1

Preface:

Here, you can find new questions in addition to our old series. Please feel free to
comment and to contact us. Again, these best of fives are not a substitute for
textbooks, please read the corresponding topics well in textbooks, and then review
and test your knowledge.

I would like to thank my dear friends in Al-Sulaimaniya general hospital (IRAQ)

for their kind help and support regarding the collection of cases from their
departments during my visit there. Thanks to: Dr. Dana Ahmad (neurologist), Dr.
Diyar Najeeb (neurologist), Dr. Dana Mahmood (physician), Dr. Saad Shwani
(neurologist), and Dr. Ahmad Saed (physician).

Please feel free to contribute and to share your experience with all. We are
welcoming new writers.
Thank you visiting our website.

Dr. Osama Amin

Head of Team Neurology4MRCP
April 2006

All Questions and answers were written by Dr. Osama Amin

Copyright2006. All rights reserved.
http://neurology4mrcp.orgfree.com

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 Contents
1. Questions (from page no .4).

2. Answers (from page no. 22).

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 Questions:

Q1:
A 23-year-old housewife presents with 2 day history of numbness and weakness in
her left arm to be followed by numbness in her right leg after 1 day. Her past medical
history is unremarkable, with no history of spinal trauma or medication abuse.
Examination revealed loss pinprick sensation in her right leg up to the lateral aspect of
her right arm and loss of position sense in the left lower and upper limbs, with left
sided up-going planter. She reported some spontaneous improvement after 5 days.
All of the following statements are wrong, except:
a. She is unlikely to have a partial Brown-Squard Syndrome.
b. Multiple sclerosis is excluded by this picture.
c. Breast examination is unhelpful.
d. MRI of the cervical cord is indicated.
e. CT scan of the head should be the top priority.

Q2:
A 67-year-old man presents with one-day history of a sudden onset of difficulty in
swallowing and vertigo. Examination revealed impaired gag reflex and sensation in
the right pharynx, coarse horizontal nystagmus, and dysarthria. His past medical
history is unremarkable. A diagnosis of ischemic brainstem stroke was done. Which
one is the correct statement?
a. He dose not need aspirin.
b. Carotid Doppler study should be omitted.
c. Asymptomatic hyperglycemia will not change the management plan.
d. Severe cervical spondylosis can be a risk factor for his stroke.
e. He should receive Thrombolytic therapy.

Q3:
A 62-year-old woman presents with a few hours history of headache and confusion.
Her non-contrast brain CT scan showed right basal ganglia hematoma. At day 4, she
developed generalized tonic clonic fit. All of the followings are true, except:
a. She needs another brain CT scan.
b. Assessing her serum sodium level is worthy.
c. He fits are likely to represent pseudo-seizures
d. The blood pressure should be monitored.
e. Careful assessment of her fluid status should not be forgotten.

4
Q4:
A 51-year-old woman who was diagnosed as having a right sided frontal lobe
meningioma recently, came with a 1-day history of irregular jerky movements of her
right arm that is at the side of the tumor. The description is consistent with a focal
motor seizure. You reviewed her brain CT scan to be sure of the site of the tumor.
How can you explain the site of the seizures?
a. She is lying.
b. The CT scan does not belong to her.
c. There is another left sided cortical tumor that is not seen by the brain CT scan.
d. You are reading the CT scan film in a reversal manner.
e. She might have an uncrossed pyramidal system of fibers.

Q5:
A 21-year-old man presents with a 2-day history of impaired consciousness. His
mother said that he has recently been diagnosed as having a psychiatric illness and is
receiving certain tablets for it. Examination revealed high fever, generalized rigidity,
fluctuating blood pressure and pulse rates. What is the likely psychiatric illness?
a. Severe endogenous depression.
b. Panic attacks.
c. Schizophrenia.
d. Hypomania.
e. He has no psychiatric illness at all.

Q6:
A 14-year-old girl is being evaluated for an abnormal gait. The final diagnosis was
Friedreick's ataxia. However, you noticed something that is against her diagnosis,
what is it?
a. Marked cognitive impairment.
b. Hyperglycemia.
c. Retinitis pigmentosa.
d. Scoliosis.
e. Negative family history.

Q7:
A 51-year-old woman came with rapid and progressive impairment in her vision
affecting her left eye only over a matter of few days. Apart from red swollen left optic
disc with severely reduced visual acuity to finger counting and Marcus-Gunn pupil,
her examination is totally unremarkable. Few days later while she is in the way of
investigations, she said that her vision is better now. Which one is the correct
statement?
a. Brain MRI with contrast is not indicated.
b. Auto antibody screen is useless.
c. Her ESR would give no clues.
d. The findings are consistent with Foster Kennedy syndrome.
e. Gradual and excellent improvement is against giant cell arteritis.

5
Q8:
A 32-year-old man presents with a 2-year history of left sided headache and ocular
pain. The pain seems to occur in severe bouts for about two hours, with eye redness
and nasal watering, day and night, with no remission, somewhat responsive to
phenacetin. He is non-smoker and non-alcoholic, on no medications or illicit drugs.
Examination revealed only gynecomastia and soft testes.Which one is the correct
statement?
a. He has migraine without aura.
b. This is malingering.
c. Brain MRI or CT scan should be done.
d. Increasing the dose of phenacetin is helpful.
e. Reassure and discharge him.

Q9:
A 5-year-old girl is being treated by chemotherapy for acute lymphoblastic leukemia,
and she is in the 4th week of remission induction phase, developed partial left sided
ptosis with diplopia, generalized headache, and progressive massive weight gain in
spite of loss of apatite. All of the followings are wrong, except?
a. Her weight gain is likely to be due to hypothalamic infiltration.
b. CSF examination is contraindicated.
c. Vincristine toxicity is the cause of ptosis.
d. Prednisolone is the cause of her obesity.
e. Cranial irradiation is of no benefit.

Q10:
A 40-year-old man was admitted because of progressive somnolence over few days.
He has a history of extensive chronic plaque psoriasis for which he is receiving
cyclosporine. Examination revealed fever, neck stiffness and obtundation. Which one
is the correct statement regarding his new presentation?
a. We should increase the dose of cyclosporine.
b. CSF examination is unnecessary.
c. Start intravenous cefotaxime and vancomycine.
d. Add azathioprine.
e. Blood cultures should be avoided.

Q11:
A 21-year-old woman, who was reasonably well and healthy, presents with 4-month
history of progressive unsteadiness in stance and gait. She denied any drug abuse or a
back trauma, and there are no risk factors for HIV infection, and no family history of
a similar condition. Examination revealed spastic paraparesis with loss of join
position and vibration sense in the lower limbs and right-sided primary optic atrophy.
No sensory level was detected. What is your next step?
a. Observation only.
b. Give high dose pulse intravenous methylprednisolone.
c. Give vitamin B12 injections.
d. Plain X ray of the lumbosacral spine.
e. Brain MRI with contrast.

6
Q12:
A 57-year-old woman with a history of mitral stenosis presents with sudden onset of
right sided weakness and global aphasia. Brain CT scan revealed massive infarction
covering the whole area supplied by the left middle cerebral artery and midline shift.
ECG showed rapid atrial fibrillation.
Which one is the correct step?
a. Omit anticoagulation for the time being.
b. Avoid digoxin.
c. No need for Echocardiography.
d. Mannitol is hazardous.
e. Immediate electrical cardioversion should be done.

Q13:
A 20-year-old woman presents with long standing history of mild lower limb
weakness that is very slowly progressive since the age of 12 years, associated with
some sensory numbness. She is not bothered by the weakness, but she is embarrassed
by her legs' appearance. Her older sister has a same condition. Examination revealed
bilateral pes cavus, hammertoes, and thin stork-like legs. The right common peroneal
nerve was palpable. Her past medical history is unremarkable. She refused to be
examined further.
What is the most likely diagnosis?
a. Chronic inflammatory demyelinating polyradiculopathy.
b. Diphtheritic polyneuropathy.
c. Relapsing Guillain Barre syndrome.
d. Rufsum's disease.
e. Charcot-Marie-Tooth disease.

Q14:
A 60-year-old right-handed man has a history of embolic stroke, and he is aphasic.
His speech is non-fluent, with impaired repetition and naming, but relatively intact
comprehension. Where is the site of the lesion that is likely to be responsible for his
aphasia?
a. Upper posterior temporal gyrus, left hemisphere.
b. Left frontal pole.
c. Left mesial temporal lobe.
d. Lower posterior inferior frontal gyrus, left hemisphere.
c. Right occipital pole.

Q15:
A 20-year-old man presents with generalized tonic clonic seizures. He is a know case
of idiopathic grand mal epilepsy and he was put on valproic acid tablets since 2 years.
All of the followings are well-know side effects of this medication, except:
a. Thrombocytopenia.
b. Pancreatitis.
c. Alopecia.
d. Tremor.
e. Weight loss.

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Q16:
An 18-year-old female, was referred to you from the psychiatric department. She was
initially diagnosed as having schizophrenia, but investigations revealed impaired liver
function tests prior to starting her conventional antipsychotic, and further work up
revealed Wilson's disease. An ophthalmologist detected Kayser-Fleischer ring in both
eyes. What is the anatomical site of this "ring"?
a. Anterior surface of the lens capsule.
b. Inner margin of the iris.
c. Descement's membrane of the cornea.
d. Stromal layer of the cornea.
e. Perilimbic area of the sclera.

Q17:
A 47-year-old man with long standing history of acromegally is seen by you in the
clinic for a routine follow up visit. Some of his nerves were palpable. All of the
following conditions can produce palpable nerves, except?
a. Primary AL Amyloidosis.
b. Charcot Marie Tooth disease type I (CMT I).
c. Rufsum's disease.
d. Guillain Barre syndrome (GBS).
e. Chronic inflammatory demyelinating polyradiculopathy (CIDP).

Q18:
A 7-year-old boy presents with poor school records for the past 2 months. He was
cleaver and has no family problems. Examination was otherwise unremarkable.
Further work up had revealed petit mal epilepsy. Which one of the following
medication can cause worsening of his condition?
a. Valproic acid.
b. Ethosuximide.
c. Carbamazepine.
d. Lamotrigine.
e. Clonazepam.

Q19:
A 28-year-old female presents with an attack of weakness in her lower limbs. She has
a history of short-lived numbness and severe blurred vision both of which improved
spontaneously. Ultimately, she was diagnosed as having multiple sclerosis. Which
one of the following conditions is against the diagnosis of multiple sclerosis?
a. Partial Brown-Squard syndrome.
b. Bilateral trigeminal neuralgia.
c. Rubral type tremor.
d. Dorsal column signs in one limb.
e. Swollen optic nerve heads with visual acuity of 6/6.

8
Q20:
A 37-year-old man presents with 2-day history of fever and confusion. His brother
said that the patient does not have any illness and on no medications or illicit drugs.
He has no risk factors for HIV infection, and no history of head trauma. Examination
documented a temperature of 38.9 C, global confusion and neck stiffness. What is
your immediate action apart from ABC (airway, breathing, circulation)?
a. Order a brain CT scan with contrast.
b. Order a Brain MRI without contrast.
c. Start intravenous Ceftriaxone and Vancomycin.
d. Send a blood sample for HIV testing.
e. Give nimodipine.

Q21:
A 17-year-old male presents with few days history of lower limb weakness in the
absence of spinal trauma. There was a bout of diarrhea before 3 weeks. There is
flaccid areflexic proximal and distal weakness with no objective sensory signs, and
flexor planters. You put a preliminary diagnosis of Guillain Barre syndrome. Which
one is the correct statement regarding this syndrome?
a. Dysautonomia is very rare.
b. Marked asymmetric signs are expected in the majority.
c. The mortality rate is around 40%.
d. A normal CSF protein level excludes the diagnosis.
e. Relapses are seen.

Q22:
A 28-year-old man brought by his family to consult you. They said that the patient
sometimes stares suddenly with embarrassing picking at clothes movements with
funny lip smacking. The patient did admit to feeling of a sudden rising sensation in
the upper abdomen with nausea but he did remember the staring or these
"embarrassing" movements. A coronal MRI of brain showed left sided mesial
temporal lobe atrophy. Which one of the following statements is the correct one
regarding his illness?
a. The patient should be referred to a psychiatrist.
b. Carbamazepine is useless.
c. The patient may be selected for anterior temporal lobectomy.
d. Hippocampal atrophy would be unusual in biopsy specimens.
e. EEG usually shows generalized spike and wave activity.

Q23:
A 51-year-old woman, with a cured early breast cancer since 10 years, presents with
many months history of generalized headache and early morning vomiting.
Examination revealed florid papillodema. Brain CT scan with contrast is consistent
with frontal lobe meningioma. Which one is the correct statement regarding this
tumor?
a. There is no association between breast cancer and meningiomas.
b. Underlying massive brain edema is seen in all cases.
c. Multiple meningiomas are seen in neurofibromatosis type 2.
d. The tumor regresses spontaneously in the majority of cases.
e. The spinal cord is the commonest site.

9
Q24:
A 63-year-old man, with type 2 diabetes and hypertension, presents with one-day
history of sudden difficulty in swallowing, vertigo, and vomiting. A diagnosis of
brainstem ischemic stroke was made. Which one of the following neurological signs
is not an indicative of brainstem pathology?
a. Vertical nystagmus.
b. Aphasia.
c. Internuclear ophthalmoplegia.
d. Horizontal gaze palsy.
e. Onion skin pattern of loss of sensation in the face.

Q25:
A 25-year-old woman presents with few weeks history of generalized headache,
vomiting, and blurred vision. Brain CT scan was normal and the CSF opening
pressure was 40 cm water. Which on is the correct statement regarding pseudo-tumor
cerebri?
a. The idiopathic variety is the commonest type.
b. Absence of headache should cast a doubt on the diagnosis.
c. Primary optic atrophy is the most fearful complication.
d. Treatment with steroids should be avoided.
e. Optic nerve sheath fenestration protects the operated eye only.

Q26:
A 7-year-old boy with few weeks' history of lethargy and progressive pallor was
found to have acute lymphoblastic leukemia L2-subtype. He is complaining of
headache and double vision. Non-contrast brain CT scan was normal. Lumbar
puncture under platelets infusion showed a CSF filled with blasts and raised protein.
Which one of the following statements is true regarding CNS involvement in
leukemia?
a. It does not affect he overall prognosis.
b. Neck stiffness is seen in the absence of pyogenic meningitis.
c. A single negative CSF sample excludes leukaemic meningitis.
d. Intrathecal methotrexate should be avoided.
e. The spine, but not the cranium, should be irradiated.

Q27:
A 67-year-old woman with a longstanding type 2 diabetes presents with transient
speech defect and right sided weakness. A diagnosis of hemispheric TIA was done.
All of the followings can be seen in anterior circulation strokes except?
a. Amaurosis fugax.
b. Motor Aphasia.
c. Transcortical sensory aphasia.
d. Nystagmus.
e Hemiplegia.

10
Q28:
A 56-year-old man patient is being evaluated after sustaining a stroke with a resultant
aphasia. Which one of the followings can result from a dominant hemispheric lesion
only?
a. Asteriognosis.
b. Urinary incontinence.
c. Hemiplegia
d. Transcortical motor aphasia.
e. Constructional apraxia.

Q29:
A 58 year old, with post-stroke epilepsy on carbamazepine tablets and is seizure free,
presents with few days' history of unsteadiness and double vision following upper
respiratory tract infection, which was treated successfully with clarithromycine.
Which one of the followings is responsible for his new presentation?
a. Hemorrhagic brainstem stroke.
b. Carbamazepine toxicity.
c. Postural hypotension.
d. Chronic subdural hematoma.
e. Clarithromycine poisoning.

Q30:
A 22-year-old diabetic man since 10 years is being evaluated for autonomic
neuropathy because of orthostasis symptoms and impotence. Autonomic neuropathy
is not seen in?
a. Amyloidosis.
b. Alcoholism.
c. Guillain Barre syndrome.
d. Motor neuron disease.
e. HIV infection.

Q31:
A 48-year-old man, who was completely healthy, presents with a 3-week history of
headache, fever, anorexia, and a double vision. Examination revealed confusion,
fever, neck stiffness, and bilateral abducense palsy with florid papillodema.
His non-contrast brain CT scan showed hydrocephalus but no mass lesion. CSF
analysis revealed an opening pressure of 30 cm H2O, cloudy fluid, lymphocytic
pleocytosis with 90% lymphocytes, protein 400 mg/dl, and sugar 20 mg/dl (random
blood sugar 110 mg/dl). His plain chest x ray showed evidence of old hilar
calcifications. He has a family history of a certain chest disease with cough and
wasting. Which one is the correct statement regarding his illness?
a. Viral meningitis is a likely diagnosis
b. Anti TB medications are better to be started.
c. His intracranial pressure is very low.
d. The hydrocephalus is due to over production of CSF.
e. Complete recovery is the rule.

11
Q32:
A 67-year-old man presents with an 8 month history of speech difficulty and
coughing upon swallowing. Examination revealed dysarthria, small conical tongue,
exaggerated gag reflex and jaw jerk. Which one of the followings is not a potential
cause of this condition?
a. Motor neuron disease.
b. Bilateral hemispheric lacunar strokes.
c. Myasthenia gravis.
d. Brainstem vasculitis.
e. High brainstem tumors.

Q33:
A 61-year-old man presents with progressive gait difficulty and abnormal speech.
Examination revealed wide spread fasciculation and muscle wasting. A preliminary
diagnosis of motor neuron disease was made. The presence of which one of the
followings is not considered to be against the diagnosis?
a. Parkinsonian features.
b. Ocular palsies.
c. Urinary incontinence.
d. Upper and lower motor neuron signs in one limb.
e. Unilateral cerebellar signs.

Q34:
A 28-year-old woman with a relapsing remitting multiple sclerosis is being evaluated
in your clinic. She has bilateral central scotomas, and fundoscopy revealed flat optic
nerve heads of whitish color and clear margins. All of the following conditions can
cause this optic nerve head picture, except?
a. Vitamin B12 deficiency.
b. Friedreick's ataxia.
c. Surpatentorial meningioma.
d. Tobacco-alcohol amblyopia.
e. Vasculitis.

Q35:
A 31-year-old woman has primary progressive multiple sclerosis since 2 years with
no remission at all. Which one of the following features is against the diagnosis of
multiple sclerosis?
a. Transverse myelitis.
b. Horner's syndrome.
c. Optic neuritis.
d. Aphasia.
e. Internuclear ophthalmoplegia.

12
Q36:
A 56-year-old man presents with 6 months history of generalized headache. He was
completely healthy and reasonably well, on no medications or illicit drugs . He is a
life long non-smoker and non-alcoholic, with no risk factors for HIV infection. His
brain MRI is suggestive of a left sided frontal lobe oligodendroglioma.
Which one of the followings is not true regarding this primary brain tumor?
a. It is mainly seen supratentorially.
b. Tumor calcification is seen in up to 90% of cases.
c. In general, it is has a favorable response to systemic chemotherapy.
d. Most tumors can be resected completely.
e. It is less infiltrative that astrocytomas.

Q37:
A 61-year-old woman, who was reasonably well and enjoyed independent life,
presents with 2-month history of depressed mood. Her son stated that his mother is
not interested in anything, and she talks very little. She did respond to a trial of
fluoxitine tablets prescribed by her general practitioner since 4 weeks. Her brain MRI
is consistent with a large right sided frontal lobe glioblastoma multiforme.
Which one of the followings is true regarding this brain tumor?
a. It is a low-grade malignant tumor.
b. Complete surgical resection is possible in the majority of victims.
c. Metastasis outside the CNS is rare.
d. The 5-year survival figure is almost 70%.
e. Gliomatosis cerebri is the usual brain MRI picture.

Q38:
A 68-year-old woman brought by her daughters to consult you regarding the memory
status of their mothers. They stated that their mother is becoming gradually forgetful
and anxious, while the patient denies this during the interview. She scored 20 on mini
mental status examination (MMSE). After an extensive work up, you diagnosed
Alzheimer's disease, and you are thinking of giving donepezil to her.
Which one of the followings is true regarding this medication?
a. It should be given 4 times daily.
b. It is markedly hepatotoxic.
c. It is not given in advanced cases.
d. Only 1-2% of patients will show modest improvement in their cognitive
functions.
e. Tachycardia is a problematic side effect.

Q39:
A 62-year-old man, diagnosed as having idiopathic Parkinson's disease since 5
months, is not responding to Sinemet tablets. Further workup disclosed a diagnosis of
normal pressure hydrocephalus (NPH).
Which one of the followings is true regarding this type of hydrocephalus?
a. A previous history of head trauma may be present.
b. There is occlusion of the Sylvius aqueduct.
c. Comprises up to 50% of cases of dementia in old people.
d. Radionuclide cisternography should be avoided.
e. Post-shunting, only 3-5% of patients will show improvement in their gait and
cognition.

13
Q40:
A 21-year-old man is extremely anxious. He stated that his father died at the age of 45
years because of Huntington's disease (HD), and he is afraid that he might be silently
affected. Which one of the followings is true regarding this fatal neurological illness?
a. It is an autosomal recessive disease.
b. There is GGG tri-nucleotide repeat expansion in HD gene.
c. Should be differentiated from benign familial chorea.
d. Seizures are frequent in adult cases.
e. Parkinsonian features are very common in late onset cases.

Q41:
A 57-year-old man, presents with 8-month history of resting hand tremor and gait
difficulty. Ultimately, he was diagnosed as having idiopathic Parkinson's disease, and
you are thinking of prescribing a medication for his illness.
Which one of the following statements is correct regarding medical therapy of this
disabling neurological disease?
a. Benztropine is an excellent option in old people with rigidity.
b. Amantidine produces a long lasting benefit.
c. Dopamine is the pro-drug of L-dopa.
d. Pramipexol is a D6 receptor agonist.
e. Tolcapone enhances the effect of L-dopa therapy.

Q42:
A 22-year-old man sustained a trauma to the upper spine following a fall from his
motor cycle. There are features of acute painful transverse myelopathy at the level of
C6,7. Imaging studies confirmed the presence of an acute hematomyelia.
Which one is the correct statement regarding this spinal cord hematoma?
a. An underlying AVM may be present.
b. Extension into the subarachnoid space never occurs.
c. Vasculitis can not cause this hematoma.
d. Treatment is mainly by surgical evacuation.
e. Selective spinal angiography should be avoided in all cases.

Q43:
A 54-year-old man presents with repetitive severe lancination pains in his lower limbs
and unsteady gait. You found bilateral small pupils with light-near dissociation. He
has a history of poorly treated syphilis 20 years ago.
Which one of the followings is true regarding tabes dorsalis?
a. The patient may present as a medical acute abdomen.
b. Rombergism is present in all cases.
c. Vibration sense in the lower limbs should be intact.
d. Urinary frequency and urge incontinence are seen.
e. Extensor planters can be elicited

14
Q44:
A 26 year old woman, was diagnosed a having relapsing remitting multiple sclerosis
(RRMS) since 1 year, and is currently receiving interferon beta therapy.
All of the followings are reasons to stop these medications in RRMS, except?
a. Development of severe depression with suicidal ideation.
b. Pregnancy.
c. Severe bone marrow suppression.
d. Development of one severe relapse after 1 year of therapy.
e. Development of secondary progression with no superimposed relapses.

Q45:
A 16-year-old schoolgirl who is a known case of idiopathic generalized tonic clonic
epilepsy, currently receiving valproic acid tablets and is seizure free for the past 3
months, is consulting you about which medications to avoid, because she was surfing
the internet and found that some mediations may worsen her epilepsy.
Which one of the following medications does not reduce seizure threshold?
a. Amphetamine.
b. Cyclosporine.
c. Acyclovir.
d. Meperidine.
e. Levitiracetam.

Q46:
A 65 year old man, was diagnosed as having amyotrophic lateral sclerosis (ALS)
since 1 month, is referred to you for consideration as whether to start Riluzole or not.
Which one of the followings is true regarding this medication?
a. It has an anti-GABA effect.
b. Is nephrotoxic.
c. Produces modest improvement in survival figure.
d. Can cause weight gain.
e. Give as an intermittent intravenous infusion.

Q47:
A 32 year old man, with post primary pulmonary tuberculosis (TB) and is receiving
anti TB medications since 5 months, presents with abnormal sensation in his feet. A
diagnosis of isoniazide-induced peripheral neuropathy was made.
All of the followings can cause sensory or predominately sensory peripheral
neuropathy, except?
a. Cisplatin.
b. Dapsone.
c. Vincristine.
d. Metronidazole.
e. Paclitaxil.

15
Q48:
A 44-year-old woman with history of watery diarrhea before 2 weeks presents with
rapidly progressive areflexic weakness in her lower limbs. A diagnosis of Guillain
Barre syndrome was made.
The followings can cause demyelinating peripheral neuropathy, but which one is the
cause of primary axonal peripheral neuropathy?
a. Hypothyroidism.
b. Vitamin B12 deficiency.
c. Chronic liver disease.
d. Waldenstroms macroglobulinemia.
e. Diphtheria.

Q49:
A 34-year-old woman with generalized myasthenia gravis complains of cramping
abdominal pains after the intake of pyridostigmine.
Which one of the followings is true regarding the treatment modalities in myasthenia
gravis?
a. Intravenous immunoglobulin is used in cholinergic crisis.
b. Thymectomy is indicated in congenital myasthenic syndromes.
c. Pyridostigmine can produce long lasting remission.
d. Prednisolone may cause a transit initial worsening.
e. Cyclosporine is contraindicated.

Q50:
A 58-year-old man, with a history of ischemic heart disease, presents with generalized
body aches and weakness that interfere with his daily job as a laborer. He is on
Aspirin, atenolol, and lovastatin. After 2 weeks of stopping lovastatin, he reported a
marked improvement in his weakness and pains.
Which one of the followings is not a cause of drug-induced myopathy?
a. Zidovudine.
b. Cyclosporine.
c. Gemfibrozil.
d. Paracetamol.
e. Penicillamine.

Q51:
A 62-year-old man presents with slowly progressive distal weakness and wasting in
both upper and lower limbs. There is absent knee jerks and flexor planters. EMG and
muscle biopsy studies confirmed a diagnosis of inclusion body myositis (IBM).
Which one is the correct statement regarding this form of inflammatory myopathy?
a. There is degeneration of anterior horn cells.
b. A positive family history should be absent.
c. Usually resistant to immune suppressive therapy.
d. The presence of rimmed vacuoles on biopsy specimens is suggestive of an
associated dermatomyositis.
e. The prognosis is excellent.

16
Q52:
A 22-year-old man is consulting you because he has a relative with a muscular
dystrophy who died because of cardiac arrest.
Which one of the following muscular dystrophies is not associated with cardiac
involvement?
a. Becker's muscular dystrophy.
b. Duchenne's muscular dystrophy.
c. Emery-Dreifus muscular dystrophy.
d. Facioscapulohumeral muscular dystrophy.
e. Myotonia dystrophica.

Q53:
A 61-year-old man presents with recurrent orthostasis symptoms. He is hypertensive
and is taking captopril and hydrochlorothiaziade tablets. He is afraid that he might
have a brain disease causing this picture.
Autonomic dysfunction can be associated with many central and peripheral
neurological diseases. Which one of the following autonomic dysfunction-causing
diseases is not associated with CNS signs?
a. Multiple sclerosis.
b. Botulism.
c. Multiple system atrophy.
d. Tabes dorsalis.
e. Parkinson's disease.

Q54:
A 29-year-old new married woman is consulting you about the likelihood of having a
child affected by mitochondrial diseases, because she has a relative with one of these
diseases.
Which one of the following diseases is not due to mitochondrial cytopathy?
a. Kearns-Sayre syndrome.
b. MERRF (myoclonic epilepsy with ragged red fibers).
c. MELAS (mitochondrial encephalopathy with lactic acidosis and stroke like
episodes).
d. Leigh's disease.
e. Machado-Joseph's disease.

Q55:
A 22-year-old man presents with 3-week history of progressive somnolence and
headache. He has a history of poorly treated frontal sinusitis before 1 month. Brain
CT with contrast revealed a large right-sided frontal mass with ring enhancement and
massive surrounding edema and midline shift.
Which one of the followings is true regarding brain abscesses?
a. In 90% of cases, no obvious source of infection can be found.
b. The surrounding edema is cytotoxic in type.
c. Fever is present in 50% of cases.
d. Lumbar puncture should be done to isolate the infectious agent.
e. After treatment, only 5% will have seizures.

17
Q56:
A 34-year-old woman, presents with 3 days history of fever, headache, confusion and
seizures. Brain MRI findings were suggestive of herpes simplex encephalitis. Which
one is the correct statement regarding this type of viral encephalitis?
a. Bilateral temporal involvement is common.
b. About 50% of PCR-proven cases will have a normal brain MRI.
c. The virus can be cultured from the CSF in 75% of cases.
d. The negative predicative value of a negative CSF PCR assay for HSV DNA is only
2%.
e. Acyclovir should be started only after CSF PCR is done.

Q57:
A 63-year-old woman presents with rapidly progressive dementing illness and
myoclonus.
Which one of the followings is not against the diagnosis of Cruetzfeldt-Jakob disease
(CJD)?
a. Fever.
b. Peripheral blood neutrophilic leukocytosis.
c. Neutrophilic CSF pleocytosis.
d. ESR of 110.
e. Positive CSF protein 14-3-3.

Q58:
A 57-year-old man presents with 3-week history of confusion, headache, vomiting,
and right sided weakness. He is a life long smoker, and recently had a chest X ray
showing a large cavitating mass near the upper right hilar area. Brain CT scan showed
multiple masses that could be metastases.
Which one of the followings is true regarding treatment of raised intracranial pressure
(ICP)?
a. Draining CSF via ventriculostomy should be avoided.
b. The head of bed should not be elevated.
c. Hemi-craniectomy is used in refractory elevated ICP.
d. Steroids are highly effective in hemorrhagic stokes.
e. Plasma osmolality should be kept above 320 mosmol/Kg when using
mannitol.

Q59:
An elderly vagrant man brought to the A/E department by the ambulance in a severe
dehydrated state, and rehydration was started. The next day, he became quadriplegic.
Which one of the followings is true regarding central pontine myelinolysis (CPM)?
a. May result from too slow correction of dehydration.
b. There is demyelination at the basis pontis without inflammation.
c. The presence of pseudobulbar palsy should suggest another diagnosis.
d. The absence of quadriparesis should cast a doubt on the diagnosis.
e. Has an excellent prognosis.

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Q60:
A 59-year-old man presents with 1-hour history of aphasia and dense right sided
weakness. Brain CT scan revealed no evidence of intracranial hemorrhage, any mass
lesion, or midline shift. A diagnosis of ischemic stroke was made and you are
considering thrombolytic therapy to be given.
The presence of all of the followings should exclude him from receiving this mode of
therapy, except?
a. Rapidly improving symptoms.
b. Blood glucose of 500 mg / dl.
c. Elevated INR.
d. Hematocrit of 20%.
e. Onset of symptoms to the time of drug administration is less 3 hours.

Q61:
A 22-year-old college student brought by his friends to the A/E department with
sudden onset of unsteady gait. He is grossly ataxic for stance and gait.
Which one of the followings is not a cause of acute cerebellar ataxia?
a. Alcohol intoxication.
b. Lithium toxicity.
c. Post-infectious cerebellitis.
d. Cerebellar hemorrhagic stroke.
e. Hypothyroidism.

Q62:
A 15-year-old male presents with slowly progressive gait unsteadiness and abnormal
speech. You detected pes cavus, scoliosis, cardiomyopathy, absent ankles, extensor
planters, and nystagmus. Your provisional diagnosis is Friedreick's ataxia.
He has two relatives affected by this disease. Which one of the followings is not
inherited as a cause of spinocerebellar ataxia?
a. Machado-Joseph disease.
b. Kearns-Sayre syndrome.
c. Episodic ataxia type II.
d. Ataxia telangiectasia.
e. Leigh's disease.

Q63:
A 21-year-old woman presents with 2-day history of headache, fever, and mild
confusion with neck stiffness. Her CSF revealed elevated opening pressure,
lymphocytic pleocytosis, raised protein and normal sugar. Viral meningitis was
diagnosed.
Which one of the following infectious viral agents is considered to be a common
cause of this type of meningitis?
a. Cytomegalovirus (CMV).
b. Ebstien Barr virus (EBV).
c. Enteroviruses.
d. Adenovirus.
e. Herpes zoster virus (HZV).

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Q64:
A 16-year-old boy presents with a 4-day history of fever, headache, and confusion.
Further workup disclosed a diagnosis of viral encephalitis.
All of the following viral agents causing encephalitides have a good prognosis in
general, except?
a. La Crosse encephalitis viral .
b. California encephalitis virus.
c. Venezuelan equine encephalitis virus.
d. Eastern equine encephalitis virus.
e. Ebstein Barr virus.

Q65:
A 21-year-old man presents with repeated generalized tonic clonic fits without
regaining consciousness. He is a known cause of epilepsy, and is taking phenytoin,
but he stopped taking his medication since 1 week because he was seizure free for
seven months and he thought that phenytoin is no more needed, as his roommate said.
Which one of the followings is true regarding status epilepticus?
a. It is not a medical emergency.
b. Intracranial hemorrhage is the commonest precipitating factor.
c. Phenobarbitone should be given immediately.
d. Lactic acidosis is seen.
e. Hypothermia is very common.

Q66:
A 34-year-old man presents with sudden onset of aphasia and right sided weakness.
Brain CT scan revealed a large hypo-dense area consistent with occlusion of the main
stem of the left middle cerebral artery.
Ischemic stroke in young people is an important subject in neurology. Which one of
the followings is true regarding the investigations done to discover the cause of this
topic?
a. Tranesophageal Echocardiography is not indicated.
b. ECG would probably add nothing.
c. VDRL can be useful.
d. CSF analysis is useless.
e. Anti-phospholipid antibodies are ordered in old people only.

Q67:
A 24-year-old woman, who was completely healthy and reasonably well, presented
with sudden severe headache and dense left sided weakness. An urgent non-contrast
brain CT scan revealed a small hematoma at the right basal ganglia.
Which one is the wrong statement regarding her illness?
a. Spontaneous hypertensive hemorrhage is the likely etiology.
b. Toxicology screen should be done.
c. Conventional cerebral angiography can be useful.
d. Coagulopathies should be excluded.
e. Congophilic angiopathy is a not a consideration.

20
Q68:
A 34-year-old man presents with 3-week history of abnormal jerky movements
involving his left hand only, which start and stop suddenly and spontaneously.
Which one of the followings is true regarding his illness?
a. Brain CT scan is diagnostic.
b. A history of head trauma is irrelevant.
c. Primary brain tumors are not a consideration.
d. A history of migraine like headache is important.
e. Carbamazepine is probably not effective.

Q69:
A 63-year-old man presented with an acute onset of right sided weakness and motor
aphasia. He is hypertensive and diabetic. Brain CT scan is consistent with cerebral
infarction.
Regarding antiplatelets and anticoagulation use in acute ischemic stroke, which one is
the correct statement?
a. Aspirin has been shown to decrease early stroke recurrence.
b. Aspirin had not been shown to decrease stroke related morbidity.
c. Heparin should be given in all acute cases.
d. Heparin is not associated with increased risk of CNS hemorrhage.
e. Clopidogrel should be combined with aspirin routinely.

Q70:
A 54-year-old woman presents with few weeks history of painful proximal muscle
weakness. Her CK is markedly elevated, and EMG is of myopathic pattern. A
provisional diagnosis of polymyositis is done.
Which one is the correct statement regarding this inflammatory myopahty?
a. Severe facial weakness is usually seen.
b. Absence of ocular involvement should cast a doubt on the diagnosis.
c. Anterior neck muscle involvement is unusual.
d. Respiratory muscles involvement may be seen.
e. A skin rash should be present to fulfill the diagnosis.

Q71:
A 6-year-old boy presents with progressive gait unsteadiness and right hand
clumsiness. His mother said that he frequently vomits in the morning. Brain MRI
discloses a right sided cerebellar hemispheric mass. Which one is the correct
statement regarding cerebellar tumors in children?
a. Infratentorial tumors tend to raise the intracranial pressure lately.
b. High grade malignant gliomas are the commonest type.
c. A cerebellar pilocytic astrocytoma has an excellent prognosis.
d. Neck stiffness indicates the occurrence of subarachnoid hemorrhage.
e. Brainstem compression never occurs

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 Answers:
Q1: Answer D
The sudden occurrence of a partial Brown-Squard syndrome in this young lady should
always prompt a search for multiple sclerosis. A compressing metastatic neoplastic
tumor (like from a breast cancer) may give a similar picture. Notice the sensory level
on lateral aspect of the right arm (C6); an MRI of the cervical cord with gadolinium is
the key imaging investigation here.

Q2: Answer D
Severe cervical spondylosis is a well-known risk factor for brain stem stroke due to
vertebro-basilar ischemia. Although there is no history of hypertension or diabetes or
hyperlipidemia, a carotid Doppler study is worthy to assess the status of the carotids.
Aspirin should be given to all patients with ischemic stroke unless contraindicated;
this patient's clinical picture clearly indicates that the patient can receive this form of
therapy; however, his history is present for about 24 hours, which excludes him from
receiving thrombolytics. Always look for and control risk factors like smoking,
diabetes, hypertension…etc.

Q3: Answer C
This woman's new generalized seizure should always prompt a search for hematoma
expansion or the development of another hematoma that causes cortical irritation.
Needless to say, hypo or hypernateremia should not be forgotten. Also, look for other
causes like severe hypoxemia, medication side effects and interactions…etc.

Q4: Answer E
Remember that up to 90% of the pyramidal tract decussates in the lower medulla to
the contralateral side. However, in a minority of normal population this decussation
could be very minor or even absent as in this patient. In those people, a lesion in this
tract above the lower medulla will not give a contalateral pyramidal signs, but instead
these will be ipsilateral. This patient had a cortical irritating tumor, which causes focal
fits at the same side of the tumor. Although other options might be true in real
practice, this congenital anomaly should not be forgotten.

Q5: Answer C
He is likely to have been diagnosed with schizophrenia and been given a conventional
neuroleptic, after which he developed neuroleptic malignant syndrome. Notice that
this syndrome develops over few days and it is not an sudden one.

Q6: Answer A
In Friedreick's ataxia, there is no impairment or only mild impairment in cognition; a
marked cognitive impairment should cast a doubt on the diagnosis. An absent family
history does not refute the diagnosis; however, its presence is an excellent clue.

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Q7: Answer E
The picture is of an acute left sided papillitis, which has a broad differential diagnosis
and causes. The pace and severity of the clinical picture, physical findings, and
careful investigations and follow up are very important. Vasculitides, infectious
agents, sarcoidosis…etc all can produce this picture, and multiple sclerosis (MS)
although unlikely it is worthy to exclude it. The visual loss in giant cell arteritis is
irreversible, and a rapid spontaneous improvement goes with MS. Foster Kennedy
gives a slowly progressive optic atrophy in one side and papilloedema in the other
side.

Q8: Answer C
Notice that the overall picture is of a cluster headache-like, but the picture is atypical
given the 2 years of non-remission and occurrence during day and night, and there are
features of hypogonadism. All of them might indicate an intracranial space-occupying
lesion in and around the sella. Actually, MRI of this patient revealed a large sellar
mass with supra-sellar extension and impingement upon the left carotid artery, which
explains both the hyperprolactinemia and the dysautonomia affecting the left side of
the face (eye redness and nasal discharge) by affecting the sympathetic fibers around
the left carotid artery. It was a prolactimona by histopathological examination after
successful surgical debulking.

Q9: Answer A
These signs indicate that CNS involvement is present, which can be documented by
CSF studies (after correcting the platelet count if severely depressed). The duration of
steroids is too short to cause such a picture of obesity. Vincristine causes peripheral
sensory neuropathy, not a cranial motor one. Those ALL patients with CNS
involvement need intrathecal chemotherapy and cranio-spinal irradiation as the
prognosis is unfortunately poor.

Q10: Answer C
This patient is immune suppressed because of cyclosporine. Given the presence of
impaired consciousness, fever, and neck stiffness, meningitis (or
meningoencephalitis) is the most likely diagnosis. We should stop cyclosporine, and
start broad-spectrum antibiotics and do CSF examination. Blood cultures can be done,
and adding another immune suppressant (azathioprine) would be a silly step.

Q11: Answer E
She has two sites of involvements: spinal cord and optic. Both are of white matter
signs. The pace is somewhat rapid, and although she did not report an improvement,
B12 deficiency and heredito-familial degenerative conditions are highly unlikely.
Instead, one should exclude multiple sclerosis by having this clinical picture and
should be differentiated from anti-phospholipid syndrome, SLE, vasculitis...etc.
Therefore, Brain imaging would be a reasonable step here.

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Q12: Answer A
This is most likely an embolic stroke to the main stem of the left middle cerebral
artery. The infracted area is large with mass effect and midline shift. So, measures to
reduce the raised intracranial pressure should be instituted without any delay like high
concentration O2, mannitol infusion...etc; while anticoagulation is definitely
indicated, it is should be omitted for the time being as there is a very high risk of
hemorrhagic transformation in this large friable infarcted area, and starting it after 1-2
weeks is reasonable. Her atrial fibrillation is rapid and she needs measures to slow it
like giving digoxin, and an echo study is needed to evaluate her heart. A DC shock is
not indicated as we don’t know the duration of the AF; besides, given the presence of
mitral stenosis, there is a very high risk of recurrence and emboli showering in the
presence of no anticoagulation coverage.

Q13: Answer E
Given the presence of such a long history, positive family history, stork-like legs and
palpable nerves in the context of little disability and no other system affection,
Charcot Marie Tooth disease is the most likely diagnosis.

Q14: Answer D
This is Broca's aphasia in a right-handed man; the posterior part of the lower left
frontal gyrus is the culprit site.

Q15: Answer E
Weight gain, not weight loss, is a side effect. Alopecia is seen with valproic acid,
while hirsutism is seen with phenytoin.

Q16: Answer C
Kayser-Fleischer ring is due to deposition of copper in the Descement's membrane of
the cornea, which is reversible upon successful therapy with copper chelators.

Q17: Answer D
CIDP, not GBS, can be a cause. Notice that CMT type I is a demyelinating disease
that produces palpable nerves; type II is an axonal one, with no such finding. Other
cause: leprosy.

Q18: Answer C
Notice that both Carbamazepine and phenytoin produce worsening in cases of petit
mal (and myoclonic) epilepsy and should be avoided. Valproic acid and Ethosuximide
are first line agents for this epilepsy, while lamotrigine and clonazepam are second
line agents.
Valproic acid is preferred because of its wide spectrum against many types of
epilepsy, which might coexist with petit mal (like myoclonic jerks and GTC seizures).

24
Q19: Answer E
This is papilloedema! Notice that papillodema is not seen in multiple sclerosis (MS).
Papillitis is seen in MS, which is seen as a red optic nerve head swelling with
SEVERE reduction in the visual acuity.

Q20: Answer C
The clinical picture is suggestive of pyogenic meningitis in a previously healthy man
with no history of head trauma; so staring iv ceftriaxone and vancomycin is the next
step while arranging for lumbar puncture. Brain CT scan is not indicated as the
history and findings are not focal. MRI is not used in critically ill and confused
patients in the emergency departments; brain CT is the first line imaging modality. He
has no risk factors for HIV, and ordering this test without a prior consent is not
accepted; besides, the result will not change our lines of management initially.

Q21: Answer E
Dysautonomias are seen in up to 65% of cases and might produce a sudden cardio-
respiratory death. Some degree of asymmetry in findings may be seen in up to 9% of
cases; however, marked asymmetry should cast a doubt on the diagnosis. Even in the
best centers, the mortality rate is around 4-5% of cases. The CSF proteins level (as is
the nerve conduction study) might be totally normal in the 1st week of illness; hence
normal studies early in the illness are not against the diagnosis. Some patients,
around 6%, may experience relapses and which might be associated with certain HLA
haplotypes

Q22: Answer C
Mesial temporal or hippocampal atrophy is a common cause of temporal lobe epilepsy
(TLE). Notice the prodromal features that are followed by the ictus (for which the
patient recollects nothing). Carbamazepine and phenytoin are effective in many
patients and should be the first line if no contraindication is present. Refractory TLE
patients are the best candidates for surgical treatment for complex focal epilepsies in
general. The EEG may show lateralized spikes in one or both temporal lobes.

Q23: Answer C
A meningioma is an extra-axial tumor that is benign in a substantial number of
victims. The presence of underlying brain edema indicates that the tumor may be
malignant, or the presence of so-called secretary meningioma, or an atypical benign
meningioma; hence massive brain edema is seen uncommonly. The tumor may stop
growing, but never regresses. The commonest sites are over the cerebral convexities
and along the falx cerebri; the infra-tentorial fossa and the spine are uncommon sites.
Intra cranial meningiomas are seen more commonly in females with a history of
breast cancer, and together with cranial irradiation in the past, are considered a risk
factor for meningioma development. Multiple meningiomas should always prompt a
search for a diagnosis of neurofibromatosis type II.

25
Q24: Answer B
Aphasia is a supratentorial sign, that almost always indicates a cortical lesion in the
dominant hemisphere; thalamic aphasia is rare and has an excellent prognosis. Certain
brainstem lesions might result in anarthria, which can be easily confused with aphasia.

Q25: Answer A
The idiopathic variety is the commonest type; other causes: intracranial venous sinus
thrombosis, medications (tetracycline, vitamin A), Addison's disease and Cushing's
syndrome. Headache free variety is well documented, and unfortunately those patients
presents lately with visual loss. The visual loss is due to secondary (not primary) optic
atrophy to a long-standing papilloedema. Although steroids can be a cause (long-term
treatments, particularly upon withdrawal), they are the agent of choice in severe
elevation of the ICP. Surprisingly, optic nerve sheath fenestration, although done in
one eye, it protects both eyes.

Q26: Answer B
CNS involvement in leukemias always portends a grave prognosis, especially when
present at the time of the diagnosis. Headache, backache, confusion, cranial palsies,
and root signs all might be clues to leukemic meningitis. A single CSF sample may
fail to show the blast cells, and repeated samples are required to increase the
diagnostic yield. Neck stiffness in leukemias could be due to leukemic meningitis,
infectious meningitis, or subarachnoid hemorrhage (due to low platelets). The patient
should be put on a certain treatment protocol that entails intrathecal chemotherapy
(methotrexate, cytosar and hydrocortisone) and craniospinal irradiation.

Q27: Answer D
Nystagmus is an infratentorial sign, and thus is due to posterior circulation ischemia
in stroke patients. Hemiplegia could be due to anterior or posterior circulation strokes.
Seizures are seen in 5-15% of stroke patients because of cortical irritation (mainly in
embolic or hemorrhagic strokes).

Q28: D
The concept of dominant and non-dominant hemisphere is important in localization in
neurology. All aphasias are due to dominant hemispheric lesions. Hemiplegia is seen
in many sites damaged by a pathology (like the cortex, internal capsule, basis
pontis…etc). Asteriognosis is a cortical sign, but can be seen in both dominant and
non-dominant parietal hemispheric lesions. Constructional apraxia is due to non-
dominant parietal lesions. Urinary incontinence is seen in lesions involving both
mesial frontal lobes as in normal pressure hydrocephalus.

Q29: Answer B
Most likely, that clarithromycine intake had resulted in enzyme inhibition and
carbamazepine toxicity manifesting as ataxia and diplopia.

Q30: Answer D
Motor neuron disease has no autonomic neuropathy as a feature. Other causes: fatal
familial insomnia, vasculitides, Fabrey's disease, and multiple system atrophy.

26
Q31: Answer B
Most likely, he has TB meningitis, and anti TB medications are better to be started
while awaiting the CSF culture for TB bacilli. The history is too long for a viral
etiology, and the intra cranial pressure is high (normal 5-18 cm H2O). The
hydrocephalus in TB meningitis could be both communicating and non-
communicating. Even with treatment, many are left with considerable degree of
neurological disability.

Q32: Answer C
This man has pseudobulbar palsy. Option C is a cause of bulbar palsy.

Q33: Answer D
Notice that the presence of cerebellar signs, ocular palsies, marked dementia,
Parkinsonian features, sensory signs and symptoms, and sphincter disturbances, all
are against the diagnosis. The combination of ALS, Dementia, and Parkinsonian
features is a very rare distinct clinical syndrome, and should not make you change
your answer in the MRCP examination regarding the classical MND features.

Q34: Answer C
Notice that this woman has primary optic atrophy. Any longstanding raised
intracranial pressure states with papilloedema (for example from a space-occupying
lesion) can produce secondary optic atrophy.

Q35: Answer D
Aphasia is cortical sign; all others are can be caused by white matter demyelination.
Noticed that the dementia that is seen in advanced MS is a sub-cortical type.

Q36: Answer B
Oligodendogliomas are primary brain tumors that are usually supratentorially in
location, less infiltrative that astrocytomas, and hence most of them are amenable to
complete surgical excision, and usually responds well to systemic chemotherapy.
Tumor calcification is usually seen in 30% of cases.

Q37: Answer C
Of all gliomas, glioblastoma multiforme is the most aggressive one, and infiltrating
the adjacent areas diffusely making complete surgical excision impossible (debulking
surgery is used to decrease its size, obtain a tissue diagnosis, and reduce the
intracranial pressure). Gliomatosis cerebri is said to be present when there is diffuse
infiltration without a clear cut mass or enhancement area on brain MRI. Needless to
say, the prognosis is very poor. Metastasis outside the CNS is rare and is usually seen
in those who are operated.

Q38: Answer C
Donepezil is a central inhibitor of acetyl cholinesterase, used in early cases of
Alzheimer's disease. About 10-20% of patients will show a modest improvement in
their cognition. The medication has a long half-life, and is given once per day.
Tacarine (central inhibitor of acetyl cholinesterase) is hepatotoxic; donepezil is not. It
can cause bradycardia, and thus contraindicated in those with advanced heart blocks.

27
Q39: Answer A
NPH is a cause of potentially reversible dementia. It is uncommon cause of dementia
(Alzheimer's disease comprises up to 50% of cases of dementia in old people). It can
be idiopathic, or secondary to head trauma, subarachnoid hemorrhage, or meningitis.
Brain MRI will show dilated ventricles (without prominent sulci) with upward
bulging of the corpus callosum and widening of the Sylvius aqueduct, in the absence
of a mass lesion. The definitive diagnosis is by radionuclide cisternography. Post-
shunting, 30-50% of patients will show improvement in their gait and cognition.

Q40: answer C
Huntington's disease (HD) is an autosomal dominant disease, due to trinucleotide
(CAG) repeat expansion at the HD gene. Both seizures and Parkinsonian features are
more common in juvenile cases, which are fortunately rare. Those who presents with
chorea in the context of a positive family history, should be differentiated from benign
familial chorea, which has a different prognosis.

Q41: Answer E
Benztropine (like all anticholinergics) is effective at treating tremor, not rigidity; and
in those who are above the age of 60-65 years, can produce confusional state.
Amantidine can be used in early cases, either alone or with anticholinergics, has a
mild short-lived effect that rapidly wears off. L-dopa is the prodrug of dopamine.
There are no D6 receptors (only D1-5). Tolcapone (and entacapone) is a COMT
inhibitor, and thus enhances the effect of L-dopa therapy.

Q42: Answer A
Hematomyelia is a hematoma in the substance of the cord, can be due to AVMs,
coagulation defects, and vasculitis. Decompression into the subarachnoid space can
occur. When an AVM is the cause, spinal angiography is done to delineate the AVM
together with evacuation of the hematoma; otherwise, the treatment is symptomatic.

Q43: Answer A
Tabes dorsalis is a form of tertiary syphilis. Rombergism is seen in 50% of cases only
due to loss of kinesthesia (and hence vibration sense should be impaired also). Loss of
bladder sensation causes urinary retention. Extensor planters are seen in Taboparesis
or general paresis of insane, not in pure tabes dorsalis. The severe lancinating pains
may cause severe abdominal pain (tabetic abdominal crises) that brings the patient as
an acute abdomen (but no surgical cause can be found, so-called medical acute
abdomen).

Q44: Answer D
The idea of giving Beta INFs in RRMS is to decrease the severity and frequency of
relapses (by one third), so the occurrence of 1 relapse after 1 year of therapy is not a
reason to stop this form of therapy; instead, 3 or more attacks within 1 year that
necessitate steroid pulses, is an indication of treatment failure, and the Beta INF
should be stopped. Options a, b, and c are true.

28
Q45: Answer E
Levitiracetam is an anti-epileptic agent. Also avoid cocaine, quinolones, and
chlopromazine.

Q46: Answer C
The precise mode of action of Riluzole is unknown, but it is supposed to have an anti-
glutamate action, and thus interfering with excito-toxic mechanisms. It is given orally
and has been found to produce a modest improvement in survival figure. It is hepato-
toxic, and can cause weight loss.

Q47: Answer B
Dapsone (usually when given in the long term, as in the treatment of leprosy, in which
the patient shows a new motor worsening after a period of improvement) can cause a
pure motor peripheral neuropathy.

Q48: Answer B
The distinction between demylinating and axonal neuropathy is very important
regarding the possible etiology and prognosis. There is no definite clinical test for this
discrimination, but a nerve conduction study can answer us. Vitamin B12 deficiency
is a cause of subacute/chronic primary peripheral axonopathy.

Q49: Answer D
The anticholinesterases have a symptomatic benefit only, and do not affect the course
of illness. Thymectomy has no place in: neonatal myasthenia, congenital myashtenic
syndromes, patients above the age of 65 years, myasthenia duration of more than 7
years, and pure ocular myasthenias. Iv Ig is used in myasthenic (not cholinergic)
crisis. Although prednisolone is effective in almost all cases, it can produce a
dramatic worsening at the start of treatment. Cyclosporine has a modest effect as an
immune suppressive agent in MG.

Q50: Answer D
Drug induced myopathy is usually under-diagnosed in clinical practice. The list is
long, but paracetamol is not implicated. Zidovudine can cause myopathy with ragged
red fibers due to mitochondrial toxicity. The risk of statine-induced myopathy (or
myositis) is greatly increased when these agents are used with cyclosporine or
gemfibrozil.

Q51: Answer C
IBM is the commonest cause of inflammatory myositis in those above the age of 50
years. Although the EMG may show evidence of denervation, there is no degeneration
of anterior horn cells. It is unfortunately resistant to immune suppressive therapy
(occasionally, many cases are diagnosed as polymyositis, which comes into light after
failure of immune suppressive therapy). The prognosis is poor. Some cases show
familial clustering. The presence of rimmed vacuoles on biopsy specimen is highly
suggestive of IBM, and there are many other distinctive features on histopathological
examination, like deposition of amyloid, presence of eosinophilic cytoplasmic
inclusions…etc.

29
Q52: Answer D
Only facioscapulohumeral muscular dystrophy (MD) is not associated with cardiac
involvement (although it may be associated with sensory neural deafness and retinal
detachment, and labile hypertension). Limb girdle MD uncommonly associated with
cardiac involvement.

Q53: Answer B
Notice that botulism is a disease of motor end plate due to interference of
acetylcholine release, and affect acetylcholine release at autonomic ganglia causing
various dysautonomias.

Q54: Answer E
Machado-Joseph's disease is a spinocerebellar ataxia type 3, due to CAG repeat
expansion.

Q55: Answer C
In 30% of cases, no obvious source of infection can be found, and the surrounding
edema is vasogenic. LP is both dangerous and unnecessary. After successful
treatment, around 50% are left with seizures.

Q56: Answer A
About 5-10% of PCR-proven cases will have a normal brain MRI (and 33% will have
a normal brain CT scan). The virus can not be cultured from the CSF. The negative
predicative value of a negative CSF PCR assay for HSV DNA is 98%, and acyclovir
should be started without delay when there is a clinical suspicion of HSV encephalitis,
as it decreases both morbidity and mortality figures.
Q57: Answer E
Positive CSF protein 14-3-3 in the context of rapidly progressive dementia and
myoclonus is highly suggestive (but not diagnostic) of CJD (it can be also positive in
HSV encephalitis but negative in Alzheimer's disease). Any fever, high ESR,
peripheral blood neutrophilic leukocytosis, or neutrophilic CSF pleocytosis should
cast a doubt on the diagnosis.

Q58: Answer C
When the ICP is recorded invasively through ventriculostomy, the CSF can be
drained to decrease the pressure. The head of bed should be elevated. Steroids can be
used in edemas associated with brain tumors or abscesses (they are useless in edema
associated with ischemic stroke or hemorrhagic stroke). Plasma osmolality should be
kept BELOW 320 mosmol/Kg when using mannitol.

Q59: Answer B
CPM results from too rapid correction of hyponatremia or hypo-osmolal states. Partial
forms are not uncommon, and may present as dysarthria, confusion, or even gaze
palsies; thus, the absence of quadriparesis is not against the diagnosis. The usual
picture is an acute onset of quadriparesis and pseudobulbar palsy in a chronic
alcoholic man who received an aggressive correction of chronic hyponatremia.

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Q60: Answer E
Option E is an "inclusion criteria". You should be familiar with both inclusion and
exclusion criteria for receiving thrombolytic therapy in this setting.

Q61: Answer E
Hypothyroidism is a cause of slowly progressive ataxia in long standing cases.

Q62: Answer B
Kearns-Sayre syndrome is a sporadic, not inherited cause of SCA.

Q63: Answer C
Enteroviruses are the commonest agents responsible for viral meningitis. All other
infectious agents are considered to be uncommon causes.

Q64: Answer D
Not every viral encephalitic process portends a gloomy prognosis. La Crosse
(California) virus, Venezuelan equine encephalitis virus, and Ebstein Barr virus have
a good prognosis in general. EEE (Eastern Equine viral Encephalitis) has a case
fatality rate of 50-75%, and at lest 80% of survivors are left with severe neurological
deficits.

Q65: Answer D
Status epilepticus is a medical emergency with high mortality rate, the commonest
precipitating event is sudden withdrawal (self or iatrogenic) of anti-epileptics. Lactic
acidosis is very common but does not warrant any treatment per se. Phenobarbitone
infusion should be used as a 3rd step (after lorazepam and phenytoin). Hyperthermia,
not hypothermia is seen.

Q66: Answer C
Ischemic stroke in young people (less than 45 years old) should prompt a thorough
search for an underlying cause, like atrial fibrillation, SBE, meningovascular syphilis,
paradoxical embolism through a patent foramen ovale…etc.

Q67: Answer A
In the absence of chronic hypertension, or in patients younger than 40 years of age,
spontaneous "hypertensive" hemorrhage is unlikely. Instead, we should look for other
causes like AVM, coagulopathy, drug abuse, head trauma, hemorrhage in
tumors…etc. Congophilic angiopathy is seen in old people.

Q68: Answer D
This is a new onset of focal motor seizures in an adult man. Intracranial space
occupying lesions should always be excluded. Brain CT scan may miss many lesions;
hence, MRI is superior to it. After head traumas, some patients may develop early and
or late onset of focal epilepsy. A migraine like headache with focal seizures should
always prompt a search for APS (anti-phospholipid syndrome) or AVM.
Carbamazepine is the drug of choice in focal seizures.

Q69: Answer A
Aspirin has been shown to decrease stroke related morbidity and mortality, and early
stroke recurrence. Heparin is indicated in persistent cardiogenic source of

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embolization. Heparin is associated with increased risk of CNS (or systemic)
hemorrhage which statistically outweighs any benefit. The combination of aspirin and
clopidogrel in the acute setting is still controversial.

Q70: Answer D
Ocular and facial muscles in polymyositis are usually spared; while the anterior neck
muscles are commonly involved (causing neck drop). Skin rash defines
dermatomyositis. Respiratory muscles involvement is fortunately uncommon but
dangerous and mainly seen in acute cases.

Q71: Answer C
Cerebellar masses in children are usually malignant brain tumors, and the pilocytic
variety of low-grade astrocytomas has an excellent prognosis and complete cure can
be achieved if removed completely. In general, infratentorial tumors tend to raise the
intracranial pressure early, unlike the supratentorial ones. Neck stiffness, with
negative Kerning's sign, indicates an infratentorial mass lesion. The most fearful event
is secondary brainstem compression and herniation syndromes.

End

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