmidt. Ch. and those that Although other. rapid-eye-movement sleep behavior disorder are The film should be kept as secure as other medical frequently associated with a significant loss of smell. 104. This is followed by behaviors to prevent with broad phenotypic variation. are classic signs of acromegaly in adults.  The patient likely suffered a temporal bone fracture relates to increased secretion of growth hormone (GH). and major without telling her is unethical. patient or impaired patient for permission is also Multiple system atrophy. and ejaculation. like mechanically or medically inducing underlying genetic change in the PLP1 gene (and its vomiting or diarrhea. patients lose their vibratory sensation and may not feel 30. and vanishing extensor indicis proprius is innervated by the posterior white matter disease (see Bradley’s NiCP. 696–712). Only 5%–10% of temporal bone which is most commonly from pituitary adenomas. dystonia. Ch. long as informed consent is obtained from the patient or his or her caregiver in cases of dementia or when 35.  The flexor pollicis longus is innervated by the only spastic paraplegia. Pelizaeus-Merzbacher disease. and erections 29. essential tremor. 262–272). unlike anorexia movements and nystagmus. the presentation. C. 58. C. interosseus nerve. innervated by the ulnar nerve (see Bradley’s NiCP. known as Hutchinson sign. pp.  The sympathetic and parasympathetic autonomic nervous systems are complementary. In: Prayson RA (ed. records and destroyed appropriately so as to not Vascular parkinsonism. The abductor pollicis brevis and pathological clue for differential diagnosis): Canavan pronator teres are innervated by the median nerve. 814–834). Pelizaeus– period and/or describe an inability to control what Merzbacher disease is an X-linked disorder of myelin they eat. much rarer. is not the sole preventer (see Bradley’s NiCP. Idiopathic the medical record and as private patient information. but it does not affect disease activity. flight” and the parasympathetic system as “rest and The presence of vesicles on the nose corresponds to the digest. Asking the demented affective disorder are not associated with loss of smell. trigeminal nerve. The disease is caused by marked by discrete episodes of recurrent binge eating impairment of the lysosomal enzyme arylsulfatase A. proactive care cause seizures as a side effect of the medicines should be taken. Colloquially. which also innervates the leukodystrophies classically involve myelin loss in the pronator quadratus and the first two flexor digitorum subcortical U-fibers (which is a good radiographic or profundus muscles. associated with PLP1 missense mutations that causes 33. only three inherited anterior interosseous nerve. If family or the 36.

rather than an antiplatelet agent. would be during an attack of hemiplegic migraine (see Bradley’s calculated at 4 (4% stroke risk per year) because of his NiCP. The scale uses age. as well as decreased motor unit Neurology Books . Elsevier. Instead. this is a 40. and recording smoking status for patients injury and parenchymal changes. E. most notably hemodialysis. the Although symptomatic regions with muscle weakness evaluation should include an MRI and assessment of and numbness may also show decreased compound anterior circulation vessels to ensure there is not a muscle action potential and sensory nerve action symptomatic carotid stenosis. 93. weakness.  The patient presented with acute onset of symptoms contentious use of EHR because it increases the risk of referable to the right hemisphere with resolution of poor documentation and even fraudulent billing. sensorineural deafness. no therapy from a point mutation in mitochondrial DNA and has been developed that reliably stabilizes or reverses causes progressive optic neuropathy and blindness. 7th edn. Although epileptiform activity should be avoided. To date. it may be seen with the syndrome of recorder or Holter monitor. and occasionally is a stipulation of receiving monetary incentives (and lactic acidosis. atrial fibrillation and a dilated right atrium. This associated paralysis may outlast the headache in these patient should have his atrial fibrillation verified on a cases. Figure from Carmody R 2013 Vascular allergy lists. and age <65 years old (see Bradley’s NiCP. Often the further intervention to reduce his risk of stroke. Copying previous notes as a M. The most been a candidate for intravenous thrombolysis given common nerve conduction abnormality in CMT1 is that he was outside of the 4. While various should be started to reduce his overall stroke risk. Ch. Ch. Evaluation should also potential amplitudes. a hereditary sequence). cerebrospinal fluid lymphocytosis. TIA. 39. Cha S. Most cases are attack (TIA). deafness. TIAs warrant admission and further autosomal dominant. D.  Meaningful use of electronic health records (EHRs) dysfunction. 7th edn. however. NiCP. Ch. BP. novel direct thrombin inhibitors or paroxysmal hemicrania. with additional risk of cardiac mental and physical regression. 867–880). Kearns-Sayre syndrome results in normal postnatal development until around 6 months progressive external ophthalmoplegia and pigmentary of age. pp. Symptoms vary widely. Leber hereditary optic neuropathy results swallow. 7th edn. male sex. inability to degeneration. Once admitted. MERRF causes myoclonic epilepsy. and BP were also elevated and warrant various voltage-gated calcium channel genes. which is 38. fibrillation can be further assessed by using the unilateral or bilateral delta activity has been reported CHA2DS2-VASc score. pp. such efficacy is not reported factor 10a inhibitors such as the one listed in choice D for hemiplegic migraine. 91. 1324–1341).  Familial hemiplegic migraine is an autosomal. The MRI. His hemoglobin A1c. myoclonus. 7-day. dominant disorder associated with mutations in LDL cholesterol. failure to stepwise fashion. but include distal hours after TIA is approximately 5%. proliferation (onion bulbs). with permission. Cajun. and progressive paralysis. Once verified. E. rapidly resolving symptoms. Smirniotopoulos J (eds. 7th edn. Kearns-Sayre syndrome is a mitochondrial incidence of Tay-Sachs is particularly high among myopathy that nearly always occurs sporadically individuals with Ashkenazi Jewish. on a loop condition. or French (thought to be related to somatic mutation in utero). diabetes. Test Three 91 options listed are reasonable considerations for new include a transthoracic echo to rule out vegetations. maintenance of (and reconciliation of) medication and 1349–1364).  Tay-Sachs is an autosomal-recessive gangliosidosis due to elevated lactic acid and restricted diffusion on associated with deficient hexosaminidase A activity. facial weakness if there were not preceding trauma and patent foramen ovale. There are many components to thrive. if paroxysmal. symptoms in 1 hour. disease (subacute necrotizing encephalomyelopathy) is Since the creation of this incentive plan in 2014. This patient would not have thin myelin sheath in the bottom left.  All of these diseases are mitochondrial in origin.5-hour window and had slowed conduction velocity due to demyelination. hypertension. often seen with atrial fibrillation. related to duplication of the evaluation. C. E. or cerebellar (with associated cherry-red spot). Although no CSF pleocytosis is described for this 12-lead electrocardiogram or. MELAS is specifically implicated by the question stem 41. In: Naidich T.) Imaging of the template for subsequent patient encounters. 103). short stature. is Brain. as well as one abnormally and 90-day stroke risk. including blindness abnormalities. or atrial clots. Canadian backgrounds. pp. ataxia. given that the stroke risk in the first 48 PMP22 gene. The correct diagnosis is a transient ischemic sensorimotor demyelinating neuropathy. transient headache and neurological deficits with anticoagulation. Castillo over 13 years old. which in this case. including the use of EHR e-prescribing from pyruvate dehydrogenase and cytochrome oxidase capabilities. The electron suggestive of a TIA. 65). headache pathologies are particularly sensitive to Because this patient also has renal failure and is on indomethacin abortive therapy. 7th edn. the devastation of Tay-Sachs disease (see Bradley’s Patients may additionally have cardiac arrhythmias. a mitochondrial syndrome that presents before 2 years meaningful-use requirements have been adopted in a of age with developmental delay. as well helps predict stroke risk in those with symptoms as tremor in a large minority of patients. not considered meaningful use. Signs of Tay-Sachs include Phenotypically. Ch. In this case the 3 temporal hematoma (see Bradley’s NiCP. when children begin to demonstrate progressive retinopathy. D. Leigh Medicaid Electronic Health Record Incentive Program.  The case and the image are demonstrative of (no restricted diffusion on diffusion weighted imaging Charcot-Marie-Tooth type 1 (CMT1). and the presence or nerve axons with surrounding concentric Schwann cell absence of diabetes mellitus to predict the 2-day. The ABCD2 scale weakness and numbness that spreads over time. which results meaningful use. duration of symptoms. leaving him with a negative MRI 43. drug-drug and drug-allergy checkers. deficiency (see Bradley’s NiCP. and ophthalmoplegia. cerebellar 42. clinical microscopy image demonstrates several myelinated features. examination showed a rhythm likely consistent with 62. It is caused by a mutation in ultimately avoiding penalties) from the Medicare and mitochondrial DNA encoding the lysine tRNA. Risk of stroke in those with atrial has not been reported with hemiplegic migraine. Ch.

The recurrent artery of Huebner is the largest (Collier sign). increases as percent resection increases). psychosis increases when 45. Mobius syndrome asymptomatic arms (see Bradley’s NiCP. neuromuscular transmission can increase the variability 49. 50. With single-fiber is given. There is no edn. 95. A stroke in this artery Neurology Books . track signs are present. 7th edn. There is no atrophy of the mesial temporal lobe and clinical measure of the increased variability in firing temporoparietal junction. pp. 74. Patients are noted from infancy to Mahadouche T. Human of one of the muscle fibers on SFEMG. and facial nerves. levels as psychosis increases when amphetamines and as opposed to an entire motor unit (which is cocaine are given (which also stimulate dopamine composed of many muscle fibers) on a regular EMG. pp. 18. 1049–1064). SN. Ch. Given the paramedian thalamus’s present at an earlier age (fifth to seventh decade). Argyll Robertson important for pathological diagnosis and molecular pupil results in bilateral. Parkinson disease would show loss of Blocking is the failure of neuromuscular transmission pigmented neurons in the substantia nigra. B. perforating branch arising from the proximal anterior and convergence-retraction nystagmus on attempted cerebral artery. of firing times and therefore increase jitter. individual muscle fibers are recorded. resonance imaging of glioma in the era of psychosis improves when dopamine is blocked. about one third of Straussler-Scheinker disease. Ch. However. the most common primary brain tumor are slow to respond to direct light reflex. due to usually has a slower course and a longer survival than ischemia in the reticular activating system. immunodeficiency virus dementia has no identifying also measured on SFEMG. which branches to supply both penetrance. 205–216). there appears to be a loss of GABA These individual fibers are stimulated by the same neurons in the hippocampus. Unilateral occlusion of the 47. 7th edn. Linetsky M. release). There may also be increased norepinephrine EMG (SFEMG). from the presynaptic terminal (see Bradley’s NiCP. with so-called boxcar ventricles. Adie’s Marie-Tooth diseases: an update. genetic prion disease of the basilar artery may also lead to coma. paralysis of upgaze and accommodation. and levodopa. increased psychosis as evidenced by disease 7th edn. pp. but generally in adults. pp. and is the anterior lentiform nuclei. Figure from Tazir M.  All of the neurotransmitters listed play a role in bevacizumab) are standard therapy for glioblastoma. Gerstmann. Serotonin is also increased (especially 5-HT2a) in with permission. PET Clinics 8(2): 163–182. chronic neuropathies. pp. frontal atrophy. Focal pp. the anterior limb of the internal capsule. A. 35. generally concurrent cranial neuropathies and/or long Ch. pp. is a congenital syndrome of underdeveloped abducens 107. D. occlusion early movement symptoms and late personality and of the artery of Percheron may lead to coma. Pope WB 2013 Magnetic dopamine agonists. This important role in a patient’s overall survival (survival syndrome is classically associated with tertiary syphilis. in which the affected pupil locations or in patients too ill to undergo major reacts consensually. but not directly. The artery insomnia) are all associated with autosomal-dominant of Percheron is a common pedicle from one posterior mutations in the PRNP gene with nearly 100% cerebral artery. Mathis S. below therapeutic levels for glioblastoma. from the posterior cerebral arteries. disorders that affect psychosis. This syndrome localizes to the dorsal midbrain caudate. Maximal surgical resection is not only respond better to accommodation. although sporadic prion diseases (see Bradley’s NiCP. Finally. as in the case of optic neuritis (see Bradley’s the fact that usual whole-brain radiation doses are NiCP. Occlusion cognitive symptoms. Chs. 1380–1421). Ellingson BM. edn. with permission.  The image demonstrates atrophy of the caudate. arising into familial Creutzfeldt-Jakob disease. estimates the number of gross pathological findings (see Bradley’s NiCP.  Genetic forms of prion disease (which are divided generally supplied by the paramedian arteries. psychosis. to light. For example. bilateral weakness with abduction of the eyes. 7th single-fiber potentials firing synchronously. E. which is characteristic of myopathies. which leads to less axon and should fire within a relatively close time of inhibition of excitatory pathways implicated in each other. 179–189 and 21. innervation to the iris sphincter. Additionally. Whole-brain radiation is rarely ever used for common localization for this syndrome is the optic glioblastoma due to the toxicity of the therapy and to nerve. Dopamine (D2 receptor mostly) leads to but almost always postoperatively (see Bradley’s NiCP.92 Neurology Self-Assessment recruitment.  The history and MRI are both compatible with remain large compared with the contralateral pupil and glioblastoma. Genetic forms of prion disease generally paramedian arteries. 7th Saunders. and region. 3rd edn. with importance in maintenance of consciousness. Stereotactic Marcus Gunn pupil is another name for a relative biopsy is reserved for tumors in otherwise inoperable afferent pupillary defect. Classically. Pick disease would show time between two motor units in the same muscle. Fiber density. and motor neuron Huntington disease. Figure from Klatt EC clinical measure of the number of quanta released 2014 Robbins and Cotran Atlas of Pathology. It delivers blood to the head of the upgaze. as described in the question stem. psychosis. Alzheimer disease may show disease can all cause similar changes. abnormally small pupils that characterization of the tumor. Journal of the pupil results from a defect in parasympathetic Neurological Sciences 347(1): 14–22. radiation and chemotherapy (temozolomide and/or 48. The most surgery. muscle fibers in the same motor unit. antiangiogenic therapy.  Whereas the paramedian thalamic blood supply is 46. Affected pupils tend to 44. 1365–1379). but also plays an respond to accommodation but not light. a serotonin agonist.  Jitter is the variability in firing time between two lysergic acid diethylamide (LSD). Ch. C. Vallat J-M 2014 have bilateral lower motor neuron facial weakness and Hereditary motor and sensory neuropathies or Charcot. with permission. E. In addition. 1791–1866).  Parinaud syndrome is composed of light-near middle or posterior cerebral arteries would not lead to dissociation of pupillary response. Houioua S. 7th edn. Ch. eyelid retraction coma. this would be less likely in the patient’s commonly seen with pineal tumors. Figure from Khan exacerbation under the influence of amphetamines. 94. 366–390). C. and fatal familial humans have variant anatomy in this region.

and mostly in girls and is caused by a mutation in the encephalopathy. 102. Test Three 93 may lead to contralateral arm and face weakness. sleep disorders. and alcohol the following: appetite change. an X-linked dominant condition that occurs 51. Pressures below this range protein (metabotropic) that inhibits adenylyl cyclase. There is no a medium half-life. metabolic derangements such as acidosis.  Normal cerebrospinal fluid pressure is between 6 considered safe to give to those with liver dysfunction. ataxia. hypoglycemia. 91. pp. L-carnitine and a low-protein diet. Fragile X the temporal lobes. In this case the tegmentum show the highest firing rates during REM supplementation of lysine and tryptophan would sleep and are considered REM-generating neurons. hypocretin choice (see Bradley’s NiCP. Figure from Kurtcan S. and seizures. Alehan F 2012 Vici syndrome hyperammonemia. and concentrations of glutaric acid and 3-hydroxyglutaric typical physical characteristics including long face. if the same chromosome hydroxylysine. caused by a maternally This is caused by deficiency of the riboflavin-dependent inherited deletion on the long arm of chromosome glutaryl-CoA dehydrogenase (GCDH). hepatically metabolized. Autism includes a broad spectrum of hemorrhages. seizures. Imaging shows enlargement of the sylvian verbal and nonverbal communication skills. Treatment consists of withholding protein from ears. the child typically present during an episode of illness with an develops Prader-Willi syndrome. hand flapping. making this the incorrect answer Norepinephrine (in the locus ceruleus). specifically glutaric acidemia type 1 (GA1). low self-esteem. The artery of of the arachnoid villi. Clinically. Ch. The diagnosis is consistent with organic a number of other neurological deficits. constellation of findings is consistent with Aicardi 7th edn. Ch. and alprazolam has a short suggestion for mania. syndrome. There is no conductance of chloride (ionotropic). D. indicate a spinal fluid leak. which is a 15. chlordiazepoxide.  Dysthymic disorder must be present for at least 22 56. and correction of Ch. generalized convulsions. All of these this range can arise from a wide variety of causes. intravenous hydration. and electrolyte associated with sensorineural hearing loss and abnormalities. medications can cause dependence if taken consistently including idiopathic intracranial hypertension. seizures. Notably. valium. Aksu B. lorazepam and temazepam have carry on daily function (such as a job). Some patients may have seizures or subdural abnormalities. artery and supplying the bulk of blood supply to the 54. Özkale Y. Ch. 89. Rett syndrome occurs ketoacidosis. seizures. C. and hyperphagia. lorazepam is uniquely 53. Major formulations of benzodiazepines have different depressive disorder or episode would not be the correct half-lives that distinguish one from another. pp. autistic features. pp. characterized by poor child abuse. with and blindness from chorioretinal lacunae. as well as increased signal intensity syndrome is caused by a mutation in the FMR1 gene in the bilateral basal ganglia. 1279–1300). 44. Gümüş A. elevated ammonia. central and should not be abruptly withdrawn. months in a 2-year period and include at least two of phobias. It includes global confirmed by identification of elevated urinary developmental delay. or slow drainage well as contralateral hemichorea. patients 15 mutation is inherited from the father.  Cholinergic neurons in the pedunculopontine development in children with GA1. hypoglycemia. as both of these typically have severe example. Brain and Development 37(5): 546–551. the diet. earlier.  The patient presented with a respiratory illness in females only (lethal to males). The different psychosis to suggest schizoaffective disorder. large acid. and gastrointestinal seen. rendering the patients unable to hold or have longer half-lives. E. 88. poor fissures and the cerebrospinal fluid spaces anterior to social interaction. as with leptomeningeal 3 Adamkiewicz is the largest radicular artery in the spine. 52. causing MR. This preserved dorsal column sensation (see Bradley’s NiCP. with shows the highest firing rate during wakefulness (see permission. 7th edn. with an early diagnosis. help to promote normal 55. Bradley’s NiCP. Erol I. indicated. B. leads to an anterior spinal artery-type syndrome. as nervous system infection. causes a very happy/pleasant demeanor with mitochondrial enzyme that converts glutaryl-CoA to associated mental retardation (MR). The diagnosis is and occurs mostly in boys. Riboflavin and L-carnitine are also laryngomalacia Pediatric Neurology 47(5): 375–378. 7th edn. 7th edn. Alkan A. acute metabolic decompensation. Iscan A 2015 MRS features during tegmental area and periaqueductal gray) are all encephalopathic crisis period in 11-year-old case with involved in promoting wakefulness. hand wringing. crotonyl-CoA in the breakdown of lysine. 1615–1685). worsen the condition. Infarction here usually spasms. serotonin (from the 1324–1341). Most are never without a depressed ligand-gated ion channels and allow for the mood and are often resistant to therapy. If toxicity Neurology Books . mass lesion. and medication works on the GABAA receptors that are hopelessness. (from the lateral hypothalamus). and enlarged testicles (see Bradley’s NiCP. 7th edn. Notably.  The case describes a girl with a history of infantile lumbar cord and cauda equina. coupled with permission. Figure from Özkale M. arising from the caudal portion of the anterior spinal pp. For answer choice. and dopamine (in the ventral Guler S. mental retardation. sleep disturbance. Patients often present accompanied by acute deterioration of his mental in infancy with infantile spasms and progress to have status. The locus ceruleus GA-1. dystonia and dyskinesia of the mouth and face are regression. Angelman syndrome. low withdrawal and to induce anesthesia. whereas pressures above Baclofen works on GABAB receptors. Extraneous movements such as MECP2 gene that leads to developmental delay. This class of energy. B. and clonazepam consequences. making bipolar disorder less half-life. Ch. dorsal raphe nucleus). The MRI paraplegia and urinary and fecal incontinence but demonstrates agenesis of the corpus callosum. whereas most benzodiazepines are likely. A.  Benzodiazepines are usually given to treat anxiety. as noted acidemia. and tryptophan. which may be confused with evidence of neurodevelopmental disorders. and repetitive behaviors. poor concentration. and 25 cm H2O in adults in the lateral decubitus The GABAB receptors are coupled to an inhibitory G position with legs extended. pp. metastatic disease (see Bradley’s NiCP. 528–572). 1261–1278). characterized by hypogonadism.

flumazenil is the antidote of choice. C6 contributes to from the radial nerve from the posterior cord).) Neurology Books . 7. 6. Magnetic Resonance Imaging Clinics of North America 20(4): 791–826. Yousem DM. 7) Mid C8 ep al in s Be r m che Te ran b l ior T1 ra er at e Inf Musculocutaneous L nerve (C5. C8. 6. e calen Inters e ria ngl Some or T ts teri 5 roo mi of Pos ians (ve n t r a l ra s) ve Contribution e c hnic spina l ner from C4 T s Dorsal scapular unk Dorsal e 3 tr cl nerve (C5) ramus a vi To phrenic Cl s Suprascapular n’t on s isi sion nerve Do i v d iv i nerve (C5. 6. 8. then merges to the posterior cord rhomboids are innervated by the dorsal scapular nerve before bifurcating to the radial nerve and axillary nerve. 6) Figure for Answer 3. T1) Medial brachial cutaneous nerve (T1) Medial antebrachial cutaneous nerve (C8. The the superior trunk. T1) Upper subscapular nerve (C5. with permission. The extensor carpi ulnaris is innervated which is derived from the posterior cord of the by the posterior interosseous nerve (C7. As seen in the figure.57 (from Mikityansky I. 7) Medial pectoral nerve (C8. 106. T1. 8. medial cord). Loevner LA 2012 MR imaging of the brachial plexus. and is a pectoral nerve (C8. 6) C5 ior d To subclavius t er rior nerve (C5. medial cord). branching brachial plexus. 6) n e lla 3 a post i C6 Ax 3 ry ar r C ds erio r Sup C7 co 3 rm Lateral pectoral A rs dle e nerve (C5. 8) Lower subscapular nerve (C5. 8. D.94 Neurology Self-Assessment occurs. 6) al M edi muscles Radial nerve (C5. The pectoralis major is innervated by the medial Ch. The adductor GABAA receptor antagonist. 6.  The deltoids are innervated by the axillary nerve. 1766–1790). pollicis is innervated by the ulnar nerve (C7–T1. 7th edn. 7. 7. 57. T1) Ulnar nerve (C7. T1) 1st intercostal nerve Median nerve Long thoracic (C5. 6. 7. pp. 6. 6) Inconstant contribution Thoracodorsal (middle subscapular) nerve (C6. Zager EL. from the C5 nerve root (see Bradley’s NiCP. T1) nerve (C5. 8) (C5. 7) r rio ste Po Contribution 1st rib from T2 Axillary To longus colli and scalene nerve (C5.

may be associated with psychosis. reducing seizures in patients with refractory confusion and amnesia without focal neurological epilepsy. most commonly caused by hypertension. B. medications encephalopathy. 7th seizures follow. otherwise. if no catheter is present. Most often. but typically has multiple severe reactions. Saunders. diagnosed by the presence of elevated phenylalanine in and even death. spasticity. 66. it should be checked to ensure it is draining MSUD is an autosomal-recessive disease due to a properly. 3rd edn. This is and cerebral vasculitis can cause intracerebral usually exquisitely responsive to levodopa (see hemorrhages but occur at significantly lower rates than Bradley’s NiCP. usually such as downward dislocation of the lens. which then used during resuscitation. infants with PKU are normal at birth but the spinal cord lesion. later develop developmental delay. Above the level of the lesion. Felbamate has the potential serious side effect transient bilateral diffusion abnormalities in the of aplastic anemia and requires frequent blood counts. glioblastoma. and supportive treatment. PKU is attacks. Treatment should commence However. but usually there are abnormal movements threatening. developmental delay. The next step is to (most common cause of death). 7th edn. Test Three 95 58. pp. not low enough to require atropine and that would not chorea. Other activity. such as homocysteine in the plasma and urine clenches the tight compressive clothing. pp. Transient one of the best tolerated antiepileptic medications. ischemic attacks cause focal neurological findings. Levetiracetam. and thromboembolism events flat can further raise blood pressure. Lorazepam may help lower Nonketotic hyperglycinemia is also autosomal recessive pain or anxiety around the episode. Phenytoin has can cause confusion and amnesia. possible. Atropine is system. and anaphylactic reactions a phenylalanine hydroxylase deficiency. seizures. dextromethorphan (which depletes the N-methyl-D- aspartate receptor). or appendicitis. analog that acts as an irreversible GABA transaminase 62. but this patient’s heart rate is leads to neonatal encephalopathy. resolve the episode. and even seizures. although generally that would point toward this diagnosis. leading to bradycardia. 7th edn. pancreatitis. an neurotransmitter synthesis. 59. and hiccups. The fecal disimpaction. cerebral amyloid angiopathy. Finding elevated ensure there are no obvious positional lesions. PKU is uncommon disorder in spinal cord injury patients that identified on a newborn screen. 3 phenylalanine buildup that inhibits protein and 60. Vigabatrin can cause severe and irreversible findings or clinical seizure activity. Treatment includes a uncomfortable position in the bed. place. 101. Typical causes include bladder distention Treatment includes dietary restriction of phenylalanine (no catheter in place. marfanoid placing the patient in the sitting position because lying habitus with thin bones. DYT-5 is 61. but this will not and is due to an enzyme defect in the glycine cleavage resolve the underlying cause of the episode. retardation and seizures just like patients with PKU. seizures. as evidenced by vision. and so on should treatment of MSUD includes immediate and lifelong be investigated. 1563–1614). aggressive behavior. often with a sweet smell of maple syrup. Alcohol withdrawal depression. which is consistent vision loss. they have other associated clinical features immediately and involve changing positions.  The patient presents with autonomic dysreflexia. field constriction. leading to episodic did not have one due to a lack of healthcare access. including arrhythmias other findings such as agitation. necessary. 968–980). edn. hypertension. followed by an attempt at ataxia. This leads to elevated glycine levels.  Vigabatrin is a gamma aminobutyric acid (GABA) with permission. Normal saline will not do anything to diet restriction with no branched-chain amino acids. tremor. and even life. 89. Ch. C. appendicitis. but other types of pain Clinically. Ch. The exact etiology is unknown. rash. Complex partial status epilepticus is Lamotrigine can cause a serious. although rarely it can months during treatment. or a limb or toe in an diagnosis of homocystinuria. and 91. and intact baroreceptor responses activate parasympathetic hypopigmentation (due to poor melanin). 1279–1300 hypertensive hemorrhages (see Bradley’s NiCP. Alzheimer Neurology Books . MSUD causes neonatal do not lower blood pressure sufficiently. bowel for life. strokes. hypotonia.  PKU is an autosomal-recessive condition caused by hepatotoxicity. They also have a musty or blood pressure and heart rate can result in heart mousy odor due to elevated phenylketones. although this patient involves a sympathetic surge. which usually starts as concentric visual with an episode of transient global amnesia (TGA). hemorrhage. 1324–1341). leukopenia. pp. but severe swings in behavioral disturbances. Quick recognition of this syndrome is the blood and decreased tyrosine in the blood. If all of these measures do not diagnosis is made from elevated leucine (branched. and severe These episodes are usually brief. (particularly with intravenous [IV] infusions). 881–903). deficiency in branched-chain alpha-keto acid catheterization should be performed. 7th edn. If a Foley is in low-methionine diet with cysteine supplementation. uncomfortable positioning). then the search for other potential chain amino acids) in the blood and urine. small stature. not guarantee improvement or even stabilization of but it may have a vascular etiology. which leads to decreased dopamine and diurnal Metastasis. and can be given to lower it. help resolve this episode. coma. or pain (tight clothing or from and is due to cystathionine beta-synthase deficiency. leading to a (see Bradley’s NiCP. Vision testing is required every 3 TGA usually occurs only once. A. Treatment includes sodium benzoate. diaphoresis. 63. D. The causes like pancreatitis. D. Homocystinuria is also autosomal recessive distention/impaction. tachycardia.  The picture demonstrates a large lobar intracerebral most often due to a tyrosine hydroxylase deficiency. intracerebral hemorrhages. dystonia that often mimics cerebral palsy. and discontinuation does occur multiple times.  The patient presented with an episode of mild inhibitor. hypertension and dry or pale skin below the level of Clinically. blocked catheter line). Profound mental retardation and treat the underlying problem (see Bradley’s NiCP. and encephalopathy. hippocampi. If these measures dehydrogenase deficiency. Figure from Klatt EC 2014 Robbins and Cotran Atlas of Pathology. and flushing. pp. sweating. pp. Ch. these patients present with mental can trigger episodes such as cholecystitis. which this patient did not have. features include seizures. Chs.

no indication here that the patient is suffering from Accepting a diagnosis despite contrary evidence is a fibromyalgia. intelligence quotient (IQ) test. a few). Ch. pp. A NIF of <30 cm H2O or forced vital diagnosis (see Bradley’s NiCP. EMG and assesses attention.  The main treatments for a myasthenic exacerbation capture the lateral femoral cutaneous nerve as it are either IV immunoglobulins or plasmapheresis.  The Trail tests evaluate executive function. Trails A examination that suggests Lyme disease. She has a very low vital capacity and nerve. meningitis in the setting of acute human immunodeficiency virus (HIV) infection or 67. Starting 69. The Clock Draw Test it is technically challenging to perform. and executive nerve conduction studies are not necessary for the function. Ch. 7th edn. out a diagnosis is a cognitive bias called confirmation arthralgia.  Type I errors are a statistical measure used to cognitive behavioral therapy along with treatment of describe an incorrect rejection of the null hypothesis. A block of the femoral nerve would not 66.96 Neurology Self-Assessment disease may show day-to-day fluctuations in cognitive capacity and NIF are on the edge of requiring abilities. not to blame other physical symptoms on migraines. As the sequential order. The PNS is involved with slowing of methylprednisolone has no role in acute myasthenic cardiac conduction. sweating. Seeking out of exclusion. 1896–1915). C.  This patient most likely has somatization disorder. both branches from the femoral nerve proximal to the of which are quite effective. and bilateral evaluates visuospatial abilities and parietal lobe studies have to be performed for comparison. They can be performed to rule out other and aphasia in which patients are to identify pictures causes like radiculopathy. and cranial neuropathies to name bias. crises because it can worsen exacerbations. 7th edn. low NIF. and have comorbid psychological generally resolve within 1 to 2 weeks (see Bradley’s disturbances of mood and anxiety. the condition can be present in the types of memory to further define specific deficits presence of normal electrodiagnostic studies. myalgia. A. 742–758 and Ch.4 L for a 70-kg patient) is 1973–1991). comorbid psychological dysfunction. 55. At the time of presentation. Prednisone immunosuppression is the condition that should force an early delivery. however. headache. there is nothing on workup or physical 65. D. decreased cardiac contractility.  The parasympathetic nervous system (PNS) will prednisone could tip the balance and lead to her increase detrusor contraction leading to micturition if requiring mechanical ventilation. IV the bladder is full. disorders typically lead to excessive and unnecessary The acute aseptic meningitis and associated symptoms medical workup. A type I error is essentially a false-positive test. at least one neurological symptom. 112. pp. This assesses processing speed. 63. D. There is type of cognitive bias called diagnosis momentum. prednisone in the short term can precipitate or worsen The lateral femoral cutaneous nerve is a purely sensory an exacerbation. Type II errors are believe the patient is malingering with hopes of the opposite of a type I error. In these patients. The decision depends on ilioinguinal ligament. which put her at a high risk of requiring Lumbar puncture would not be helpful in the intubation. Patients may have cranial neuropathies in which there is usually a history of physical and/or in this setting as well.  This patient presents with meralgia paresthetica. so acute treatment of her myasthenia followed by recovery of normal cognitive function (see should be a priority. The lateral femoral cutaneous of objects. Ch. there are multiple testing (enzyme-linked immunosorbent assay or recurrent somatic complaints of pain in at least four Western blot) may be negative. constriction of pupils. HIV emotional abuse. E. Somatization treatment should be deescalated with negative results. and the patient 68. Bradley’s NiCP. 7th edn.  This patient is most likely suffering from aseptic pp. The Wechsler Adult Intelligence Scale is an nerve can be evaluated by nerve conduction. Local injections to the nerve can 43.” Treatment involves 64. which entraps the lateral femoral cutaneous nerve. indicative of impending respiratory failure. seroconversion. Although a patient may begin sexual complaint. Although this This occurs when a test is positive showing a patient patient does endorse headaches. 1102–1120). The Wechsler Memory Scale evaluates several Furthermore. The Boston Naming Test is a test of language diagnosis. 920–967). Trails pregnancy progresses. and Azathioprine is used in long-term management of lacrimation. Unless there is an obvious reason to cognitive bias called anchoring bias. the enlarging abdomen places B involves drawing a line from alternating letters and pressure on the ilioinguinal ligament. Ch. C. must correctly draw a line from 1 to the last number in This is a common condition in pregnancy. but fitting clothing around the waist can also be beneficial. the question stem does not provide sufficient Accepting a prior diagnosis without confirming it is a detail to meet diagnostic criteria for migraines. however. one should reserve this as a diagnosis hypothesis is false but is not rejected. two gastrointestinal symptoms. involves numbers scattered on a page. note that these symptoms are not intentional. Further. involved with pupillary dilation. concentration. Further. although Lyme disease can cause confirmatory evidence without ordering tests to rule a number of symptoms and signs (fever. In the case of pregnancy. in which the null secondary gain. her vital cardiac conduction and contractility. 109. can worsen myasthenic exacerbations (see Bradley’s NiCP. this is not a treatment. and empiric therapy until cultures and PCR testing returns. so no weakness is involved in this condition. The sympathetic nervous system is myasthenia to help reduce chronic steroid use. but after seroconversion different sites or functions along with at least one it will be positive. constriction of bronchioles. the comorbid conditions and availability of each main treatment is delivery. pp. 77. capacity of <20 mL/kg (1. It is important to NiCP. pp. but there are not discrete episodes of amnesia ventilation. 65. 7th edn. increasing Although the patient may need antibiotics. which then numbers in sequential order until completed. is primarily clinical. function. fluoroquinolones. pp. 511–527). Diagnosis patients might have (see Bradley’s NiCP. and dilation of Neurology Books . Finally. especially ciprofloxacin. one must be careful has a disease but they do not actually have that disease. Loose- mainstay of long-term treatment for myasthenia. nor do they imply the patient is “faking. Ch. D. be helpful.

Ch. none have reported that patients regain In dementia with Lewy bodies there is significant the ability to ambulate. year. B. However. and areas of 75. and proprioception. including central cord.  Fluorodeoxyglucose PET scans are increasingly associated with neuropathy (see Bradley’s NiCP. mucosal ulcers and pathergy more strongly commonly associated with small cell lung cancer. Fluorodeoxyglucose (FDG) is metabolized by the active brain. 26. should be avoided long term. Cord loss but no weakness. he has radicular or cardiac arrhythmias. Ch. Although sensory ganglionopathy (also known as sensory sarcoidosis is a consideration in the differential neuronopathy or sensory polyganglioneuropathy). personality changes. and striatum. surgically reversible compression is present. Test Three 97 the bronchioles (see Bradley’s NiCP. 106. conservative contusions often result in venous congestion of the management is recommended initially. there may be significant develop spasticity. 273–278).  Rituximab is a CD20 monoclonal antibody that is fine touch. however. Ataxia used in a variety of autoimmune conditions such as from spinal cord injury often relates to injury of neuromyelitis optica and refractory myasthenia gravis. disease causes hypometabolism in the frontal lobes numbness. It is most common in individuals from the pp. antibody used in the treatment of multiple sclerosis. including swinging blood pressures traumatic in origin. Ch. and may therefore affect the upper limbs activity modification. pp. There is an additional contribution from this spinal although the evidence is more limited for these. 7th edn. In frontotemporal dementia and primary Within weeks. through which joint position Fingolimod is a sphingosine-1-phosphatase inhibitor sensation is transmitted to the cerebellum. studies have In Alzheimer disease. Patients pp. and antiepileptics (phenytoin.  The substantia gelatinosa is mostly a column of steroids may be used in those who have persistent interneurons. or human enhancement on MRI. 107. common cause. 1867–1895). Ch. severe radicular pain that is refractory to other agents. which involves chorea.  Behçet disease is an immune-mediated small vessel the treatment of multiple sclerosis. stereognosis. E. This is diagnosis. 7th edn.  This patient presents with signs and symptoms of a nervous system. pp. In this setting. 41. Middle East and Asia. 7th edn. indicate Behçet’s. lamotrigine. Only column of producing natural opiates. may have severe autonomic instability with severe 72. thiamine toxicity. Upon presentation. Ch. Systemic or epidural 76. it generally causes enhancing purely peripheral sensory disorder consistent with a inflammatory lesions as described. A. preceding infection or immunodeficiency virus. and decreased sphincter tone. Vibratory Ch. typically from the ANNA-1 antibody Neurological complications of lupus are present in (anti-Hu antibody). C. Paraneoplasia is the most vaccination. and refractory seizures and is not 71. B. 814–834). transmitting noxious stimuli afferently.  The patient has an L5 radiculopathy. Multiple sclerosis would have multifocal motor neuropathy. 63. 1791–1867). 7th edn. but edn. Alemtuzumab is a CD52 monoclonal antibody used in 3 70. Anti-Jo antibody is seen in 25%–50% of patients. lone remains incompletely understood. However. 58. 486–503). uveitis. neurological decline with weakness is surgical Monoplegia and absent reflexes would be a result of discectomy an option (see Bradley’s NiCP. especially with patient pain in the L5 distribution with corresponding sensory manipulation or with bowel movements. E. pathergy reaction. carbamazepine. which is likely spinal cord injury. Multiple vasculitis associated with mucosal ulcers. along with 73. Corresponding spinal anatomy is shown (see Bradley’s Natalizumab is an alpha-4-integrin adhesion molecule NiCP. 881–902). 7th used to assist in the diagnosis of undifferentiated edn. response. 108. Ch. memory complaints. When Behçet’s affects the central 74. and is not associated with mucosal ulcers. 7th edn. but would not have associated syndrome. nonnarcotic pain medication more than the lower limbs. Anti-GM1 antibody is commonly seen in Behçet’s than lupus. 676–690). the concurrent inflammatory myopathies associated with interstitial symptoms in this patient are more suggestive of lung disease. areflexia. 7th medication may also be used in the short term. ADEM generally has associated Sjögren syndrome. Opioid pain level and sphincter tone loss (see Bradley’s NiCP. Clarke’s column. Ch. leading to papules at sites of minor and valproic acid) are voltage-gated sodium channel cutaneous trauma due to robust inflammatory blockers (see Bradley’s NiCP. which reduce the if the pain is refractory or the patient has progressive production of substance P from nociceptive neurons. pp. pp. Anti-NMDA receptor antibody is seen in ulcers or pathergy (see Bradley’s NiCP. tone increases and patients progressive aphasia. the posterior parietotemporal shown small gains in sensory and motor outcomes at 1 region and posterior cingulate are often affected first. used in the treatment of multiple sclerosis. On examination. pp. Traumatic spinal cord injury hypometabolism in the occipital lobes. sensation travels in the nucleus proprius.  Although the effect of high-dose methylpredniso­ hypometabolism suggest decreased functional activity. Neurology Books . injury to the ventral horn motor fibers. 51. Huntington often presents with “spinal shock” with plegia. B. pp. 7th edn. Anti-GQ1b antibody is enhancing lesions and vision problems (optic neuritis seen in the Miller-Fisher variant of Guillian-Barré rather than uveitis). but still have a sensory with NSAIDs. and physical therapy. 1766–1790). any acute spinal hypometabolism in the frontal lobes and anterior cord injury should have an immediate MRI in case a temporal lobes (see Bradley’s NiCP. NMDA receptor encephalitis.

nausea. Nimodipine is used to reduce the risk of left-handedness or eyeglass prescriptions. et al. Pt. 2013 Detecting subarachnoid posttraumatic stress disorder from her experience.  The CT scan and history suggest a subarachnoid 79. pp. patients with coiling of the aneurysm. with permission. Clarke‘s column (T1-L3) I II Lateral basal nucleus III IV Spinal reticular zone V Intermediolateral cell column. vomiting. European Journal of Radiology 82(9): 1539– amnesia. characterized by the inability to recall personal or and meningismus. Spinal Cord and Peripheral Motor and Sensory Systems. Vol. typically associated with a xanthochromia on lumbar puncture if >12 hours after traumatic event.76 (from Jones HR. Depersonalization is the feeling of detachment from Treatment of ruptured aneurysms includes clipping or one’s own body or self. seizures. nausea. vomiting. The Fisher grade is a differential.  α-latrotoxin is the neurotoxin produced by black hemorrhage (SAH). which causes release and blocks Neurology Books . Burns E. Other dissociative disorders listed include hydrocephalus. and even right/ deficits. such as headache. (see Bradley’s NiCP. drugs. Although there is memory impairment. Figure from Verma RK. but others will remain amnestic to the kidney disease. Causes of SAH include trauma. or 67. It is often seen on CT scan or by situational information. Complications of SAH include event forever. which is a scale of 1–5 The current case does not mention external trauma to that takes the patient’s clinical appearance and helps the head that would make concussion high on the predict prognosis and outcome. and is or more distinct identities. SAH is graded and unable to control their own actions or movements. 7. C. pp. Pomeroy S 2013 The Netter Collection of Medical Illustrations: Nervous System. 981–995). Often. and dissociative identity disorder (previously called multiple vasospasm. but hemorrhage: comparison of combined FLAIR/SWI the initial diagnosis is better explained by dissociative versus CT. Aminoff MJ. rebleeding. scale that classifies the radiological appearance of SAH there are no other features of concussion mentioned. thoughts. VI sympathetic preganglionic neurons (T1-L2) X VII Intermediomedial cell column VIII IX IX Flexors IX Motor neurons of limbs (cervical Extensors and lumbar enlargements of cord) Distal part of limb Proximal part of limb Motor neurons of trunk and neck (C1-3 and T2-12) Figure for Answer 3. one should consider the possibility of a defense mechanism. photophobia. ruptured aneurysm. pp. Saunders. the patient may later develop Andereggen L. 2. with each identity unaware associated with worsening of neurological examination of the others. 7th edn. gender orientation. nausea. D. Finally. 78. vomiting. or periods and go beyond that of typical amnesia. This exists when a person has two (peaks at 1 week) from the onset of bleeding. using the Hunt and Hess scale.  Dissociative amnesia is a dissociative disorder thunderclap headache. hyponatremia.98 Neurology Self-Assessment Nuclear cell columns Laminae of Rexed Nucleus posterior marginalis (marginal zone) Substantia gelatinosa (lamina II) Nucleus proprius of posterior horn Nucleus dorsalis. Vasospasm typically presents 3–15 days personality disorder). 49–78. Given her history of kidney Memories are still present but repressed as part of a disease.) 77. Kottke R. phonophobia. photophobia. which typically presents with a widow spiders. 1545. There may be differences in speech. 742–757 and such as headache. Gaps of memory often exist for long onset. along with management of depersonalization will feel as if they are in a dream the complications of SAH. Chs. Typically. with permission. or new focal attitudes. vasospasm and is often taken for at least 14–21 days. E. 55. Some will eventually recall details ruptured aneurysm in association with adult polycystic of the event.

52. The Saxitoxin works similar to tetrodotoxin. can persist for longer. A trial of carbidopa/levodopa would be dopaminergic agents or agents such as calcium channel appropriate for dopamine-responsive dystonia. Although chorea can (2) at times of inactivity (3) or in the evening (4) that be mistaken for tics. the anterior pituitary gland. inhibiting action potential formation in somatic and with permission. antistreptolysin-O and anti-DNase B titers. radiculopathy. Further. RLS may also be secondary and associated clinical syndrome described. more commonly associated with semicircular canal 80. of vertigo with oscillopsia and frequent nausea. dysphagia. A. leading to gravitational settling. given the as latrodectism. and hyperactivity.  Restless legs syndrome has five diagnostic criteria. Symptoms diabetes insipidus. involve the posterior pituitary as well and lead to cholinergic. pp. chorea is usually asymmetrical and can be associated 83. postpartum hemorrhage and associated hypovolemia. Cold allodynia and possibly no nystagmus with this maneuver at all. 1237–1253). Simultagnosia is the loss of 21. then preexisting pituitary adenoma or lymphocytic pain and involuntary muscle contractions in the hypophysitis. tics are usually accompanied by a is usually relieved by movement (5) and is not premonitory urge followed by a sense of relief after the accounted for by another medical or behavioral action is performed. also known hypothyroidism. with each movement 81. Habit reversal therapy is 84. and selective serotonin The hemorrhage is thought to result from ischemia in reuptake inhibitors (see Bradley’s NiCP. 7th edn. and aminergic nerve endings. Saunders. adrenal insufficiency. this is known postpartum headaches. Ch. Evaluation typically includes Prosopagnosia is the inability to recognize faces. lithium. C. The lateral canal otolith position more likely paresthesias that begin around the mouth and spread produces down-beat nystagmus with Dix-Hallpike or to the limbs and perineum. 7th edn. is consistent with Sheehan with iron deficiency. and ataxia. caused by otolith malposition in the semicircular dysarthria. paradoxical temperature reversal (sensing cold as Cerebellar vermis lesions tend to cause gait and trunk unbearably hot) are common. causing symptoms of sudden. Respiratory failure may occur due to other information regarding endocrinological diaphragm involvement. This. and relates to obsessive-compulsive disorder.  Astereognosia is the inability to identify objects by with other behavioral issues such as irritability. pp. 1422–1460). Ciguatoxin is found upright to a prone position with neck extended. 102. The in the ciguatera fish and modulates the voltage-gated Dix-Hallpike is best suited to detect posterior canal sodium channels by allowing increased sodium dysfunction. 86. Neurological symptoms often include ground. 7th after a streptococcal throat infection in children. uremia. Figure from Ferri F 2009 selectively blocks voltage-gated sodium channels. and range from mild weakness to failure to lactate. Treatment is whereas visual agnosia is the broader inability to secondary prevention with penicillin G until the age of recognize objects visually. touch despite having intact sensation. Periodic limb movements of not be associated with choreiform movements (see sleep (PLMS) are characterized by EMG activity seen Bradley’s NiCP. rapidly moving from a seated position with head ataxia. which would as a polysomnogram. D. or other 12.  The computed tomography (CT) image shows lasting 0. whereas crystal malpositioning is the posterior canal due to brevetoxin activates these channels. caffeine. Hemorrhages may occasionally as pseudotumor cerebri) and should be discontinued Neurology Books . RLS is thought to be due to the low indicated if Huntington disease was high on the accumulation of brain iron and is usually treated with differential. hypothyroidism. Symptoms associated with cerebellar hemispheric lesions. It parietal dysfunction. regularly every 20–40 seconds. 7th common. and idiopathic intracranial hypertension (IIH. pp. autonomic nerve fibers. Both canals. delayed nystagmus with the Dix-Hallpike maneuver is Ch. but are not likely. patients have a 3 often begin with a target lesion around the bite. and diffuse dysautonomia is dysfunction in the patient (see Bradley’s NiCP. 583–604). 96. syndrome. Commonly. Headache. Ch. but identify numbers or letters written on the hand. Test Three 99 reuptake of neurotransmitters at noradrenergic. Symptoms include perioral 82. severe paroxysms saxitoxin and brevetoxin are found in shellfish. Agraphesthesia is the inability to typically resolves spontaneously within 3 months. D. pp. Ferri’s Color Atlas and Text of Clinical Medicine. 696–712). and therefore increased membrane toward the affected ear when that ear is toward the depolarization. Ch. which highly associated with RLS but not required for the likely represents hemorrhage. 7th edn. by blocking most common semicircular canal location of otolithic activation of voltage-gated sodium channels. performed by rotating the head 45 degrees and then Both cause perioral and acral paresthesias. neuropathy. or for at least 10 years after the disease. fatigue. Treatment of the chorea entire picture or scene (see Bradley’s NiCP. whereas nystagmus is more commonly mild diffuse weakness are also common. 122–127). The edn. It typically used for the treatment of Tourette syndrome is (1) an often uncomfortable urge to move the legs and would not be indicated here. which leads to lateral beating nystagmus permeability at rest. alpha2-delta ligands.5–10 seconds on polysomnogram. The diagnosis of RLS is made Stimulant medications are typically used for treating clinically and does not require diagnostic testing such attention-deficit/hyperactivity disorder. Ch. The other answer choices are causes of abdomen that later spread to the limbs. 1615–1685). could include prednisone. which is caused by pituitary necrosis after diabetes. Tetrodotoxin is found in puffer fish and edn. A. C. PLMS is hyperdensity in the area of the pituitary gland. may last for up to 1 week (see Bradley’s NiCP. Genetic counseling would be condition. paired with the diagnosis. pp.  Benign paroxysmal positional vertigo is a condition and acral paresthesias. to reduce ability to see multiple objects or an inability to see an the risk of rheumatic carditis. 46. followed by ascending weakness. Ch. calcium channel blockers. 7th edn.  Isotretinoin is associated with the development of amenorrhea. tetrabenazine. and respiratory depression. The Dix-Hallpike maneuver is membrane depolarization and action potential firing. antidopaminergic drugs.  Sydenham disease typically presents up to 6 months localization than cerebellar (see Bradley’s NiCP. and occasionally paralysis. pp. headaches. Symptoms vary widely. 85.

Autistic children can have odd Z Z behaviors with inappropriate emotions. Relaxed usually increased agitation or aggression. 89. E.  The sarcomere is a repeating unit within muscle separate into different hemispheres.  The N22–P34 interpeak latency corresponds with Immunosuppressed L. pp. prolonged with a cervical cord lesion. bargaining.  The five phases of grief are denial. Adolescent through adult S. This patient has a presumed thoracic Neurology Books . and quinolone antibiotics have been described in The P37 would also likely be prolonged. influenzae compression by tumor. causing a large fibers leading to muscle contraction. influenzae. A parasagittal meningioma may cause nocturnal headaches. but does not cause gelastic seizures (see Bradley’s NiCP. arteriovenous malformation. 110. including nebulin. monocytogenes Chs. Although the most common cause of TABLE FOR ANSWER 3.  This patient presents with gelastic seizures. 7th edn. this is not Toddler S. holoprosencephaly in which the brain does not 86. long chains of myosin are attached and make up the but both hemispheres are present. The sarcomere is central ventricle. Bradley’s NiCP. lasting for <1 minute. D. Contracted 91. an answer choice. The thin filament is corpus callosum in this sample. and acceptance. 7th edn. pp. 3rd edn. Elsevier.100 Neurology Self-Assessment upon diagnosis. neuralgia have multiple sclerosis. Projection is an immature Textbook of Medical Physiology. and is rarely used as therapy for exacerbations. Large-volume interpeak latency (brainstem to cortical response) lumbar puncture may be helpful in the diagnosis of would be normal. When this occurs.  Approximately 5% of patients with trigeminal shown here.86 (from Hall JE 2015 Guyton and Hall depression. Optic nerve lesion. Mesial temporal lobe sclerosis does not cause primarily laughing seizures. 1563–1614). transverse myelitis. Levetiracetam can cause behavioral changes. compressing Newborn Group B streptococci. Ch. trauma. Sjögren syndrome (see Bradley’s NiCP. Gelastic seizures are most commonly associated with hypothalamic hamartomas. Many times it can take Z Z years for the right diagnosis because the seizures are confused with behavioral episodes. 89.92  trigeminal neuralgia is a blood vessel pressing on the Age or comorbidity Bacteria trigeminal nerve as it exits the brainstem. tetracycline. E. H. N.  A chart with common bacterial causes of meningitis based on age range or comorbidities is 87. 1279–1300). but there is nothing in the question stem to suggest this patient is I A I on levetiracetam.  The specimen demonstrates alobar Ch. 90. From this frame. 1686–1719). Nocturnal headaches suggest increased intracranial pressure. 1686–1719). the motion of Bradley’s NiCP. multiple proteins. which is made up of facial defects like cyclopia. pp. Lissencephaly is a When a muscle is activated. E. Ch. pp. and seizures. cleft lip. N9–N13 peaks are symptoms. mental sarcoplasmic reticulum binds to troponin. L. or inflammatory diseases such as Elderly S. meningitidis. 7th edn. and about 2% of patients with multiple sclerosis will have trigeminal neuralgia. coli. 7th edn. 1915–1956). These I A I present with episodes of either uncontrollable laughter or crying. coli and damaging the nerve sheath over time. maximal therapy (see Bradley’s NiCP. but nocturnal headaches should point toward the possibility of an intracranial mass. aureus 88. pp. Less common causes include nerve H. pneumoniae brainstem. indicating a lesion in the dorsal IIH. 1159–1186 and 103. Similarly. C. B. calcium released from the genetic disorder that results in pachygyria. columns of the spinal cord. with permission. Saunders. holoprosencephaly the best answer. pneumoniae. improving both headaches and vision conduction or the spinal roots. vision when intracranial pressure remains high despite 7th edn. H. An absent N22 would Acetazolamide is the mainstay of therapy for mild cases suggest a problem with either peripheral nerve of IIH. influenzae. pp. S. the entire brain is made up of actin polymers. N20 is the cortical peak in upper extremity sheath fenestration is a last-resort therapy to preserve SSEPs and would not be affected (see Bradley’s NiCP. 34. Ch. actin binding to myosin-heavy chains will shorten the Figure from Klatt EC 2014 Robbins and Cotran Atlas of sarcomere and therefore shorten the muscle (see Pathology. monocytogenes conduction between the lumbar roots and the Sinusitis or otitis media H. meningitidis infection.) 92. making polymers and blocks the binding of actin to myosin. and cleft palate. with defense mechanism. permission. which would prolong this latency. 7th edn. pneumoniae. Pachygyria involves few smooth gyri along the brain. Neurosurgical patients S. 348–366). Although there is no structure of the thick filament. Troponin sits on the actin abnormal with a single ventricle. Polymicrogyria involves troponin from actin and allowing actin to bind to many very small gyri on the surface of the brain (see myosin-heavy chains. Figure for Answer 3. pneumoniae. Ventriculoperitoneal shunting is reserved involved in upper extremity SSEPs and would be for intractable headaches associated with high pressure. amiodarone. influenzae. 13th edn. S. C. pp. pneumoniae. releasing retardation. not a thoracic even after treatment with acetazolamide. 103. A. and therefore the possibility of a tumor. Ch. 80. but P34–P37 association with IIH development. A. 101. N. anger. This is usually associated with midline bordered by a Z-line on each side.

A. timing and size of dosage increases 96. pp. and coma. Saunders. and few lesions on Methanol ingestion is associated with bilateral initial magnetic resonance imaging of the brain. Headache is the initial symptom.  Metastatic tumors from systemic cancers are the 102. diarrhea. Treatment is usually agricultural industry in pesticides and herbicides. B. 158–162). Carbon monoxide poisoning relate to the formation of vein of Galen malformations. C. between first and second attacks. these two factors do not directly from acetylcholinesterase. accounting for about one third of tumors. Inhalation leads to early neonatal period. Although dehydration and infection may lead to venous which binds the organophosphates. and respiratory exposure comes from manufacturing molded plastics distress). Although each of the error was severe enough to result in termination. D. Ch. resulting in many slightly less common than glioblastomas. It is frequently Neurology Books . 91.  The patient clinically has Lambert-Eaton myasthenia most common intracranial tumors. Organophosphates are predominantly used in the seizures. pp. although the (manifested by tachypnea. prognosis (see Bradley’s NiCP. required and may consist of ventriculoperitoneal shunt Organophosphates are acetylcholinesterase inhibitors if hydrocephalus is present and/or vasoocclusive that cause nausea. As such.  The most appropriate response is to leave the order progress to extrapyramidal deficits. 7th edn. and organomegaly. Ch. there is no same area. and possibly worse phenotype. 87. especially the female gender. Figure from Swaiman K 2011 employees. pp. glioblastomas. pp. salivation. pp. Secondary treatment of bradycardia.  Prolonged carbon monoxide exposure may lead to have not been associated with symptom exacerbation necrosis of the globus pallidus. C. Ch. Figure accompanying question text from Liu GT. peroxisomal fatty acid metabolism (see Bradley’s NiCP. Pituitary adenomas are syndrome have no peroxisomes.  Rapid increases in steroid doses are known to the mistake. Pralidoxime the high-output heart failure may also be required. schwannomas. pp. 1324–1341). 2nd edn. symptoms. 40–74. Figure sclerosis. 1159–1186). LEMS presents in the 40s through intracranial tumors. coma. B. and long-term to arteriovenous shunting. Test Three 101 93. occur. meningiomas are the most 60s with progressive proximal weakness. or garage as these form prenatally. hemorrhages (rarely). photograph. pp. and as originally intended. with permission. cognitive delay. occurring at about half the peroxisomes. temporal lobe lesion. miners. No fraudulent billing has occurred. although home exposure from a Swaiman’s Pediatric Neurology: Principles and Practice. seizures. which correlates with a contralateral may be diagnosed through measurement of very-long. seizures. and myoclonus. apnea.  Hexacarbon solvents like n-hexane are commonly during fetal development and are often identified in the found in paints. Ch. and glue. Changing the order when death. 39. placing the patient at risk of an chronic dialysis patients in which aluminum exposure adverse event. DR2) are associated with increased risk of multiple causing parkinsonism. 2nd edn. craniofacial 98. 7th edn. These malformations form at approximately 6–11 weeks 99. causing a variety of use leads to peripheral neuropathy and dysautonomia. not paints and lacquer. associated with brain atrophy. E. 7th edn. Alcohol is multiple sclerosis include younger age at disease onset. treatment of the malformation. lacquers. with permission. or cardboard. 97. Living in a northern have manganism than carbon monoxide poisoning (see latitude may increase one’s overall risk of multiple Bradley’s NiCP. Medicare or the insurer does not need to be contacted. seizures. cyanosis. typically occurs in gas workers. malpractice has not occurred. 7th edn.  Factors associated with favorable prognosis with microvascular disease substantially. Zellweger syndrome sky” field loss. 411–458). it is up to the facility to determine whether send a patient into myasthenic crisis. headaches. This includes impaired tumors. Nerve sheath developmental abnormalities. although the patient should be informed of 100. or hydrocephalus. as seen in the (see Bradley’s NiCP. seizures. Ch. 109. but is not specifically correlated with from Yachnis AT 2012 Neuropathology. which can 95.  Zellweger syndrome is an autosomal recessive common. Elsevier. Aluminum toxicity can result in cognitive contrast was not felt to be necessary is performing decline. strokes from steal phenomena or mass effect. These malformations often lead a sense of euphoria and hallucinations. Without an injury to the patient. sensory onset (rather than motor). and may indirectly lead to mammillary complete remission after first attack. headaches. cerebellum. but increases the risk of stroke and 101. E. 80. a category that includes vestibular neuronal migration and abnormal brain development. D. with permission. Galetta SL 2010 Neuro-Ophthalmology: Diagnosis and 94. Tobacco does not cause any specific brain injury. Volpe 7th edn. releasing them sinus thrombosis. 5th malfunctioning furnace or wood-burning stove can edn. is the antidote used in organophosphate poisoning. C.  The visual field shown demonstrates a “pie in the abnormalities. Saunders. Although it does deposit in the sclerosis. the other drugs may be used in the treatment of myasthenia gravis. which may indicate impaired (see Bradley’s NiCP. The following figure summarizes chain fatty acids (especially C26:0 and C26:1) in lesion localizations related to various visual field deficits cultured fibroblasts. NJ. including high-output cardiac failure Acrylamide can have similar symptoms. This is seen in excessive testing. but may be seen in welders exposed to unnecessarily. putamen necrosis. 1896–1914). and may actually mistake. Although the radiology technician made a exacerbate myasthenia symptoms. weakness. 7th edn. the patient described would be less likely to such association with MHC class I. disorder associated with mutations in several PEX Glioblastomas are the second most common 3 genes that encode the proteins required for intracranial tumor.  The images depict a vein of Galen malformation. Among primary syndrome (LEMS). Ch. so aluminum dust (see Bradley’s NiCP. occur about half as often as Patients frequently have symptoms of hypotonia. and increasing medical expenses is not limited. 16. impaired feeding. Manganese is most often inhaled by Although certain MHC class II alleles (such as HLA- welders and accumulates in the globus pallidus. Management. long remission body hemorrhage through thiamine deficiency. 1254–1260). individuals with Zellweger incidence of meningiomas.

transmission. 3rd edn. usually small cell lung In this case the lowered INR resulted in recurrent cancer. 13. D.98 (from Goetz CG 2007 Textbook of Clinical Neurology. With sustained or repeated contractions. In this condition.102 Neurology Self-Assessment Location Field defect Left eye Right eye Comment 1 Left optic nerve No light perception left eye R L 2 Chiasm Bitemporal hemianopsia 3 Right optic tract Incongruous left homonymous hemianopia 1 4 Left lateral geniculate Right homonymous nucleus sectoranopia (lateral choroidal artery) 5 -or- Incongruous right 2 homonymous 3 hemianopia 4 5 Left temporal lobe Right homonymous upper quadrant defect (“pie in the sky”) 6 6 Left parietal lobe Right homonymous 7 defect. 1563–1614). A. so it has the potential to of calcium into axons. Bulbar symptoms are uncommon. Figure from Howard JF 2013 from lesions to the inferior parietal lobule and Electrodiagnosis of disorders of neuromuscular posterior superior temporal lobe (see Bradley’s NiCP. pp. but it is a strong hepatic enzyme present. immunosuppression. As the saying goes. unlike myasthenia. and reducing synaptic release of increase the INR by decreasing metabolism of warfarin acetylcholine. posterior lateral frontal lobe. the CMAP amplitude will side. with permission. LEMS never presents with purely ocular inducer. Saunders. This leads to a stronger contraction with repeated seconds electrical activity is suppressed. 101. On nerve conduction studies. Valproic block presynaptic calcium channels. which can be shown and inhibition of electrical excitability occurs after 4–6 electrodiagnostically with 50-Hz repetitive stimulation minutes. usually on the left stimulation or exercise. Physical Medicine and Rehabilitation Clinics 7th edn. of LEMS patients have cancer. Ch. 128–144). The phenomenon is synaptic excitability occurs within the first 2–4 minutes. Neurology Books . time is brain. the compound muscle action 105. which allows more calcium to build lobe. D. Lamotrigine. pp. calcium will build up in the axon terminal and will 104. with permission. and gabapentin are (VGCC) antibodies are diagnostic. of North America 24(1): 169–192. 109. which then leads to aphasia. 3. Inhibition of effort and the return of reflexes. patients are able to write. In 12–15 cleft. 1895–1914).) associated with autonomic symptoms such as dry 103. Conduction diaminopyridine (3. Ch. known as facilitation. These antibodies not significant enzyme inducers or inhibitors.  Pure alexia without agraphia is a syndrome that can potential (CMAP) of a clinically weak muscle should occur with lesions to the splenium of the corpus have a low amplitude. (see Bradley’s NiCP. lacosamide. Fifty to seventy percent result in a lower international normalized ratio (INR). which can increase warfarin’s metabolism and symptoms. Anti-presynaptic voltage-gated calcium channel stroke. they cannot read even something they have just Treatment includes searching for and treating the written. This results from loss of connections from primary cancer.4 visual input centers and language centers. stopping the flow acid is an enzyme inhibitor.  No significant neuronal changes occur within the lead to an increase in acetylcholine in the synaptic first few seconds of cerebral blood flow arrest. and 3. Broca aphasia arises from lesions of the more acetylcholine release (see Bradley’s NiCP. 7th edn.4 DAP). but can be partial seizures.  Carbamazepine is an excellent medication for mouth. but increase by more than 200%. denser inferiorly 9 7 Left occipital lobe Right homonymous lower 8 (upper bank) quadrantanopia (macular sparing) 8 Left occipital lobe Right homonymous upper (lower bank) quadrantanopia (macular sparing) 9 Right occipital lobe Left homonymous hemianopia (macular sparing) Figure for Answer 3. Wernicke’s aphasia results Ch. but with 50-Hz repetitive callosum and medial occipital lobe. pp. as shown in the tracing. 7th edn. or a brief 10-second period of exercise.4 DAP acts to prolong aphasia comes from lesions to the superior temporal action potentials. Lesions to the angular gyrus result in anomic up in the presynaptic terminal.

Astrocytes B. besides loss of balance for many years. Macrophages following is the inheritance pattern? D. Subthalamic nucleus (STN) C. Autosomal recessive C. She recently saw a segment on television about someone with a condition similar to her’s who had deep brain stimulation (DBS). Rituximab pregnant presents to your office for evaluation of headaches. drinking. Glatiramer 6. These hurt over the left side of her head with a throbbing pain. Ventral intermediate (VIM) thalamus Figure for Question 4. Lipid solubility 5. Which target in the brain would be most appropriate for her? A. She has attempted numerous combinations of medications for her tremor. Autosomal dominant E. A 25-year-old female who is currently 16 weeks B. and are associated with nausea and photophobia. Mitochondrial blood–brain barrier (BBB)? E. which of the C. Which cell type is predominantly lost in the center of a demyelination plaque in multiple sclerosis? 1. Fingolimod has noted more frequent and severe headaches.1. Magnetic resonance imaging of the brain is Neurology Books 103 . including propranolol. Humoral mediation of leukocytes passing the blood– D. Her headaches are occurring frequently enough that she is missing several days of work per week. primidone. She is without cognitive dysfunction or general medical issues. she E. A patient presents with a high-amplitude action tremor of both arms that prevents her from eating. or dressing herself. topiramate. She is now completely dependent on her loved ones for care. Neuron demonstrated the findings shown here. Over the last several weeks.  B. Dimethyl fumarate and was previously treated with valproic acid for D. Natalizumab migraine prevention.Test Four QUESTIONS 3. Molecular weight E. each with intolerable side effects or lack of efficacy. Oligodendroglia A. X-linked dominant 4. Globus pallidus interna (GPi) 2. Immunogenicity B. and she has been unable to help take care of her 3-year-old son. Charge C. Nucleus accumbens A. Autopsy A. Which of the following is not a consideration in determining whether a substance will cross the D. T-cells form of the most likely diagnosis. She has a history of migraine headaches C. Presence of specific transporter D. A 66-year-old woman with dementia suffered a large intracranial hemorrhage and passed away. and clonazepam. Centromedian (CM) thalamus brain barrier is the purported mechanism of which of the following drugs? E. X-linked recessive A. In the heritable B.

Diagnosis can be confirmed by lumbar puncture E. Increased risk of leukemia abnormal motor responses with conduction blocks C. C8 radiculopathy deleterious to cerebral myelin? 12. Electromyography shows D. Restart valproic acid A. D. he does not make eye contact with the painless weakness in his right arm. but now involves a and has decreased tone but normal strength. Always associated with autism spectrum disorder present. Duchenne muscular dystrophy 10. Posterior interosseous neuropathy Figure for Question 4. Steroid myopathy E. extensor digitorum. Only present if mutation received from father fasciculations and decreased recruitment in ulnar and E. This condition responds dramatically to most likely taken from a patient with which disease? plasmapheresis. Hypocalcemia school. Hypoglycemia an elongated face. A 27-year-old woman presents with subacute D. C. She has never had any similar episodes of weakness or numbness before. Pathergy test C. On general examination. which ascended up her legs. He was never on C. Initially this began examiner. The photomicrograph of a biopsied thigh muscle is D. A. Anti-GQ1b antibody B. Which of the following is the most likely diagnosis? A. and he was the B. Anti–HTLV-1 antibody E. Inclusion body myositis paraparesis over the past 2 days. Polymyositis decreased sensation in her toes. He wrist drop. Hypokalemia product of an uncomplicated birth. Which of the following is reasonable next step? true about this condition? A. and extensor indicis muscles. C. with weakness of grip strength. A 62-year-old male presents with a 6-month history of Neurologically. with 5/5 strength elsewhere in the arm. A. Anti–aquaporin-4 antibody D. This condition is caused by a virus in the B. 7. and currently requires special education at D. Which of the following is the best test to diagnose her underlying condition? A. which was present upon awakening 2 weeks ago. Hypernatremia schedule with motor or cognitive milestones as a small child. although last year she had a 2-week episode of severe vision loss and pain in her right eye that resolved without treatment. Which of the following would be the most radial innervated muscles. Recommend no preventative medications C. X-linked recessive Neurology Books . Which of the demonstrates weakness in muscles innervated by the following is true of the underlying cause for his ulnar and radial nerves along with fasciculations. is moderately delayed in verbal output. along with pain in her left shoulder.  C. His examination demonstrates stereotypies with his hands. Nerve conduction studies in the A. Radial neuropathy at the spiral groove 8. Recommend daily ibuprofen condition. These conduction blocks are outside of typical sites of compression. He denies any numbness. She has numbness on the dorsal portion of the left thumb. Posterior cord brachial plexopathy D. Hyponatremia His parents are intellectually normal. Correction of which of the following is most classically E. Anti-GM1 antibody 11. A 63-year-old female presents to the office for evaluation of left arm weakness. His symptoms? triceps reflex is absent.104 Neurology Self-Assessment normal.7. Lower trunk brachial plexopathy B. he is noted to have E. large ears. Caused by CGG triplet repeat right arm demonstrate normal sensory responses and B. Anti-GM1 antibodies are frequently found in this B. Her examination is notable for 0/5 strength in the left brachioradialis. Riluzole is the recommended treatment for this condition. A 15-year-old boy is evaluated for developmental delay. Start acetazolamide demonstrating cytoalbuminological dissociation. Normal muscle Picornaviridae family. and macroorchidism. She has a normal biceps reflex and triceps reflex. but the brachioradialis reflex is not present on the left. then difficulty with urinary continence. and finally an inability to walk due to weakness. She first noted E. Start propranolol for migraine prevention B. 9.

Pelizaeus-Merzbacher disease the following would not be effective in this scenario? B. and circulatory support. blindness. Reduce amplitude of all waveforms D. Foramen rotundum equally along the axon. Talking to one’s self in public D. Reduce sweat artifact C. Eccentric clothing C. Sodium and potassium channels are distributed B. 8 E. Hypervolemia C. 6 C. pale optic discs. At birth. An 18-year-old male is brought into the emergency evaluation? department after a motor vehicle accident. E. Hypertension D. Intraarterial beta-blockers Neurology Books . On day 7. Which statement about myelinated axons is true? 18. T-tube trial A. and impaired motor skills. After a severe motor vehicle accident.  resonance image (MRI) demonstrates diffuse symmetric hyperintensities of bilateral white matter regions with involvement of the U-fibers. his motor response to noxious stimulus weakens significantly. Intracranial pressure monitoring withdraws his extremities to painful stimulation. increasing the high-pass filter will result in which of the following? A. A 1-year-old Ashkenazi Jewish girl who was born at full term presents with developmental delay. Cerebral palsy D. After the assurance of proper respiratory 20 m/s. Myelinated axons conduct axon potentials with nerve exit the skull base? 4 statutory conduction. Superior orbital fissure B. Acceptance of magical thinking B. The next day. Hemodilution E. A. Jugular foramen depolarization to occur. and poor feeding. Transcranial Doppler evaluation is the patient’s Glasgow Coma Scale score? D. intubated. Which of A. suggestive of the correct diagnosis? cerebral vasospasm. Increase amplitude of all waveforms E. whereas potassium channels reside under man arrives at the emergency room unconscious and myelin sheath. Reduce electromyography (EMG) artifact B. What C. Foramen lacerum D. as well as spine imaging. 5 E. She has a long history of seizures. C. Increase electrode impedance 16. Balloon angioplasty E. On A. Canavan disease C. A magnetic resonance 20. transcranial Doppler N-acetylaspartic acid (NAA). 7 D. Which of the following is demonstrates increasing velocities. Alexander disease A. On examination. makes incomprehensible sounds. There is hyperreflexia and positive Babinski signs bilaterally. A magnetic Figure for Question 4. Assuming others will exploit one’s self 17. All of the following are features of schizotypal personality disorder except? A. Electroencephalogram B. a 50-year-old Ranvier. Normal conduction speeds in myelinated axons are shown here. propagating the action potential. she has macrocephaly. she had hypotonia. Voltage-gated potassium channels allow D. poor head control. Cerebral angiogram examination he will open his eyes to painful stimulation. Through which of the following does the mandibular A. Pervasive discomfort with close relationships E. and spasticity in the bilateral lower extremities. A computed tomography scan of his brain is E. Rett syndrome B. In an electroencephalogram (EEG). and B. Foramen ovale C.19. Test Four 105 13. which of the following is the next most appropriate 14. The aforementioned patient remains critically ill (MR) spectroscopy shows an increase peak of and intubated. 9 15. Sodium channels are clustered in the nodes of 19.

Autosomal-dominant nocturnal frontal lobe trauma. Slow rolling eye movements C. Delta waves comprising >50% of epoch E. but the remainder of his neurological examination is normal. He denies any B. Which myasthenia gravis. Her D. with altered mental status. A 47-year-old male with a recent history of the following is the most likely diagnosis? endocarditis presents with progressive weakness in his A. Administer hydrocortisone E. and strength is mildly reduced. On examination.  episodes with twitching of one side of his face and retained consciousness. which have progressively worsened over the last week. He has a history of Her magnetic resonance image (MRI) is shown. MRI of the thoracic spine with and without contrast obesity with multiple bruises.106 Neurology Self-Assessment 21. Laboratory tests reveal E. An 8-year-old male presents to the office for evaluation of seizures. He has severe pain in his lower back and is tender to the touch over his vertebral processes. What is the most appropriate next best step to stabilize the patient? A. A 23-year-old African American female presents to the A. She has been unable to control her bladder C. round facies.21. Rationalization E. In the last 2 months he has had several nocturnal Figure for Question 4. and striae D. On examination. Transverse myelitis neurological examination reveals nearly flaccid E. Cervical spinal angiogram is hypotensive and encephalopathic. Projection D. Aquaporin-4 antibody hyponatremia. His wife states that he has poor compliance with his medications. Vertex sharp waves 25. Vertebral fracture paralysis of her legs and loss of sensation in her legs to all modalities. Sublimation B. D. His parents state that he has reached all developmental milestones and does well in school. he B. Juvenile myoclonic epilepsy White blood cell count is 14 and erythrocyte E. he has a fever of 102. He has not been sick A.6. A 45-year-old man presents to the emergency room pin prick in her hands. Delta waves comprising 20%–50% of epoch D. Anterior spinal infarct emergency department with leg weakness. epilepsy weakness in his legs. A sample from his electroencephalogram (EEG) is shown. Denial C. MRI of the brain with and without contrast on his abdomen. Intellectualization 26. Elevated oligoclonal bands with infectious symptoms recently. She has sensation loss to vibration and 23. Which of 22. K complexes and spindles B. but his immunosuppressant of the following tests would uncover the diagnosis? regimen is unknown. Which of the following is characteristic of stage 2 sleep? A. falls. Plasmaphoresis B. Elevate sodium 24. Lower potassium D. reduced reflexes. Which of the following is an example of a mature coping mechanism? A. Guillan-Barré syndrome numbness. and metabolic acidosis. Spinal epidural abscess and had several episodes of incontinence. Intravenous immunoglobulins (IVIG) C. Which of the following is the most likely diagnosis? Neurology Books . He has central C. hyperkalemia. and B. Benign epilepsy with centrotemporal spikes legs. sensory loss to all modalities in C. Landau-Kleffner syndrome sedimentation rate (ESR) is 75 mm/h. and loss of sphincter tone. Juvenile absence epilepsy his legs.

Test Four 107


Figure for Question 4.26. 

27. The abnormality indicated by the black arrow in the
figure is most likely related to an abnormality in which
of the following?
A. Glucose metabolism
B. Cell growth pathways
C. Myelin production
D. Cerebrospinal fluid drainage
E. Neuronal migration
28. The woman in the figure has which cranial nerve palsy?
A. Right oculomotor nerve palsy
B. Left trochlear nerve palsy
C. Right abducens nerve palsy
D. Left abducens nerve palsy
E. Left oculomotor nerve palsy
2 cm

Figure for Question 4.27. 

Figure for Question 4.28. 

Neurology Books

108 Neurology Self-Assessment

29. A 17-year-old female presents to her psychiatrist alone C. Ensure confidentiality of information obtained
for evaluation of anorexia. Her weight is stable in from research subjects
recent months, but her body mass index is 16.5. Recent D. Ensure optimal statistical analysis to answer
complete blood count and metabolic panel were research question posed
normal. At the end of the appointment you realize that
the patient’s parents are casual friends whom you see E. Ensure appropriate informed consent is obtained
regularly. After disclosing this to the patient, she for research
requests that your conversation remain confidential. 32. A 34-year-old female with no past psychiatric history
Which of the following is the most appropriate presents to the office for evaluation of her mood. Two
response? months ago a military plane crashed and, although she
A. Inform her that as a minor her parents are entitled did not know any of the people on the flight, she was
to her medical information. jolted by the incident because her husband is a
B. Encourage her to keep the appointment secret. military pilot. Since that time, she has had
uncontrollable episodes of crying. She has been unable
C. Contact her parents after the appointment because to return to work and is unable to take care of her
anorexia is a threat to her health. family. She feels as if she is “on edge” and has snapped
D. If her parents ask, tell them no information can be at her children over minor disagreements. She denies
disclosed. any suicidal ideation or hallucinations. She denies any
E. Dismiss the patient from the clinic. nightmares or flashbacks. Which of the following is the
most likely diagnosis?
30. A 33-year-old female with a history of migraines calls A. Acute stress disorder
the office because of a severe migraine at 32 weeks of
pregnancy. There are no complicating features to her B. Posttraumatic stress disorder
headache, and this headache is similar to all her prior C. Adjustment disorder
migraines. Which of the following abortive therapies D. Major depressive disorder (MDD)
would be the most appropriate for this patient?
E. Normal bereavement
A. Oral ibuprofen
B. Oral sumatriptan 33. A 16-year-old male presents for evaluation of cramping
and weakness in his legs. A periodic acid-Schiff (PAS)
C. Intravenous valproic acid stained muscle biopsy is shown here. Which of the
D. Intramuscular ketorolac following is the most likely diagnosis?
E. Hydrocodone A. McArdle disease
31. Which of the following is not a principal goal of an B. Becker muscular dystrophy
institutional review board (IRB)? C. Polymyositis
A. Protect the rights of human subjects participating in D. Myotonic dystrophy
research E. Charcot-Marie-Tooth type 1a
B. Ensure minimal risk to human subjects by
proposed research

Figure for Question 4.33. 

Neurology Books

Test Four 109

34. Which of the following is most consistent with urinary 39. Which of the following most closely correlates with the
dysfunction related to a conus medullaris lesion? age of symptom onset with Huntington disease? 4
A. Normal flow A. Gender
B. Detrusor-sphincter dyssynergia B. Specific base pair affected by individual point
C. Urinary frequency mutation
D. Urinary urgency C. Gender of affected parent
E. Large postvoid residual D. Parental age of symptom onset
E. Triplet repeat length
35. Where do lower extremity sensory afferents, which
transmit joint position and vibration sensation, 40. A 65-year-old right-handed man is sitting at his
synapse in the central nervous system? grandson’s baseball game when he begins to feel
A. Anterior commissure of the spinal cord heaviness in his right arm and leg, slurred speech,
and difficulty communicating. His daughter, who
B. Substantia gelatinosa immediately notices the onset of his symptoms, calls
C. Rexed lamina VI 911, and the patient is brought to the nearest
D. Nucleus gracilis emergency room. The patient arrives 30 minutes after
E. Ventral posteromedial nucleus the onset of his symptoms and is noticed to have a
National Institutes of Health Stroke Scale score of 17,
36. A 63-year-old man with a history of hypertension, including difficulty following commands, dysarthria,
hyperlipidemia, and tobacco abuse presents with right left forced gaze, global aphasia, right-sided facial
upper and lower extremity numbness and clumsiness. droop, and right hemiparesis. There is also an extensor
A magnetic resonance image demonstrates a left plantar response on the right. His blood pressure is
thalamic stroke, and the patient is started on aspirin 173/85 mm Hg, pulse is 102, temperature is 98.6, and
and appropriate medical management. He is later oxygen saturation is 98% on 2 liters of oxygen. What is
discharged to an acute rehabilitation facility. the next best step in his management?
Approximately 3 weeks later, he begins to have right A. Initiate aspirin 325 mg
upper and lower extremity pain and burning, even to
nonnoxious stimuli such as the sheets touching his B. Give nicardipine to decrease blood pressure
arm. Which of the following is most likely the C. Administer intravenous (IV) tissue plasminogen
diagnosis? activator (tPA) at a dose of 0.9mg/kg
A. New thalamic infarct due to unrecognized atrial D. Obtain a noncontrast head computed tomography
fibrillation (CT)
B. Compressive ulnar and peroneal neuropathy of the E. Give mannitol for impending herniation
right hemibody 41. DiGeorge syndrome (also known as velocardiofacial
C. Migrainous phenomenon after his infarct syndrome) is associated with all of the following except:
D. Dejerine-Roussy syndrome A. Agenesis of corpus callosum
E. Melkersson-Rosenthal syndrome B. Tetralogy of Fallot
37. Immediate primary surgical repair (reattachment) is C. Cleft palate
indicated for which of the following types of peripheral D. Hypoparathyroidism
nerve injury? E. Thymic aplasia
A. Ragged nerve tear by propeller blade
42. Opportunities for physicians to advocate for patients
B. Compressive neurapraxia (e.g. Saturday night palsy) include all of the following except:
C. Clean nerve transection by knife A. Writing to legislators about an issue
D. Stretch injury B. Meeting with hospital administrators about an issue
E. Incomplete axon-loss radial nerve injury from C. Telling patients to learn to deal with their health
fractured humerus issue
38. A 50-year-old man suffers headaches that he describes D. Joining physician advocacy organizations
as “a hot poker” behind his left eye. These tend to E. Speaking to local patient groups about an issue
occur about twice per day, each lasting a half-hour, and
recur at around the same time each day. He has 43. Within 3 weeks, a 42-year-old man develops a right
concurrent lacrimation on the left. The current series of wrist drop, a left foot drop, and then a numb patch on
headaches has been ongoing for nearly 2 months, the back of his right leg. A series of laboratory tests was
though he’s had several similar headache cycles over performed, and he ultimately underwent a right sural
the last few years. He feels a need to walk around the nerve biopsy. Based on the pathological image shown
house to cope with the severe pain. Based on his most here, which of the following is the most likely
likely underlying diagnosis, which of the following is concurrent laboratory abnormality?
the best choice of first-line preventive therapy? A. Antigliadin antibodies
A. Verapamil B. Antineutrophil cytoplasmic antibody
B. Levetiracetam C. Vitamin B12
C. Indomethacin D. Hemoglobin A1C
D. Inhaled oxygen E. Copper
E. Amitriptyline

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110 Neurology Self-Assessment

44. A 43-year-old woman received surgery and high-dose
focal radiation 2 years ago for the treatment of a left
nasopharyngeal carcinoma. She now presents with
neurocognitive deficits and imbalance. Examination
reveals no local recurrence of cancer in her
nasopharynx. Her magnetic resonance imaging (MRI)
of the brain with contrast is shown. Based on the most
likely diagnosis, which of the following is true about
this condition?
A. The patient should be observed.
B. Antifungal therapy should begin immediately.
C. Radiation therapy should begin immediately.
D. Evaluation for paraneoplastic antibodies should be
E. Treatment with steroid or antiangiogenic therapy
Figure for Question 4.43.  may be necessary.

Figure for Question 4.44. 

45. Which of the following conditions could cause the C. Right lateral pontine stroke
pattern seen in this blink reflex? D. Right trigeminal neuropathy
A. Left Bell palsy E. Right internal capsule stroke
B. Left trigeminal neuralgia

Blink reflex R2
Right OOc

Left OOc

right side

Right OOc

Left OOc

left side
Figure for Question 4.45. 

Neurology Books

Ophthalmic artery 51. On examination.  E. Dilated fundoscopy was performed in the emergency room. History of mild motor milestone delay because of a change in behavior. she B. Aside from insomnia. Dysautonomia the following is shown in his pathological specimen? B. Ataxia C. Schizophreniform disorder C. Three days prior. Myoclonus D. monocular vision loss. Which of 4 A.  50. Schizophrenia B. and an autopsy is performed. Since the accident. Internal carotid artery D. He reports two recent episodes of transient vision loss in the same eye. Normal neurological examination Upon attempting to help the child. Schizoaffective disorder D. painless. Which of the following is the most likely cause of his symptoms? A. Bereavement Neurology Books . Common carotid artery Figure for Question 4. frequently crying when the incident is mentioned. Anterior ischemic optic neuropathy Figure for Question 4. Cowdry A inclusions 47. Spongiform changes D. Lewy body E. Negative family history for seizures her house and even talks to him in front of her family.49. Seizure duration more than 15 minutes witnessed a car hit a child while crossing the street. Test Four 111 46. which is the most prominent 49. she is unkempt and disheveled. Neurofibrillary tangles C. Cognitive decline B. she realized he had D. Which of the following is not characteristic of a simple febrile seizure? 48. Parkinsonism A.47. Only one seizure per 24 hours succumbed to his injuries and passed away. profound. The anterior choroidal artery is a branch of which of the following? A. A 32-year-old woman with no prior psychiatric history is brought to the emergency room by her family A. At this point in time. she has seen the child walking throughout E. Anterior cerebral artery C. Brief psychotic disorder E. Negri body E. Optic neuritis E. Central retinal vein occlusion C. A 65-year-old man with a long smoking history presents with sudden. A 67-year-old male dies after having dementia for the early symptom of fatal familial insomnia? past 5 years. Paraneoplastic retinopathy B. C. Middle cerebral artery B. a picture from which is shown here. Central retinal artery occlusion D. what is most likely her diagnosis? A.

Bipolar 1 disorder A. Clinically. Polymicrogyria 58. A 31-year-old man presents to the emergency room 55. and has even thought of experimenting with drugs. Dentate 54. sodium phenylacetate. Hypercoagulability E. Macrocytic anemia symptoms of schizophrenia. Granular cells an increased libido. Which of the following cell types provides the primary after being unable to sleep for 1 week. D. Basket cells sitting still. C. Which of the following conditions is depicted in the B. he appears tired but has a difficult time E. Vigabatrin Figure for Question 4. Holoprosencephaly D. Porencephaly respiratory alkalosis. Mania A. Mossy fibers him. His ammonia is elevated. has had A. and requires intubation for E. and he is talking to the devil to help him out of suicide attempt with depression when he was a this situation. His wife states output of the cerebellum? that he has been rearranging his garage. Women with OTC deficiency can be normal or have headaches. sepsis is ruled out and antibiotics are discontinued. fit of psychosis. What is the most likely diagnosis? placing the healthcare workers in danger. Emboliform magnetic resonance image (MRI) shown? C. Phenobarbital C. which is uncharacteristic of B. and arginine hydrochloride are all treatments for OTC deficiency. She also recently found out that he has been C. which is autosomal recessive. Fastigial B. Pregabalin E. There is low potential for acute extrapyramidal side vitamin B12 (cobalamin)? effects. Sodium benzoate. E. he is hypothermic. 59. schizophrenia comes to the emergency room (ER) in a When asked about psychological problems in the past. Which of the following is true regarding B. He believes there are cops trying to kill his wife thinks he had a prior hospitalization for him.54. Schizencephaly E. although liver function is normal. Which of the following is not true regarding urea cycle defects? A. and there is no evidence of acidemia. A. Which of the following cerebellar nuclei send efferent D. teenager. Cerebral edema is a common cause of coma. All have an X-linked inheritance except ornithine transcarbamylase (OTC) deficiency. It has low potency action on the D2 receptor. and has significant vomiting. There is no 56. Cognitive decline 57. 53. Cyclothymic disorder C. Periventricular nodular heterotopia first 2 days of life. Demyelinating myeloneuropathy information via the superior cerebellar peduncle? E. E. It is not available in an injectable form. Schizophrenia physician administers haloperidol in combination with lorazepam. Which of the following antiepileptic medications does not enhance the activity of gamma aminobutyric acid (GABA) in the central nervous system? A. A newborn becomes increasingly somnolent over the D. A 27-year-old man with a history of poorly controlled evidence of illegal substances on his urine drug screen. Which of the following is not a likely effect of low D. D. Purkinje cells examination. B. but not the negative. The ER A. It is useful for the positive. A urea cycle defect is suspected.  Neurology Books . Although he was initially placed on broad-spectrum antibiotics. C. Globose A. His speech is sensible but tends to be fast and jumps from one topic to the next. Stellate cells gambling away their savings with online poker. and ophthalmoplegia. hypotonic. Major depressive disorder haloperidol? C. Tiagabine D. Bipolar 2 disorder B. It is an atypical antipsychotic.112 Neurology Self-Assessment 52. Clonazepam B. vomiting. All of the above C. On D. He is throwing chairs in the room. B. Arginine supplementation is needed for arginosuccinic acid synthetase deficiency.

and loss of reflexes. A 37-year-old female is intubated due to hypoxic respiratory failure. Botulinum toxin symptomatic lesion? A. he develops flaccid weakness in his arms and legs. Nerve conduction attenuated inversion recovery (FLAIR) abnormalities studies were normal in the arms and legs. necessary. Guillain-Barré syndrome with symptoms of fever. and C. falling well behind her peers by the time she was 30 months of age. Which of the following is the most likely cause 4 weakness. If left untreated. Which of the following is the most recover. neck stiffness. Myasthenia gravis confusion. she ignores their C. in bed. A 48-year-old male with a history of alcohol abuse genetic syndromes should be considered? presents to your clinic with his family for evaluation of A. A 32-year-old female tragically lost her husband in a motorcycle accident 3 weeks ago. except for in the periaqueductal gray matter and mammillary absent F waves. He has suffered three falls in that time. Test Four 113 60. Which stage of grief is she likely experiencing? 65. After admission. Critical illness neuropathy garden this summer. Which of the following 62. Which of D. Her examination shows prevent development of the disorder. memory may not fully activity was seen. Contact the hospital risk management team. On morning rounds. Central portion of the cervical cord due to episodes of hypotension. Landau-Kleffner syndrome examination demonstrates an ataxic gait and bilateral D. His family has noticed short-term memory loss. Poliovirus and position sense in the left leg. This disorder is more common in patients with the for 1 week before her admission and spent several days APOE4 allele. and apathy over the last B. moderate weakness of the left leg. After a brief evaluation. Dravet syndrome partial abducens nerve palsies. These increased in frequency. D. Tell the nurse to give the medications because the physician has ordered them. A magnetic resonance image E. His C. it is felt that she is going E. Left side of the cervical cord 64. Report the nurse to her charge nurse. She refuses to give C. and no spontaneous E. Change the orders to appease nursing concerns. diffuse weakness in her extremities. Bargaining antiepileptic medications. When family B. Posterior columns in the thoracic cord medications until her concerns are addressed. Her husband states she had diarrhea B. and later absence seizures B. A 31-year-old man in Texas who is current in his routine medical care presents to the hospital in July B. D. disorientation. whereas she has loss C. Magnetic resonance imaging may show fluid double vision. A 36-year-old female with a history of multiple with retained bulbar strength and normally reactive sclerosis presents to the emergency department with pupils. Acute disseminated encephalomyelitis (ADEM) Which is the most likely localization of her E. On neurological examination she is noted to of his symptoms? have new weakness of the left finger flexors and A. West syndrome few weeks. members talk to her about her loss. and normal strength on the right. A young girl first developed generalized tonic-clonic A. Left lateral pontine lesion the following is the best response? E. They are concerned about her being depressed. Generalized epilepsy with febrile seizures plus true of this condition except? (GEFS+) A. Educate the nurse on why the medications are alive. Anger seizures with fever during her first year of life. Right side of cervical cord A. but Neurology Books . Denial developed as well. All of the following are E. Rett syndrome memory loss. a nurse expresses concerns about the blood pressure medications ordered for a patient B. 66. West Nile virus extensors. likely diagnosis? A. bifacial weakness. 61. this can lead to progressive encephalopathy and death. Acceptance of 18 months. Administration of thiamine before glucose can and could not get out of bed. She was unable to activate any muscles bodies. She became quite refractory to C. through the normal grieving process. on electromyography (EMG). Depression cognitive development began to slow at about the age E. her D. questions and talks about her husband as if he is still D. Despite adequate treatment. Today she complained of shortness of breath C. Guillain-Barré syndrome of temperature and pin prick sense in the right leg. He has been spending long days in the D. headache. Acid maltase deficiency (MRI) of the brain is unrevealing. Along with this. Polymyositis 63. She has loss of vibration B.

Cerebellar ataxia both ears. A magnetic resonance image (MRI) of the brain is 68. Which of the following locations would be expected to show hypometabolism on FDG PET? Neurology Books . Which of the following is the most likely diagnosis? A. as well as recently learning D. Deep inhibitory postsynaptic potential will lead to but no falling. Marchiafava-Bignami disease examination reveals mild numbness of the right arm C. Radial dipoles are not recorded well with scalp A. B. He kicks his wife sometimes while sleeping. His physical examination reveals mild rigidity Figure for Question 4. Multiple sclerosis that time. On examination. Since A. Manganism performed and is shown here. diagnosis? E. abnormal reflexes. A 56-year-old man presents with acute hearing loss in C. C. D. A 26-year-old female presents to your office for obtained and shown here. but is able to stand dipoles with scalp electrodes. with a knee-buckling gait and unsteadiness. A 49-year-old woman presents with 1 month of gait 69. without difficulty. What is the most likely 6 cm2 of underlying cortex. displayed. Conversion disorder over the last 3 months. His family has noticed that his “internal clock” seems to be off because some days he will sleep all day and other days he is awake all day. or B. Choriocarcinoma C. Vertically oriented dipoles are the main recordable unable to lift her legs off the bed. A single electrode detects electrical activity from of her husband’s infidelity. she has also noticed shortness of breath. Subacute ischemic stroke Figure for Question 4. physical and sexual abuse. A fluorodeoxyglucose (FDG) position emission tomography (PET) scan was obtained. Conus medullaris syndrome 70. When ambulating. Superficial excitatory postsynaptic potential will Babinski sign. Acute flaccid paralysis from the West Nile virus electrodes. as well as an acute episode of E. but no other significant abnormalities. His family reports progressive confusion D. which began abruptly. cough. Creutzfeldt-Jakob disease and face. she is A.68. great effort is lead to scalp negativity. All of the following are true about difficulty. She cause of his symptoms? suffered a spontaneous abortion 3 months ago. Her neurological B. Meningioma D. Somatization disorder visual field cut in the right eye 3 weeks prior. A 66-year-old male presents with vivid visual hallucinations and memory loss. There is a Hoover sign with no evidence of increased tone.  71. Further questioning reveals a history of scalp positivity.114 Neurology Self-Assessment 67.  in his upper extremities and slow finger taps. and blood in her sputum. Which is the most likely evaluation of headaches and left arm numbness. There is no history of electroencephalogram (EEG) recording except? bowel or bladder incontinence. Susac syndrome Magnetic resonance imaging (MRI) of her brain was E. Glioblastoma multiforme B. Multiple sclerosis E.70.

Which of the following has the least amount of data as a risk factor for concussion? A. Frontal lobes the last year to impress a guy. A. often E. Sphingosine-1-phosphatase inhibitor E. Result of a ruptured berry aneurysm. α-galactosidase A deficiency D. Prior concussion 76. Gender B. A. Temozolomide C. A gamma D. her body mass index is 14 (normal is 72. headache. Males are most prominently affected in this condition. D. Position played D. Which of the following is not a common cause of 18. Arylsulfatase-A deficiency 75. There are no medications that have been shown to C. A 22-year-old male with a history of kidney disease and prior stroke presents for evaluation of numbness A. His physical examination shows a dark-red C. An 18-year-old girl is evaluated for fatigue. Body weight is usually normal in this condition. Alpha-4-integrin adhesion molecule antibody diuretics. D. Group B Figure for Question 4. However. Amenorrhea is often associated with this condition. his examination is normal. nausea. the ankles and mild sensory loss in his feet. Which of the B. A 73-year-old diabetic man presents with headache and E. Anterior temporal lobes broke up. she felt obsessed with her appearance. May occur with thrombosis of the sagittal sinus. He then awakes with confusion. A beta C. A result of tearing bridging veins. most patients with this condition fingolimod? purge through self-induced vomiting. A alpha B. or A. Posterior temporal and parietal lobes roommate accompanies her today and contributes to 4 the history by stating that the patient has lost weight in C.78. Vincristine with prominence of her bony structures. 73. even after they D. laxatives. Occipital lobes 77. Paclitaxel A. CD20 monoclonal antibody confusion 3 days after a fall. Associated with cerebral amyloid angiopathy last several months and involves burning paresthesias (CAA). E. Which of the following is the mechanism of action for D. PMP22 duplication B. There is diminished muscle bulk throughout. in the feet. She has thin hair and has goosebumps on chemotherapy-induced peripheral neuropathy? her skin. in his feet. The rest of D. a 14-year-old football player loses consciousness for 3 minutes. He has reduced reflexes at region. Bortezomib B. and vertigo.  Neurology Books . Which of the following abnormalities would be seen in this patient? E. Test Four 115 A. Striatum feeling overweight despite being told the contrary. He had a similar episode 3 months before.5–24. After eating. Which of the following is associated with the diagnosis shown in the image? 74. PMP22 deletion C. Sport played C. Acetylcholinesterase inhibitor 78. A delta E. On examination. Associated with a skull fracture over the temporal rash around his waistline.9). This has been slowly progressive over the B. Equipment worn E. After an excessively rough tackle. Her B. Which of the following nerve fiber types transmit proprioceptive information? A. B. Cisplatin following is true regarding the patient’s diagnosis? C. Dihydroorotate dehydrogenase inhibitor help treat this condition. Excessive phytanic acid accumulation E.

Involve frequent cardiac arrhythmias D. Idiopathic inflammatory condition hours. Then today he developed acute vertigo described as a spinning sensation with associated B. Trial of ropinirole B. Obsessive-compulsive disorder (OCD) E. you past health problems except for heavy menstrual cycles. He denies hearing loss or ear pain. he pain. A-alpha fibers pituitary gland. A 75-year-old man with 100-pack-year smoking history presents with midface pain and horizontal diplopia. A 23-year-old female presents to the office with complaints of difficulty falling asleep. or obtained. Which of of vertigo. Which of the feels anxious unless he performs the action and has a following would be the best course of action? sense of relief after performing the action. Hyperglycemia imbalance and nausea. B.” A magnetic resonance imaging of the brain is E. Which of the following is a tauopathy? myopathy except? A. Attention-deficit/hyperactivity disorder (ADHD) C. Multiple embolic events smoking. C fibers D. A-gamma fibers E. Her husband complains that she kicks him frequently sniffed for no reason and began to flick his during the middle of the night. Which of the following is uncommon of classic migraine E. Which of the following is a comorbidity of this condition? B. aching pain? B. Prechiasmal right optic nerve lesion A. and autoimmune conditions E. Multiple system atrophy C. Left occipital lobe lesion D. at which point he stopped A. On examination. Which of the following would correlate with this testing. Which of the following is not true about his current condition? 86. They are A. He easily maintains fixation on a target with bilateral head thrust 88.116 Neurology Self-Assessment 79. basic metabolic panel. she develops cramps in her legs. Visual disturbance usually monocular and improve when he stands up. Each night when 80. appreciate the noted mannerisms. hemorrhages. All of the above auras? A. Stimulation of which types of fibers produces poorly A. Progressive supranuclear palsy A. A. Serum ferritin level D. Dementia with Lewy bodies B. weakness. otolaryngologist for frequent sniffing despite a lack of forcing her to stand up and walk around to relieve the congestion or seasonal allergies. Autosomal-dominant. Most die in adolescence E. B fibers are other possible etiologies. She has no significant finger throughout the day. cognitive. Labyrinthitis B. he has no E. Poor impulse control D. Viral infection cranial nerve deficits. A 12-year-old boy is brought to the neurologist for pressure-like headaches that worsen when he lies down B. 87. Amyotrophic lateral sclerosis D. An 11-year-old boy is referred to neurology by his lying down to sleep. D. Posterior circulation stroke E. frequent throat clearing. A 62-year-old patient with poorly controlled diabetes 82. Generally similar symptoms with repeating attacks or gait difficulty. Nicotine withdrawal Neurology Books . The patient reports that he and liver function tests were normal. On examination. Fungal infection had any recent infections. For the last year. Which is the most likely cause of his full-field visual evoked potential? symptoms? A. but does endorse frequent urination. He reports a few passing moments of vertigo the following is the most likely cause of the symptoms? over the last 3 days. Mutated genes encode nuclear envelope proteins 84. D. and also note Recent complete blood count. comes to the emergency room with a chief complaint left facial numbness. localized. and B. Self-injurious behavior 85. Right occipital lobe lesion B. All of the following are true of Emery-Dreifuss 83. Treatment involves desmopressin. A-delta fibers C. dull. Nerve conduction study C. Benign paroxysmal positional vertigo (BPPV) D. He denies weakness C. Parkinson disease X-linked varieties exist C. May be preceded by prodrome of changes in mood His mother states that he “just can’t stop drinking or eating fluids. Prechiasmal left optic nerve lesion E. Overnight polysomnogram A. Trauma. Contracture is a common early symptom E. Duration <30 minutes 81. Vestibular neuritis C. sensory. autosomal-recessive. and left facial weakness. Serum sodium is typically high. speech. Incidence is high after surgical manipulations of the C. which has been ongoing for 3 C. Urine osmolality is typically high. May include visual. and has not D. and the patient is diagnosed with a Rathke gait symptoms cleft cyst. Arterial Doppler of the lower extremities also suppressible. or numbness. Chiasmal lesion C.

his parents noted his right arm was stiff. 4 years E. as well as dysarthria. Nightmares 91. which is the MPz–MI best next step? GI neg up 4 µV A. A. 2 years weather D. Airborne isolation and directed antimicrobial therapy 89. Gram stain and India ink are negative. and tongue deviation to the left. afterward he is confused. A head computed tomography (CT) is unremarkable. and more recently has developed time his parents arrive in the room. Sturge-Weber syndrome A. followed by new-onset diplopia. and headache. Hypomelanosis of Ito Which of the following is the most likely diagnosis? B. lip smacking. A. 9–12 years 92.94. He is noted to have bilateral abducens palsies. 2–4 years C. he is afebrile. 18–24 months B. What is the most likely episodes. Ataxia-telangiectasia B. A 12-year-old boy presents to clinic for evaluation of 94. dysphagia. 18 months C.  E. Classically presents with psychosis E. white blood cells of 223 cells/mL (lymphocytic predominance). Begin oral antifungal therapy and plan daily Oz–Fz lumbar punctures C. Von Hippel-Lindau disease epilepsy E. 6–9 years E. Juvenile myoclonic epilepsy E. Begin intravenous immunoglobulin and monitor respiratory function Figure for Question 4. Whole body position emission tomography- computed tomography (PET-CT) and radiation Oz–MI oncology consult B. Acute attacks may be triggered by certain stand on tiptoes. protein of Figure for Question 4. 3 years D. Test Four 117 Left eye Right eye 140 mg/dL (normal is 15–45 mg/dL). 4–6 years D. his strength and sensation are normal. resulting in bruises and scrapes.88. A 56-year-old homeless Indian immigrant man presents to the hospital with weeks of cough. At what age should a child be able to say sentences 93. Autosomal-dominant nocturnal frontal lobe D. Associated with decreased production of heme B. Which of the following is not true of porphyria? with two to four words. He has minimally delayed language. and bicycling of his legs and unresponsiveness for 30 automatisms. As an infant. and 4 Fz–MI red blood cells of 0 cells/mL. Mesial temporal lobe sclerosis D. and by the have infantile spasms. they witness complex partial seizures with staring. Classically presents with strokes 90. build a tower of four blocks. He has even fallen out of bed with these skin examination is shown. 1 year B. Otherwise. The episodes start with a noise. At what age does a child first sense the finality of death? A. His last 3 months. A 12-year-old boy presents to the neurology clinic due recurrent abnormal episodes that occur at night while to a history of seizures. demonstrates features of motor stereotypies and poor These have occurred several times per week over the eye contact. Associated neuropathy may be triggered by cold C. and a chest x-ray reveals a right upper lobe consolidation.  Neurology Books . Night terrors C. Begin antitreponemal therapy and identify sexual 50 100 150 200 50 100 150 200 contacts msec msec D. Tuberous sclerosis C. malaise. he was noted to he’s asleep. On examination. Cerebrospinal fluid (CSF) analysis reveals a glucose level of 4 mg/dL (normal is 45–80 mg/dL). A thorough neurological examination seconds before it stops. Based on your greatest suspicion of the underlying cause of this patient’s illness. With the last diagnosis based on history and examination? episode. and demonstrate parallel play? anticonvulsants A.

Diabetic cachexia D. A 66-year-old female presents for evaluation of extremity pain. A medical student has been showing romantic interest toward one of his neurology attendings. Nucleus of Edinger-Westphal C. She started on metformin and insulin and then developed painful burning paresthesias in her fingers and toes. A E. Electromyography (EMG) and nerve conduction studies in the arms and legs are normal. Inferior division of the middle cerebral artery B.96. Vitamin B12 B. Right parapontine reticular formation (PPRF) B. Artery of Heubner E. Ask the couple to be discrete until he graduates. Lenticulostriate arteries D. C. she has a right facial droop with right neurological symptom of diplopia on right lateral gaze.98. Anterior cerebral artery C. Vitamin B3 B 97. Diabetic amyotrophy 99. Diabetic distal sensory polyneuropathy Figure for Question 4. Ignore the relationship. arm weakness and mild sensory changes in the right Notable findings from extraocular examination are arm. Her nystagmus of the right eye in panel B. and is unable to name several objects. Remove the attending from a supervisory role. Vitamin B1 D. Expel the student. As chairperson of the department. Which of the comprehension is intact. A 35-year-old healthy man presents with a first-time examination. Which of the following arteries is the most these findings? likely cause of her stroke? A. Insulin neuritis E. Which of the following is the most likely cause of her symptoms? C A. Adverse effect of metformin B. Which of the following was likely deficient in the patient for whom an autopsy photo is shown? A.118 Neurology Self-Assessment Figure for Question 4.  95. Left abducens nucleus E. A 62-year-old female presents for stroke evaluation due to acute right hemiparesis for the last several hours. Vitamin B6 C. Left medial longitudinal fasciculus A. Superior division of middle cerebral artery Neurology Books . B. Vitamin D E. She is noted to have effortful and fragmented demonstrated in the figure and include horizontal speech. They have been seen on two dates. Fire the attending. but she has difficulty with following lesion localizations most corresponds with repetition. On 98. Left oculomotor nucleus D. what is the most appropriate response? A.  C. 96. D. She was recently diagnosed with diabetes after laboratory work showed a hemoglobin A1C of 11%.

Tumefactive multiple sclerosis B. 15 mL/100 g/min E. Prosopagnosia pupils. Osteopenia proteins are cleaved. Muscle biopsy would reveal necrotic muscle fibers 101. Balo’s concentric sclerosis A. diagnosis for the images shown from a 45-year-old E. decreased tone. Which of the following best describes his condition? Examination demonstrates a well-developed child with A. An 8-month-old girl presents to the emergency room is able to identify and name people by their voices. Multiple abscesses C. Muscarinic receptor agonist D. 65 mL/100 g/min B. he 102.101. This patient has progressive motor neuron weakness paraneoplastic disorders. Soluble NSF attachment protein receptor (SNARE) A. 4 a clinical risk to be addressed or monitored? B. Optic aphasia few movements. This is inherited in an autosomal-dominant woman? manner with anticipation. Hemorrhagic cystitis C. Acute disseminated encephalomyelitis (ADEM) pyridostigmine? C. he is unable to recognize faces. Developing new malignancy D. Infertility synaptic vesicles to release acetylcholine. Reversible acetylcholinesterase inhibitor E. She spent C. Anomic aphasia the last few days with her grandparents where she had tried new foods. Although he has intact visual Figure for Question 4. leading to an inability of B. which of the following is not from a mutation in the SMN1 gene. A. with poor feeding and weakness over the last 3 days. Voltage-gated calcium channel agonist D. and dilated B. Which of the following is the mechanism of action for B. C. Which of the following is true of this D. Metastatic lung cancer 103. Which of the following cerebral blood flow levels correlate with impaired but reversible neuronal function? A. Nicotinic receptor antagonist E. Immunosuppression nerve conduction studies. 45 mL/100 g/min C. ptosis. 35 mL/100 g/min D. Cortical blindness condition? E. E. Irreversible acetylcholinesterase inhibitor 104. A 55-year-old male presents with an acute stroke in the right fusiform gyrus. In using cyclophosphamide for the treatment of A. 5 mL/100 g/min 105. This patient should have increased cerebrospinal fluid (CSF) protein and conduction blocks seen on D.  fields. Test Four 119 100. Interestingly. The parents deny any recent illnesses. Which of the following is the most likely underlying and ragged red fibers. Aphemia Neurology Books .

  Oligodendroglia. Rituximab obscurations. vacuoles with basophilic granules. Generally. pp. 7th polydipsia). T-cells and inappropriate antidiuretic hormone secretion. or excessive intake of inflammation within active plaques. 7th edn. intravascular volume depletion and dehydration. 676–695). the cells responsible for axonal 110. Both the GPi and the STN have been 1. 37. Further. A. polymyositis would show lymphocyte and macrophage pp. Overly rapid correction of severe edn. Ch. 51. The pp. 551–572. with myelin loss in the center of a demyelinated plaque. Ch. as is autoimmunity. In: Practical pp. DBS could be considered. amino acids) may cross. the system (CNS).120 Neurology Self-Assessment ANSWERS common target for essential tremor is the VIM nucleus of the thalamus. with the highest risk class C. which is thought to sequester with accumulation of myelinoid bodies and amyloid lymphocytes in lymph nodes. cerebral macrophages are a prominent component of the salt wasting. Ch. Fingolimod is a structural analog of a a mixture of myofiber degeneration and inflammation. Churchill 6. exact mechanism of action of dimethyl fumarate is not central nuclei. a cell adhesion molecule necessary for are avoided during pregnancy. hyponatremia may lead to central pontine myelinolysis (or osmotic demyelination). substances with correction not to exceed 10–12 mEq/L per day. alteration of consciousness.  This patient has an action tremor consistent with with correction of hypernatremia. infiltration of the endomysium (between and around individual myofibers) (see Bradley’s NiCP. 7th edn. This and slower progression of disability for natalizumab patient’s headaches are severe enough that postponing compared with placebo. neuronal axons are 8. the patient’s glatiramer has been noted to induce a population headaches are not characteristic of IIH. but in cases where leukocytes to exit blood vessels into the central nervous patients are unable to perform their daily activities. The nucleus with mutations in the amyloid precursor protein (APP) accumbens is being evaluated in medically refractory gene. charge. C. there is necrosis. Surgical Neuropathology: A Diagnostic Approach. Hypernatremia is most commonly seen with diabetes However. A biopsy from autoimmunity (see Bradley’s NiCP. specific transporters (e. 80. Steroid myopathy is characterized by diffuse mediation on the HCA2 receptor. A. Finally. Ch. although they are preserved or even increased at the periphery of a plaque. hypertonic saline 4. In the absence of medical levels is not associated with demyelination. As such. thereby reducing the deposition. Hereditary CAA is thalamus is being evaluated in medically refractory tic autosomal dominant and is most commonly associated disorders such as Tourette syndrome.  Hyponatremia may be related to the syndrome of preserved within a demyelinated plaque.  The case and image are consistent with cerebral considered for the treatment of Parkinson disease. which would switch from proinflammatory Th1 cells to regulatory generally include nocturnal headaches. potentially through dystrophy. Acetazolamide is used for idiopathic are similar to the myelin basic protein. 401–406). are reduced proportionate to Color Atlas and Text of Clinical Medicine. is a monoclonal antibody against CD-20. Generally migraine preventative medications α4-integrin. which is more cause an immunological diversion. and papilledema (see Bradley’s NiCP. although its use is associated with decreased and fat. Ch. Glatiramer is a but can lead to medication overuse headaches and synthetic protein whose exact mechanism of action is premature closure of the ductus arteriosus in the third unknown. threat of osmotic demyelination is significantly lower 5. Figure from Ferri FF 2009 Ferri’s myelination in the brain. 88.  Natalizumab is a monoclonal antibody against her child. patients this patient. The four amino acids composing the drug trimester. circulating lymphocytes and overall lymphocytic infiltration into myofibers. The CM hereditary cases when present. 1261–1278). but correction of calcium clothing. or people taking migraine headaches. Daily ibuprofen may be helpful. pp. transient visual Th2 cells through an unknown mechanism. By targeting CD-20. This drug is known to increase treatment would not be the best option. and dressing.  Molecular weight. her quality of life is severely rather than neurological. although previously effective in in previously immunosuppressed patients. as Neurology Books . which are consistent with Duchenne muscular granulocyte invasion into the CNS. the B-cell population is reduced. Although the most Generally. A. Alternatively. Valproic acid. with fiber atrophy worse in fast fibers on speculation of an antioxidant role of dimethyl ATPase staining. C. Inclusion body myositis is notable for fumarate. 1973–1991). immunogenicity is not an important factor insipidus or medication effect and is associated with in BBB crossing (see Bradley’s NiCP. pp. it is important to identify dystonia (generalized and segmental). Studies demonstrated reduced relapses benefits of treatment may outweigh the risks. The 7. that are keeping her from working and taking care of 2. 7th edn. Elsevier. 1159–1186). natalizumab for more than 2 years. 1915–1954). Propranolol is the risk of JC virus infection. and increased endomysial fibrous tissue known. amyloid angiopathy (CAA). and lipid solubility are all should only be used with great caution in the setting important components of passive diffusion of a of severe and symptomatic hyponatremia with molecule across the BBB. medication effect.g. D. and number of active. with permission. D. a surface 112. Ch.  The muscle biopsy shows variation in fiber size. However. Additionally. At this point. causing progressive regarded as reasonably safe during pregnancy and is multifocal leukoencephalopathy. sphingosine. pp.  This pregnant patient has severe migraine headaches Livingstone. Reactive astrocytes hypotonic fluids (as in alcoholism or psychogenic are also prominent in plaques (see Bradley’s NiCP. medications. neurodegenerative disorders in adults. 3. contraindications. 7th edn. Hypocalcemia may lead to affected due to her dependence on others for feeding. The most hypoglycemia should be corrected rapidly. protein expressed on B-cells. A. The risk of overly essential tremor and has tried and failed numerous rapid correction of hypokalemia is mainly cardiac. which may intracranial hypertension (IIH). is considered pregnancy class X for with positive JC virus antibodies. the GPi is considered for the treatment of common form is sporadic. permission. common in pregnancy. Figure from Ma JM 2010 Biopsy pathology of depression (see Bradley’s NiCP.

distal radial muscles are affected (as with a major artifact coming from sweat. A lower trunk plexopathy would frequency sweat artifact. whereas potassium channels are under the demyelinating polyneuropathy (CIDP). 349–391). NMO is sounds. but should not cause sensory loss or 15. Data from Teasdale G. below the selected frequency.5 Hz or less.  The combination of optic neuritis and transverse 14. stimulation. Ch. motor neuropathy. B. Neurology Books . 1791–1866). often with ulceration. and results in deltoid weakness and Wernicke encephalopathy (see Bradley’s NiCP. 742–758). A 4 possible. it is not commonly associated with 1 = None recurrent symptoms. This is commonly 12. triceps Increasing this filter will further reduce the low- strength is spared. Chs. pp. An increased risk of leukemia is seen with least two motor nerves with conduction blocks outside various genetic disorders. the low-pass filter (high-frequency filter) can brevis. 34. these patients have anti-GM1 antibodies. and areflexia. 50. frequency filter. In lesions at the Low-frequency waveforms on EEG are usually artifacts. Test Four 121 prolongation of hypoglycemia may cause permanent does not affect the brachioradialis or cause changes in neuronal injury. syndrome. nerve (as outlined in the stem). D. To reduce this dorsal interosseous muscle and abductor pollicis artifact. patients should be given thiamine before posterior cord plexopathy affects both the axillary and dextrose-containing fluids in order to reduce the risk of radial nerves. Notably. pp. Pathergy is a phenomenon Best motor Best verbal associated with Behçet’s disease in which minor Eye opening response response cutaneous trauma is associated with robust 4 = Spontaneous 6 = Obeying 5 = Oriented inflammatory response. responds to intravenous immunoglobulin and not 1301–1323). Ch. 2 points for incomprehensible (NMO. motor and sensory deficits (see Bradley’s NiCP. also known as the low- sphincter dysfunction (see Bradley’s NiCP. plasmapheresis or steroids. Ch. especially Down syndrome. Autism spectrum electrodiagnostic findings. 7th edn. The full scale is shown here (see Bradley’s Anti-GQ1b antibodies are associated with Miller-Fisher NiCP.  The high-pass filter. C. which flows entity from CIDP. (MMN) with conduction block. 58. 7th edn. Approximately 50% of although not necessarily with FXS (see Bradley’s NiCP. and is not myelin sheath. previously known as Devic disease). pp. A. A. radial neuropathy at the spiral groove. pp.  Sodium channels are clustered in the nodes of dissociation is present in chronic inflammatory Ranvier. gene. in the range of 30 Hz or greater. of typical areas of entrapment. Chs. D. This is usually conduction testing by normal sensory studies in a C8 increased by poor electrode–skin contact. MMN 7th edn. EMG artifact is typically very affect median and ulnar muscles. either radiculopathy but abnormal ulnar and medial from improper attachment or lack of conducting antebrachial cutaneous sensory responses in a lower gel (see Bradley’s NiCP. 7th edn. 7th edn. which is ophthalmoplegia. Posterior interosseous neuropathy pp. therefore. Clinically.  The patient scores 2 points for opening eyes to myelitis is commonly seen in neuromyelitis optica painful stimulation. However. This clustering of sodium channels typically present in MMN. D.  Fragile X syndrome (FXS) is an X-linked dominant misdiagnosed as amyotrophic lateral sclerosis (ALS) disease caused by CGG triplet repeats within the FMR1 because it is a pure motor disorder with fasciculations. pp. merely pricking the skin with 2 = To pain 4 = Withdrawing 3 = Inappropriate a needle to evaluate for this reaction as evidence of 1 = None 3 = Flexing 2 = Incomprehensible Behçet disease. Cytoalbuminological 13. and cause numbness in the medial forearm. (see Bradley’s NiCP. Normal myelinated and causes a pure motor neuropathy. 10. 107. 648–675 and 90. Polio is in the Picornaviridae family from one nodal region to the next. This results in outlined in the stem) and sensation loss is confined to waves that are usually 0. spiral groove.5 Hz or 1 Hz. 814–834). The keys to discerning MMN from ALS include a lack although this phenomenon may be seen with Prader- of upper motor neuron findings in MMN and key Willi and Angelman syndromes. but is not always hallmark of MMN is normal sensory responses with at present. Lancet 2: 81–84. Although MMN is an allows depolarization to occur at the nodes. nor with ocular symptoms. 98. Although HTLV-1 infection may cause a 2 = Extending 1 = None viral myelitis.14  Glasgow coma scale for classical triad in this stem. pp. The 3 = To speech 5 = Localizing 4 = Confused pathergy test is. be reduced to 30 or 35 Hz from a standard of 70 Hz. assessment of coma and impaired consciousness ataxia. when safely and quickly sensation. a lower trunk plexopathy can look similar to Electrode impedance is a measure of resistance a C8 radiculopathy. The electrodiagnostic disorder is commonly seen with FXS. Although sensorimotor problems may present with Miller-Fisher syndrome. Jennett B 1974 Assessment of coma and Anti-GM1 antibodies are associated with multifocal impaired consciousness. a variant of Guillain-Barré syndrome.  The patient’s symptoms fit anatomically with a selected frequency’s amplitude in the range of 50%. pp. 348–365). 7th edn. 1159–1186). pp. but there would fiber conduction speeds are in the range of 40–60 m/s be no conduction blocks on nerve conduction studies (see Bradley’s NiCP. reduces the amplitude of waveforms Ch. such as the first fast. 9. 55. but they are differentiated on nerve between the skin and the electrode. 7th edn. 34–35. 7th edn. and 4 points for withdrawal to painful associated with antibodies against aquaportin-4. numbness over the shoulder in addition to radial Ch. leading autoimmune neuropathy. There is no parental imprinting in this disease. This disease is an autoimmune motor neuropathy that may mimic amyotrophic lateral sclerosis. but only reduces the 11. because it is a purely motor nerve. 1484–1518). A standard the region supplied by the superficial radial sensory low-frequency filter is usually set at 0.  This patient has multifocal motor neuropathy Ch. there is no mention of the TABLE FOR ANSWER 4. 80. trunk plexopathy. it is considered a distinct to saltatory (not statutory) conduction.

Patients typically The jugular foramen is the exit for the present with fever and spinal tenderness in the setting glossopharyngeal nerve. angioplasty. Rett syndrome.” which includes hypertension. sulcal human immunodeficiency virus. papaverine. features are not prominent in schizotypal personality and hemodilution. with close relationships. symptomatic vasospasm. accumulation of NAA in the brain.  For symptomatic vasospasm.  Although all of the other evaluations may prove Frequently the ESR is elevated. Oligoclonal bands can point toward a Merzbacher disease is a leukodystrophy that spares the diagnosis of multiple sclerosis (MS). and ophthalmic nerve. E. LETM should point toward white matter leukodystrophy with involvement of the the diagnosis of neuromyelitis optica (NMO). MRI of the brain Cerebral palsy is a term that describes a number and MRI of the thoracic spine might be performed in of conditions generally related to brain damage this patient and may show more demyelinating lesions. and posterior meningeal artery.  The mandibular nerve exits the skull base through Deramond H. imaging of the spine will show the abscess.  This patient presents with a severe transverse development of blindness (from optic atrophy). and would be the best answer (see Bradley’s NiCP. Spinal angiogram can changes with a frontal predominance. and extraspinal infections. intravenous drug use. the early swelling. issue. recent spinal intracranial pressure monitoring the most pressing surgery/procedure. and may myelitis can cause similar symptoms. including neonatal lethargy. hypotonia. Figure from Parrilo JE. along with the accessory meningeal in neuromyelitis optica and recurrent longitudinal artery and the lesser petrosal nerve. although the evidence personality disorder are suspicious of others remains uncertain. edn. These recessive leukodystrophy most often seen in the include calcium channel blockers (nimodipine. the ventilator. Transverse for seizures if there is clinical suspicion. 2014 Critical Care Medicine: Principles of Diagnosis and and often talk to themselves in public. but is not an represent an eccentric group who are uncomfortable urgent need (see Bradley’s NiCP. which affects the gray matter. microcephaly (rather than cord lesion. pp. Risk factors include useful in this patient. however. but could be treatment and surgical drainage. not an immediate need. Ch. Angiography is also used for disorder. such as beliefs in magic or telepathy. 981–995). et al. or associated with aspartoacylase deficiency. and stereotyped hand movements. for deployment of balloon 17. gram-negative bacilli. would expect for an ischemic injury. E. blocker injection (see Bradley’s NiCP. but the focal provide insight into cerebral activity in case the spinal tenderness and fever should point toward an Neurology Books . Treatment is supportive. Ch. 1324–2341). Figure from Cassinoto C. but these “triple-H. trochlear 199–205. 62. Empiric antibiotics omitted in this traumatic case. in addition to a peak in NAA on MR frequently aquaporin-4 antibodies are found in these spectroscopy. 7th edn. a poor suck. with permission. Their clothing Management in the Adult. standard therapy includes and assume most will exploit them. or for various intraarterial drugs. Cerebral emboli can seed the vertebrae or meninges and lead to angiogram is an important early step in the case of an abscess. sensory loss to all modalities. and temporal horn enlargement make state. 867–880 and 67. E. Ch. patients. abducens nerve. prominent in this condition. Chs. and U-fibers. sigmoid sinus. The 22. C. inappropriate or strange. Journal of Neuroradiology 36(4): fissure is the exit for the oculomotor nerve. intraarterial beta- presentation given is classic for Canavan disease. 7th pp. Canavan disease is verapamil. be helpful if an ischemic etiology is suspected. an autosomal. not a a mutation in the GFAP gene and has white matter longitudinally extensive lesion.  Spinal epidural abscesses require quick diagnosis to foramen rotundum is the exit for the maxillary nerve. as there is a risk of lacerum. Treatment involves both antibiotic nontraumatic subarachnoid hemorrhage. nicardipine). vascular dissection may be indicated after medical streptococci. Patients with paranoid 20. Often they will have strong pp. Transcranial Doppler monitoring may be from an endemic area). when the patient is awake and ready to wean from Guillain-Barré can cause weakness and sensory loss. but is weakness and sensory loss to pain and temperature.122 Neurology Self-Assessment 16. not an anterior cord syndrome as you macrocephaly). there is no evidence for. leading to an milrinone. 91. at or before birth and is static rather than but the aquaporin-4 antibodies are specific for NMO progressive (see Bradley’s NiCP. D. Generally. Electroencephalogram may help evaluate but does not cause loss of sphincter tone. pp. MRI of the spinal cord the foramen ovale.  Individuals with schizotypal personality disorder patient does not regain consciousness. Alexander disease is a leukodystrophy caused by segment or partial segment of the cord. nerve. but lumbar The internal carotid artery exits through the foramen puncture is not recommended. B. Instead. 1159–1186). 62. diabetes. and tuberculosis (if stabilization.  This patient has Canavan disease. myelitis as evidenced by lower extremity paralysis and spasticity. MRIs show a Seizures and poor feeding and nutrition are also longitudinally extensive transverse myelitis (LETM). and loss of developmental milestones. Anterior spinal infarcts cause done to evaluate for early signs of vasospasm. macrocephaly followed by 21. hypervolemia. Ashkenazi Jewish population. C. 18. Olindo S. the clinical presentation suggests a complete milestone regression. prevent severe neurological damage. control. 7th edn. poor head pp. 7th edn. immunocompromised effacement. with matches their eccentric nature and may be permission. 80. vagus nerve. puncturing the abscess and spreading the infection. MRI shows a diffuse spanning ≥3 spinal levels. spinal accessory of a progressive myelopathy. 4th edn. amrinone. Pelizaeus. U-fibers and is caused by deleterious PLP1 genetic but transverse myelitis in MS is usually a small events. A T-tube trial will be useful but preserved proprioception and vibration sensation. 19. The superior orbital extensive myelitis. Mosby. which is possible. Dellinger RP unusual beliefs. The case or standard practice that includes. Notably. tends to present with However. 867–880). evaluation for must include coverage for Staphylococcus aureus. Magnetic resonance nerve. an external ventricular drain should be Endocarditis presents a significant risk because septic placed with pressure-monitoring capability.

with permission. Although administering steroids can precipitate a myasthenic crisis.  The woman in the figure demonstrates a right eye the loss of the awake alpha posterior dominant rhythm with full horizontal ductions and a left eye with and the presence of slow eye rolling movements. will cause a phase reversal in the referential montage—a fairly unique feature to BECTS. if other physicians in the demonstrates bilateral independent centrotemporal area can provide similar specialized care and the spikes with a horizontal dipole during sleep. her request should be years of age and resolve in the early to middle teens. 25. the physician painful situation as purely academic. which pp.  Adolescents are given some freedom to make mechanism. as normal adduction but no abduction. D. not focal spikes with Cushing syndrome. This patient. but <20% of each epoch. Test Four 123 abscess rather than merely a transverse myelitis. and mechanism in which patients make excuses for their mental health. the administration of steroids (see Bradley’s NiCP. B. acceptable. Oculomotor nerve palsy leads to inferior 3 and 4 are frequently put together and called slow and abducted resting position of the eye. it should be performed 27. Denial is a neurotic defense mechanism. likely related to chronic as is present with BECTS.  Defense mechanisms are classically divided into with horizontal diplopia when looking down with an three categories. Ch. 7th edn. seizures typically start between 5 and 8 injury or death. 1720–1735). Seminars in 24. wherein a patient attributes his or her own healthcare decisions for themselves. best seen physician is a close friend of the family and believes Neurology Books . Most epileptic spikes have a Vertebral fractures can cause a similar clinical picture vertical dipole with the negative surface component 4 with weakness and focal tenderness. A. Closmann JJ. ptosis and mydriasis in some cases.  The EEG and clinical scenario are most consistent suffering from a serious condition. Stage 3 is marked by delta waves nerve innervates the lateral oblique muscle of the eye. hyperkalemia. 52. Projection is an immature defense 29. the physician should encourage the followed by clonic movements of the mouth. but the EEG adrenal insufficiency and administer stress-dose demonstrates continuous slow wave and spike steroids. with permission. spindles and K complexes. 1563–1614). adduction and elevation. 7th 100. 102. painful reality. status and hypotension. 696–712). It is also associated with pp. pp. formerly known as benign rolandic epilepsy. 79. Ch.  The arrow indicates a subependymal in this setting given the high morbidity and mortality hamartomatous nodule. including mature. It is not necessary to dismiss consciousness. Rationalization is a neurotic cases of pregnancy. although 26.  The examination findings of central obesity. Sublimation is the only mature defense pp. 101. present in 20%–50% of the recording. and is exemplified by channeling and Baus MR 2008 Idiopathic unilateral cranial nerve one’s unacceptable impulses into something VI palsy: a case report and review of the literature. Stage 4 is and abducens nerve palsy causes ipsilateral loss of composed of >50% delta waves in the recording. Autosomal-dominant frontal prednisone therapy for myasthenia gravis. mechanism listed. and endocarditis type of phase reversal is seen in typical montages but should lead you to an epidural abscess as the not the referential montage.  Stage 2 sleep is characterized by the appearance of Perinatology 39(1): 36–43. Pomeroy SL 2016 Bradley’s Neurology in Clinical and metabolic acidosis are also consistent with adrenal Practice. Ch. Most seizures arise out of sleep and can the patient from the clinic as long as confidentiality secondarily generalize on occasion. however. pp. so she is not in immediate danger of serious this syndrome. Figure from Hsu CS. In normal. Delta waves can be present. 1147–1158). as these will self-correct with and survival pathway (see Bradley’s NiCP. In this lobe epilepsy has seizures that arise out of sleep. Jankovic J. A. In cases of minors who can make decisions These episodes usually involve paresthesias of the face for themselves. 7th edn. 1282– and is seen when a patient avoids becoming aware of a 1286. but the patient tubers (left frontal operculum and right parasagittal). due to medication noncompliance. but not usually and not keep them secret. A Tuberous sclerosis is a disease of cellular overgrowth correction of the electrolyte abnormalities is not the related to overactivation of PI3K/Akt/mTOR cell growth best answer in this case. 7th edn. Plasmapheresis and IVIG are both Further pointing toward this diagnosis are two cortical options to treat a myasthenic crisis. Brain overgrowth in disorders of RTK-PI3K-AKT signaling: a mosaic of malformations. and round facies are consistent generalized spike and wave discharges. Ch. has been with benign epilepsy with centrotemporal spikes maintaining her weight and her laboratory work is (BECTS). insufficiency. absence epilepsy and juvenile myoclonic epilepsy have multiple bruises. with impaired wave sleep (see Bradley’s NiCP. Trochlear nerve palsy leads to resting hyperopia on the affected side. and adducted eye (see Bradley’s NiCP. neurotic. of adrenal crisis. abnormalities such as hyponatremia. 1615–1685). Stage 1 sleep is marked by 28. This are not associated with fevers. Ch. Elsevier. 7th edn. given that the patient has altered mental spikes are seen frontally without a horizontal dipole. Stage abduction. striae. The onset of adrenal insufficiency in this case discharges along with language dysfunction (see was likely prompted by abrupt withdrawal of steroids Bradley’s NiCP. Electrolyte Figure from Daroff RB. Intellectualization is also a neurotic immediate threat to her health and she requests that mechanism in which feelings are avoided by seeing a the information remain confidential. The EEG can be respected. Figure from Hevner RF 2015 edn. as is seen in tuberous sclerosis. immature. 104. As such. 7th edn. especially in the feelings to someone else. 7th edn. 1538–1562). one should think about Landau-Kleffner also arises out of sleep. Ch. D. pp. However. Juvenile 23. on a referential montage. both a positive and negative surface component. but scenario. Mazziotta JC. Journal of Oral and Maxillofacial Surgery 66(6). A horizontal dipole has diagnosis (see Bradley’s NiCP. This can patient to discuss their conditions with their parents impair the ability to speak. A. with permission. As long as the patient is not an actions or feelings. D. they and a positive component deeper in the brain. The abducens well as vertex waves. should respect this request. does not have symptoms of myasthenic crisis. sexually related health issues. respected.

A. MDD can involve a low mood. and mild opiates. and significant information from the muscle spindles. nucleus cuneatus (for the arms). Suprapontine lesions (as due to the risk of early closure of the ductus arteriosus. including opiates. D. C. IRBs are governed by the Office of Human Research Protections within the Department of Health 35. with permission. this case for a severe headache. a helplessness. and informed consent is pp. MDD is not usually in stroke. and autosomal-recessive disorder caused by a defect in hypersensitivity to little or no stimuli. Ch. 1973–1991). but may also have interrupted stream and vasoconstriction may put the mother at a higher risk incontinence due to detrusor-sphincter dyssynergia of stroke or heart attack. tricyclic inflammatory infiltrate with CD8+ lymphocytes. 7th edn. often described as burning. The anterior commissure members can result in significant distress and even of the spinal cord crosses the midline of the spinal occasional hallucinations in normal bereavement. The second-order The behavioral symptoms (either anxiety or neurons form the medial lemniscus. Becker muscular dystrophy is the somatosensory cortex.  There are three neurons in the major pathway of and Human Services. E. Ch.  In general. which stains brightly miscommunication between the afferent pathway and with PAS staining. neurogenic bladder dysfunction can be and ketorolac should not be used in the third trimester grouped by lesion location. 7th edn. D. though.  Adjustment disorders involve the development of in the medulla at the nucleus gracilis (for the legs) and behavioral symptoms within 3 months of a stressor. and often involves trials fibrosis. (see Bradley’s NiCP. antidepressants.124 Neurology Self-Assessment this personal relationship will affect professional that can cause fiber type grouping and group atrophy judgment. poor stream. and changes to appetite condition developing weeks to months after a thalamic and sleep. Figure from Klatt EC 2014 Robbins and Cotran Atlas of 30. This results in a buildup of is thought to stem from a stroke-induced glycogen below the sarcolemma. referral to another physician could be on muscle biopsy and onion bulbs on nerve biopsy considered. Death of close friends or family to the primary sensory cortex. 7th edn. and vasoconstriction of the (poorly coordinated activity of detrusor and sphincter placenta may result in relative ischemia to the fetus muscles). An acute stress the head of the dorsal horn. Research is ongoing with regard to the use of pathological features. 7th edn. tramadol. 112. pp. in this case the stressful event was not pain and temperature sensation to the contralateral closely related to her. Third-order neurons go from the thalamus expected response. Unfortunately.  The clinical scenario describes Dejerine-Roussy feelings of worthlessness. Polymyositis is associated with an endomysial of multiple agents. Ch. and notably large postvoid risk must be matched by sufficient benefit to patients. The diagnosis of myotonic deep brain stimulation to modulate pain in thalamic dystrophy is usually made by testing the DMPK gene pain syndromes. The reason this scenario does not on chromosome 19 for CTG repeats. are 31. seen with stroke) are often associated with urinary Valproic acid has the most risk in early pregnancy due frequency and urgency due to detrusor overactivity. recurrent thoughts. Notably. 47. or topical analgesics such as lidocaine sizes and central nuclei but has no characteristic patches. anticonvulsants such as gabapentin or Myotonic dystrophy is associated with variation in fiber pregabalin. 26. or those at the conus medullaris or cauda equina. Saunders. This carries However. E. These lesions may or may not result in (see Bradley’s NiCP. month. 110. 3rd edn. the specific analyses thereof may be outside dorsal root ganglion. Patients tend to have severe and chronic pain response to a particular stressful event. disorder. Lesions below the level of the pons but above the Sumatriptan can be used during pregnancy. to neural tube defects. which decussates depression) typically cause significant functional and travels to the ventral posteromedial nucleus of the impairment and seem to be out of proportion to the thalamus. Posttraumatic stress disorder interneurons that synapse on motor neurons to make involves similar clinical symptoms to an acute stress spinal reflexes (see Bradley’s NiCP. tingling. Although IRBs do review the sensation for joint position sensation and vibratory scientific method posed for a given research study. resulting in performance of human research. also known as thalamic pain syndrome.  McArdle disease is type V glycogenosis. out of proportion to the environmental stimulus 33. Charcot-Marie. which houses its cell body in the achieved. Rexed lamina VI receives of increased anxiety and arousal. long-term cognitive effects if used later in pregnancy. D. cord anterior to the gray matter of the cord. This syndrome myophosphorylase. 1915–1955). anhedonia.or third-line agent after with urinary frequency and urgency due to detrusor acetaminophen or mild narcotics because overactivity. 605–621). 273–278). residual volumes (see Bradley’s NiCP. The a tragedy such as a plane crash but not an inability substantia gelatinosa of the spinal cord is located in to continue with her regular life. These neurons synapse 32. obtained. Other options include acetaminophen. in sensation. guilt. but is sacrum (as seen with spinal cord injury) are associated considered a second. Ibuprofen 34. projection that bypasses synapses in the spinal cord and forms the dorsal columns. symptoms cerebellum or thalamus. syndrome. Ch. but there are concerns with with normal flow and minimal postvoid residual.  Hydrocodone is the most appropriate medication in Pathology. Infrasacral lesions. thalamic pain associated with atrophic fibers. human rights are respected. pp. an (allodynia). for which minimal hesitancy. hypertrophic fibers. One would expect sadness from side to form the anterior spinothalamic tract. The first-order neuron is the actual sensory order to ensure that a scientifically sound result may be receptor in the skin. and is very challenging to treat. which are the clinical impairment. which receives a variety of disorder must begin within 4 weeks of a stressful event mechanical and sensory information to relay to the and involves flashbacks. except the symptoms must be present for >1 pp.  IRBs are committees empowered to ensure the safe associated with detrusor underactivity. represent a new stroke is that new infarcts develop Tooth 1a is a hereditary demyelinating polyneuropathy acutely and tend to cause “negative symptoms” such as Neurology Books . 36. These cells have a central the scope of an IRB. decreased libido. suicidal ideation. elevated postvoid residual.

  For the long-term prevention of episodic cluster branchial pouches during embryogenesis lead to headaches. should be performed (but not primary nerve repair). E. INR not consistent with a peripheral cause of pain in this >1. including facial deformities like pp.6. 107. generally have earlier onset than the parent. the history and localization are improving symptoms. 7th edn. Mazziotta JC. see Bradley’s NiCP. pp. ratio (INR) should be immediately obtained. Ch. surgical resection may change or structural lesions. There is little to suggest a and is without exclusion criteria. and the prevention of other headache types. leading to earlier disease onset. Further. Stretch injury similarly would not be benefitted by surgical nerve repair. It is associated with 6 months (see Bradley’s NiCP. Neither although clear differentiation is often difficult by MRI the gender of the patient nor of the affected parent alone. Although poststroke patients can develop stroke within the last 3 months. 96). 4 case. with permission. Generally such effects are long-term is a specific CAG triplet repeat. 903–919). Ch. 7th edn. 648–675). Ch. the triplet repeat length increases over subsequent 44. C. after radiation therapy. Additionally. 39. Amitriptyline is often beneficial in the 43.5 hours of the last known normal patient is symptomatic. Laboratory tests such as a be considered. in which case fasciotomy stroke (malignant cerebral edema. phenomenon is known as genetic anticipation. If symptomatic. 103. blood pressure were above 185 mm Hg systolic or as with a propeller. 1791–1866).  Telling patients to learn to deal with a given issue hemicrania. Ch. except in the case of swelling in the next week if there were a large territory compartment syndrome. image shows an epineural blood vessel with fibrinoid but is generally not helpful for cluster headaches. and the rest given over 1 hour. There is no that they can be easily identified in 2–3 weeks for evidence for impending herniation in this particular primary nerve graft repair. swelling of the face and lips. otherwise. 314–323). if IV tPA is to be given. indomethacin.  The length of the triplet repeat in the Huntingtin Pomeroy SL 2016 Bradley’s Neurology in Clinical Practice. Although observation alone might be point-of-care glucose and international normalized appropriate if radiation necrosis is found incidentally. Test Four 125 numbness. C. but genetic anticipation means that the patient will radiation necrosis tends to be less nodular than tumor. usually seen in the pediatric have a better prognosis than those who receive it later. Although paroxysmal 42. In addition. generally need to be surgically 110 mm Hg diastolic. anomalies of the third and fourth 38. Radiation therapy would likely initially. but rather tolerating a cephalgias. 65. E. problem. Ch. a thalamic > 185 mm Hg.  The next best step in this case is to obtain a necrosis can first be treated with steroids. this is not useful for preventive therapy. such as migraine. intracranial hemorrhage. 1686–1719). 7th edn. or if debrided first. at least 24 hours. if IV tPA is given. 7th edn.000. More ragged nerve tears. Photon scatter from high-dose radiation for (parental imprinting) affects symptom onset in head and neck cancers often leads to adjacent cerebral Huntington disease. Bradley’s NiCP. aspirin should not be administered for 37. pp. as with a knife or scalpel. necrosis of its wall and perivascular and transmural Levetiracetam has little evidence in headache inflammation. Nerve endings may be tacked to fascia so above 220 mm Hg systolic otherwise. B. repair chromosome 22q11. 7th edn. Figure from Daroff RB. 920–967). Ch. Similarly. occurring a few years or. but the patient would be at risk of intracerebral does not require surgical repair. Parental heterogeneous enhancement with fluid attenuated age of onset may provide a rough guide for a patient.  Clean nerve transection. and is commonly inherited in an is indicated. but it would only be needed if the clean and well-perfused injury. population (see Bradley’s NiCP. decades (see Bradley’s NiCP. pp. Nicardipine is an option to decrease is generally repaired immediately due to the relatively blood pressure. not a point mutation complications. platelet count <100. 7th edn. 50. 64. If meaningful nerve regeneration 41. but paraneoplastic diseases often cause such focal. Neurology Books . radiation 40. Ch. rarely. are not limited to.  DiGeorge syndrome results from a deletion on is not seen by 5 months after humerus fracture. this should not be pp. Melkersson-Rosenthal syndrome is characterized by and the risk of death is similar. those who receive IV tPA earlier and a furrowed tongue. A. nor do out hemorrhage.  The history and MRI are compatible with radiation generations. Compressive neurapraxia case. gene is associated with age of symptom onset. All of the other items listed in the answer cluster headaches are generally not responsive to choices are routes to advocating for patients. various anomalies. 7th edn. D. Ch. major surgery or head trauma in the last 2 weeks. rapidly enhancing lesions (see Bradley’s NiCP. then IV tPA should be fungal infection in this case. A. so antifungal therapy considered at a dose of 0. 30. inversion recovery (FLAIR) signal change.9 mg/kg. 7th edn. systolic blood pressure headaches or migraines after their infarct. with or without recurring facial paralysis. treatment. verapamil is generally considered first-line congenital absence of the thymus and parathyroid (see therapy. In general. There is no suggestion of without first obtaining a noncontrast head CT to rule paraneoplastic disease in the case description. Jankovic J. The cause of Huntington disease necrosis. administered before a CT of the brain. or aneurysm. Notably. Although aspirin is needed. 75. noncontrast head CT to rule out cerebral hemorrhage Antiangiogenic therapy (with bevacizumab) has been and to ensure there is not already major ischemic useful in some cases. On imaging. another of the trigeminal autonomic is not advocating for them. 7th edn. and known arteriovenous malformation pain syndrome is the most likely etiology in this case.  The case is one of mononeuritis multiplex. Examples of exclusions include. with a 10% bolus would not be necessary. but not exacerbate the problem. pp. Although inhaled oxygen is efficacious for Bradley’s NiCP. Elsevier. is exquisitely sensitive to indomethacin. If the it would not be appropriate in this case given that the patient is within 4. The risk of bleeding is around 1%–6%. cleft palate and cardiac anomalies like tetralogy of Fallot. this can present as diffuse. This necrosis. and generally has best results before autosomal-dominant pattern. consistent with a vasculitic process (see prevention (see Bradley’s NiCP. abortive therapy for cluster headaches.

Central retinal Bouras C 2009 Neuropathology of Lewy body vein occlusion is often acute and painless. but can also be disease course. 163–178). including cytoplasmic inclusions formed from the aggregation of tachycardia. hyperhidrosis. posterior communicating artery. Arteritic AION is R2 response is seen both ipsilaterally and associated with giant cell arteritis. most often seen in Alzheimer disease and are 1365–1379). unstable plaque in the carotid artery. Motor alpha synuclein and ubiquitin.  Central retinal artery occlusion classically presents inclusions seen in rabies. Typically. The two of the visual field is affected in the eye.  The correct answer.  The branches from the internal carotid (in order veins.5 seen in Parkinson disease. virus (see Bradley’s NiCP. whereas the output of CN VII on the left edn. ophthalmic artery. pp. 7th edn. Brain Research Bulletin 80(4–5): 203–210. and a normal permission. These are seen most and cognitive symptoms tend to come later in the often in dementia with Lewy bodies. with an average survival of about 1. Fundoscopy shows tortuosity and dilation of retinal 50. neuromyelitis optica. With left-sided stimulation there Waheed NK. pp. whereas the occasional flame hemorrhages. R1 is seen ipsilateral to stimulation. In the given be transient or acute and persistent. including sudden. Ch. although this may be severe supraorbital nerve is stimulated independently with blurring rather than darkness. Anterior ischemic optic neuropathy (AION) may be either arteritic or 45. Immediate stroke evaluation is important due to risk of 1380–1421). Schizoaffective disorder has prominent mood Figure from Aminoff M 2012 Aminoff’s Electrodiagnosis symptoms and at least 2 weeks of psychotic symptoms in Clinical Neurology. with low-amplitude ipsilateral R2 response. hallucinations. with oculi. Fundoscopy in arteritic AION is likely to response. often with example. Figure from Kövari E. Fundoscopy with nonarteritic AION are electromyography responses from the orbicularis generally reveals optic disc edema and hyperemia. 7th both working. Nonarteritic AION is more common. 920–967). each painless vision loss. if left ipsilateral R1 response. and then bifurcation into the symptoms. and schizophrenia for 6 months or more. Patients generally present with progressive. C. only 3 days after the accident. 7th edn. Absent responses with unilateral hallucinations with disorganized behavior. pp. and the symptoms are not accounted for by patterns of abnormalities—usually the loss of R2 mood or substance exposure. with flame hemorrhages. a delayed and OCT: Optical Coherent Tomography. but generally has a more gradual onset of vision anterior choroidal artery. 7th edn. Cowdry A inclusions are Fundoscopy is often associated with a cherry-red spot eosinophilic inclusions seen in the nucleus of a surrounded by retinal whitening.126 Neurology Self-Assessment pp. eosinophilic severe insomnia and dysautonomia. Pontine strokes can cause various month. Spongiform changes are with a sudden. robust R2 response contralaterally. In this testing. seems to be affected. Horvath J. there is a return to the premorbid psychiatric state after a brief 46. profound. elongated filamentous intracellular inclusions made of tau protein. as is most likely in this case. 35. Figure from Winn H 2011 Youmans of optic neuritis include multiple sclerosis or Neurological Surgery. opposed to profound vision loss with CRAO). but disorders. The most common causes NiCP. pp. with permission. generally a moderate decrease in visual acuity (as with permission. with right-sided stimulation there is a normal contralateral vision loss within 14 days. and check the function of cranial nerves (CN) V and VII may present similarly to CRAO. psychosis precludes bereavement. pp. with in the absence of mood symptoms. 17. Ch. This demonstrates that the left CN V and brainstem are working because 48. she needs to would indicate a trigeminal neuropathy on the side have one or more delusions. A. Her psychosis started after VII is not working well because of the poor ipsilateral witnessing a horrible accident and consists of visual R1 and R2 responses. 366–390). Optic neuritis is generally associated with anterior and middle cerebral arteries (see Bradley’s subacute central vision loss. which altitudinal pattern. a spongelike appearance. C.  Lewy bodies are relatively large. genetic prion disease associated with mutations in the PRNP gene. This shows the right-sided CN V and VII are and/or cotton wool spots (see Bradley’s NiCP.  Fatal familial insomnia is an autosomal-dominant psychotic disorder. Ch. and/or grossly disorganized or internal capsule strokes would not cause blink reflex catatonic behavior. Neurology Books . The presence of permission. The vision loss can contralaterally in a normal response. The duration is >1 day but <1 abnormalities. Saunders. round. The two most common causes are vasculitis or many large extracellular vacuoles. A. 94. Neurofibrillary tangles are years (see Bradley’s NiCP. and hyperpyrexia. a normal ipsilateral R2 untreated. (see Bradley’s NiCP. Negri bodies are small intracellular 47. 95. C. 7th edn. often only part recording electrodes over the orbicularis oculi. see the figure) are the retinopathy is associated with antiretinal antibodies. Trigeminal neuralgia and disorganized speech. is of the robust contralateral R2 response. Further. 1065–1083). with absent responses.  Blink reflexes are uncommonly used tests that nonarteritic. 49. pale disc with retinal whitening response. Saunders. Figure from Duker JS. Paraneoplastic from proximal to distal. Management may neuron in herpes simplex virus or varicella zoster involve ocular massage or tissue plasminogen activator. In order to stimulation but normal responses on the other side meet criteria for brief psychotic disorder. generally in an responses seen are the R1 and R2 responses. 65. Schizophreniform responses or significant delay in R2 responses disorder is characterized by psychosis for >1 month but bilaterally with stimulation of the affected side <6 months. Saunders. D. Ch. but the left CN brief psychotic disorder. painless vision loss in one seen in prion diseases and involve the presence of eye. Goldman D 2014 Handbook of Retinal is an almost-absent R1 response. and the brainstem relay systems. giving the tissue atherosclerosis. 6th edn. Ch. and a delayed low-amplitude contralateral R2 show an edematous.

  Haloperidol is one of the typical antipsychotics goal-directed behavior. B. often resulting in seizures postictal period. ischemic. distractibility. and cognitive decline. which usually consists of associated with a systemic infection.  The Purkinje cells are the major output cells of the electroencephalogram. 101. minutes and has no focal postictal features. or more than one seizure per (see Bradley’s NiCP. 1226–1236).  The MRI shown depicts schizencephaly. A. Additional considerations include macrocytic anemia and hypercoagulability due to External carotid artery resultant hyperhomocysteinemia. The followed by semilobar. 24-hour period. 53. subacute combined degeneration of the spinal cord.  The patient is in the midst of an acute manic The Purkinje cells are GABAergic and send inhibitory episode with history of depressive disorder in the past signals to the deep cerebellum and brainstem. Izumi T 2012 The seizures. Treatment involves 4 Anterior artery artery mood stabilizers. consists of multiple shallow gyri. The mood disturbance must be cerebral cerebral severe enough to cause impairment. This is in younger than 5 years of age and are generally contrast to porencephaly. one must have at least three or cerebellar degeneration (see Bradley’s NiCP. 34(7): 546–552. E. cerebellar cortex. 1563–1614). a normally developed child with no neurological Polymicrogyria is a migrational abnormality that abnormalities and no family history of seizures. associated with abnormally placed islands of gray family history of seizure. The alobar subtype is the most severe. simple febrile seizures are usually generalized. 7th edn.  Febrile seizures occur in 2%–4% of children open-lipped (shown) and closed-lipped. as this patient has mania (not hypomania) and has met prior criteria for major depressive episode. They are divided cysts lined by white matter. Simple or developmental insult. Periventricular nodular who does not meet the aforementioned criteria.  Vitamin B12 is a key cofactor to many biochemical Cervical reactions in the body. usually associated Notably. Holoprosencephaly diagnosis of simple febrile seizures is contingent upon can sometimes be associated with septooptic dysplasia. so its deficiency may cause segment Internal carotid artery many unwanted effects. 85. which usually has milder Supraclinoid Posterior symptoms and lack of significant functional segment communicating Ophthalmic impairment in contrast to mania. developmental conditions such as Aicardi syndrome Complex febrile seizures are diagnosed in anyone and DiGeorge syndrome. with permission. with permission. E. Seizure prophylaxis is cerebellum. 7th edn. Ch. Elsevier. causing loss of proprioception and ataxia. C. antipsychotics. lined with gray matter. Figure for Answer 4. Bipolar 2 disorder artery consists of hypomania. Ch. D. pp. 7th edn. prolonged matter near the ventricles. but only if an episode of Petrous mania occurs. The episode of acute artery artery mania would preclude a diagnosis of major depressive Cavernous disorder. and more elaborate workups may be pursued. Cyclothymic disorder is unlikely. increased 56. Test Four 127 Middle Anterior pleasurable activities. Maeda T. then lobar. Sensory information arrives through but may be considered in complex febrile seizures the mossy fibers and is first processed by granular cells (see Bradley’s NiCP. There are two types of schizencephaly: 51. and in the molecular layer by the stellate and basket cells layer. mania is not generally seen (see Bradley’s NiCP. Purkinje that required hospitalization. more of the following for at least 1 week in duration: Ch. Holoprosencephaly is caused febrile seizures are characterized by no more than one by failure of the brain to divide into the left and right seizure in a 24-hour period. including lumbar puncture and/or 55. 7th edn. Brain & Development relatively higher than with simple febrile seizures. To meet criteria for cells are the main target in diseases like paraneoplastic bipolar 1 disorder. heterotopia is also a migrational abnormality that is including patients with developmental abnormalities. caused by a toxic.) 54. 89. 4th edn. pp. which in turn send signals to Purkinje cells. pressured speech. a developmental abnormality that consists of a cleft extending from the ependyma to the pia mater. pp. 1279–1300). a major depressive episode may segment be a part of bipolar disorder. flight of ideas. 1461–1483). Conversely. the likelihood of an underlying diagnosis differences in epileptic characteristics in patients with of epilepsy or central nervous system infection is porencephaly and schizencephaly. pp. focal seizures. There is no psychosis in this case to segment suggest schizophrenia. inflated self-esteem. Chief among these for the neurologist are demyelinating myeloneuropathy. and selective choroidal serotonin reuptake inhibitors. with congenital cytomegalovirus or other with no focal features or preceding automatisms. or excessive involvement with whose mechanism of action involves blocking the D2 Neurology Books . into either simple or complex categories. They are located in the middle layer of the generally not needed for simple febrile seizures.50 (from Caplan LR 2009 Caplan’s Stroke: A Clinical Approach. decreased need for sleep. 97. which lasts less than 15 hemispheres. In patients with complex febrile Figure from Shimizu M. Although depression can be seen with vitamin B12 deficiency. Ch. 52.

and ophthalmoplegia. the prick below the level of the lesion. Ch. pp. Ch. B. vomiting. this is called subacute combined degeneration. ataxia. Vigabatrin irreversibly the cerebrum. whereas memory and ataxia take much including intermittent headache. If left untreated. Lazaro RT. Response for OTC deficiency. and arginase emergent setting. 26. and disorientation.128 Neurology Self-Assessment receptor. gray matter. especially in the postpartum Korsakoff syndrome. there is also a ammonia. with permission. and is involved in posture contralateral loss of sensation to temperature and pin control and voluntary movement. women can also have symptoms. urinary retention. Clonazepam and phenobarbital bind GABA receptors. anticholinergic properties. nucleus and receives input from the vestibular system. Many patients are left with movement abnormalities. A lesion on the right side of the cervical cord Intermediate zone would cause right-sided weakness. The APOE4 allele is associated Neurology Books . pp. Other FLAIR or T2 hyperintense lesions in the periaqueductal symptoms may include vomiting. coma. The position and vibratory sensation bilaterally without orientation of the deep cerebellar structures is loss of strength. It is one of the high-potency D2 antagonists. medial thalami. 273–278).  Deep cerebellar nuclei transmit information back to increasing GABA activity. resolve quickly. 6th edn. transmitted through mossy and climbing fibers. deficiency. which involves ipsilateral weakness. Anger Emboliform is the second stage in which they become easily nucleus Globose frustrated or question why this loss has befallen them. Figure for Answer 4. Lesions of the posterior columns cause loss of posture and proximal limb movements. 91. the syndrome will deficiency. which avoidance of dietary protein.  This patient has a lesion on the left side of her immediately medial to the dentate. nucleus Bargaining is the third stage in which people try to make deals to take away the loss that they have Fastigial suffered. When it occurs in the setting of B12 demonstrated in the figure. The Tiagabine inhibits the reuptake of GABA into main efferent pathway from the cerebellum is the presynaptic terminals (see Bradley’s NiCP. period with the triad of hyperammonemia. sodium benzoate. 61. A. However. 7th edn. Central cord emboliform and globose are known as the interposed syndrome involves weakness of both upper extremities nuclei. B. 1563–1614). Cerebral edema can be treated with high potential for acute extrapyramidal side effects mannitol. A lesion of the left side of the pons would cause ipsilateral facial weakness and contralateral extremity Medial zone weakness. 7th edn. but higher leading to antiepileptic and antinociceptive effects. tectal plates. Together. The fastigial is the most medial deep cerebellar more so than the lower extremities. lateral nucleus. It is commonly seen in alcoholics or patients with 58. 62. and left-sided loss of pin prick and temperature sensation (see Bradley’s NiCP. and sensory dysfunction of varying degrees. confabulation. Others (especially after gastric bypass surgery). Roller M. with decreased dietary intake or absorption of thiamine OTC deficiency being the most common. 57. E. superior cerebellar peduncle. and medial to the emboliform. or sodium phenylacetate. Even those infants treated early are still such as acute dystonic reactions (responsive to highly likely to develop mental retardation (see benztropine or diphenhydramine). A. The high-potency agents arginine. The low-potency agents have a binds calcium channels in the central nervous system.) thiamine deficiency (vitamin B1) leading to the triad of memory loss. Korsakoff syndrome can be acutely precipitated by argininosuccinic acid synthetase deficiency.  Wernicke-Korsakoff syndrome is caused by a pp. D. In this stage. Most urea cycle disorders present in the newborn retrograde amnesia. people will choose nucleus to ignore the loss or live in an alternate reality. but ocular symptoms tend to deficiency is X-linked. and projects to the cervical spinal cord resulting in Brown-Seqúard red nucleus and lateral thalamic nucleus.57 (from Umphred DA. as well as chronic Bradley’s NiCP. extrapyramidal symptoms such as tardive orofacial 59. are good for injection and for reducing positive Hemodialysis may also be helpful to remove excessive psychotic symptoms acutely. which involves anterograde and state. or eye longer to improve. Burton G 2013 Neurological Rehabilitation. as well as treatment with are two low-potency agents. which is X-linked. pp. Although OTC to treatment is variable. Magnetic resonance imaging abnormalities include encephalopathy. which can ultimately contributing to the corticoreticulospinal and result in a capelike loss of sensation with cervical vestibulospinal systems. The dentate is the most 101. The mammillary bodies. but it dyskinesia or dystonia. 631–652. administering glucose without thiamine in an argininosuccinic acid lyase deficiency. modulating syndrome. All of these are autosomal recessive except progress to encephalopathy and even death. Wernicke- include carbamoyl phosphate synthetase deficiency. hypothermia. 1324–1341). and respiratory alkalosis. and bilateral or seizures (and may be confused with sepsis). 7th edn. The globose is immediately ipsilateral loss of vibration and position sensation.  Denial is considered the first stage in the Kübler- Dentate Ross stages of grief. and the nucleus fifth stage acceptance. The emboliform is 60.  Pregabalin’s exact mechanism is not known. Elsevier. right-sided loss of Lateral zone vibration/proprioception. voluntary movements. and controls voluntary movement planning and initiation. Ch. lower risk of extrapyramidal side effects. generally through excitatory output inhibits GABA metabolism to increase GABA activity. leading to modulation of lesions. The fourth stage is depression.  There are five primary urea cycle disorders. clinical approach to urea cycle disorders includes the as opposed to chlorpromazine and thioridazine.

motor units may not be States. Nurses are justified in questioning orders by physicians. hypsarrhythmia. examination and symptoms are mostly neurological in dominant focal epilepsy with continuous spike and nature rather than multisystemic as in somatization wave discharges in the dominant temporal lobe during disorder. and seizures continue beyond 6 years of age or develop associated with significant edema. Ch. this develops during a prolonged intensive care unit ADEM may follow illness. There is resistance) but no localizable neurological findings. There is no indication of dysmetria. Rett horn cell injury (i. lungs and the brain. absent reflexes. and mental retardation. Critical illness neuropathy can to discern from West Nile virus. B. nerve conduction abnormalities are not seen 4 syndrome (see Bradley’s NiCP. Landau- 68. 1226–1236). diagnosis of Guillain-Barré syndrome (GBS). including lumbrosacral plexus. Ch. 1563–1614). and she has a believe is inappropriate. which is located in the periphery near the mutations. conversion (AMA Code of Medical Ethics). truncal instability. have confusion. motor units should be myelitis. and activated and would be of short duration and low the summer months present the greatest incidence due amplitude. or a molar 66. Guillain-Barré syndrome may be difficult recruitment changes. pp. 7th edn. but does not usually cause diffuse weakness and loss of reflexes. may pass before abnormal spontaneous activity is seen. and has no known association to Wernicke-Korsakoff Generally. 85. Walking requires great effort. found in females with normal development early on. and this individual has been vaccinated. for the first several days with GBS. GEFS+ is a condition in which febrile gray-white junction. stiff neck. spontaneous abortion. D. and intense function declines as seizures become intractable. pp. and fibrillations would be present. West syndrome ataxic dysarthria. Treatment is supportive. which corresponds temporally with a new also have the obligation to not provide care they stressor. Nurses disorder. 101. Glioblastomas are primary brain tumors. 78. Reduced recruitment should be seen immediately in 63. This is most into epilepsy without regard to fever. cognitive behavioral therapy. although deficits may persist. cognitive inhibitors. 107. 67. diffuse spontaneous activity. no need to contact the risk management team or report Observation shows that even though the patient the nurse to her charge nurse. history of preceding diarrheal illness. It commonly occurs after a pp. represents the triad of infantile spasms. After 1 month of symptoms. myotonia and small-amplitude. Finally. 1121–1146). Choriocarcinoma is with mutations in the sodium channel–encoding gene a rare cancer that frequently metastasizes to both the SCN1B (see Bradley’s NiCP. and either rhombencephalitis or no effect on F waves. 7th edn. would not have changes in reflexes or F waves. or wide-based. This disorder can present with precipitous accompanied with bulbar weakness. Rett syndrome is bowel/bladder changes. Dravet physical therapy. attempting to flex the contralateral leg against not changing orders to appease her concerns. she does so when give medications that she is uncomfortable giving is an moving about in bed. If the Poliovirus infections are exceedingly rare in the United exacerbation is severe enough. but rarely has ongoing fever stay. chromosome in some cases. Polymyositis would have including encephalitis. D. Ch. Acid maltase deficiency is a treatable metabolic and would be apparent on MRI. and constipation (see Bradley’s NiCP. disorder is most likely.  The patient likely has a West Nile virus infection weak muscles from GBS. movements of the hands. Ch. but there should be no spontaneous activity or this unlikely. premature labor. and the cauda equina. is an autosomal. and significant positive waves and its mosquito vector. Nerve conduction studies would be disturbance (including loss of pupillary expected to be normal. B.  Dravet syndrome (early-onset epileptic stressor no longer is required to meet criteria for encephalopathy) is associated with early generalized conversion disorder.  This patient has a contrast-enhancing lesion on her Kleffner syndrome is associated with GRIN2A MRI. round. Myasthenia gravis mortality is low. including prominent 7th edn. 7th edn. short-duration motor 64. Landau-Kleffner syndrome. and possibly bilateral lower associated with MECP2 mutations. making seen. West syndrome extremity (true) weakness. Keep in mind that a preceding 65. 1791–1866). D. pp. A. 742–758 and Ch. The best response is to educate Hoover sign (lack of leg extension into the bed while the nurse on why certain medications are necessary.e.  The relationship between physicians and nurses units (see Bradley’s NiCP. This lesion is small. West Nile virus) would not be syndrome is a neurodevelopmental disorder usually intermittent and not have normal tone and reflexes. but unfortunately this patient causing diffuse central nervous system symptoms. should be focused on appropriate care of the patient. Given these findings. Test Four 129 with an increased risk of developing Alzheimer disease and absent F waves on nerve conduction studies. could not activate any units. The tumor can occur in the spine. Botulinum toxin myopathy in which there is a deficiency of alpha- ingestion may cause flaccid weakness. has been associated with ARX mutations on the X ataxic gait to suggest a diagnosis of cerebellar ataxia. 55. Texas is a common location for this virus. and would Neurology Books . Conus medullaris syndrome would be expected to have but later milestone loss and loss of functional low back pain with decreased perianal sensation. interictal one would expect tone abnormalities (upper motor electroencephalograph abnormality known as neuron signs). Additionally. however. and up to 2 weeks pp. Demanding the nurse reports the inability to lift her legs. somatization is not likely given the patient’s also known as epileptic aphasia. but is usually glucosidase. It is associated consistent with a metastatic lesion. along with abuse of power within the physician–nurse relationship a history of physical and sexual abuse. or ongoing fever. autonomic respiratory failure.  The patient has several features that point to a pregnancy. Further. Similarly.  The patient has an acute onset of movement especially if these orders are out of usual care. although EMG would show responsiveness). sleep that causes speech delay or regression. Treatment is aimed at treating tonic-clonic seizures. A flaccid paralysis from an anterior syndrome is associated with SCN1A mutations. followed by additional seizure comorbid depression with selective serotonin reuptake types later in early childhood.

130 Neurology Self-Assessment

not have spread to the lungs. Multiple sclerosis can parietal lobes can be seen in dementia with Lewy
cause round lesions that enhance with contrast, but bodies and Alzheimer disease, but Alzheimer disease
they are not typically associated with significant edema, does not typically affect the occipital lobes.
and usually have incomplete ring enhancement. Hypometabolism of the anterior temporal lobes and
Meningiomas are benign tumors outside of the frontal lobes is seen in frontotemporal dementia.
parenchyma of the brain. These can cause cerebral Hypometabolism of the striatum is commonly seen
edema if large enough and typically enhance in Huntington disease (see Bradley’s NiCP, 7th edn,
homogenously with contrast. Ischemic strokes do not Ch. 41, pp. 486–503).
cause the pattern seen on the MRI. Subacute strokes 72. E.  Temozolomide is not a common cause of
(2–4 weeks old) may show enhancement, though not chemotherapy-induced peripheral neuropathy. The
ring enhancement, and most edema would be resolved other answer choices mentioned are common culprits
at that point (see Bradley’s NiCP, Ch. 112, pp. 1973– for a peripheral neuropathy, but different
1991). Figure from Kang TW, Kim ST, Byun HS, et al. chemotherapeutic agents can lead to different
2009 Morphological and functional MRI, MRS, neuropathic syndromes. For example, paclitaxel and
perfusion and diffusion changes after radiosurgery of bortezomib often lead to a painful sensory neuropathy.
brain metastasis. European Journal of Radiology 72(3): Vincristine may be associated with a relatively painless
370–380, with permission. sensorimotor and autonomic neuropathy. Cisplatin
69. C.  Deep inhibitory postsynaptic potentials cause a may lead to a relatively painless large-fiber and/or
positive charge to develop in the body of the neuron, sensory ganglionopathy. The neuropathy can occur at
making the more superficial portion of the neuron any point in time while on the chemotherapy, but
appear more negative, and therefore the EEG electrode occurs usually in a dose-dependent fashion and
records a negative potential. The same is seen with worsens when the patient has other factors or
superficial excitatory postsynaptic potential, which conditions that can predispose to nerve injury (such
causes negativity in the superficial portion of the as diabetes mellitus). The neuropathy generally
neuron and is recorded as a negative scalp discharge. improves when the offending agent dose is lowered
Vertically oriented dipoles are recorded well on scalp or discontinued. Most of the time, the neuropathy
electrodes, whereas radial-oriented dipoles are not improves after the chemotherapy has stopped and
seen well (see Bradley’s NiCP, 7th edn, Ch. 34, can be treated with the same agents that treat other
pp. 348–366). painful peripheral neuropathies (gabapentin,
70. D.  Susac syndrome is a small-vessel vasculitis that pregabalin, nortriptyline, venlafaxine). No trials
commonly presents with sensorineural hearing loss, have shown clinically meaningful benefit for any
branch retinal artery occlusions, and encephalopathy. agent to prevent chemotherapy-induced peripheral
MRI classically shows “cannonballs” of T2 neuropathy (see Bradley’s NiCP, 7th edn, Ch. 107,
hyperintensity within the corpus callosum related to pp. 1791–1866).
demyelination. Multiple sclerosis causes T2 73. B.  Fingolimod is one of the newer disease-modifying
hyperintense areas of demyelination throughout the treatments for multiple sclerosis. It is a sphingosine-1-
brain, commonly including the corpus callosum. phosphate analog that binds to the sphingosine-1-
Although optic neuritis is associated with multiple phosphatase enzyme, resulting in sequestration of
sclerosis, this does not generally cause partial field cuts lymphocytes into lymph nodes. Natalizumab is a
and has no associated hearing loss. Marchiafava- monoclonal antibody that binds to the alpha-4-
Bignami disease is a progressive demyelinating disease integrin adhesion molecule, preventing lymphocytes
that generally affects the corpus callosum of alcoholics. from crossing the blood–brain barrier used in the
Although the cause is not known, it is believed to treatment of multiple sclerosis. Teriflunomide is a
relate to a nutritional deficiency. There is no specific dihydroorotate dehydrogenase inhibitor used in the
association of hearing loss or vision loss. Creutzfeldt- treatment of multiple sclerosis that works by inhibiting
Jakob disease is a prion-related spongiform synthesis of pyrimidines. Pyridostigmine is a reversible
encephalopathy that may cause rapidly progressive acetylcholinesterase inhibitor and has no role in the
dementia. MRI findings include T2 hyperintensity in treatment of multiple sclerosis, but is used in
the basal ganglia, thalamus (pulvinar sign), and insula, myasthenia gravis. Rituximab is a CD20 monoclonal
as well as restricted diffusion along the cortex. antibody originally used in the treatment of
Manganism is an accumulation of manganese in the lymphoma, but is now also used in neuromyelitis
basal ganglia, which may present with parkinsonism. optica, multiple sclerosis, myasthenia gravis, and other
MRI demonstrates accumulation of manganese, which B-cell–mediated autoimmune neurological conditions
may be hyperintense on both T1 and T2 (see Bradley’s (see Bradley’s NiCP, 7th edn, Ch. 80, pp. 1159–1186).
NiCP, 7th edn, Ch. 65, pp. 920–967). Figure from 74. C.  This patient presents with a history of kidney
Allmendinger A, Mallery RM, Magro CM, et al 2014 failure, strokes, angiokeratomas, and neuropathy at a
Cauda equina involvement in Susac’s syndrome. Journal young age. This presentation should be concerning
of the Neurological Sciences 337(1–2): 91–96, with for Fabry disease. This disorder is caused by a
permission. deficiency in a lysosomal enzyme, α-galactosidase A.
71. A.  Dementia with Lewy bodies is marked by PMP22 duplication causes Charcot-Marie-Tooth type
fluctuating levels of wakefulness and alertness, visual 1a, whereas the deletion causes hereditary neuropathy
hallucinations, mild parkinsonian features, and with predisposition to pressure palsies. Metachromatic
memory loss. Although brain biopsy is the only leukodystrophy is associated with an arylsulfatase-A
definitively diagnostic test for dementia with Lewy deficiency. Phytanic acid accumulation occurs in
bodies, FDG PET scans can provide important clues, Refsum disease, which causes night blindness,
including characteristic hypometabolism in the visual field abnormalities, hypertrophic palpable
occipital lobes. Hypometabolism in the temporal and nerves, distal leg weakness, hearing loss, and

Neurology Books

Test Four 131

cardiomyopathy (see Bradley’s NiCP, 7th edn, associated with trauma, especially in older or alcoholic
Ch. 107, pp. 1791–1867). patients with frequent falls. Radiographically, SDHs 4
are not restricted by cranial sutures, but rather are
75. C.  According to the 2015 American Academy of
restricted by dural reflections (such as the falx).
Neurology consensus guidelines regarding concussion
Treatment depends on the patient’s clinical status and
and traumatic brain injury, there are several clear risk
the amount of mass effect from the bleed. This can
factors for sport-related concussion. The type of sport is
include clinical observation, hematoma drainage
highly associated with concussion risk, and is greatest
through burr holes, or even evacuation by craniotomy.
in football, rugby, hockey, and soccer. Gender is also
Epidural hematomas are convex hemorrhages that
associated with risk, but differs by sport, with greater
result from the tearing of the middle meningeal artery,
risk in females playing soccer and basketball. History
usually due to a skull fracture in the temporal region.
of prior concussion is strongly associated with risk for
There is often loss of consciousness due to trauma
additional concussions, possibly within 10 days of the
followed by a lucid interval that precedes a period of
first. There is moderate evidence that certain well-fitted
coma. CAA usually results in a lobar hemorrhage,
helmets effectively reduce the risk of concussion,
which is parenchymal. Hemorrhages can also occur as
whereas soft head protectors provide no benefit in
a part of venous sinus thrombosis, but are usually
sports such as soccer or basketball. However, there is
located in the deep regions of the brain or in the
insufficient data to date to establish position played as
draining distribution of the sinus. Ruptured berry
a risk factor in specific sports. Similarly, there are
aneurysms usually lead to subarachnoid hemorrhages,
insufficient data that age or competition level
not subdural hematomas (see Bradley’s NiCP, 7th edn,
contributes to overall concussion risk (see Bradley’s
Ch. 39, pp. 411–458). Figure from Johnstone EC,
NiCP, 7th edn, Ch. 61, pp. 860–866).
Owens DC, Lawrie SM, McIntosh AM, Sharpe MD 2010
76. A.  Proprioception is transmitted through afferent A Companion to Psychiatric Studies, 8th edn, Churchill
alpha fibers, and somatic motor innervation is sent Livingstone, with permission.
through the efferent A alpha fibers. Touch and pressure 79. C.  Emery-Dreifuss myopathy is most commonly
are transmitted through afferent A beta fibers, and inherited through an X-linked pattern, with autosomal-
efferent A beta fibers contribute to somatic motor dominant transmission less frequent, and autosomal-
innervation. Pain, cold, and touch are transmitted recessive quite rare. The disease is caused by mutations
through afferent A delta fibers. The A gamma fibers are in either the EMD or LMNA genes, which encode
specialized efferent fibers, sending input to intrafusal nuclear envelope proteins. The earliest symptom of
muscle fibers and muscle spindles. Group B fibers are Emery-Dreifuss myopathy is generally contractures of
preganglionic autonomic nerves. Both group A and the elbows, ankles, and neck, followed by progressive
group B fibers are myelinated, but group B fibers are weakness and cardiac arrhythmia. With a pacemaker
smaller and have a slower conduction velocity. Group preventing cardiac death, most patients live into
C fibers are small and unmyelinated, and therefore adulthood, although some die prematurely from
have slow conduction velocity. They are involved in progressive cardiac or pulmonary disease (see Bradley’s
pain, temperature, and reflex responses in the dorsal NiCP, 7th edn, Ch. 110, pp. 1915–1955).
spinal roots, as well as postganglionic sympathetic
output. 80. E.  The boy in this case has Tourette syndrome,
manifested by both multiple motor tics and at least
77. C.  The patient in this question most likely has one phonic tic for at least 1 year in someone younger
anorexia nervosa. This is a disorder that affects 1% of than 21 years of age. Notably, the question details the
adolescent girls. Only 5%–10% of patients with criteria for a tic, in that they are suppressible actions
anorexia nervosa are men. Most have weight loss with a premonitory urge accompanied by a sense of
associated with a fear of being overweight that often relief after the action. Many times psychological
leads to starvation and excessive exercise. Patients conditions coexist with Tourette syndrome, including
refuse to maintain body weight at or above the normal ADHD, poor impulse control, OCD, and self-injurious
weight for age, and also have an altered perception of behavior. The treatment usually consists of
their body weight. In those who have begun their tetrabenazine, topiramate, or antidopaminergic
menstrual cycles, there is often amenorrhea, defined by medications for the motor symptoms. Stimulants,
the absence of three consecutive menstrual cycles. clonidine, or guanfacine can be used to treat comorbid
Patients with anorexia nervosa can also binge eat ADHD. Selective serotonin reuptake inhibitors are
and purge, so long as they are still underweight. usually used to treat comorbid OCD (see Bradley’s
Alternatively, patients with bulimia nervosa must have NiCP, 7th edn, Ch. 96, pp. 1422–1460).
recurrent episodes of binge eating (eating more than
81. A.  The patient has central diabetes insipidus (DI) as
what others would eat in a discrete period) and
a result of pituitary stalk compression from his
purging (through vomiting, laxatives, diuretics, enemas
Rathke cleft cyst. Urine osmolality is typically low
or exercise), but have a normal body weight and
(not high) in central DI, due to the pituitary’s inability
generally poor control over eating habits. Binge eating
to release antidiuretic hormone (also known as
and purging must occur at least two times per week for
vasopressin). Patients will often present with an
at least 3 months for bulimia nervosa. Treatment
increased sensation of thirst and polyuria with very
includes nutritional counseling, psychotherapy, and
dilute urine. Etiologies of central DI include cerebral
selective serotonin reuptake inhibitors.
hemorrhages, hypophysitis, compression by mass
78. A.  The image depicts a crescent (or concave) lesions such as craniopharyngiomas or Rathke cleft
hemorrhage located between the dura and the cysts, trauma, or postoperatively from sellar surgeries.
arachnoid, best known as a subdural hematoma (SDH). Treatment includes desmopressin or thiazide
This is caused by tearing of bridging veins that drain diuretics (see Bradley’s NiCP, 7th edn, Ch. 52,
into the dural venous sinuses. Most commonly, SDH is pp. 696–712).

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132 Neurology Self-Assessment

82. D.  Several items in this case point toward stroke as the minutes and tends to dissipate as the headache begins.
etiology of his vertigo. First, one should recognize his Although visual symptoms are the most common aura
stroke risk factors of age and heavy smoking history. complaint, rarely patients may have any of the
Further, recurring episodes of vertigo over recent days is previously mentioned symptoms instead (see Bradley’s
suggestive of transient ischemic attacks, and acute NiCP, 7th edn, Ch. 103, pp. 1686–1719).
vertigo onset suggests a vascular cause. Finally, normal 86. B.  C fibers are unmyelinated fibers with a relatively
bilateral head thrust test is highly sensitive for stroke. small diameter and slow conduction velocity. These
In patients with a vestibular cause of vertigo, such as fibers are present in the dorsal root ganglia and
labyrinthitis or vestibular neuritis, individuals cannot transmit nociception in a nonspecific and poorly
maintain target fixation when the head is turned localized way. They are also present in postganglionic
toward the affected side, although they perform fibers in the autonomic nervous system. A-delta fibers
normally when the head is turned toward the are fast myelinated fibers that transmit sharp localized
unaffected side. The key distinction between pain and temperature signals. A-alpha fibers include
labyrinthitis and vestibular neuritis, both of which are the alpha motor neurons involved in muscle
generally viral in nature, is the presence or absence of contraction and sensory fibers to the muscle spindles.
hearing loss. Vestibular neuritis affects the vestibular A-gamma fibers innervate the muscle spindle, which
nerve only, thereby causing vertigo and balance helps sense the level of muscle contraction (see
disturbance only, whereas labyrinthitis also affects the Bradley’s NiCP, 7th edn, Ch. 54, pp. 720–741).
cochlear nerve and thereby causes hearing loss as well.
BPPV is a condition caused by otolith malposition in 87. D.  The listed symptoms all localize to the cavernous
the semicircular canals, and is associated with sudden, sinus, including oculomotor, trigeminal, and facial
severe vertigo, prompted by movement of the head and nerves. A diagram of the cavernous sinus is shown here.
lasting only a few minutes per episode. The findings of Mucormycosis is associated with infiltrating cavernous
a Dix-Hallpike maneuver are often abnormal with sinus infections in immunocompromised patients,
posterior canal otolith displacement, which is the most including diabetic patients. Poorly controlled diabetes is
common location. In such a case, otolith repositioning, associated with embolic events in cranial nerves,
such as with the Epley maneuver, may be therapeutic. although the likelihood of multiple involved nerves at
Nicotine withdrawal may be associated with headaches, once is unlikely. Hyperglycemia may cause focal
anxiety, depression, or difficulty with concentration. neurological deficits, but is less likely to cause multiple
However, vertigo is not a common symptom (see ipsilateral cranial neuropathies. Tolosa-Hunt syndrome
Bradley’s NiCP, 7th edn, Ch. 46, pp. 583–604). is an idiopathic, steroid-responsive granulomatous
condition that affects the cavernous sinus, but would be
83. A.  Tauopathies include Alzheimer disease, progressive less likely than an infectious process in this case. Viral
supranuclear palsy, frontotemporal dementia, and infection may cause multiple cranial neuropathies, but
corticobasal degeneration. Alpha-synucleinopathies not generally localized only to nerves traversing the
include Parkinson disease, dementia with Lewy bodies, cavernous sinus (see Bradley’s NiCP, 7th edn, Chs. 79,
and multiple system atrophy (see Bradley’s NiCP, 7th pp. 1147–1158 and 104, pp. 1720–1735).
edn, Ch. 96, pp. 1422–1460).
84. E.  Restless legs syndrome (RLS) is diagnosed clinically CORONAL VIEW
when five criteria are met: (1) an often uncomfortable
urge to move the legs (2) at times of inactivity (3) or Pituitary Sympathetic IIIrd cranial
in the evening (4) that is usually relieved by movement gland plexus nerve
(5) and is not accounted for by another medical or
behavioral condition. RLS is frequently associated with IVth cranial
periodic limb movements of sleep. If she had pain and nerve
cramping in her legs with activity, rather than at rest,
an arterial study could be helpful. RLS is not diagnosed Ophthalmic
by polysomnogram, but is rather a clinical diagnosis. nerve
Nerve conduction studies can help determine whether
there is a neuropathy or radiculopathy present, but VIth cranial
these conditions would not be common in a 23-year-
old. Ropinirole or other dopamine agonists are the
initial medical treatment for RLS, but this is not the Maxillary
next best course of action. Iron deficiency is the most Internal carotid nerve
common cause of secondary RLS. If the ferritin is <50,
iron supplementation is recommended and could be Figure for Answer 4.87 (from Kaiser PK, Friedman NJ, Pineda II R
curative (see Bradley’s NiCP, 7th edn, Ch. 102, 2014 Massachusetts Eye and Ear Infirmary Illustrated Manual of
pp. 1615–1685). Ophthalmology, Saunders, with permission.)
85. B.  Although the precise pathophysiology of migraine
aura is not known, the phenomenon is cortical in 88. A.  Visual evoked potentials measure the conduction
origin. As such, common visual disturbances such as time of the visual system by stimulating the retina with
scintillating scotoma or fortification spectra are either flashes of lights or, more commonly, a reversing
generally binocular but homonymous. However, checkerboard pattern on a screen. Responses are
similar to other homonymous symptoms, patients may recorded over the occipital region, with the P100 as the
erroneously report a monocular disturbance. Migraine main diagnostic waveform. This is a positive wave,
auras may be preceded by up to 1 day of prodromal which occurs approximately 100 ms after the stimulus.
symptoms, including changes in mood, energy, hunger, The eyes are stimulated independently and then
or excessive yawning. The aura itself is usually <30 responses are compared with each other. The visual

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Test Four 133

evoked potential demonstrates a delayed P100 wave in picking movements, not bicycling movements (see
the right eye, whereas the left eye is normal. This is Bradley’s NiCP, 7th edn, Ch. 101, pp. 1563–1614). 4
consistent with a prechiasmal lesion of the right optic
91. D.  Comprehension of death develops in a step-wise
nerve, such as optic neuritis. Other causes of a
fashion for children. Before the age of 3 years, there is
unilateral prolonged P100 include ischemic optic
generally no understanding of death. Then, until
neuritis, nutritional or toxic optic neuropathies,
around the age of 6 years, children often have magical
hereditary neuropathies, optic nerve tumors,
thinking in which they believe that their own actions
compressive tumors, refractive errors, glaucoma, and
or thoughts caused another person’s death and that
retinopathies. Bilateral delayed responses usually
death is a reversible process. Between the ages of 6 and
represent a lesion posterior to the chiasm but are not
9 years, magical thinking is replaced by a greater
specific to the right or left occipital lobe. Hemifield
understanding of the finality of death. Between the
stimulation, which is essentially never performed these
ages of 9 and 12 years, a child’s understanding of death
days, can help localize postchiasmal lesions, but with
becomes similar to an adult’s understanding thereof.
the easy access to neuroimaging, this is of limited value
today. Chiasmal lesions can show prolonged P100 or 92. E.  The most likely underlying diagnosis is tuberculous
absent P100 bilaterally (see Bradley’s NiCP, 7th edn, meningitis. The patient’s risk factors include
Ch. 34, pp. 348–366). Figure from Daroff RB, Jankovic homelessness and immigration from India, where the
J, Mazziotta JC, Pomeroy SL, editors, 2016 Bradley’s incidence of tuberculosis (TB) is relatively higher. The
Neurology in Clinical Practice, 7th edn, Elsevier, with right upper lobe consolidation on chest x-ray is also
permission. suspicious for TB. Tuberculous meningitis often causes a
basilar meningitis, leading to cranial neuropathies as a
89. C.  At 2 years of age, most children will be able to
presenting symptom. Common CSF findings include
name parts of the body, identify objects in pictures, say
profound hypoglycorrhachia (low glucose), elevated
short sentences, and follow simple instructions. They
protein, and pleocytosis with lymphocytic
are able to sort shapes, play make-believe, and build a
predominance. Although the Gram stain is negative in
tower of four or more blocks. Physically, children of
this case, the acid-fast stain would likely be positive.
this age are able to stand on their tiptoes, kick a ball,
Proper treatment would include airborne isolation, given
throw overhand, and begin running. Two-year-old
his cough and chest x-ray consolidation, and directed
children demonstrate parallel play in which they play
antituberculous therapy until confirmatory microbiology
next to other children, but not with other children. At
testing returns. Whole-body PET-CT would be reasonable
18 months a child will say single words, point to
if this patient had a new diagnosis of cancer, although a
objects he or she wants, follow one-step commands,
tissue diagnosis should be obtained (if possible) before
walk, eat with a spoon, and drink from a cup. At 1
beginning radiation or chemotherapy. Although fungal
year, a child will be nervous when away from parents
meningitis causes hypoglycorrhachia and lymphocytic
or around strangers, play games like “peek-a-boo,” use
pleocytosis, India ink staining is generally positive for
simple gestures to communicate, have very few words,
these infections. Further, this patient has none of the
rise to a sitting position without help, and may stand
usual risk factors for fungal meningitis, including
alone or even walk. At 3 years, children will copy
human immunodeficiency virus, posttransplant
friends and adults, show affection, take turns, follow
immunosuppression, chronic steroids, or diabetes.
two- to three-step commands, have mostly
Finally, oral antifungal therapy would be insufficient in a
understandable speech, play make-believe, copy a
case of fungal meningitis; instead, one should use broad
circle, or build a tower of six blocks. Three-year-olds
spectrum intravenous therapy until a source is identified.
should be able to pedal a tricycle. At 4 years, a child
Although tertiary syphilis may cause basilar meningitis,
should be able to play “mom” and “dad,” would rather
it does not generally cause hypoglycorrhachia or such
play with children than adults, has difficulty
severe pleocytosis in the CSF. Although Bickerstaff
determining what’s real or make-believe, can tell
encephalitis or Miller-Fisher syndrome (both of which
stories, understands time, remembers parts of a story,
may lead to respiratory muscle weakness) may be
draws a person with two to four body parts, plays
considered in this case, the lack of ataxia or motor
board games, and stands on one foot (see Bradley’s
disturbance argues against these diagnoses (see Bradley’s
NiCP, 7th edn, Ch. 8, pp. 66–72).
NiCP, 7th edn, Ch. 79, pp. 1147–1158).
90. B.  This patient presents with episodes that are
93. E.  Porphyrias are a group of disorders resulting from
consistent with frontal lobe seizures. These are usually
various enzyme deficiencies in the breakdown of
brief episodes that arise out of sleep, and can involve
porphyrins to produce heme. A buildup of porphyrins
bicycling of legs, kicking, thrashing, or other odd
results in multisystem dysfunction, including anemia,
behaviors. At times, tonic posturing can be seen. Of the
abdominal pain, cold-induced neuropathy (due to
options mentioned, the syndrome of autosomal-
crystallization of porphyrins), seizure, and psychosis.
dominant nocturnal frontal lobe epilepsy best fits with
Porphyria crises may be triggered by various
this patient’s episodes. This disorder is genetically
medications that also lead to porphyrin precipitation
heterogeneous; however, the most common mutations
from the blood, including certain anticonvulsants
affect the neuronal nicotinic acetylcholine receptor.
(carbamazepine, phenytoin), certain antibiotics, and
This syndrome usually presents between 8 and 20 years
other classes of medications. Although hypertension
of age, and is commonly misdiagnosed as a
and vasospasm may occur with porphyria attacks,
parasomnia, nightmares, or a psychogenic disorder.
stroke is a rare symptom of this disease (see Bradley’s
Seizures in juvenile myoclonic epilepsy can occur at
NiCP, 7th edn, Ch. 10, pp. 92–114).
night, but these tend to be generalized convulsions,
not partial seizures with bicycling. Mesial temporal 94. E.  The history consists of a boy with history of
sclerosis can also cause nocturnal seizures, but these infantile spasms, complex partial seizures, and
seizures involve automatisms such as lip smacking and language delay with autistic qualities. The image of his

Neurology Books

528–572). Neurology Books . Magnetic resonance significant unintentional weight loss and severe imaging typically reveals hemiatrophy with neuropathic pain in the setting of poorly controlled leptomeningeal angiomatosis and calcifications diabetes. variable immunodeficiency with acidosis when combined with computed tomography susceptibility to sinopulmonary infections. Vitamin D deficiency is associated with lenticulostriate arteries are small arteries off of the increased risk of central demyelination. 7th edn. and congenital abnormalities. nails.  An internuclear ophthalmoplegia (INO) is autosomal-dominant disease characterized by visceral characterized by impaired adduction of the affected cysts and tumors. The Edinger-Westphal nucleus is a parasympathetic Academic Press. diarrhea. EMG and nerve conduction studies show a length- Patients may also have malformations affecting the dependent sensorimotor axonal polyneuropathy. to be present. usually a leg.  The superior division of the middle cerebral artery immediate dismissal of the student or the attending. dermatomes. and cutaneous manifestations.  Vitamin B1 (thiamine) deficiency is associated with area. either TSC1 (encodes hamartin) or TSC2 (encodes Patel MC. limbs. C. as with middle cerebral artery that pass into the caudate. 44. complications. a lesion here disrupts the parasympathetic (constrictive) outflow to the 95. The inferior division of the middle cerebral artery Wernicke-Korsakoff syndrome with bilateral supplies the lateral temporal lobe and Wernicke’s area. mental retardation (MR). depression. pp. C. MR. Insulin neuritis presents with rapid-onset infantile spasms). EMG and nerve conduction treatment of giant cell astrocytomas. Von Hippel-Lindau disease is an 98. and truncal paresthesias. hair. Ignoring the relationship or acknowledging it and pp. hands. Ch. Focal neurological deficits may occur due fingers and toes in the setting of rapid control of to brain tumors. Saunders. and hemiparesis. otherwise. including retinal and central nervous eye. pheochromocytomas. mammillary body hemorrhages. with permission. and fluorescing under a Wood lamp. is an autosomal-recessive disorder caused by a which help differentiate it from insulin neuritis. contrast. 7th edn. the adduction deficit). A. ocular and cutaneous telangiectasias. TS is an brain (see Bradley’s NiCP. teeth. 5th edn. Rapamycin is often used in the previously high glucose. Other amyotrophy. 2014 Aminoff’s Neurology and General Medicine. clinical features include facial angiofibromas. The system hemangioblastomas. mutation in the ATM gene and is characterized by Metformin has many side effects. Sturge-Weber syndrome is characterized by weakness of one limb. 39. diabetic subungual (fingernail or toenail) fibromas. Ch. Patients polyneuropathy involves paresthesias and numbness often have hypopigmented or white macules along the beginning in the toes and moving higher over the lines of Blaschko. supplies the lateral frontal lobe. and polyneuropathy. and Shagreen patch. or autism. pp. small fiber neuropathy. with a “down and out” is to remove the physician from the supervisory role to position of the affected eye on primary gaze. nucleus of the oculomotor nerve. to neurological presentations include seizures (most often name a few. or face/ Diabetic amyotrophy presents with rapid painful skull. Vitamin B3 (niacin) deficiency is and one area in the middle back consistent with a associated with pellagra (dermatitis. The neuropathy. 97. and significant angiomas that involve the leptomeninges as well as the weight loss. Ch. including Bradley’s NiCP. affected limb. Ataxia-telangiectasia involvement. Patients may have plexopathy or brachial plexopathy. Figure from Perkin DG. 411– autosomal-dominant disorder caused by mutations in 458). regardless of oculomotor nucleus would cause ipsilateral ptosis the situation. Diabetic cachexia presents with glaucoma. feet. and lesion responsible for an INO is generally within the multiple organ cysts. including distal sensory retinal hamartomas. 7th edn. Figure from Kanski JK. Lesions of the supervisors and trainees are unacceptable. diabetic cachexia. The classic cutaneous manifestation is show findings consistent with a lumbosacral a nevus flammeus (port-wine stain).  Diabetes has a plethora of neuromuscular subependymal nodules or giant cell astrocytomas. Bowling B 2011 asking for discretion still allows for conflicts of interest Clinical Ophthalmology: A Systematic Approach. Although most facilities have policies Saunders. Vitamin B6 the anterior cerebral artery that supplies the caudate (pyridoxine) deficiency is associated with peripheral and anterior portion of the internal capsule. usually in the V1 and V2 distributions of the controlled diabetes. pp.  Romantic or sexual relationships between medical pupillary constrictors in the light reflex. 100. The artery of Heubner is a branch off of nerve and dorsal column demyelination. Distal sensory seizures. all of which have malignant ipsilateral medial longitudinal fasciculus (same side as potential (see Bradley’s NiCP. typically occurring in patients with poorly skin. but painful paresthesias are not seen (see malignancies. which involves Broca’s 96. with abduction nystagmus of the opposite eye. depending on the developmental delay. Miller DC. 1791–1866). there is no need for 99. Hypomelanosis of tests are normal unless there is an underlying Ito is an autosomal-dominant disorder characterized by neuropathy already present. cardiac rhabdomyoma. 7th edn. findings. A PPRF lesion generally causes 1538–1562). The appropriate response in this situation and weakness of adduction. painful paresthesias and hyperalgesia in the distal like behavior. the ipsilateral eye (see Bradley’s NiCP. It is often associated with autonomic ipsilateral to facial manifestation. Aside from the aforementioned cutaneous 3rd edn. Lesions of avoid any conflicts of interest. including severe lactic cerebellar ataxia. Lane RJM. Ch. with permission.134 Neurology Self-Assessment skin shows hypopigmented macules (ash-leaf spots) multiple sclerosis. Josephson SA slow or weak saccades toward the side of the lesion. Figure from Aminoff MJ. regarding romantic relationships. EMG and nerve conduction studies trigeminal nerve. Often alpha- fetoprotein is elevated. visual loss. the the abducens nucleus cause weakness of abduction in relationship must end during the period of supervision. with permission. 7th edn. 107. and insulin neuritis. palate. D. focal seizures. E. Vitamin B12 No aphasia is noted with strokes to the anterior (cobalamin) deficiency is associated with peripheral cerebral artery. these are classic cutaneous dementia) but no specific pathological changes in the manifestations of tuberous sclerosis (TS). Hochberg FH 2010 Atlas of Clinical Neurology. usually covering more than two course of years until eventually involving the hands. tuberin).

Optic aphasia is the inability to shown. There are several different botulinum toxins. and myotonic dystrophy (autosomal-dominant temporal Occipitotemporal gyrus sulcus Occipitotemporal disorder with anticipation). In experimental models. B. and 100. Some patients are unaware of their differentiate from the other listed lesions.  Cyclophosphamide is a nitrogen mustard alkylating carbamate insecticides. D. Hintzen RQ 2009 Spinal cord Bradley’s NiCP. 7th edn. Ch. which binds to are opened by a change in membrane potential. This presents with diffuse weakness. 109. 101. Guillain-Barré (conduction blocks and elevated CSF protein). These target SNARE proteins (SNAP25. Recognition usually associated with significantly more mass effect than is comes from voice. 65. Mellema J.105 (from Vanderah T. but would not cause (fusiform) gyrus pupillary abnormalities like botulism does (see Figure for Answer 4. Imaging often shows bilateral describe the use of the object.) Neurology Books . Immunosuppression is common after pp. The other answers point toward spinal temporal gyrus muscular atrophy (SMN1 gene). Abscesses are generally ring enhancing with central Aphemia is a variant of Broca aphasia in which the hypointensity and marked mass effect (see Bradley’s patient is initially mute. 80. but recovers the ability to NiCP. 4 arteries do not cause aphasia (see Bradley’s NiCP. with permission. skin is the threshold for electrical failure. pp. The Human Brain. These disorders could cause some of the symptoms. pp. although it would generally be about each face. as organophosphate insecticides like malathion and well as various autoimmune disorders (including parathion.  This patient has botulism. 109. Long-term risks of 50–55 mL/100 g/min. hypointensity on T2/fluid attenuated inversion which commonly comes from bilateral temporoparietal recovery. autonomic dysfunction. donepezil. leading Superior to weakness. 122–127). cranial Lingual nerve abnormalities. which can come from ingestion of spores that are in various foods such as honey. although it is deficits and will confabulate when asked to describe generally associated with significant mass effect. with associated ringlike enhancement. Test Four 135 posterior internal capsule. 1895–1914). although right-sided lesions in the fusiform presence of these rings makes this more likely than any gyrus (shown in the figure below) can cause the of the other options. and bladder cancer. and is often quite aggressive. Journal of the Neurological Sciences 279(1–2): 114–117. or vesicle- associated membrane protein) are involved in vesicular binding to the presynaptic membrane. each with its own target. Voltage-gated calcium of hemorrhagic cystitis. with permission. and often the goal of therapy 104. 1896–1914). 103. syntaxin. 1159–1186). To prevent this. Nicotinic paraneoplastic disorders). synaptobrevin. involves bilateral occipital lobe lesions. rivastigmine. For about 24 hours around receptor antagonists include curare. most channels do not have any specific agonists because they institutions use prophylactic mesna. ADEM is most common in children after name an object with retained ability to demonstrate or infection or vaccination. Ch. Irreversible inhibitors include agent used in the treatment of various malignancies. pp. 13.  Normal cerebral blood flow at rest is for autoimmune disorders. is not returned in a timely fashion (see Bradley’s NiCP. Anomic aphasia is the white matter lesions with ring or incomplete ring inability to name objects or people. this stops the release of acetylcholine from the synapse. Figures from speak with phoneme substitutions and pauses (see Kreft KL. different visual stimuli (known as Anton syndrome).  Pyridostigmine is a reversible acetylcholinesterase Strokes to the artery of Heubner and lenticulostriate inhibitor used in the treatment of myasthenia gravis. galantamine. neostigmine. blood cyclophosphamide include infertility and secondary flow between 8 mL/100 g/min and 18 mL/100 g/min malignancies such as leukemia. The lesions. 1896–1914). 920–968). 109. Ch. 7th edn. but are associated with cyclophosphamide use. lymphoma. leading to an Tumefactive multiple sclerosis may be difficult to inability to see. B.  Prosopagnosia is the inability to recognize faces. and lentiform nucleus. When these proteins are damaged by a botulinum toxin. and gyrus dilated pupils. 102. C. A. cyclophosphamide. and nerve agents like sarin gas. not and detoxifies the bladder-toxic cyclophosphamide ligand binding (see Bradley’s NiCP. pp. 7th Other reversible acetylcholinesterase inhibitors include edn. Ch. 12. 7th edn. pp. Gould D 2016 Nolte’s Bradley’s NiCP. Blood flow below a rate of often seen with steroids (see Bradley’s NiCP. Collateral mitochondrial myopathy (biopsy with ragged red Inferior sulcus fibers). 128–144). like the haircut. although it is not irreversibly damaged. 7th edn. This is the cancer. pp. 7th edn. there is a risk antagonists include atropine. metabolite. 8 mL/100 g/min can result in cell death if blood flow Ch. Cortical blindness enhancement and occasionally with hemorrhage. ileus. Osteopenia is not penumbra range. 7th edn. concentric rings of hyperintensity and isointensity or 105. so neurons quit functioning. This disorder makes patients unable to sclerosis. B. Balo’s is a rare variant of multiple syndrome. Ch. Ch. involvement of Balo’s concentric sclerosis. Elsevier. Muscarinic the time of cyclophosphamide infusion.  Balo’s concentric sclerosis generally appears with 7th edn. Metastatic lung recognize family members or the faces of famous cancer may have multiple lesions with ring people unless they are able to memorize certain details enhancement.

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4.  Neurology Books 137 . Which is the best treatment for the condition described in Question 5. Carvedilol D. and phonophobia. Ischemic stroke C. Intravenous valproic acid E. Highly active antiretroviral therapy (HAART) demonstrates flaccid paralysis of his lower extremities with absent reflexes. Norepinephrine D. Which of the following neurotransmitters is considered vibration and proprioception are intact. There is no evidence of weather just before symptom onset. A 27-year-old female who is currently 8 weeks pregnant presents with a severe headache. A 42-year-old woman with relapsing remitting multiple He is resuscitated and placed in the intensive care unit sclerosis was previously treated with mycophenolate for further monitoring. he is extubated and and now with natalizumab over the last 3 years. Human immunodeficiency virus (HIV) encephalitis D. Anterior spinal infarct E. He has sensory loss to pin prick E. The headache is located over the right side of her head with a severe. Dopamine B.Test Five QUESTIONS 3. Gradually. Acute inflammatory demyelinating B. He is referred to neurology because of sudden jerks in confusion. Acute disseminated encephalomyelitis E. She has an urgent magnetic resonance imaging of the brain that is also normal. John Cunningham (JC) virus reactivation 6. Acute multiple sclerosis flare E. throbbing pain associated with nausea. A 53-year-old man with a history of hypertension is A. Clonazepam B. Carbamazepine A. Acetylcholine A.2. Carbidopa/levodopa is the likely cause of the demonstrated problem? B. Acetamino