Clinical Neuropsychiatry Editors Thomas H. Jobe, MD Associate Director, Neuropsychiatric Division Department of Psychiatry University of Illinois at Chicago Chicago, Ilinois Moises Gaviria, MD Director, Neuropsychiatric Division Department of Psychiatry University of Ilinots at Chicago Chicago, Minos Antony Kovilparambil, MD Attending Neuropsychiatrist Saint Bernard Hospital Attending Psychiatrist Loretto Hospital Chicago, linois b Blac SeieneeBlackwell Science Baitorial of: 350 Main Stret, Maken, Massachusetts 02148, USA ‘Osney Mead, Oxford OX2 0, England 25 John Steet, London WCIN 2BL, England 23 Ainslie Place, Edinburgh EHS 6AJ, Scotland 54 University Steet, Caton, Victoria 3053, Australia (Other Editorial Offices Arnette Blackwell SA, 224, Boulevard Saint Germain, 75007 Paris, France Blackwell Wissenschaft Verlag GmibH Kurflrstendamm 57, 10707 Berlin, Germany Zeetnergasse 6, -1140 Vienna, Aust Distributors usa Blackwel Science, Ine 350 Main Steet Malden, Massachusets 02148 (Telephone orders: 800-215-1000 oF 617-388-4250; fax orders: 617-368- sm) anata Copp Clatk Professional 20) Adelaide Sueet, West, 3rd Floor ‘Toronto, Ontario MSH 17 (Teephon orders: 416-597-1616 or 1-800.818:9417; Fox orders: $16-597- 117) Avetain Blackwell Science Py, Le 54 University Street Carlton, Vistoria 3053 (Telephone order: 0993470800; Fax orders 13-9349-3016) Outside North Arericn and Austain Blackwell cence, Lt «/o Marston Book Seevices, Lid P.O. Box 269 Abingdon, (Oxon OXI4 AYN England (Telephone orders: 44-01235-465500; Fax orders: H-O1235-45555) Acquisitions Editor: Chris Davis Production Editor: Heather Garrison Manufacturing: isa Flanagan “Typeset by Best-ot Typesetter Li, Hong Kong Pinte and bound by Hamillon Printing (© 1997 by Blackwell Science, Ine Printed in the United Stats of America 96.97 9899 543.21 All rights reserved. No part ofthis book may be reproduced in any form lorby any electronic or mechanical means, including information storage and retrieval systems sithout permission in writing from the publisher, ‘exept by a reviewer who may quote bref passages in a review. ‘The Blackwell Science logo is a tase mark of Blackwell Science Lt, registred atthe United Kingdom Trade Marks Registry Lary of Congress Cataloging in-Publication Data Clinical neuropsychistry / editors, Thomas Jobe... [ea Bom Incluss bibliographical references and index. ISBN 0.86542-528-0 1. Neuropsychiatry. 1, Jobe, Thomas IDNLME 1. Organic Mental Disorders, Paychotic—dagnoss, 2 Organic Mental Disorders, Peychotic—therapy. 3. Bran Diseases— psychology. 4, Brn Injures—peychology. 5. Neuropsychology — methods. WM 220 C6416 1997 RC3ELCH826 1997 6168—de21 DNLM/DLC. for Library of Congress 48558 arTO BORIS ASTRACHAN whose efforts made possible the development of neuropsychiatry at UIC AND TO HYMAN MUSLIN a valued teacherContents Preface Diagnostic Assessment 1. The Mental Status Examination Ovidio A. De Leon, MD 2. The Neurologic Examination Xabier Beristain, MD 3. Neuropsychological Assessment John A. Bartok, PhD Eileen M. Martin, PhD 4, Functional Brain Imaging, Michael J. Blend, PhD Antony Kovilparambil, MD Xabier Beristain, MD Cognitive Disorders 5. Delirium. Chamlong Disayavanish, MD Kevin M. Furmaga, PharmD 6, Dementia Ramén Cacabelos, MD 7. Neuropsychiatric Aspects of Ischemic Cerebrovascular Disease Moises Gaviria, MD Christopher G. Lis, MPH Chamlong Disayavanish, MD Kevin M. Furmaga, PharmD 8. The Diagnosis of Stroke Cathy M. Helgason, MD David O. Staats, MD Camilo R. Gomez, MD 9. Human Immunodeficiency Virus Infection and the Central Nervous System R, Craig McKenna, MD 7 2 123 11 160 m1. Vv. 10, Traumatic Brain Injury Shobha Sinha, MD Addictions 11, Alcoholism R. Craig McKenna, MD 12, Drug Addiction William W. Weddington, MD Neurologic Disorders 13. Epilepsy ‘Nutan Atre-Vaidya, MD 14, Tourette's Syndrome Anne Leach, MD 15, Parkinson's Disease Herbert F. Vandenberg, MD 16, Frontal Lobe Syndrome John A. Bartok, PhD Moises Gaviria, MD Psychiatric Disorders 17. Schizophrenia Miguel A. Gonzalez Torres, MD 18, Dissociative Disorders Michaele Johnson, LCSW ‘Thomas H. Jobe, MD Kevin M. Furmaga, PharmD 19, Affective Disorders: Biochemical Aspects Lucia Inchausti, MD Rafael Segarra Echevarria, MD 170 179 181 194 8 229 236 2a7 249 283 301Contents VL. Special Conditions 323 VIL. Pharmacologic Treatments in Neuropsychiatric 20, Geriatric Neuropsychiatry 35 Lenin head Kathleen Kim, MD 23, Neuropsychopharmacology 355 21. Legal Aspects of Neuropsychiatry a4 Chamlong Disayavanish, MD Philip Pan, MD Kevin M. Furmaga, PharmD 2, Stress 32 24, Neuroleptic Malignant Syndrome 382 Debra Klamen, MD Surinder Nand, MD LaGenia Bailey, PharmD Index 389Contributors Nutan Atre-Vaidya, MD ‘Associate Professor of Psychiatry Department of Psychiatry and Behavioral Sciences Finch University of Health Sciences ‘The Chicago Medical School Chicago, Ilinois LaGenia Bailey, PharmD Department of Psychiatry Veterans Administration, West Side Medical Center Clinical Assistant Professor Department of Pharmacy Practice University of Hlinois at Chicago Chicago, linois John A. Bartok, PhD Assistant Professor of Psychology Rush Neurobehavioral Center ‘Chicago, Iinois Xabier Beristain, MD Research Fellow, Department of Neurosurgery University of Hlinois at Chicago Chicago, Hlinois Michael J. Blend, PhD, DO Professor of Radiology /Nuclear Medicine Director of Nuclear Medicine University of llinois at Chicago Chicago, Iinois Ramén Cacabelos, MD, PRD, DMSci Director and Chairman EuroEspes Biomedical Research Center Institute of CNS Disorders Santa Marta de Babio Bergondo, La Coruna Spain vidio A. De Leén, MD Assistant Professor of Psychiatry Director of Neuropsychiatric Unit ‘Neuropsychiatry Division University of Ilinois at Chicago Chicago, Illinois Chamlong Disayavanish, MD Professor of Psychiatry Faculty of Medicine Chiang Mai University Chiang Mai Thailand Rafael Segarra Echevarria, MD Department of Psychiatry Hospital de Basurto Bilbao Spain Kevin M. Furmaga, PharmD, BCPS Assistant Professor of Pharmacy Practice Clinical Assistant Professor of Pharmacy in Psychiatry University of Illinois at Chicago Chicago, Iiinois Moises Gaviria, MD Director, Neuropsychiatric Division Department of Psychiatry University of Iinois at Chicago Chicago, Iinois Camilo R. Gomez, MD Associate Professor of Neurology Director of Cerebrovascular Service Department of Neurology University of Alabama at Birmingham Birmingham, AlabamaContributors Cathy M, Helgason, MD Professor of Neurology Director of Cerebrovascular Service Department of Neurology University of Illinois at Chicago Chicago, linois Lucia Inckausti, MD Hospital de Puis Guipuzcoa Servicia de Psychiatria Bilbao Spain ‘Thomas H. Jobe, MD Associate Director, Neuropsychiatric Division Department of Psychiatry University of Minois at Chicago Chicago, Iinois Michaele Johnson, LCSW ‘Neuropsychiatric Division Department of Psychiatry University of Ilinois at Chicago Chicago, Iinois Kathleen Kim, MD Associate Professor of Psychiatry Associate Director of Clinical Services Department of Psychiatry University of Illinois at Chicago Chicago, Iinois Debra Klamen, MD Associate Professor of Psychiatry Director, Division of Undergraduate Education in Psychiatry Department of Psychiatry University of Chicago Medical School Chicago, Ihinois, Antony Kovilparambil, MD Attending Neuropsychiatrist Saint Bernard Hospital Attending Psychiatrist Loretto Hospital Chicago, Iinois ‘Anne Leach, MD Assistant Professor of Psychiatry ‘The Psychiatric Institute Department of Psychiatry University of Iinois at Chicago Chicago, Iinois Christopher G. Lis, MPH Research Associate Division of Neuropsychiatry University of Illinois at Chicago Chicago, Iinois Eileen M, Martin, PhD Associate Professor of Psychology in Psychiatry University of Illinois at Chicago Chicago, Iinois R Craig McKenna, MD Associate Professor of Clinical Psychiatry Director of Consult-Liaison Service Veterans Administration, West Side Medical Center Chicago, Iinois, Surinder Nand, MD Chief of Psychiatry Veterans Administration, West Side Medical Center Chicago, [inois Philip Pan, MD. Assistant Director of Education Isaac Ray Center Chicago, Hlinois Shobha Sinha, MD Assistant Professor of Clinical Psychiatry Neuropsychiatric Division Department of Psychiatry University of Chicago at Illinois Chicago, [linoisContributors David O. Staats, MD Herbert F, Vandenberg, MD Associate Professor of Clinical Medicine Department of Psychiatry Geriatric Medicine University of Illinois at Chicago University of Illinois at Chicago Chicago, Ilinois Chicago, Illinois William W. Weddington, MD Miguel A. Gonzalez Torres, MD Director of Addiction Program Chief, Department of Psychiatry ‘Veterans Administration, West Side Medical Center Hospital de Basurto Chicago, Iinois Bilbao SpainPreface This volume emerged from requests by residents and col- leagues for information that was of immediate clinical relevance but could be presented within a broad neuro- scientific perspective. As a result, this volume contains a combination of highly theoretical as well as highly prac- tical material. The background for each chapter is well referenced to the several excellent recent texts and journal articles in the field of neuropsychiatry. We are often asked how neuropsychiatry differs from behavioral neurology, general psychiatry, geriatric psychiatry, and biological psychiatry. Neuropsychiatry differs from behavioral neurology because behavioral neurology focuses primarily on the brain origins of disturbances in normal functions while neuropsychiatry focuses on the brain origins of abnormal functions. Therefore, the neuropsychiatrist focuses on symptoms of impulsive behavior, aggression, delusions, or hallucina- tions while the behavioral neurologist focuses on, for example, speech problems due to aphasias and other neuropsychological deficits. Neuropsychiatry differs from biological psychiatry by focusing on neurocomputational models, such as neural networks, rather than receptor theories of mental illness. Also, neuropsychiatry differs from both general psychia~ try and biological psychiatry because it depends less on highly controlled epidemiological studies and more fon case reports and small case sample reports. Neuro- psychiatric patients have multisystem disease and do not lend themselves easily to the limitations of inclusion criteria, With regard to pharmacology, neuropsychiat differ from both general psychiatrists and biological psy- chiatrists by looking for new uses for old drugs rather than awaiting the arrival of the latest computer-modeled, “cleaner” drugs for a given receptor type. Neuropsychiatry complements general psychiatry, especially in the area of the trauma model. Neuro- psychiatrists seek to discover not only how the brain is damaged from head injury suffered during physical trauma but also how the limbic system is damaged by such traumas as childhood sexual abuse. With regard to geriatric psychiatry, neuropsychiatry concerns itself with the early application of new basic neuroscience research directly to problems of older patients, Finally, the international character of neuropsychiatry should be mentioned. The international character of neuroscience research has helped to bring together indi- vidual national societies and programs with less cultural and political bias than other mental health areas. There is overlap and some conflict between neuro- psychiatry and each of the related specialties. This is to be expected in a new field that is based on an old tradi- tion but is entering a new growth phase. Neuropsychiatry is positioning itself with these other fields and with neuropsychology so that they all may exploit the ex- pected yields in brain imaging and clinical neuroscience techniques. It will require the cooperative efforts of all related specialties to form a coherent research program in the explosive areas of brain imaging and related tech- niques in order to apply this research to the immediate benefit of our patients. THY, MG. AK,Part! Diagnostic AssessmentChapter 1 The Mental Status Examination Ovidio A. De Leén Brain disease has a diverse effect on human behavior and. higher cortical functions. The mental status examination, which is the systematic assessment of the cognitive and emotional functions commonly disturbed in patients with organic brain diseases, reveals these effects. Although the examiner can obtain important information on cogni- tive functions during the history-taking and patient- interviewing process, in many cases, deficits are not apparent, It is therefore only by the deliberate probing entailed in the mental status examination that deficiencies in memory, language, constructional abilities, and other higher cortical functions are disclosed. Neither psychiatrists nor neurologists routinely per- form a detailed mental status examination. According to Strub and Black (1), however, a full examination is a ne~ cessity in patients with a known or suspected brain lesion and in patients with either vague behavioral complaints or the recent onset of psychiatric symptoms. The resulting recognition of cognitive and emotional deficits secondary to traumas, vascular accidents, and other neurologic dis- eases then helps in the planning of rehabilitative treat- ‘mentand averts frustration on the part of both the patient and family. On the other hand, headaches, behavioral changes, or the recent onset of seizures can herald the development of a brain tumor, small infarcts, or sub- dural hematomas, lesions sometimes undetected by a neurologic examination. Frontal or temporal tumors, hydrocephalus, and cortical atrophy can, in many pa- tients, be the cause of depression, memory problems, or other subtle emotional and behavioral symptoms. ‘The physical and neurologic examination must precede the mental status examination to make sense of the infor- mation obtained during the mental status examination and to determine a probable diagnosis. The mental status ‘examination that follows should be comprehensive, done systematically, and performed in a hierarchical manner. It begins with the evaluation of consciousness, the most basic function. The examiner first needs to establish whether the patient is able to sustain attention before attempting to evaluate other functions requiring the skills of anattentive person. The examiner then moves on to the evaluation of speech, because an impairment in com- ‘munication will make it difficult or impossible to assess the patient’s other higher cognitive functions, such as memory, construction ability, calculation, gnosis, and abstract thinking, ‘The Mini-Mental State Examination is a brief, widely used screening test that superficially assesses several dimensions of language and cognitive functioning. The maximum score is 30, with scores below 24 con- sidered abnormal and indicative of diffuse cognitive dysfunction (2). Nothing is more critical in the conduct of a mental status examination than to establish a good physician- patient relationship. It is only after the patient feels that the clinician has a genuine interest in and respect for him that he will engage in a cooperative and trusting relation- ship. The neuropsychiatris, just as any other clinician, can never expect to obtain useful information until this most important goal is achieved. After greeting the pa- tient, either in the office or on the hospital floor, the neuropsychiatrist structures the interview by first intro- ducing himself and then explaining the purpose of the interview. Sometimes confidentiality issues also need to be discussed before the examination can proceed. The physician should avoid making a cold opening full of medical jargon as he reads the patient's chart. A caring, warm, and supportive manner needs to be displayed at all times and the patient's feelings recognized. Phrases that serve this purpose are: “I will try to bother you as little as possible,” “I will be asking you a bunch of silly questions, but they will help us understand how different parts of your brain are working,” or “Maybe you are tired of being asked the same questions by different people.” 3Part I Diagnostic Assessment Being responsive tothe patient's feelings should not at the same time be done in such an aimless and purposeless way as to impede the structured, systematic, and thor- ‘ough investigation of cognitive functions. Discussed in this chapter are bedside tests used in clinical situations. A discussion of specialized neuropsychological tests and procedures is beyond the scope of this chapter, however. History ‘The history-taking needs to address not only those changes in behavior usually indicative of an organic process in the traditional sense, but also the symptoms typical of functional psychiatric conditions. It is also im- portant to investigate carefully any medical conditions that not only affect mental functioning, but also necessi- tate treatment with medications whose effects on the ner- ‘vous system frequently spawn the symptoms of a mental condition. Finally itis important to obtain information on the patient's level of education, work history, and ethnic and social backgrounds, as well as to assess the patient's premorbid personality and prior level of functioning, The examiner needs to record the following elements of the history: 1. Identifying information: Age, sex, handedness, ethnicity, religious affiliation, marital status, occupa tion, and reason for and source of referral. Chief complaint: Specific complaint in patient's own words, as well as complaints noted by family. 3. History of present illness: Date and type of onset, symptoms, sequence of behavioral and personality changes, and treatments attempted. Bizarre, life- threatening, or dangerous behaviors should in particu- lar be noted. A patients difficulty with attention pro- cesses, memory, language, judgment, reading, writing, and calculation points toward the existence of an of- ganic condition. It is also important to note the status of appetitive behaviors, such as sleeping, eating, and sexual interest. Classic psychiatric symptoms such as hallucinations, depression, anxiety, or delusions need to be placed in the context of other clinical findings. 4. Past medical and psychiatric history: Inciudes a sys- tematic review of the patient's symptoms, previous medical or psychiatric problems, use of alcohol or 2 drugs, and use of medications (including over-the- counter medications). This also includes any trauma, seizures, or central nervous system infections suffered by the patient and the nature of any exposure to toxic substances. 5. Personal history: Birth and developmental landmarks, educational and work history, and status of family and social relationships. 6. Family history: The presence of neurologic or psychiat- ric diseases in other family members, the treatments implemented for them, and their success, General Observation ‘The careful observation of the patient’s behavior and de- ‘meanor usually provides important initial clues that help the clinician not only to direct his attention toward impor- tant aspects of the examination, but also to guide his own behavior during the assessment. ‘The patient’s general appearance provides the first clinical information. This consists of the patient's physical characteristics, such as his body type, the nature of any asymmetries or minor physical anomalies, his personal hygiene, and the way in which he is dressed and groomed. The assessment of the patient’s attitude fo- uses on the degree of the patient's cooperation with the interview process and the status of his or her relationship with the examiner. The extent of eye contact, the degree of cooperation, excessive familiarity, and any suspicious- ness or unrealistic expectations exhibited begin to reveal important information about the patient's disorder. ‘The observation of the patient’s motor activity includes the level of activity (hyperkinesis versus hypokinesis) and a search for catatonia, abnormal postures, tics, extrapyra~ midal symptoms, involuntary movements, or obvious neurologic deficits, such as hemiplegia, ataxia, dysmetria, and gait difficulties ‘The examination of the patient's affect entails an evalu- ation of the quality of his mood, the patient's range of emotional expression and his interpersonal relatedness, any autonomic indicators of anxiety, and the patient's somatic preoccupations. The interviewer can watch his, own internal responses to the patient, as these can mirror the patient's subjective affective state. Disturbances of af-Chapter 1 The Mental Status Examination fect are prominent in some organic conditions, such as frontal lobe dysfunction. Insight and judgment begin to be assessed when the patient is asked why he sought help. Demented patients may seem unconcerned or bewildered by this question. Patients with other organic brain disorders may be unable to appraise their shortcomings. They also fail to under- stand the reason for the consultation, their diagnosis, the treatment prescribed, or the effect of their illness on their ability to function, According to Kirshner (3), replies to these questions reveal more about these functions than do proverb interpretation, similarities and differences test- ing, and asking the patient to show how he would react to certain artificial situations. Consciousness Consciousness is the level of arousal of an organism that takes place within the course of a cyclic pattern of oscillation. All of the psychic functions are superimposed on this fundamental neurophysiologic state. The brain- stem reticular formation and the diffuse thalamic pro- jection system activate the cortex to enable the person to relate to his environment (including his inner self) Without the input from these systems, the cortex is unable to function adequately and specific lesions of the reticular formation induce a state of coma. Metabolic disturbances, drug intoxications, hemorrhages, infarcts, and sepsis are common causes of such disturbances of consciousness. Alterations in consciousness range from full alertness to deep coma, with lethargy, obtundation, and stupor intermediate points on the continuum. The alert person is aware of his surroundings and able to establish meaning ful interpersonal interactions. The lethargic patient is hypersomnolent and his spontaneous movements are de- creased, but he can be aroused easily. Nevertheless, he tends to fall asleep when not continuously stimulated. Patients who are difficult to arouse are obtunded. If aroused, they are confused and no valid evaluation of their mental functioning is possible. The stuporous pa- tient responds only to intense or painful stimulation, after which he makes only restless, purposeless movements or mumbles. No behavioral response is obtained in a coma- tose patient even with painful stimulation. There is some degree of subjectivity in these states, and for this reason it is useful to describe the nature of the stimulation being applied and the behavioral response of the patient Teasdale and Jennett (4) were responsible for developing the Glasgow Coma scale, which assigns a numerical value that quantitates the level of consciousness. ‘The imprecision and subjectivity of the foregoing termi- nology that arise if careful behavioral descriptions are not employed are best illustrated by the term stupor, whose central feature is the absence or reduction of action and speech. In a history of the psychiatric concept of stupor presented by Berrios (5), it was noted that the ancient Greeks considered stupor a state of non- responsiveness. During the nineteen century a progres- sive “psychologization” of the term took place, with an emphasis on the use of regression as an explanatory ‘model. Wernicke’s introduction ofthe term akinesia subse- quently inaugurated a tendency to distinguish the disor- ders of motility seen in psychiatric conditions from those seen in neurologic conditions. In the general use of the term, however, both organic and functional conditions are encompassed and it is presumed that the pathologic ‘mechanisms involved are different. In functional stupor the disorder of consciousness is considered to be related to its content and not to the neurophysiologic processes underlying arousal. Bleuler (6) believed that the patient With catatonic stupor has lost his will to: exercise his speech, motor systems, and consciousness, whereas these systems become inefficient and impotent in the patient with depressive stupor so that they cannot enact the patient's volitional design. The psychiatric literature con- siders hysterical stupor, nowadays extremely infrequent, to result from the removal of conscious control, by means Of dissociation, of motor and speech activities. Rogers (7) has recently postulated that psychiatric psychomotor re- tardation and neurologic akinesis are indistinguishable. Hicks and Birren (8) had already suggested that the same brain mechanisms underlie the psychomotor slowing that ‘occurs in the settings of aging, psychosis, and brain dam- age. Fisher (9) also proposed that varying degrees of psychomotor retardation constitute a continuum, start- ing with the full-blown syndrome of akinetic mutism, and suggested that the malfunction resided in a mes-Part I Diagnostic Assessment encephalofrontal activating system, similar to the moti- vation circuit postulated by Mogenson et al (10). Attention Attention is the capacity to control information process- ing in the brain. Wilhelm Wundt was the first to conduct serious systematic research on the topic of attention, and his laboratory was an important center for studies on priority entrance and the fluctuation of attention, Wundt’s disciple, Emil Kraepelin, went on to distinguish between passive attention (Auffassung) and active or vol- untary attention (Aufmerksamkeit). He considered that the first was altered during the acute or terminal stages of dementia praecox, whereas compromise of the latter was a consistent feature of the illness, These processes corre- spond to some of the components of attention that Posner and Boies (11) have proposed. According to these authors, attention comprises four distinct capacities: selectivity, vigilance, span, and alertness. Selectivity is the mechanism that leads to the further processing of one or few stimuli out of the many that impinge on the sensory organs and seems to be a function of the left hemisphere (11), Vigilance is the ability to sustain the scrutiny of one stimulus over time and is a function of the right parietal lobe. Some authors define concentration as the capacity to attend consistently to internal stimu. Patients with corti cal damage are unable to screen out irrelevant stimuli and have difficulty focusing and maintaining attention. Span of attention is the scope or amplitude of attention and is subserved by the left hemisphere. Aleriness is the capacity to scan the environment and disattend one stimulus when another seems to be more important. This adaptability is the result of frontal lobe functioning. Frontal lobe-damaged patients would per- form well in digit repetition but fail the “A” Random Letter test owing to problems with perseveration and an inability to make shifts in response patterns. ‘The limbic system endows the process of attention with, emotional meaning, and deficits in this function can be observed in patients with Iesions involving subcortical structures. Sometimes during the history-taking, the patient, the family, or both, point out difficulties with concentration. 6 Observation of the patient's behavior can also reveal evi- dence of such distractibility. A common bedside test to assess a patient's level of attention is the Digit Repetition test. In this test the patient is presented with digits in a normal tone of voice ata rate of one per second, beginning with a two-digit string and increasing until the patient fails. Compromise of attention processes can be presumed in a nonaphasic, nonretarded patient who is unable to repeat more than five digits ‘A test of selective attention and concentration is the Stroop Color Word test. It consists of three pages of 100 words each. The words red, green, and blue are printed in black in random order on the first page. The patient is asked to read as many of the words as possible in 45 seconds. The second page has seties of Xs printed in red, green, and blue, and the patient is asked to identify as many of the colors as possible in 45 seconds. The words red, green, and blue are printed on the third page, but not in the color of ink that matches the color denoted by the word. The patient is asked to identify the colors, not read the words, as fast as possible in 45 seconds. Frontal Jobe-damaged patients typically make many errors on this test Vigilance tests demand that the patient discriminate the ‘occurrence of a target stimulus from the occurrence of other similar stimuli, The “A” Random Letter testis such a test, In this test the patient is presented with a series of random letters and is told to indicate whenever he hears the letter A. No errors of omission or commission are expected in normal people. Concentration can be tested by asking the patient to name the days of the week or the months of the year in reverse order. Concentration can also be tested by having the patient spell a simple five-letter word such as world or arth and then ask him to spell the same word backward. ‘The Serial Sevens Subtraction test involves having the patient count backward starting at 100 and repetitively subtracting 7 from the remainder, or starting at 20 and repetitively subtracting 3 from the remainder. This test assesses a patient’s ability to sustain the task in mind. Successful completion may also reflect the patient's socioeconomic status or scholastic achievement, however, 30 this must be borne in mind when interpreting, the results ‘The examiner can identify at bedside an inattention toChapter 1 The Mental Status Examination one side of the body, usually the left, ifthe patient ignores one half of the space, Double simultaneous stimulation usually reveals the deficit, but mild degrees of neglect can be recognized using a line bisection task. In this test the patient is asked to mark the midpoint of a horizontal line drawn by the examiner. The examiner can also test for this by showing the patient a sheet of paper containing mul- tiple short lines and ask him to cross every one of the lines, The pattern of the lines left uncrossed can easily show the presence of neglect Language ‘The communication of information requires the use of symbols. The ability to encode, decode, and interpret the semantic and syntactic elements of those symbols consti- tutes a high cortical function closely related to thought named language. Ross etal (12) characterized four compo- nents of language: gestural, prosodic, semantic, and syn- tactic. Gestural language consists of the body movements and facial expressions used to convey meaning, Prosody is the melody, inflection, rhythm, and timber that convey emotional meaning. Semantic language is the symbolic ‘meaning of words. Syntax is the relational aspects of lan- guage and involves the use ofthe different types of words such as prepositions, adverbs, and adjectives that permits the appropriate ordering of words to produce a gram- ‘matically correct expression. Aphasia, which is an ac- quired disorder, is the disruption of language functions caused by lesions in a more or less specific brain region. ‘The term dysphasia is reserved for the congenital or devel- ‘opmental disorders that interfere with the proper learn- ing of language. During history-taking the examiner needs to establish that a patient’s language skills were intact before the onset of the present iliness. It is also important for him to differentiate between a language dysfunction and a motor or articulatory dysfunction called. dysarthria, in which the symbolic functions of lan- guage are not affected. Sometimes it is also important to discern whether the dysfunction involves only the voice, a condition called dysphonia. If the patient can write correctly or his verbalizations can be transcribed and read normally, the patient is not phasic. The mute patient, on the other hand, may or may not be aphasic. The ability to write normally indicates the absence of aphasia in these patients. The aphasic patient tries to communicate by gestures or sounds, and usually there is some language output that corroborates the diag- nosis. Oral apraxia sometimes interferes with the patient's ability to produce speech sounds, but if the patient cannot pucker the lips and pretend to blow out a lit match when asked to do so, this can establish the diagnosis as well. Schizophrenic patients also exhibit abnormal language expressions that are a reflection of bizarre thought pro- cesses, and this can be easily discerned, The language abnormalities seen in demented patients are typically ac- companied by other cognitive impairments indicative of a more widespread brain disease Dominance is the term given cerebral hemisphere spe- ialization in language and speech. Laterality is the term ven a specialized, objectively measurable central func- tion of a paired faculty, such as that involving the use of the eyes, ears, hands, oF feet. Preference is the name given the subjective experience of the person when expecting a motor act. Clinically, one may be merely testing prefer- cence that may depend more on the peripheral organ than ‘on any central mechanism. It is also important to deter- ‘mine the patient's handedness because of the association between cerebral dominance and language, About 99% of right-handed and most left-handed people have relative Janguage dominance in the left hemisphere, and the finding of aphasia in right-handed patients usually indi- cates the presence of a lesion in the left hemisphere. Left- handed aphasics may have lesions in either hemisphere, but crossed aphasia in dextrals is a rare occurrence. Hand- ceciness can initially be assessed during the history-taking and the observation of behavior. The family history of handedness and a tendency for the patient to use either hand to perform skilled movements offer important infor- ‘mation. Having a patient flip a coin, throw a ball, or hold a knife can also be used to demonstrate handedness. The Wada test, which involves the intracarotid injection of amobarbital (Amytal), can often establish the status of dominance. ‘The bedside language examination includes an assess- ment of spontaneous speech, naming, repetition, compre- hension, reading, and writing, During it the examiner first listens to the patient's spontaneous speech, observing carefully the fluency, articulation, prosody, and content of the speech, Fluency alludes to the flow of speech, which 7Part I Diagnostic Assessment ‘can be free and unimpeded or sparse and effortful. Mild defects can be assessed by the Animal-Naming test and the FAS test. In the former test the patient is asked to name as many animals as possible during 60 seconds; the normal person is able to name from 18 to 22 animals. In the FAS test the patient must name words that begin with the letters F, A, or § during three 60-second trials, Normal people can name at least 12 words beginning with each letter. Articulation refers to the enunciation of the speech. Such formation of sounds can be clear and distinct or slurred and garbled. Aprosodic speech lacks inflections and typically is seen in patients with right frontal cortical lesions. Comprehension dysprosody is the inability to discern differences in the meaning implied by different intonations and is associated with right temporoparietal lesions. Motor dysprosody is the inability to convey dif- ferent meanings through the use of different intonations. An analysis of the content of speech focuses on such er- rors as semantic or phonemic paraphasias, neologisms, agrammatism, and circumlocution. Naming difficulties constitute a reduced capacity to retrieve the words used in everyday speech. This function can be tested by showing the patient different objects and asking him to name them. The objects used should in- clude common as well as uncommon objects. The finding of normal repetition indicates that the primary language circuit is intact, that is, that auditory perception, the decoding of words in Wernicke’s area, transmission to Broca’s area, and the activation of articu- lation are all occurring. Repetition remains intact in the setting of aphasic syndromes that result from damage to other cerebral regions that give and receive information to and from the language primary circuit. Testing is per- formed with items of increasing difficulty. Unfamiliar or ‘grammatically complex phrases such as “no ifs, ands, or buts,” “whence, whither, and wherefore,” or “beat the kettle drum slowly” are met with difficulty in patients whose primary language circuits are intact, but who have lesions in ancillary language circuits that control gram- mar and sentence formation, Comprehension is initially assessed during history- taking. The formal testing of comprehension requires a strategy that isolates this function from other language functions. This can be accomplished by posing questions requiring minimal verbal responses, such as “yes” or 8 “no” answers or pointing to an increasing number of objects. It is important to remember that patients with brain lesions frequently tend to perseverate, and therefore questions should alternate randomly between those re- quiring a “yes” or “no” answer. Reading aloud and for meaning, should be tested, pro- vided the patient has an adequate educational back- ground. This involves having the patient read single words, then phrases, then paragraphs. To test compre- hension the patient is asked to execute commands given in printed form. Writing is tested by dictating numbers and words to the patient, then later asking him to write a short sentence using these numbers and words. The ‘examiner looks for paragraphias, or gross spelling errors. ‘Alexia is the loss or impairment of the ability to com- prehend written language. Agraphia is the loss or impair- ment of the ability to produce written language. Anterior alexia occurs in the presence of an abnormality of the dominant frontal lobe and accompanies Broca’s aphasia. Central alexia (alexia with agraphia) occurs in the setting of lesions involving the inferior aspect of the dominant parietal lobe and is consistently present in patients with Wemicke's aphasia. Posterior alexia (alexia without agraphia) is associated with disorders of the dominant hemisphere occipital lobe and is commonly seen in right- handed patients with an occlusion of the left posterior cerebral artery. Several combinations of normal and abnormal lan- guage functions can be discerned and constitute distinct aphasic syndromes. Global aphasia is the most severe form of aphasia and is characterized by nonfluent speech and impaired repetition, comprehension, naming, gram- mar, and prosody. The cause is a large lesion that dam- ages most of the anterior and posterior language areas. In Broca’s aphasia, comprehension is intact but speech is nonfluent and repetition, grammar, and prosody are im- paired. Naming may also be impaired. This type of apha~ sia is caused by a lesion in the anterior speech area. The ‘speech seen in patients with transcortical motor aphasia is also nonfluent and naming, grammar, and prosody are abnormal, but comprehension and repetition are nor- mal, The lesion involved typically affects the dominant supplementary motor area or the white matter pathway connecting to Broca’s area, or both sites. Patients with Wernicke’s aphasia are capable of fluent speech, butChapter 1 The Mental Status Examination comprehension, repetition, and naming are impaired Prosody is normal and grammar may be intact. The lesion is in the posterior language area. Patients with transcortical sensory aphasia have fluent speech, but com- prehension and naming are impaired. Repetition and. prosody are normal, and grammar may be intact. In this type of aphasia the lesion involved disconnects, Wemnicke’s area from surrounding temporoparietal asso- ciation cortices. Conduction aphasia isa fluent aphasia in which repetition is severely impaired and naming is im- paired but comprehension is normal. Prosody is normal, grammar may be intact, writing is plagued with errors, and reading aloud is abnormal, but comprehension dur- ing silent reading is normal. This syndrome is most com- monly caused by lesions in the fasciculus arcuatus and, related association fibers connecting Wernicke’s area to Broca's area, Anomic aphasia is characterized by distinct word-finding difficulties and an inability to name objects, ‘on confrontation. Lesions in several parts of the dominant hemisphere can cause this disorder, but itis rather severe in patients with lesions affecting the second and third temporal gyri. Finally, subcortical aphasia occurs in pa- tients with vascular lesions in the basal ganglia, and such patients typically present with mild anomia and compre- hension deficiencies. Memory ‘The fund of information derived from formal and infor- ‘mal education and common social experience constitutes the content of memory. Memory research attempts 0 ‘understand the different psychological and neurophysi- ologie processes responsible for the storage of infor- mation and its recall at a later time. Signoret (13) has proposed five different processes involved in memory. ‘The first of these isa holding process, which is responsible for the momentary retention of information before further processing takes place. The second is a process of acquir- ing, which invoives the gathering of information and the encoding of selected mnemic traces that can be linked to other bits of information. The third is a consolidation, process in which information is stored in a permanent or semipermanent archive. The fourth is a retrieval process involving the recapture of previously stored information for appropriate use. The fifth is a scanning process in ‘which the person reviews an assortment of mnemic traces and selects them in connection with current situations. Each memory process involves separate, but intercon- nected, neuroanatomic structures. No discrete syndromes are found in clinical practice, however, because the dis- ruption of one memory process occurs in conjunction with a malfunction of other critical memory and mental processes. Its also important to remember that the cause of memory dysfunction is not always organic. This was shown by psychoanalysts long ago who demonstrated that memories are subject to repression and distortion. Some prerequisites are necessary for the successful in- terpretation of memory tests. First, an alternative source should be sought out to verify the accuracy of the per- sonal information supplied by the patient. Second, the patient's premorbid intellectual and the educational level should be taken into account when asking historic and general knowledge questions. Third, the results of ‘memory tests are invalid if the patient is noncooperative or if he shows an impairment of other basic processes such as consciousness, attention, or sensory, motor, or language functions. Psychiatric conditions such as mood, disorders, psychosis, or anxiety can also result in poor memory performance, and stable memory deficits should not be inferred on the basis of the testing results alone. The term pseudodementia has been used to describe the disorder in patients whose memory deficits are not re- lated to brain damage but rather to perturbations associ- ated with a psychiatric condition. From the clinical point of view, three memory functions are widely recognized: immediate recall, recent memory, and remote memory. Immediate recall is the ability to reproduce accurately information just received (14). This process is performed by the language cortex sur rounding the sylvian fissure and does not require the intervention of limbic system structures. This ability is influenced by factors such as recency, distraction, and ‘quantity and is usually examined by digit repetition testing, A short verbal story can also be read after the patient is told that he will be expected to retell the story from memory. An example might be: “George is a TV news reporter./ He was covering an earthquake/ in Los Angeles./ He interviewed/ a woman,/ named Carol,/ whose car/ was parked on a street/ which caved in/ during the tremors./ The car/ disappeared/ into the 9Part I Diagnostic Assessment earth” (15). Remembering, at least eight of the 15 ideas presented would be considered a normal performance. An alternative test is the Babcock sentence: “One thing fa nation must have to be rich and great is a large, secure supply of wood.” This is also a test for language repetition. Recent memory is the process of acquiring and storing {information that permits people to learn new information and to retrieve this material after an interval of minutes, hours, or days. Limbic structures (hippocampi, the mamillary bodies, and the dorsal medial thalamic nucleus) are required to secure storage and retrieval. Ori- entation to place and time requires the capacity to learn the ongoing changes that take place in the environment, and its impairment suggests recent memory deficit. The patient should be asked his name, age, and birth date to test his orientation to person, Then he should be tested for his place orientation by questioning him about the present location (name, kind, and specific locale of the place), city, state and country, and finally his address. Time orientation is tested by asking the patient to give the date, day of the week, time of the day, and season of the year. Normal people may get the date of the month or the day of the week wrong, however, and performance is also dependent on the level of education, A visual ‘memory test can be useful for assessing recent memory in phasic patients. In this test, five objects are hidden in the patients room while he is watching. After 5 minutes he is asked to name and to indicate the location of the objects. New learning ability can be tested by giving the patient four unrelated words to remember: brown, honesty, tulip, and eyedropper, He is then asked to repeat the words after 5, 10, and 30 minutes. Memory storage can be assessed by giving the patient verbal cues when spontaneous recall fails. A retrieval defect is indicated if the patient's perfor- ‘mance improves even slightly. Paired-associate learning canbe examined by reading the patient alist of four pairs of words and then asking him to remember the words that go together. After the patient has been given the instructions, he is presented with one word and expected to repeat the paired word, An example is: weather-box, high-low, house-income, book-page. ‘Remote or tertiary memory consists of a vast amount of information maintained for a long time, sometimes for a lifetime, that is more or less readily available. The re- 10 trieval of this material may be of limited accuracy as the result of distortion, repression, or sublimation, however. Most of the information is stored throughout an extensive network of neural structures in both hemispheres, which explains the resilience of complex individwal memories in the setting of focal brain damage. Priming is an important event that occurs before limbic and frontal circuits are activated to participate in retrieval. Information learned many years previously, however, can be retrieved when the limbic circuitry is no longer functioning, The examina- tion of this function involves asking the patient to recall historical and personal events. The most-used questions focus on the names of the present and past presidents, important national events such as wars, the names of famous persons or the patient's family members, and per- sonal information. Constructional Ability The ability to draw, construct, or manipulate shapes and figures in two and three dimensions is called con- structional abilily. This nonverbal function requires the integration of frontal, parietal, and occipital lobe func- tions. The left hemisphere contributes to a person’s ability to draw the details of specific things, whereas it is the right hemisphere that perceives the spatial configuration ‘of the whole image (15). The integrity of multiple cortical areas, as well as intact vision, good motor coordination, normal strength, and preserved tactile sensation, are re- quired for correct performance, For this reason, abnor- ‘mal results to tests of constructional ability cannot be presumed to indicate disruption in the integrative higher cortical functions in the presence of motor or sen- sory deficits. Likewise, the ability to draw may be signifi- cantly impaired but constructional ability intact in a patient with chorea, Test results should also be inter- preted with caution in patients who are socially deprived, patients whose academic performance has been poor, and patients with a history of developmental disabilities. De- spite the importance of constructional abilities for the performance of everyday activities, an impairment can go unnoticed if the patient avoids tasks he is no longer ca- pable of doing, Useful and simple bedside constructional ability tests, requiring only paper and pencil, include the reproductionChapter 1 The Mental Status Examination of designs and the drawing of pictures on verbal com- mand. In the former test the patient is asked to copy designs of increasing difficulty, beginning with simple geometric figures, such as triangles or diamonds, and advancing to three-dimensional squares and intersecting pentagons. In clock drawing the patient is asked to draw a clock with the numbers and hands on it. Sometimes the examiner chooses a time to be drawn that involves the placement of the hands in neither a perpendicular nor overlapping fashion. Deficits in constructional ability can be easily discerned when the patient distorts the clock face, places the hands so that they indicate a time other than the one requested, spaces the numbers unevenly, or places them outside the clock, This test is also able to detect neglect. This disorder is evidenced when the pa- tient ignores the left side or squeezes the numbers onto the right side. Sometimes motor perseveration interferes with the task, rendering the patient unable to complete the drawing, In addition, left hemisphere lesions may hinder a patient's capacity to draw details, whereas right hemisphere injuries may cause a patient to distort the figure and create a lack of harmony among its elements. The results of constructional tests alone are not always conclusive when it comes to ascertaining the side of the lesion, unless unilateral neglect can be demonstrated. The drawing of a daisy in a flowerpot or a house in perspec- tive, so that two sides and the roof are seen, are also useful ways to assess visuospatial deficits. ‘Visuospatial integration is mainly a function of parietal lobes, and constructional impairment is rarely seen in patients with lesions that spare areas posterior to the rolandic fissure. Abnormalities in visuospatial tasks ob- served in patients in confusional states are usually transi- tory. On the other hand, they can be an early sign of cortical dementia. Other neurologic findings are also present in patients with vascular dementia Higher Cognitive Functions A person’s ability to perform higher-order cognitive func tions becomes impaired in the early stages of cortical damage, before attention, language, and memory pro- cesses are compromised. Abstraction, conceptualization, and the use of Ieamed information in problem solving, constitute the so-called higher intellectual functions and are uniquely human abilities. The results of the evalua- tion of these functions must be interpreted with caution, however, and take into consideration the patient's inteli- gence, education, and cultural background The status of acquired information is assessed by tests of vocabulary and general information. Strub and Black (1) have proposed a list of 10 questions, the patient's, responses to which can be scored. According to them this test provides a reasonable estimate of the patient's fund of information. The questions are: 1) How many weeks are in a year? 2) Why do people have lungs? 3) Name four people who have been president of the United States since 10940, 4) Where is Denmark? 5) How far is it from New York to Los Angeles? 6) Why are light-colored clothes cooler in the summer than dark-colored clothes? 7) What is the capital of Spain? 8) What causes rust? 9) Who wrote the Odyssey? 10) What is the Acropolis? Calculation ability is the ability to manipulate old knowledge and requires the use of basic arithmetic con- cepts and correct spatial alignment. The patient can be asked to solve single-digit calculations first, then advance to two-digit calculations, and finally, to written complex operations. Dyscalculia may be the result of unilateral or bihemispheric damage. Benson (14) has described three major variations of calculation disorders: 1) aphasic acalculia, in which the patient is unable to handle number language; 2) visuospatial acalculia, in which the patient cannot maintain appropriate place-holding values; and 3) anarithmetria, in which the processes of computation are disturbed. Aphasic acalculia occurs primarily as a result of dominant (left) hemisphere damage. Visuospatial acal- culia can result from abnormalities of the posterior non- dominant hemisphere. Its difficult to localize the neuro- pathologic defect responsible for causing anarithmetria, apparently because computational ability depends on ‘widespread cerebral function. This disorder has been ob- served in the context of brain disorders involving a dif- fuse disorder such as Alzheimer’s disease. A dominant parietal lobe lesion frequently leads to the development of Gerstmann’s syndrome (dysgraphia, dyscalculia, finger agnosia, and right-left disorientation), Lesions of the frontal, parietal, temporal, or occipital lobe may cause calculation deficits, However, these deficits are more fre- quent in the setting of parietal lobe damage because pari- etal lobe function is responsible for the understanding of uPast I Diagnostic Assessment numerical concepts and the correct spatial alignment of numbers. Abstraction and conceptualization constitute an ad- vanced stage of thinking that corresponds to Piaget's for- ‘mat operation period of cognitive development. Both hemispheres contribute to abstract thinking, albeit in different ways, The left hemisphere is responsible for for- ‘mulating verbal concepts in a logical way. The right hemi- sphere is responsible for appreciating an integrated image of the spatial and emotional elements. As a consequence, information is analyzed, comparisons are made, hypoth- eses are formulated, and thinking using inductive and deductive reasoning takes place. These functions are as- sessed by proverb interpretation, similarities testing, and conceptual series completion. Proverb interpretation is used to evaluate divergent reasoning. The patient may need to be told what a proverb is:a saying with a broader meaning. Ifthe patient can interpret simple proverbs (eg., “the grass is greener on the other side” or “do not cry over spilled milk”), harder ones may then be presented (e.,, “loose lips sink ships,” “every cloud has a silver lining,” or “a rolling stone gathers no moss"). The person should be able to provide an abstract generalization and not a literal interpretation. When the patient is asked how a pair of objects are alike, he needs to be able to conceptualize the category to ‘which the two items belong, This task requires the ability to discover relations and to make use of verbal concepts. ‘Common similarities posed to patients in clinical practice, in order of difficulty, are turniplemuliflower, carjairplane, Aeskibookcase, poem/novel, and horsejapple. Conceptual se- ries completion requires the ability to reason at a high level. In this task the patient is presented with an in- complete series that requires him to add a letter or number for completion, Examples are ABCD_;14710_; AZBY CX D_ (1). There are no specific anatomic correlates for these igher cognitive functions. Their representation is dif- fuse, and lesions located in several parts of the brain, including cortical and subcortical structures, can cause these functions to be impaired. Bilateral cortical disease is the disorder most likely to produce these deficits. Owing to its effect on verbal reasoning, dominant hemisphere dysfunction frequently causes high-order operations to 2 be disrupted. Also documented has been the disruptive effect of nonfrontal unilateral lesions on these functions. In addition, damage to either the occipital or pari- etotemporal brain areas leads to a far greater intellectual deficit than quantitatively comparable frontal lobe dam- age. Higher Mental Control Functions ‘There are important differences between higher cognitive functions and higher mental control functions. Although both involve the manipulation of knowledge, the latter is concerned with monitoring the input, pondering potential response consequences, and initiating an ad- equate behavior. This complex process is called executive control. ‘The classic case of Phineas Gage, published in 1868, is still one of the finest illustrations of the serious behavioral consequences of executive control dysfunction, Benson (14) has proposed the existence of five higher control functions that reside in the prefrontal cortex. These are sequencing, drive, executive function, future memory, and self-awareness. Sequencing is the maintenance of the serial order of information, its organization into salient sets, and its subsequent integration with previously learned data. The lateral aspects of the prefrontal cortex are crucial for the acceptance and maintenance of data in a prescribed order. Drive is the function that stimulates responses made through basal and posterior systems. The cingulate gyrus and the supplementary motor area are implicated in the initiation and maintenance of both mo- tor and mental activity. Executive function includes an- ticipation, the planning of responses, response selection, and finally, monitoring of the actual response. This func- tion operates as a supramodal control over sequencing and drive, as well as over the basic basal and posterior functions. Future memory represents the preplanning of potential future actions or the anticipated memory of al- ready formulated plans, Self-awareness is the ability of the mental system to monitor itself and can be considered the highest mental control function. Defects such as men- tal shallowness, inability to plan ahead, impaired self- concern, and unconcern about the effect of personal behavior on future relationships are frequently described as sequelae of frontal lobe damage.Chapter 1 The Mental Status Examination Testing for primitive reflexes can provide initial sug- gestive information regarding the status of higher mental control functions. The snout reflex is elicited by tapping the mouth gently with a reflex hammer and observing for puckering of the lips. The palmomental reflex is obtained ‘when the palm of the hand is scratched briskly across its center, which elicits a contraction of the mentalis muscle on the same side of the chin. The grasp reflex is elicited by stroking the patient's toward fingers while the patient is distracted, causing the patients fingers to flex. There is some doubt that such reflexes are of pathologic signifi- cance, and it seems as though they lack semiologic value in more than half of healthy people. Landau (16) reviewed the literature on these reflexes and concluded that these reflexes indicate the existence of widespread cerebral dys- function. However, their semiologic value increases if two or more are found together and they are intense and unilateral ‘The impairment of executive functions leads to the de- velopment of disorders affecting the initiation and orga- nization of actions, such as imitation behavior, utilization behavior, motor impersistence, motor perseveration, dis- inhibited behaviors, and abulia. Autonomy in the performance of individual actions is lost after frontal lobe damage; affected patients become depencient on their environment (17). In patients showing imitation behavior, the patient imitates the gestures and behavior of the examiner, even if asked to stop. Unlike echopraxia, which is an automatic phenomenon, imita- tion behavior is deliberate and willful. It can be tested by the examiner flexing his wrist up and down and asking, “What is this called” The patient will respond by flexing, his wrist up and down, Utilization behavior is an extreme state of depending, ‘on the environment for the regulation of behavior and consists of the pseudovoluntary use of objects present in the patient's surroundings. For example, seeing a glass of water will induce the patient to drink. This can be tested for by showing a pen to the patient and asking “What is this called?” The affected patient will respond by taking the pen from the examiner. ‘Motor impersistence is the incapacity to sustain activi- ties the patient is capable of beginning, This dysfunction is present if the patient is found to be unable to keep his eyelids closed, his tongue protruded, his mouth open, or his gaze to one side. Frequently, but not always, impersistence is related to right hemisphere damage. A related symptom is the involuntary resistance to, for ex- ample, an examiner's attempts to move a limb, a symp- tom named paratonia or Gegenalter Motor perseveration is the repetition of previous ac- tions or its elements. Luria’s tasks are well known as bedside maneuvers to detect this dysfunction. Examples Of these maneuvers are to form a ring and a fist with one hand in sequence, to alternate from making a fist to plac- ing the fingers at the edge of the hand then to the palm, and to change from an outstretched left fist and right palm to an outstretched right fist and left palm. In con- frontation naming, old elements recur when the task is changed; for example, a patient affected by motor per- severation correctly names the first object shown but continues to repeat this name when other objects are presented. Reciprocal action tests also elicit a switching difficulty. For example, the patient is asked to point with ‘one finger when the examiner points with two, and vice versa, or to tap once when the examiner taps two, and vice versa, Affected patients are unable to do this. Finally, a writing sample frequently elicits the phenomenon, The patient will repeat the same word or letters indicating perseveration. It is common to elicit examples of disinhibited behav- ior during history-taking, The lack of the capacity for ‘onganized action renders the patient a prisoner of his impulses. This functional defect may be explored using a go and no-go task. This may involve asking the patient to squeeze the examiner's hand when he says “red” and to relax his hold when he says “green.” Affected patients are unable to do this. The abulic patient, on the other hand, shows a loss of spontaneity, reduced initiative, monosyl- labic speech, and poor disposition to engage in any task. Other Cortical Functions Higher cognitive functions are exquisitely integrated with motor function. A process called mofor planning occurs ‘between the time of perceptual recognition and the per- formance of a motor action and involves the perceptual judgment of the relevant situation, the formulation of a rotor plan, and the smooth execution of a sequence of motor programs. Apraxia is the inability to carry out pur- 13Part I Diagnostic Assessment poseful movements on command in the absence of sen- sory, motor, or language dysfunction. The most frequent type of apraxia, known as ideomotor apraxia, is the inability to perform in response to a verbal command an act that could easily be carried out spontaneously by a normal person. Motor, sensory, or language disorders need to be ruled out in such patients. One of three anatomic sites is damaged in patients with ideomotor apraxia: 1) the white ‘matter beneath the left supramarginal gyrus, 2) the motor association cortex of the dominant hemisphere, or 3) the anterior corpus callosum or interhemispheric pathways connecting left and right motor association areas. In other words, normal praxis requires the integrity of the system that links the posterior language areas, the dominant (usually ipsilateral) motor association area, and the ho- mologous motor association areas of the opposite hemi- sphere with their connecting pathways. Testing for ideomotor apraxia includes asking the patient to show how to salute, comb his hair, lip a coin, brush his teeth, hammer a nail, drink through a straw, or blow out a lit match (14), ‘The inability to maintain a sequence of movements de- spite a demonstrated ability to carry out each component has been named ideational apravia. For example, if such a patient is presented with a pipe, tobacco, pouch, and matches and asked to fill the pipe, light it, and smoke it, he will inevitably leave out one of the steps, even though he has shown he is able to comprehend the task and to execute each one of the steps individually. This disorder is seen in the setting of diffuse cortical disease. Some investigators have suggested the culprit lesion is located in the parietal lobe, but others point out that an impair- ment of sequential motor activities is considered a pre- frontal disturbance. Such patients are examined by giving them some simple task to perform that consists of several steps, such as folding a letter, placing it into an envelope, sealing it, and sticking a stamp on it. ‘The same exquisite integration that exists between higher cognitive and motor functions also exists between higher cognitive functions and sensation. A simple sensa- tion provides the raw data for mental life, but an inereas- ingly complex process must take place before these elementary sensations become constituents of thought. This process consists of four steps: 1) reception, or the neural activities that take place starting with the primary “ receptor and ending with the primary cortical sensory areas; 2) discrimination, or the comparison of sensory stimuli received through the modality-specific reception channels; 3) unimodal association, or the comparison of the discriminated stimuli with previously processed stimuli stored in memory; and 4) heteromodal associa- tion, or the correlation of a percept with information from other sensory and motor modalities. At this point a sen- sory stimulus is fully appreciated and consciously recog- nized (14). An abnormality in this higher-level sensory process is known as a disturbance of gnosis. Nonverbal auditory agnosia is the inability to perceive nonverbal sounds and usually results from structural damage to the primary auditory cortex of the right tempo- ral lobe and to the pathways feeding sensory information to it. The affected patient knows that a sound has been made but cannot distinguish between, for instance, clap- ping, a bell sounding, or a dog barking. Verbal auditory agnosia, or pure word deafness, is a total inability to comprehend verbal language, although the ability to rec- ognize nonverbal sounds is intact, The lesion causing the disorder is located in the posterior language area, usually bilaterally. Pain asymbolia is considered a higher-level somesthetic disturbance and is characterized by a decreased response to deep pain, This disorder has been noted after partial severance of the connections between the thalamus and the primary cortical sensory areas. Astereognosis is the inability, despite intact basic sensory function, to identify objects placed in one's hand on the basis of tactile sen- sation alone. This deficit usually indicates the existence of a lesion in the contralateral anterior parietal lobe. Agraphesthesia is the inability, despite intact basic sen- sory function, to identify through tactile stimulation num- bers traced on the palm of the hand and indicates dysfunction of the contralateral parietal lobe. Visual agnosia is the inability to recognize objects or pictures of objects presented visually, but in the absence of aphasia and despite intact visual acuity, In appercep- tive visual agnosia the patient has difficulty forming vi- sual percepts and uses other sensory cues to accomplish recognition. Bilateral damage in the visual association cortex has been identified as the cause of this disorder. In associative visual agnosia the patient is unable to name the object despite normal recognition. In this case, visual(Chapter 1 The Mental Status Examination percepts are intact but the patient cannot carry out higher- Jevel associations. Bilateral lesions involving the inferior temporal occipi- tal junction and subjacent white matter or damage to the left occipital lobe and posterior corpus callosum can cause this type of agnosia Prosopagnosia is the inability to recognize familiar faces. Bilateral occipitotemporal lesions are responsible for causing this disorder. Topographagnosia is the inabil- ity to orient oneself in an abstract spatial representation such as a map. The culprit lesion is considered to be located at the dorsal convexity of the right parietal lobe. Rating Scales Neuropsychiatric rating scales are useful for quantifying, diverse aspects of a patient's behavior. Despite the fact that many of the abnormal behaviors treated by neu- ropsychiatrists consist of subjective constructions, rating, scales provide a means to measure these constructs. The development of these scales usually involves the per- formance of careful studies that provide statistical evi- dence of the validity and reliability of the scale. The usefulness of these measurements cannot be overempha- sized. They complement clinical judgment when it is necessary to establish the severity of symptoms or dys- functions and to evaluate the effectiveness of treatment ‘and when itis important to obtain reliable data for com- parison. Sometimes itis necessary to establish the patient's glo- bal impairment; at other times it is important to assess, more specific aspects of dysfunction, The Mini-Mental, State examination is unparalleled in its ability to briefly and objectively evaluate a patient with a suspected cogni- tive impairment (2). A shortcoming of this instrument, however, is its lack of sensitivity, that is, the high rate of false-negative results associated with its use. The Glasgow Coma scale, described by Teasdale and Jennett in 1974 (4), can quantify the comatose state and can be used longitudinally to obtain important prognostic infor- mation. On a 15-point scale this instrument documents the degree of eye opening, verbal responsiveness, and ‘motor response to stimuli. In the same vein, Trzepacz.et al (18) presented a 10-item clinician-rated symptom-rating scale for delirium=the Delirium Rating scale, They ob- tained evidence that this instrument was able to differen- tiate delirious patients from a control sample of demented and schizophrenic patients, while considerable overlap ‘was noted for the Mini~Mental State examination ‘The Galveston Orientation and Amnesia testis a practi- cal scale that assesses the status of cognition after brain injury and is useful for documenting the recovery of ‘memory in patients suffering from posttraumatic amnesia (19), Apathy is a common neuropsychiatric symptom and is defined as a lack of motivation not attributable to a decreased level of consciousness, cognitive impairment, or emotional distress. Marin et al (20) provided evidence of the reliability and validity of the Apathy Evaluation scale in measuring the apathy construct. Royall et al (21) developed the Executive Interview, a 25-item, 15-minute interview that involves a standardized clinical encounter in which the behavioral sequelae of executive dyscontrol can be observed. It is common knowledge that an exami- nation of executive function is frequently overlooked dur- ing routine mental status examinations, and this scale can provide valuable information in patients suspected of having these disturbances, ‘The examiner can also use scales to evaluate more cir- cumscribed symptoms. One such symptom is pathologic crying or laughing (without feelings of sadness or happi- ness), sometimes also referred to as emotional incontinence, emotional lability, or pseudobulbar affect. Robinson et al (22) developed the Pathological Laughter and Crying scale to evaluate this disorder and provided evidence of its reli- ability and validity in patients who have suffered a stroke. This is a 16-item interview-rated instrument that quantifies various aspects of laughter and crying, includ- ing the relation of the episodes to extemal events, the duration of episodes, the degree of voluntary control over the laughter or crying, their inappropriateness in relation to events, and the degree of resultant distress. They also provided evidence showing that the scale is useful for evaluating the outcome of treatment, Another common neuropsychiatric symptom that is amenable to objective ‘quantification is aggression. Yudofsky et al (23) devel- coped the Overt Aggression scale for this purpose and provided evidence of its reliability and validity. This scale is an operationalized instrument that can be used to docu- ment the aggressive episodes and is useful for treatment outcome studies. 15Part | Diagnostic Assessment Finally, some rating scales that are commonly used in psychiatry to examine depressed patients can also be helpful in the evaluation of patients with neuro- psychiatric conditions. The Hamilton Rating scale (24) and the Beck Depression Inventory (25) are the best known of these scales. The latter instrument sometimes is not suitable for neuropsychiatric patients, however, because of its emphasis on intact cognitive functions. References 1, Strub RL, Black FW. The Mental Status Examination in newrol- ‘gy, 3rd ed. Philadelphia: Davis, 1993. 2, Folstein MF, Folstein SE, McHugh PR. Mini-Mental State: practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-198. 3, Kirshner HS. Behavioral neurology: a practical approach. New York: Churchill Livingstone, 1986 4, Teasdale G, Jennett B. Assessment of coma and impaired consciousness: a practical scale. Lancet 1974;2:81-84, 5, Berrfos GE. Stupor: a conceptual history. Psychol Med 1981113677688, 6. Bleuler E. Textbook of psychiatry. 1916 [Translation by Brill ‘AA. New York: Macmillan, 1951] 7, Rogers D. Motor disorder in psychiatry. Towards a neurological psychiatry. New York: Wiley, 1992 8, Hicks LH, Birren JE. Aging, brain damage and psychomotor slowing, Psychol Bull 1970;74:377-396, 9, Fisher CM. Abulia minor vs. agitated behavior. Clin Neurosurg 1983;319-31 10, Mogenson GJ, Jones DL, Yim CY. From motivation to action: functional interface between the limbic system and the motor system. Progr Neurobiol 1980;1469-97. 11. Posner Ml, Boies SJ. Components of attention. Peychol Rev 1971,78:391-408. 12, Ross ED, Harney JH, de LaCoste-Utamsing C. How the brain integrates affective and propositional language into a unified 16 15. 16. v7. 18, 18. 21 brain function: hypothesis based on clinicopathological cor- relations. Arch Neurol 1981,38:745-748 Signoret JL. Memory and amnesias. In: Mesulam MM, ed Principles of behavioral neurology. Philadelphia: Davis, 1985: 169-192, Benson DF. The neurology of thinking. New York: Oxford Uni- versity Press, 1994, Trzepacz PT, Baker RW. The psychiatric mental status ‘examination. New York: Oxford University Press, 1998. Landau WM. Reflex dementia: disinhibited primitive think: ing. Neurology 1989;39:133-137. Lhermitte F, Pillon B, Serdaru M. Human autonomy and the frontal lobes. I: Imitation and utilization behavior: a neuropsychological study of 75 patients. Av Neurol 1986; 1926-334, ‘Trzepacz PT, Baker RW, Greenhouse J. A symptom rating scale for delirium. Psychiatry Res 1988;23:89-97. Levin HS, O'Donnell VM, Grossman RG. The Galveston Orientation and Amnesia test: a practical scale to assess cognition after head injury. J Nero Ment Dis 1979;167:673— 684. Marin RS, Biedrzycki RC, Firinciogullari 8. Reliability and validity of the Apathy Evaluation scale. Psychiatry Res 1991/38:143-162. Royall DR, Mahurin RK, Gray KF, Bedside assessment of executive dyscontrol: the Executive Interview (EXIT). J Am Geriatr Soc 1992;40'1221~-1226. Robinson RG, Parikh RM, Lipsey JR, et al. Pathological laughing and crying following stroke: validation of a mea- surement scale and a double blind treatment study, Am J Psychiatry 1993:150:286-293, ‘Yudovsky $C, Silver JM, Jackson W, etal. The Overt Aggres- sion scale forthe objective rating of verbal and physical ag- gression. Am J Psychiatry 1986;143:35-38. Hamilton M.A rating scale for depression. Neurol Neurosurg Psychiatry 1960;38:745-748, Beck AT, Ward CH, Mendelson M, et al. An inventory for measuring depression. Arch Gen Psychiat 1961,4:561— 571,Chapter 2 The Neurologic Examination Xabier Beristain (One problem residents and specialists other than neurolo- gists have in common is that they find an understanding of the origin of neurologic disorders something remote and mysterious and the neurologic examination compli- cated and difficult. They complain that it is difficult to remember what to do, they are not sure what they are looking for, and many times they do not know the way in Which to describe their findings. All this has caused many physicians to assume that all neurologic problems are the exclusive province of the neurologist or neurosurgeon, and as a result, they make litte effort to diagnose nervous, disease. However, this perception is quite erroneous, be- cause neurologic disorders are commonly encountered in primary care, as well as in psychiatric patients and as a part of multisystemic disorders. It is true that the diagno- sis of some neurologic disorders requires vast experience and skill in the practice of neurology, but this does not justify a neglect of the neurologic examination as a part of the general evaluation of a patient. Furthermore, a neurologic examination is a necessity when examining neuropsychiatric patients because it can orient the clini- cian to the nature of the underlying neurologic disorder and help him understand the cognitive and behavioral changes affecting their patients, The Neurologic History As it is for most specialties, the history-taking is fre- quently the most important part of the neurologic exami nation because it often holds the key to diagnosis, A skillfully taken history, with a careful and thorough analysis of the chief complaints and of the course of the illness, can indicate the probable diagnosis, even before any physical or laboratory examination has been done. Recording a patient's history is a lengthy task, and be- cause of time limitations, itis often necessary to perform a second or even a third interview to clarify the nature of the patient's problem. These second or third interviews can supply additional information or may give further insight into information already given. There are several important aspects to the history- taking process: 1. Identifying information: This includes the patient's name, sex, age, race, handedness, date and place of birth, nationality, residence, marital status, religion, and reason for and source of referral 2. The presenting complaint and history of the pres- cent illness: The nature of the chief complaint, or complaints, and their duration should be recorded carefully, However, often patients have difficulty ex- plaining the nature of their main complaint. For this reason, the examiner must be sure he has understood what the problem is and what the patient means when he says, for instance, that he is suffering from dizzi- ness. Does this mean he has vertigo or is suffering seizures or syncopal episodes? Or that he is referring to 2 sensation of unsteadiness? In these cases, letting the patient talk about the main problem for several min- utes without interruption can shed light on the nature of the specific problem. At other times, however, the examiner must guide the patient’s description by ask- ing questions as “When did all this start?” or asking other questions to draw out important, relevant infor- ‘mation and prevent misunderstandings. At the end of the interview, the ansivers to all these questions need to have been made clear. 3. The past medical history: The status of the patient's ‘general health before the onset of the present illness is recorded on, as is his history of illnesses, operations, accidents, or injuries. All of this information is impor- tant because it can often give a clue to the identity of the present complaint. However, the examiner must be cautious about giving too much credence to the diag- noses patients may give their own medical and psychi- wvPart I Diagnostic Assessment atric problems (e.g.,a patient may say he has theuma- toid arthritis when he actually has osteoarthritis). 4. Family history: This is important in those patients ‘with conditions that point to the existence of a genetic disorder. Questions about the medical history of other relatives are particularly indicated in patients suffering from headaches, epilepsy, hyperkinesia, ataxia, cerebellar disorders, neuropathy, or muscular dystrophies. Head and Neck Examination ‘The examination of the head and neck focuses on the vasculature supplying the brain; the cervical spine, in- cluding signs of meningeal involvement; and the cranial nerves. Vascular Examination ‘The main purpose of the vascular examination is to detect signs of disease in the aortocranial vessels that could cause stroke, especially in the carotid arteries. This is done at the bedside through the palpation and auscultation of the neck vessels Carotid Palpation and Auscultation After explaining the purpose of the examination to the patient, the examiner should place one or two fingers gently on first one of the carotid arteries and then on the other one. Both carotids should never be palpated at the same time. Once he identifies the pulse, he should next assess the pulse along the course of the vessel. After this the examiner should place the bell or the diaphragm of the stethoscope on the artery to listen for bruits. If any ate found, he should determine their frequency (pitch), duration, amplitude, and origin. (The bell is better for hearing low frequencies and the dia- phragm for hearing high-pitched sounds.) The examiner should also determine the section of the artery where the bruit is heard and the changes in the pitch and amplitude of the bruit along it. High-pitched bruits are more likely to stem from a tight stenosis. However, because heart mur- ‘murs may sometimes radiate to the carotid artery, the examiner should make sure that this is not what he is hearing. 18 Subclavian and Vertebral Arteries ‘To examine the subclavian arteries, the examiner palpates the brachial or radial pulses in both arms atthe same time, looking for asymmetries. Ifthe pulse in one of the arms is noted to be weaker and delayed, this is probably the result ofa proximal stenosis ofthe subclavian artery. Next he should auscultate the supraclavicular fossae in search of bruits related to subclavian stenosis. Usually there are zo symptoms of brainstem dysfunction, even in the case of a subclavian stenosis, because of the collateral blood supply that forms. However, there may be such symp- toms in some cases of severe subclavian stenosis as 2 result of the reversal ofthe direction ofthe blood flow in the vertebral artery to supply the metabolic needs of the arm. This phenomenon is known as the subclavian steal syndrome. Iti also mandatory that the blood pressure be ‘measured in both arms. Cervieal Spine Before starting any maneuver, the examiner should determine whether the patient is holding his neck in an unnatural posture. If the posture is abnormal, the usual cause is muscle spasm. After this he should palpate the muscles of the spinal region to search for any tenderness, spasm, or abnormal consistency and also assess the flexion, extension, and lateral flexion of the neck. It is important to always remember not to perform, any manipulation if there is a possibility of cervical instability It is also important to search for possible foraminal compression resulting from a possible discal herniation. This is done with the patient's neck hyperextended and laterally flexed toward the extremity the pain or neurologic symptoms are radiating to and applying pres- sure on the top of the patient's head, An aggravation of symptoms is considered a positive result, Similarly, trac- tion of the neck can be used to test for discal herniation, ‘but in this case relief of the pain is considered a positive result. Meningeal irritation causes anterior flexion to be limited, and this results from a spasm of the posterior muscles of the neck. It is usually associated with pain during spontaneous motion. The degree of resistance is variable, ranging froma slight resistance to an inability to flex the neck. To examine such a patient, the examinerChapter 2 The Neurologic Examination Figure 2.1. Tests for meningeal iritation. (A) Flexion of the knees as the patient tries to flex the neck (Brudzinski’s sign). (B, C) Two different ways to elicit Kernig’s sign. should place his hand gently behind the patient's head and try to flex it, at the same time paying attention to the position of the patient's knees. If curing the attempt to flex the neck the patient flexes his knees, this is consi ered a sign of meningeal irritation, a sign known as Brudzinski's sign (Fig. 2.1A). This occurs because the meninges are stretched during flexion of the neck, caus- ing a discomfort that is relieved by flexion of the knees. Another way to identify meningeal irritation is to extend the patient’s knee with the thigh flexed at the hip. This, ‘will elicit resistance and pain in the patient with meningeal irritation, a sign known as Kernig’s sign (Fig, 2.1B). Another way to elicit Kernig’s sign is by straight-leg raising (Fig. 2.10). Cranial Nerve Examination ‘The cranial nerves comprise 12 pairs of nerves, and, ex- cept for two of them, the olfactory and optic nerves, they either arise from or arrive at the brainstem (Table 2.1). Examination of these nerves is one of the most important parts of the neurologic examination because it can yield important information useful for the localization of a neu- rologic lesion; it may also indicate the presence of a dif fuse process such as vasculitis or a generalized infection. The accurate localization of lesions affecting cranial nerves requires some skill in the performance of the neu- b a Table 2.1, Cranial Nerves and Their Related Functions Ceanial Nerve No. Name Function 1 Olfactory Smell B Optic Vision IW Qculomotor Eye movements, pupillary size changes IV Trochlear ye movernont downward and Vo Trigeminal Sensation of the face, mastication, corneal reflex VI Abducens [Abduction of the eye vil Facial Facial expression, taste Vil Acoustic Auditory acuity, balance, head-eye coordination IX Glossopharyngeal Sensation of posterior third of tongue, pharynx; motor controt for stylopharyngeus muscle X Vagus ‘Movement of palate, pharynx, larynx; autonomic contol Xt Spinal Shrugging of the shoulders, turning and flexion of the neck Xt Hypoglossal Movement ofthe tongue rologic examination and a knowledge of the anatomy of the brainstem and the nerves that arise from it. Cranial nerve abnormalities may arise from specific lesions in the nerves, from a lesion in the nucleus ot a lesion in the 19Part I Diagnostic Assessment pathways communicating with the brainstem nuclei, or as a generalized problem of nerves or muscles. As a result, these lesions can be classified as infranuiclear, nuclear, or supranuclear, depending on the location of the lesion pro- ducing the deficit. Olfactory Nerve Examinations of the olfactory nerve are rarely done in clinical practice, but when done, each nostril must be tested separately, using any aromatic, nonirritative sub- stance. Agents often used include peppermint, camphor, coffee, and rosewater. This test should be done when a patient complains of a loss of the sense of smell or taste or when a frontal lobe abnormality is suspected. If the pa- tient is able to identify smells appropriately, the olfactory nerve is considered normal. Ifthe patient cannot recog- nize the scents of the agents offered but can recognize the scent of ammonia, anosmia is diagnosed. Unilateral brain lesions do not ordinarily cause a loss of the sense of smell unless the olfactory tracts are damaged. Optic Nerve Examination of the visual system can reveal many impor- tant clues to the diagnosis of neurologic diseases because of the localizing value of visual deficits. Such examina- tions include assessments of visual acuity, the visual fields, the fundi, and the pupillary light reflex, Visual Acuity Visual acuity is an expression of the function of the ‘macula and its neural connections. Although visual acu- ity does not need to be tested routinely, to do so, each eye should be tested separately for distant and near vision using a Snellen test chart, which is placed 20 feet (6 meters) from the patient. The number on the right side of each line indicates the distance (in feet) at which a person with normal vision could read the line. Acuity is rendered as a fraction, such as 20/20, meaning that the patient is able to read at 20 feet what a person with normal vision could read at 20 feet; a score of 20/30 indicates that the patients able to read at 20 feet whata person with normal vision could read at 30 feet, and so forth, Itis essential to test a patient's visual acuity while he is wearing corrective lenses, if any. 20 Visual Fields ‘The visual fields are that portion of the space in which objects are visible during fixation in one direction. Each eye has its own field, overlapping to a large extent the field of the other eye. The visual fields are divided verti- cally through the point of fixation into the temporal and nasal fields. They are also divided into the central and peripheral fields. The central field constitutes the area immediately surrounding the point of fixation and in- cludes the zone of maximal acuity. The peripheral field consists of the remaining field of vision To assess for major field defects, the examiner has the patient look with both eyes at his eyes. Then, placing his hands approximately 50cm apart on either side of the patient's head and 30cm above his eye level, he tells the patient to indicate which index finger he is moving to the right, left, ot in both directions, The examiner then repeats the maneuver with the hands 30m below the patient's eye level. To assess the peripheral visual defect in a more refined way, each eye can be tested individually using a white- headed pin. This involves having the patient cover his left eye and look at the examiner's left eye, who then brings the pin into the patient's line of fixation from the periph- ery. The patient tells the examiner when he first sees the pin, The stimulus should be moved in an arc into the field of vision. This is done for each of the four quadrants, all the while making sure that the patient does not move his hhead or his eyes. The same testis repeated on the right eye. Table 2.2. Types of Visual Field Defects 1. Hemianopia: This isa visual defect that affects half ofthe visual field, either the nasal or temporal it refers to the area ‘of space that is seen and not the impaired portion of the retina or visual pathway. Hemianopia is usually homony- ‘mous, meaning thatthe field defect affects the same area in both eyes (Fig 2.2) In heteronymous hemianopia the nasal or temporal fields on both sides are afected. It can also be an altitudinal defect, affecting the upper or lower field of vision 2. Quadrantanopia: This is similar to hemianopia, except that in this case a superior or inferior quadrant is affected, either in the ight or let visual fel. 3. Scotoma: This is an area of impaired vision within the visual fieldChapter 2. The Neurologic Examination If the patient does not cooperate properly during these confrontation tests, then starting from behind, the inter- viewer can bring his face into view from each side with- out emitting any sound or other clues to his presence. If this elicits a glance toward the examiner, this indicates, perception in that field. The visual threat test is used in patients with a low level of consciousness. This involves, making some quick motion such as a quick thrust of the hand toward the patient's eye, which may evoke a blink reflex when the intact field is stimulated, This response must be interpreted with caution, however, because sometimes thrusting the hand produces an air flow that stimulates the sensory terminals instead of the visual pathway. The types of visual field defects are listed in Table 22 and depicted in Figure 2 Oculomotor, Trochlear, and Abducens Nerves The oculomotor, trochlear, and abducens nerves inner- vate the extraocular muscles and are examined together (Table 2.3). These muscles are coordinated precisely and smoothly and in such a way that they are able to produce conjugate gaze. When assessing ocular movements, the examiner should first look at the resting position of the patient's eyes to see if they move together full and equally inall directions or have a tendency to look in one direc tion, He also looks for abnormal movements such as rhythmic oscillations and jerks. Eye Movements The examiner should start the formal examination of eye movements by asking the patient to look at the tip of a Temporal (Mase Nasal 0 Temporal x, f \ / Figure 22. Visual field defects resulting from lesions at different points in the visual pathway. (A) Complete blindness in the let eve. (8) The usual effect is a left junction scotoma in association with a right quadrantanopia. The latter results from interruption of the right retinal nasal fibers that project into the base ofthe left optic nerve, (C) Bitemporal hemianopia, (D) Right homonymous hemianopia,(E, F) Right superior and inferior quadrant hemianopia. (G) Right homonymous hemianopia. (Reproduced by permis- sion of Adams RD, Victor M. Principles of Neurology, Sth ed. New York: McGraw-Hill, 1995.) 21art I Diagnostic Assessment Table 2.3. Muscles Supplied by the Third, Fourth, and Sixth Cranial Nerves Cranial Newe Muscles Supplied ‘Medial rectus Superior rectus Inferior rectus Inferior oblique Levator palpebrae Superior oblique External rectus 1 (oculomotor IV (rochlear VI (abducens) Right eye Tl I VI m1 vit Vv Figure 23. Representation of the eye movements and the nerves involved, showing the influence of different nerves in the control of eye movement. The third cranial nerve is responsible for stimulating elevation of the eye, both when looking to either side and in the midposition. It is also responsible for stimulating adduction of the eye and looking, downward as the eye is turned outward. The sixth cranial nerve stimulates abduction and the fourth cranial nerve, inward rotation and downward looking. [pen or his finger, which is held 50cm away, and to follow it as he moves it about. (These are called pursuit move- ments.) The examiner should move the pen or his finger so that the patient has to look up and down from the center, ‘up and down at the extreme of lateral gaze, and side to side; this checks the function of different muscles in- volved in eye movements (Fig, 2.3). During the examina- tion the examiner should also look at the patient's eyes to see if they move together and smoothly over the complete range of motion. The patient should be told to let the ‘examiner know if he sees double when the finger or pen is in any particular position. If the patient complains of double vision, the examiner should then establish which eyeiis affected, that is, which one has the weak or affected 2 muscle, To do this the patient should be asked to close one of his eyes to see if the double vision disappears. Ifit does, it the next step is to establish which is the affected eye and involved muscle. The rules pertaining to diplopia are given in Table 2.4. The speed of saccadic eye movements is checked by asking the patient to look to the right, to the left, and up and down as fast as he is able to, The ability of the patient to converge both eyes is assessed by having him look at the tip of a pen held 50 em away and bringing it toward his face. Other ocular movements to be taken into account are nystagmus, which is a slow drift of the eyes in one direc- tion with a fast correction in the opposite direction. The direction of the nystagmus is defined according to its fast component (eg, upbeat, downbeat). During the examina- tion, the patient is asked to follow the pen with his eyes while the examiner looks for the appearance of nystag- ‘mus, Jerky movements of the eyes can sometimes be seen in extreme positions of lateral gaze, but these are not nystagmus. If this happens, the examination should be Table 2. Rules of Diplopia 1. Double vision is maximal inthe direction of gaze of the muscle affected, 2. The outer image is the false one. 3. A false image arises in the affected eye. ‘Table 2.5. Explanation for Some Forms of Nystagmus Optokinetic nystagmus: Tis is a chythmic jerk nystagmus that ‘occurs while a person is watching a moving object (eg., looking ‘out a window on a moving train). Ifthe optokinetic nystagmus i present, the patient is not blind. (Ths is a useful finding for ‘diagnosing malingering and psychogenic blindness.) Pendular nystagmus: This isa binocular nystagmus occurting in tone plane and is related to a variety of conditions in which central vision is lost (e.g, albinism) Convergence nystagmus: Ths isa rhythmic abduction of both ‘eyes followed by a quick adduction. This kind of nystagmus is ‘observed in patients with lesions ofthe upper midbrain. ‘Ocular bobbing: Ths isa fast jerk ofthe eyes downward followed by a slow drift upward. It is usually due to pontine lesions.‘Chapter 2 The Neurologic Examination repeated so that no extreme position of gaze is elicited (Table 25). Lesions of Individual Ocular Nerves A complete third-nerve lesion causes ptosis on the ipsilat- eral side and a slight drooping of the eyelid on the con- tralateral side. There is also difficulty in moving the eye inward, upward, and downward. Midriasis (a wide pupil) occurs if the parasympathetic fibers, located peripherally in the third nerve, are affected as well. The size of the pupil in a patient with third-nerve palsy helps in making the distinction between a compressive third-nerve palsy (a midriatic pupil) and a metabolic third-nerve palsy (normal-sized pupil). The latter palsy has been given the name metabolic because it is frequently. seen in patients with diabetes mellitus, which is re sponsible for producing an ischemic lesion of the third ‘A fourth-nerve lesion renders a patient unable to look inward and downward at the same time, producing ver 2 @® IV Oo Or "-® Oo tical diplopia. Commonly these patients complain of difficulty reading or going downstairs. They may also be observed to tilt their heads, a finding known as Bielschowsky/s sign. This is done to compensate for their inability to rotate their eyes inward and downward. AA lesion of the sixth nerve is the easiest to diagnose because it only supplies the external rectus muscle, which produces a pure outward horizontal movement. How- ever, the finding of a dysfunction of this nerve is of little value asa localizing sign because it may result from many causes, such as elevated intracranial pressure (Fig. 24. Pupils When examining a patient's pupils, the examiner should pay attention to their size, symmetry, and shape, remem- bering that elderly people tend to have small pupils. A bright light should be used to examine the pupils and only one eye illuminated at a time. As the light is shone into the patient's eye, the examiner should note whether this eye constricts (direct photomotor reflex) and also Dilated pupil, ptosis and lateral deviation of the eye. The right eye does not move downward when the eyes are tumed to the left in case of paresis of the right IV nerve Inability to look outwards due to right abducens palsy Figure 24. Examples of disconjugate gaze. Figure shows the positon of the eyes in the setting of different nerve lesions. Arrows indicate the direction in which the patient is attempting to move both his eyes. 23Part I Diagnostic Assessment whether the contralateral eye does so (consensual light reflex). The examiner should make sure that the patient is ooking into the distance and not into the light, because the pupils tend to dilate at short distances, The same procedure should then be repeated for the other eye, After this the examiner should swing the light repeat- edly from one eye to the other at 1-second intervals and observe the pupillary response. The consensual and direct reflexes should be equal in normal people. Asymmetries between these reflexes may stem from a lesion of the pupillary reflex arch. If there is damage to the afferent limb of the arch, the response to the light will be found to be defective or absent in both eyes when the light is shone into the affected eye, but both eyes will respond to the light when the contralateral (unaffected) eye is illu- minated. (E.g.: A section of the optic nerve in one eye ‘cannot affect the consensual reflex in this same eye.) Ifthe efferent limb of the reflex arch is damaged, the affected eye will respond poorly to the light, both when the light is shone in it and in the other eye. However, illumina- ting the eye with a damaged efferent limb elicits a Basal Normal DD ®D ® ii @D @® T normal consensual light reflex in the opposite (un- affected) eye. When the light is swung rapidly from one eye to the other, the eye that showed a consensual reflex may dilate ‘when illuminated directly. This means that the afferent limb of the arch is not able to maintain the same degree of pupillary constriction as it does during consensual con- striction, indicating the existence of a relative afferent pupillary defect; this is sometimes called the Marcus Gunn pupil (Fig. 2.5). Another pupillary defect is Adie’s pupil, which is due to ciliary ganglia degeneration. In this set- ting the reaction to light is slow; even minutes may go by before there is a change in pupillary size. The affected pupil responds promptly to common miotic drugs, how- ever, but is unusually sensitive to low concentrations of pilocarpine (0.1%) that almost do not affect normal eyes. ‘This phenomenon is indicative of a denervation supersen- sitivity. Another pupillary disorder is seen in Horner's syndrome and consists of a combination of ptosis, enophthalmos, and miosis (see Fig. 2.5). It is due to a lesion of the sympathetic fibers and can arise at different Marcus-Gunn pupil DOD ®D ® T @ @® Y Horner's syndrome Figure 25. Pupillary response in normal and pathologic conditions. In the normal response, both the direct and consensual reflex are the same when each eye is illuminated. In the setting of a Marcus Gunn pupil, the left eye is unable to maintain the same degree ‘of pupilloconstriction as the consensual response when the right eye is illuminated. A Marcus Gunn pupil indicates a lesion in the afferent pupillary response to light. Therefore, more pupillary constriction occurs when the affected pupil is consensually stimulated, not directly stimulated. Horner's syndrome isan interruption of the sympathetic nerve supply to the eye with a resulting triad of ptosis, miosis, and enophthalmos. 4Chapter 2 The Neurologic Examination levels, including the hypothalamus, pons, upper cervical cord, and superior cervical ganglitum, as well as along the carotid artery. A classic pupillary disorder seen some- times in patients with neurosyphilis or diabetes is the Argyll Robertson pupil. In these patients there is no re- sponse to light but still a response to accommodation. Trigeminal Nerve ‘The trigeminal nerve is a nerve that conveys both sensory and motor stimuli. The motor portion innervates the muscles involved in mastication, mainly the masseter, temporalis, and pterigoid muscles. The sensory supply ‘comes through the three divisions of the trigeminal nerve: the ophthalmic, maxillary, and mandibular branches. The motor portion is tested by asking the patient to clench his teeth. As he does so the examiner palpates the bbulk of the masseter and temporalis muscles. The patient is then asked to open his mouth against resistance with the examiner's middle and index finger on the chin, With the patient's mouth slightly open the examiner then strikes the lower jaw with the reflex hammer to see if this elicits the jaw jerk. The jay jerk isa normal reflex that can be exaggerated in upper motor neuron disease. The sensory portion is examined division by division and includes light touch, pinprick, and temperature test- ing. (This is discussed in greater detail in the description, of the general examination of the sensory system.) The corneal reflex arch consists of an afferent limb that goes through the trigeminal nerve and an efferent limb that comes through the facial nerve, The comeal reflex is, tested by approaching each eye laterally and touching the cornea with a wisp of cotton, The normal response is a bilateral blink, (The examiner should look for asymme- tries.) The sclera should not be touched because this will stimulate the conjunctiva. The eye should also not be approached frontally because then the eye will close in response to a visual stimulus and not in response to stimulation of the cornea. Facial Nerve The facial nerve supplies the muscles of facial expression and the stapedius muscle, mediates taste for the anterior two thirds of the tongue, and provides the parasympa- thetic supply to the lacrimal gland and the sublingual and, submaxillary glands. The examiner should pay attention to the patient's face during the interview and the examination. The finding of sned palpebral fissure and flattened nasolabial fold may indicate the existence of a facial palsy. To evaluate the function of this nerve, the patient should be asked to wrinkle his forehead, look up toward the ceiling, bare his teeth, whistle, and screw up facial asymmetry with a wi his eyes and resist attempts to open them with the examiner's fingers. It is important to distinguish between central and peripheral palsy. In central palsy there is weakness of the lower face on one side but the upper part of the face is spared because the nerve supply for the upper face comes from both cerebral hemispheres. In peripheral palsy the weakness affects the upper and lower face on one side. When the lesion is peripheral, Bell's phenomenon can be elicited (j.e., upon attempted closure of the lids, both eyes roll upward, but the one on the paralyzed side remains visible). If the nerve of the stapedius muscle is involved, there is hyperacusis (painful sensitivity to loud sounds). Ifthe geniculate gan- glion is involved, lacrimation may be reduced. Taste is rarely checked in the usual clinical setting but can be done using a cotton-tipped applicator dipped in a salt, sugar, or bitter solution. Each of the hemitongues should be tested, both the anterior one third and the posterior two thirds. Acoustic Nerve ‘The acoustic nerve consists of an acoustic and a vestibular division. When testing the acoustic division, one ear should be tested ata time. This is done by placing a watch close to the patient’s ear and asking, him if he can hear it. Instead of a watch the examiner can rub the tips of his thumb and index finger together. If this simple test re- veals an asymmetry, he should then use a 516-Hz. tuning fork to perform Rinne’s and Weber's tests (Table 2.6). Rinne's Test ‘To perform Rinne's test the examiner places the vibrating fork on each of the mastoid processes and tells the patient to let him know when he is no longer able to hear it through bone conduction. As soon as this occurs, the fork should be removed from the mastoid and placed in front of the ear IF the patient is then still able to hear it, this is a positive result and indicates preserved air conduction, 3Part I Diagnostic Assessment Table 2.6. Interpretation of the Results of Rinne's and Weber's Tests Result Conclusion Rinne’s Test’ Weber’s Tes? Conductive deafness Negative Positive (deaf ear) Sensory deafness Positive Positive (good ear) Normal Positive Negative » Positive, aie better than bone conduction; negative, air worse than bone conduction, " Positive, lateralization; negative, no lateralization, Weber's Test To perform Weber's test the vibrating fork is positioned ‘on the vertex of the patient’s head and the patient asked whether he hears the sound better in one ear than in the other. It should not lateralize in normal people but should be heard in the middle of the head. The sound is louder in the affected ear of a patient with an air conduc- tion problem. To test the vestibular division the examiner should first note the way the patient walks and his stance and whether there is nystagmus. After this the patient should be examined while supine and with the head elevated 30 degrees to keep the lateral semicircular canals vertical After this cold water (30°C) is instilled into the outer ear canal with a syringe. As this is done the examiner should observe the patient for the nystagmus that should appear after several seconds. If the function is normal, the fast component of the nystagmus will beat away from the instilled ear (ie, “avoids the cold”). The same test can be done using warm water (40°C), only in this case the nys- tagmus will normally beat toward the instilled ear (i.e, “seeks for warmth”). If there is a reduced response to cold and warmth, this is a pathognomonic sign of canal paresis. The normal responses in the unconscious patient, however, are reversed: tonic movement of the eyes toward the cold water and tonic movement away from the warm water. Barany’s Maneuver Barany’s maneuver is a test designed to investigate posi- tional vertigo. This is done with the patient sitting on a bed in such a way that when he is placed supine his head 26 will extend beyond the edge of the bed. With the patient in the sitting position, the examiner should turn the patient's head to one side and have him look to this side. ‘Then, the examiner should rapidly lower the patient down onto the bed while supporting his head and looking for nystagmus. If this elicits vertigo and nystagmus, the result is positive, though itis important to remember that there isa delay between the performance of the maneuver and the appearance of vertigo or nystagmus in the setting of benign positional vertigo. In addition, if the maneuver is repeated several times, the response will tend to lessen and disappear in this form of vertigo. Turning Test To perform the turning test the patient is told to close his eyes and extend both arms straight out in front, then to step in place several times. If there is a lesion, the patient will tun gradually toward the side on which the lesion is located. Glossopharyngeal and Vagus Nerves The glossopharyngeal nerve conducts gustatory stimuli from the posterior one third of the tongue and the pharynx, supplies sensation to the middle ear, and provides an autonomic supply to the salivary glands. The vagus nerve supplies sensation to the tympanic mem- ‘brane, the external auditory canal, and external ear and furnishes a motor supply to the muscles of the palate, pharynx, and larynx. It also conducts autonomic afferent fibers from the carotid baroreceptors and a parasympa- thetic supply to and from the thorax and abdomen. When testing both nerves, the examiner should pay attention to the position of the uvula to see if itis central ‘or deviated. To do this the patient is asked to say “Al.” In normal people it should move upward and not deviate. If it deviates to one side, this means that the contralateral vagus is impaired (peripherally and centrally). If it stays, in position, the paresis is bilateral. ‘To investigate the gag reflex the examiner touches the pharyngeal wall behind the pillars of the fauces and notes the resulting position of the uvula. The response should be similar to that elicited when a person says “Ah.” Ifthe uvula goes up when the patient says “Ah” but not when the pillars are touched, this means that the glossopharyn- geal nerve is hypoactive or paretic.Chapter 2 The Neurologic Examination Table 2.7. Patters of Muscle Weakness Pattern Manifestations Upper motor neuron Weakness ‘pyramidal syndrome) Hypertony Hyperelexia Babinski’ sign Lower motor neuron Weakness Hypotony Fasciculations Atvophy Hyporeflexia No Babinski’ sign Muscle disease Wasting Impaired oF absent reflexes Hypotonia Neuromuscular junction Fatigability Normal or diminished tone Normal reflexes ‘The patient is also asked to cough. A sudden onset to the cough is a normal response; a gradual onset ("bovine cough") is not. The latter is typical of vocal cord palsy and indicates that the vagus nerve is hypoactive, Hypoglossal Nerve The hypoglossal nerve is an exclusively motor nerve that supplies the intrinsic muscles of the tongue. Each hypoglossal nerve innervates the corresponding half of the tongue. To investigate its function the examiner should check the patient’s tongue while itis at rest in the ‘mouth, looking for fasciculations and atrophy of the hemitongues that indicate a deficit of the inferior motor ‘The patient should than be told to stick out his tongue to sce if there is any deviation of the tip. If there is a deficit of one of the hypoglossal nerves, the tip of the tongue will deviate toward the affected side, just the ‘opposite of what happens in the settings of facial palsy or a deviated uvula. ‘The examiner next asks the patient to protrude the tongue into the cheek and tests the power of this by push- ing against it with his fingers, comparing the responses on the right and left sides. Spinal Nerve This spinal nerve innervates the trapezius and sterno- cleidomastoid muscles. Each cerebral hemisphere inner- vates the ipsilateral stemocleidomastoid muscle and the contralateral trapezius muscle. To test this nerve, the pa- tient is asked to shrug his shoulders while the examiner applies downward pressure on them. The patient is then asked to turn his head while the examiner applies re- sistance on one side of his forehead, taking care that this a turning motion and not one causing lateral flexion. As the examiner does this he watches the opposite sternocleidomastoid muscle and palpates its bulk. If the trapezius and sternocleidomastoid muscles on the same side are both weak, the lesion is located peripherally. If the epsilateral sternocleidomastoid and contralateral tra- pezius muscles are noted to be weak, the lesion is on the ‘upper motor neuron on the ipsilateral side. The Motor System Examination of the motor system is performed to deter- mine both the severity of the weakness (partial: paresis; total: plegia) and its pattern, the latter being the more important of the two because ofits ability to give informa- tion about the location of the underlying disorder. There are four patterns of muscle weakness (Table 2.7). The severity of weakness can be graded according to a scale of from 0 to 5, with normal strength being 5 and absence of contraction being 0 (Table 2.8). An examination of the motor system involves assessing, strength in different muscular groups, including an examination of muscle tone and the reflexes. Before Table 2.8. Gradation of Strength Grade Description ° ‘Absence of any muscular contraction 1 Contraction but without being able to flex or extend the joint 2 Contraction with movement of joint but only with ‘gravity eliminated 3 Able to move against gravity but not ater applying resistance 4 Able to move against resistance but less than roxmally 5 Normal strength 27Part I Diagnostic Assessment performing any formal examination, however, the ‘examiner should note the position of the patient to see if there is any abnormal positioning of the limbs or dimin- ished spontaneous motility. He should also look for signs of wasting and for fasciculations (spontaneous contrac- tions of groups of muscle fibers visible upon inspection of the muscles; this can be elicited by tapping the muscles). It addition, he should compare the size of the thumbs; a smaller hand could be related to a cerebral disorder during childhood, but this can also give a clue to hand dominance, Strength To test strength the examiner should assess each of the four limbs, going from the proximal to the distal aspect. ‘At the same time he should compare the findings on the right and left sides and grade strength from 0 to 5, as just described. If weakness is found in a limb, a comparison between the proximal and distal strength in that limb can provide some indication of the cause. (As a general rule, proximal weakness is associated with myopathies and distal weakness with neuropathies.) Upper Limb ‘The examination of the upper limb should start with ask- ing the patient to extend his hands straight out in front of him with his hands extended and fingers spread apart. Table 2.9. Nerve Supply of the Upper Lim Muscles The patient should then be told to close his eyes and hold this position. With the patient in this position, the exam- {ner should look to see if one or both arms drift and the hand flexes, as happens in the setting of upper limb weak- ness, Patients with weakness will also show bending at the elbow and pronation of the forearm, Malingerers and patients with “functional” deficits may show a drift in which the stiff arm is gradually lowered. After this the patient should be asked to abduct his shoulder against resistance (the first 30 degrees of abduction depends on the supraspinous muscle and the remainder on the del- toid). Then, with his arm supinated the patient is asked to flex his elbow against resistance applied to the wrist, which involves the use of the biceps brachii. After this the patient is asked to extend the elbow, which requires the use of the triceps brachii. The examiner then fixes the ppatient’s hand and asks him to keep his fingers straight as the examiner applies pressure against them; this requires the use of the extensor digitorum. Next the examiner closes his fingers around the patient's fingers and tries to extend them as the patient resists this; this makes use of the flexor digitorum, The second finger is abducted to test the strength of the first dorsal interosseous muscle. Ab- duction of the thumb is tested by completely supinating the patient’s arm with the palm flat and trying to pull the thumb backward toward the patient's nose. To evaluate the anterior serratus muscle the examiner has the patient Muscle Nerve Nerve Root Function Deltoid Axillary cs Shoulder abduction Biceps brachii Musculocutaneous 3,6 Elbow flexion ‘Triceps brachii Radial 6, 7, C8 Elbow extension Extensor digitorum Posterior interosseous 7,8 Finger extension Flexor digitorum supericialis Median, ulnar ce Finger flexion ‘and profundus First dorsal interosseous Ulnar n [Abduction of second finger Second palmar interosseous Ulnar u Adduction of second finger ‘Abductor pollicis brevis Median 1 “Thumb abduction Serratus anterior Long thoracic 5, €6, C7 Fate scapula to chest Rhomboids Dorsal nerve of scapula C4, 5 Lift medial margin of scapula Supraspinatus Suprascapular cs Shoulder extension Infraspinatus Suprascapular C5, C6 External arm rotation Brachioradialis Radial ro Flexor digitorum profundus (third Ulnar ce Flexion of the distal phalanx of and fourth fingers) third and fourth fingers 28Chapter 2. The Neurologic Examination push against a wall with one arm at a time and looks to see if there is any “winging” of the scapula. The rhom- boids are evaluated by having the patient place his hands on his hips and then try to bring his elbows backward as the examiner tries to hold them in place. Examination of, the infraspinous muscle requires that the patient flex both arms at the elbows and place his arms against his side, then try to rotate the elbows outward against resistance. (See Table 2.9 for a summary of the nerves that supply the upper limbs, together with the muscles and nerve roots involved and the resulting function.) Lower Limb The examination of a lower limb starts with having the patient flex his leg at the hip against resistance while the knee is flexed; this tests the strength of the iliopsoas muscle. With the patient supine and his legs straight, the patient is then asked to push his heel downward with the ‘examiner’s hand placed beneath the buttock on that side. This tests the gluteus maximus. Next flexion of the knee, which tests the hamstring muscles, and extension against resistance, which tests the quadriceps, is done. The exam- iner then places his hand on the sole of the patient's foot, land asks him to make a plantar flexion, which involves the use of the gastrocnemius muscle. After that, the ‘examiner places his hand on the upper surface of the patient's foot and the patient is asked to cock his ankle back; this tests the anterior tibial muscle. The patient is Table 2.10. Nerve Supply of the Lower Limb Muscles ‘muscle Newe Root ltiopsoas Lumbosacral plexus LT, L2 luteus maximus Inferior gluteal 1s, si Gluteus medius and minimus Superior gluteal L415 Adductors Obturator GB Quadriceps Femoral Bu Hamstring Sciatic Ls, si Tibialis anterior Deep perone ua ts Gastrocnemius Posterior tibial st flexion Tibialis posterior Tibial Ws Extensor digitorum longus Deep peroneal b Extensor digitorum brevis Deep peroneal 5, s1 Peroneus longus Superficial peroneal U5, SI Peroneus brevis Superficial peroneal, St then asked to extend his big toe against resistance, which tests the extensor digitorum longus, and next the rest of the toes, which tests the extensor digitorum brevis, Additional examinations include an assessment of ab- duction of the hips, to test the strength of the gluteus ‘medius and minimus, and of their adduction, to assess the adductors. Inversion of the foot is tested by asking the patient, who is lying supine, to turn his foot inward. This assesses the tibialis posterior. Eversion is tested by having the patient turn his foot outward. This assesses the peroneus longus and brevis muscles. (See Table 2.10 for a summary of the nerves that supply the lower limbs, together with the muscles and nerve roots involved and, the resulting function.) Tendon Reflexes ‘The tendon reflexes are elicited when the stretch receptors are stimulated, and this is done by striking the particular tendon with the reflex hammer; the muscle then contracts in response to this stimulus. The method of scoring these reflexes is shown in Table 2.11. The proper way to use the reflex hammer is to hold it by the end of the handle, with the wrist loose, then letting it swing down to percuss the tendon Tendon Reflexes in the Arms Following isa description of the way in which to elicit the tendon reflexes in the arms, The nerves and the nerve Function Hip flexion Hip extension Hip abduction Hip adductors Knee extension Knee flexion Foot dorsiflexion Foot plantar Foot inversion Big toe extension Toe extension Foot eversion Foot eversion 29Part I Diagnostic Assessment Table 2.11. Scoring of Tendon Reflexes Score Finding 0 ‘Absent +f Obtainable with reinforcement + Norma) a Increased 3+ Clonus roots involved in these reflexes are summarized in Table 2.12 and the types of response and the associated neuro- logic disorder are summarized in Table 2.13. The biceps reflex is elicited by flexing the elbow ap- proximately 100 degrees and placing it on the abdomen of the supine patient. The examiner then places his index finger on the biceps tendon and swings the hammer onto the finger. The normal response is flexion and pronation of the forearm, The triceps reflex is elicited with the pa- tient in a similar position and involves striking the triceps tendon directly with the hammer. The normal response is extension of the arm. To elicit the supinator reflex the ‘examiner strikes the distal portion of the radial bone with the patient's arm supinated. The normal response is el- bow flexion Tendon Reflexes in the Legs To elicit the knee reflex the examiner places his arm under the knee of the patient, who is lying supine and relaxed, then strikes the tendon below the patella. A normal response is extension of the knee, The ankle reflex is tested by positioning the patient supine, then turning his foot inward 90 degrees with the knee partially flexed and Table 2.13. Common Reflex Responses Seen in Clinica! Practice the medial malleolus facing the ceiling. As the examiner applies some pressure on the tip of the sole (to stretch the tendon), he strikes the Achilles tendon directly. The nor- ‘mal response is a plantar flexion. Another way to elicit this reflex is with the patient kneeling on a chair or bed, with the feet hanging over the side. Gee also Tables 2.12 and 2.13.) Not infrequently it is difficult to elicit reflexes. One of the most common reasons for this is a lack of proper relaxation, This can be overcome by distracting the pa- tient with casual conversation. If the reflex is still not obtainable, the patient should perform a reinforcement maneuver. This involves having the patient clasp the end of the right or left hand with the other hand and pull in opposite directions. If it is the upper limb reflexes that are being tested the patient should be told to clench his teeth. To demonstrate the presence of clonus at the ankle, the examiner places his hand on the sole and dorsiflexes the foot briskly. If there is clonus, this will produce a series of rhythmic contractions of the gastroc- nemius. To determine whether there is clonus at the knee, with the patient supine and the leg straight, the Table 2.12. Nerve and Roots Involved in Tendon Reflexes Reflex Response. Increased reflex or clonus ‘Absent reflex Reduced reflex Widening of the reflexogenic area Inverted reflex (the reflex under study is absent but there is spread toa lower level) Pendular reflex (the limb continues to swing for several beats) Reflex Newe Root Ankle Tibial 51, 2 Biceps Musculocutancous C5, 6 Triceps Radial o Supinator Radial ce, G Knee Fomorat Bus Abnormality UMN lesion Peripheral neuropathy, nerve, root Peripheral neuropathy, muscular, cerebellar Lack of reflex indicates level ofthe lesion and an UMN disease lesion below; indicates a spinal location Cerebellar lesion UMN = upper motor neuron, 30Chapter 2 The Neurologic Examination Figure 26. The method for eliciting Babinsk's sign. With the tend of the handle of the reflex hammer a ine is drawn starting at the heel and going along the external border of the sole, then across to the head of the first metatarsal bone, examiner takes ahold of the patella and briskly brings it downward. Babinski’s Sign The Babinski’s reflex is elicited by taking the tip of the handle of the reflex hammer or another blunt pointed object and using it to draw a line across the patient's foot pad, starting near the outer aspect of the heel and. following along the extemal border then across to the head of the first metatarsal bone, forming a sort of ques tion mark (Fig. 2.6). The response in normal people is flexion of the toes. However, patients with a pyramidal syndrome will slowly extend the big toe and spread the other toes apart (Babinski’s sign). Sensory System ‘The testing of the sensory system during a regular neuro- logic examination assesses several primary sensory mo- dalities as well as some discriminating ones requiring an appreciation of not only the simple stimuli involved but also of more complex qualities. Among the first group are the following senses: vibration, pain, temperature, joint position sense, and touch. The second group in- cludes graphesthesia, stereognosis, and two-point dis- crimination. Vibration, joint position sense, and light touch are conducted through the posterior column and depend on the large fibers for transmission. Pain and temperature sensation are conducted through the spino- thalamic tracts and depend on the small fibers for trans- mission. Pain Pain is examined using a sharp disposable object such as a safety pin (hypodermic needles are not recommenced because they ate too sharp and could easily injure the patient; if they are used, they should be made blunt first. Using the safety pin the examiner asks the patient to distinguish between sharp and dull sensations, the latter élicited using the opposite end of the safety pin. (This is done to make sure that what the patient is reporting is a pain sensation and not touch.) The patient keeps his eyes closed as this is done. The next step is to establish the threshold of pain by determining the minimal stimulus the patient is able to distinguish. It is important to com- pare the threshold in different areas, between the right and left sides, and distally versus proximally. The exam- ner should avoid repeating the stimuli too close in time or space to prevent spatial and temporal summation phe- nomena from occurring. Temperature The temperature sense is not routinely measured. As a screening method, however, the examiner can touch the patient in the same place on opposite sides of the body with the tuning fork and ask him if he feels any difference. To formally test temperature, the examiner takes two test tubes and fills one with warm water and the other with cold water, then applies the tubes in random order against the patient's skin, The patient is asked to distin- guish between the warm and cold test tubes. Vibration Examination of the vibration sense is quick and easy to do in the alert patient and involves placing the 128-Hz tun- ing fork on a bony prominence and asking the patient if he feels a buzzing sensation while the fork is vibrating, To make sure that the patient is feeling the vibration and not just the touch of the fork, the fork needs to be placed randomly in both a vibrating and nonvibrating mode. The 31Part 1 Diagnostic Assessment Figure 2.7 The way to hold the finger or toe when testing joint position sense. fork is placed in a distal to proximal pattern, starting at the tips of the toes, then following with the metatarsal phalangeal joint, the medial malleolus, the tibial tuberos- ity, and the anterior superior iliac spine. To test the upper limbs the examiner starts at the fingertips and proceeds to each interphalangeal joint, then the metacarpal pha- langeal joint, the wrist, the elbow, and the shoulder. As with the testing of the other senses, the examiner com- pares the responses on the right and left sides as well as the proximal and distal findings. Joint Position Sense To test joint position sense the examiner takes the patient’s big toe, holding the distal phalanx at the side (Fig. 2.7) so that he does not place pressure on the upper and lower surface of the toe that could interfere with the patient's ability to distinguish spatial location using proprioception, The patient is told to close his eyes and indicate whether the joint is down or up. The examiner should start with large movements in either direction and gradually reduce the angle moved. If the joint position sense is not normal for the big toe, the other toes should then be tested. Another way to test joint position sense is to ask the patient to indicate the smallest movement he is able to distinguish (eg., “Now I am going to move your finger 32 and you have to tell me when you start feeling it moving”). Light Touch Light touch is tested using cotton wool, which should be dabbed onto the patient's skin in such a way that the stimulus is consistent. It should not be dragged across the skin because a moving stimulus will create the spatial summation of impulses, increasing the chance of percep- tion. The patient’s eyes should be closed as the examiner touches his skin in random places. The patient should be told to say “yes” each time he feels the contact of the cotton wool. Some patients with a psychogenic loss of sensation may say “no” when touched in the affected area. If a single touch is not felt, the examiner should augment the stimulus by using a stroking motion. Lesions that produce light touch deficits are commonly associated with a variety of positive symptoms (e.g., numbness). Graphesthesia Graphesthesia is defined as the ability to recognize num- bers and letters drawn on the skin, To explore this com- plex sensory modality, the examiner draws a letter or number on the patient's palm with his finger and the patient is asked to identify the character. This can also be done on the patient's arms, legs, or face. Stereognosis Stereognosis is the ability to identify objects through touch. Usually keys and coins, and the like, are used. The patient should be told to touch the object very well and to ‘name the object without looking at it. The way the patient ‘manipulates objects can give extra information about his proprioception and motor coordination. ‘Two-point Discrimination ‘The two-point discrimination test assesses the ability of the patient to distinguish between two closely situated stimuli, A compasslike device is used to test this function. ‘The sensitivity depends on the region of the skin tested. It is maximal on the tongue and poor on the trunk, espe- ally the back. When testing the palm, the examiner should start with 2 to 3 cm and reduce the distance until the patient reports only one stimulus (a bit less that 1m). On the fingertips, two points separated by few millime-