Chapter 2 Clinical Neurology For questions 1 to 7, match the eye movement with the description.Each response may be used once, more than once, or not at all. A. convergence nystagmus B. dissociated nystagmus (internuclear ophthalmoplegia) C. downbeat nystagmus D. impairment of optokinetic nystagmus E. ocular bobbing F. seesaw nystagmus G. spasmus mutans 1. a common sign of multiple sclerosis 2. most often associated with large destructive lesions of the pons 3. seen exclusively in infants 4. associated with lesions of the cervicomedullary junction 5. associated with lesions of the parasellar region 6. associated with lesions of the parietal lobe 7. associated with lesions of the pineal region 8. Which of the following is false of seizure foci? A. Epileptic foci are slower in binding and removing acetylcholine than normal cortex. B. Firing of neurons in the focus is reflected by periodic spike discharges in the electroencephalogram (EEG). C. If unchecked, cortical excitation may spread to the subcortical nuclei. D. Neurons surrounding the focus are initially hyperpolarized and are GABAnergic. E. The change in seizure discharge from the tonic phase to the clonic phase results from inhibition from the neurons surrounding the focus. 9. An abnormal optokinetic response is more likely to be obtained by rotating the optokinetic nystagmus drum A. away from an occipital lobe lesion B. away from a parietal lobe lesion C. toward an occipital lobe lesion D. toward a parietal lobe lesion E. toward a temporal lobe lesion For questions 10 to 14, match the EEG wave with the description.Each response may be used once more than once or not at all. A. alpha B. beta C. delta D. theta E. 3-per-second spike and wave 10. 4 to 7 Hz 11. normally may be present over the temporal lobes of the elderly 12. recorded from the frontal lobes symmetrically 13. associated with absence seizures 14. attenuated or abolished with eye opening or mental activity 15. Which of the following drugs is least effective in the treatment of trigeminal neuralgia? A. baclofen B. carbamazepine C. clonazepam D. phenytoin E. ketorolac tromethamine (Toradol) 16. Which of the following is true of papilledema? A. Absence of venous pulsations is a reliable indicator of papilledema. B. Pupillary light reflexes remain normal. C. The congested capillaries derive from the central retinal vein. D. Unilateral edema of the optic disk is never seen. E. Visual acuity usually decreases. 17. Which of the following can occur in glossopharyngeal neuralgia? I. pain in the throat II. syncope III. pain in the ear IV. bradycardia A. I, II, III B. I, III C. II, IV D. IV E. all of the above 18. Features of trisomy 13 (Patau’s syndrome) include I. microcephaly II. hypertonia III. cleft lip and palate IV. dextrocardia A. I, II, III B. I, III C. II, IV D. IV E. all of the above 19. Which of the following is not a feature of Parinaud’s syndrome? A. dissociated light–near response B. lid retraction C. nystagmus retractorius D. paralysis of upgaze E. third nerve palsy 20. Which of the following is true of tuberculous meningitis? A. Headache is usually absent. B. If untreated, the clinical course is self-limite C. The inflammatory exudate is confined to the subarachnoid space D. The inflammatory exudate is found mainly at the convexities. E. The protein content of the cerebrospinal fluid (CSF) is almost always elevated 21. Which of the following CSF findings is least suggestive of acute multiple sclerosis? A. an IgG index greater than 1.7 B. increased myelin basic protein C. increased protein to 200 mg/dL D. presence of oligoclonal bands E. slight to moderate monocytic pleocytosis 22. Each of the following is true of myasthenia gravis except A. A decrementing response to peripheral nerve stimulation is typical. B. Aminoglycoside antibiotics may worsen the symptoms. C. Females are more frequently affected in the < 40 age group. D. Females predominate in the subset of patients with a thymoma. E. Ten to 15% of patients have no antibodies to the acetylcholine receptor. 23. A defect in mitochondrial DNA is found in each of the following disorders except A. Kearns-Sayre syndrome B. Leber’s hereditary optic atrophy C. Leigh’s subacute necrotizing encephalopathy D. Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke (MELAS) E. Menkes’ syndrome 24. Symptoms of spontaneous carotid artery dissection include I. dysgeusia II. eye pain III. tongue weakness IV. Horner’s syndrome A. I, II, III B. I, III C. II, IV D. IV E. all of the above 25. Memory impairment is caused by discrete bilateral lesions of which of the following structures? I. amygdala II. hippocampal formation III. mammillary bodies IV. dorsomedial nuclei of the thalamus A. I, II, III B. I, III C. II, IV D. IV E. all of the above 26. Genes responsible for cavernous malformations have been mapped to chromosomes A. 1 and 3 B. 3 and 5 C. 3 and 7 D. 4 and 5 E. 5 and 7 27. Each of the following is characteristic of a diabetic third nerve palsy except that A. it develops over a few hours B. it spares the pupil C. it is usually painless D. the lesion involves the center of the nerve E. the prognosis for recovery is good For questions 28 to 36, provide the best match of the toxicities with the description.Each response may be used once, more than once, or not at all. A. arsenic poisoning B. lead poisoning C. manganese poisoning D. mercury poisoning E. phosphorus poisoning 28. transverse white lines in the fingernails 29. black lines at the gingival margins 30. Later symptoms resemble those of Parkinson’s disease 31. treated with atropine 32. Pencillamine is the treatment of choice in the chronic form. 33. characterized by mood changes, tremors, and a cerebellar syndrome 34. treated with ethylenediaminetetraacetic acid (EDTA) and dimercaprol (BAL) 35. increased excretion of urinary coproporphyrin 36. Diagnosis can be made by the examination of hair samples. 37. Which of the following is not a characteristic of Adie’s syndrome? A. degeneration of the ciliary ganglia and postganglionic parasympathetics B. more common in women than in men C. no reaction to 0.1% pilocarpine solution D. paralysis of segments of the pupillary sphincter E. Pupil responds better to near than to light. 38. Characteristics of infantile seizures include I. lip smacking II. hypsarrythmia III. generalized tonic-clonic activity IV. myoclonic head jerks A. I, II, III B. I, III C. II, IV D. IV E. all of the above For questions 39 to 42, match the disease with the description.Each response may be used once, more than once, or not at all. A. myasthenia gravis B. myasthenic syndrome of Eaton-Lambert C. both D. neither 39. Muscles of the trunk and lower extremities are more frequently involved than the extraocular muscles. 40. Poor response to anticholinesterase drugs 41. An incrementing response (marked increase in the amplitude of the action potential with fast rates of nerve stimulation) is typical. 42. associated with antibodies to the presynaptic voltage-dependent calcium channel 43. The dorsal scapular nerve innervates the I. supraspinatus II. rhomboids III. subscapularis IV. levator scapulae A. I, II, III B. I, III C. II, IV D. IV E. all of the above For questions 44 to 50, match the peripheral nerve with the muscle it innervates.Each response may be used once, more than once, or not at all. A. axillary nerve B. dorsal scapular nerve C. subscapular nerve D. suprascapular nerve E. none of the above 44. teres major 46. subscapularis 47. levator scapulae 48. supraspinatus 49. infraspinatus 50. rhomboids 51. The motor unit potential in myopathy is of A. decreased voltage and decreased duration B. decreased voltage and increased duration C. decreased voltage and normal duration D. normal voltage and decreased duration E. normal voltage and increased duration 52. Which is true of myotonic dystrophy? A. Frontal balding occurs only in men. B. Lens abnormalities are rare C. The congenital form is inherited only from the maternal line D. The inheritance is autosomal recessive E. Weakness always predates the myotonia. 53. Subacute combined degeneration of the spinal cord is caused by a deficiency of A. cobalamin B. folic acid C. nicotinic acid D. pyridoxine E. thiamine 54. The marker linked to the Huntington gene is localized to the short arm of chromosome A. 4 B. 11 C. 17 D. 22 E. none of the above A. left geniculocalcarine tract and corpus callosum B. left geniculocalcarine tract and Wernicke’s area C. left geniculocalcarine tract, corpus callosum, and Wernicke’s area D. right geniculocalcarine tract and corpus callosum E. right geniculocalcarine tract and Wernicke’s area 56. Deviation of the eyes to the right is most likely to occur with occlusion of the A. calcarine artery bilaterally B. calcarine artery on the contralateral side C. contralateral paramedian branch of the basilar artery D. ipsilateral superior cerebellar artery E. superior division of the contralateral middle cerebral artery 57. Which of the following antiepileptic drugs has the shortest half-life? A. carbamazepine B. ethosuximide C. phenobarbital D. phenytoin E. valproate For questions 58 to 60, match the description with the disease A. amyotrophic lateral sclerosis B. syringomyelia C. both D. neither 58. weakness and atrophy of the hands 59. hypo- or areflexia 60. absence of sensory changes 61. Biochemical studies of neurons from a seizure focus have shown all of the following except A. increased levels of extracellular potassium in glial scars near seizure foci B. decreased rate of binding and removing acetylcholine in the foci C. deficiency of γ-aminobutyric acid (GABA) D. decreased glycine levels E. decreased taurine levels 62. The most reliable indicator of an intracellular cobalamin (vitamin B12) deficiency is A. low vitamin B12 on a microbiologic assay B. low vitamin B12 on a radioisotope dilution assay C. low vitamin B12 on a Schilling test D. the finding of hypersegmented polymorphonuclear neutrophil leukocytes (PMN) in bone marrow smears E. the finding of increased serum concentration of methylmalonic acid and homocysteine 63. Each of the following is true of radiation myelopathy (delayed progressive type) except A. Absence of pain is typical early in the course B. It occurs 12 to 15 months after radiation. C. Magnetic resonance imaging (MRI) shows abnormal signal intensity; decreased on T1 and increased on T2. D. Sensory changes usually develop after motor changes. E. The most severe parenchymal changes are typical of infarction. 64. Fasciculation potentials indicate A. motor nerve fiber irritability B. motor nerve fiber destruction C. motor unit denervation D. muscle atrophy E. reinnervation of muscle units For questions 65 to 70, match the description with the potential. A. fasciculation potential B. fibrillation potential C. both D. neither 65. di- or triphasic pattern 66. 5 to 15 ms in duration 67. may take the form of positive sharp waves 68. seen in poliomyelitis 69. usually develops 24 to 36 hours after the death of an axon 70. may be visible through the skin 71. What characteristics of motor unit potentials are typical soon after reinnervation? A. prolonged, high amplitude, and polyphasic B. prolonged, low amplitude, and polyphasic C. shortened, high amplitude, and polyphasic D. shortened, low amplitude, and polyphasic E. none of the above 72. Which of the following ocular findings is not seen in myasthenia gravis? A. abnormal pupillary response to accommodation B. normal pupillary response to light C. weakness of extraocular muscles D. weakness of eye closure E. weakness of eye opening 73. Risk factors for carpal tunnel syndrome include I. acromegaly II. amyloidosis III. hypothyroidism IV. pregnancy A. I, II, III B. I, III C. II, IV D. IV E. all of the above 74. Which of the following is true of neurologic findings in sarcoidosis? A. Cranial nerve VI is most frequently involved B. Sarcoidosis occurs in 25% of cases of sarcoid C. Polydipsia, polyuria, somnolence, and obesity are common features. D. The granulomatous infiltration is most prominent over the hemispheres. E. Visual disturbances are usually secondary to lesions in the occipital cortex. 75. All of the following are associated with narcolepsy except A. increased total number of hours per day spent sleeping B. cataplexy C. hypnagogic hallucinations D. sleep paralysis E. sleep patterns beginning with the rapid eye movements (REM) stage 76. Which of the following signs or symptoms occurring in a young person is the most suggestive of multiple sclerosis? A. bilateral internuclear ophthalmoplegia B. gait ataxia C. Lhermitte’s sign D. optic neuritis E. vertigo 77. The muscles most often involved in thyroid ophthalmopathy are the A. inferior, superior, and medial recti B. inferior rectus and superior oblique C. lateral and superior recti D. lateral rectus and superior oblique E. medial rectus and inferior oblique 78. Most cases of “idiopathic”hemifacial spasm are thought to result from A. ephaptic transmission B. hypersensitivity of facial muscles C. hypocalcemia D. psychiatric disorders E. recurrence of latent viral infection 79. The diagnosis of neurosarcoidosis is based on A. biopsy evidence of sarcoid granulomas in non–central nervous system (CNS) tissue and neurologic findings B. computed tomography (CT) scan showing meningeal involvement C. increased sedimentation rate and hyperglobulinemia D. increased serum levels of angiotensin-converting enzyme E. MRI findings of periventricular and white matter changes For questions 80 to 84, match the paraneoplastic syndrome with the description.Each response may be used once, more than once, or not at all. A. limbic encephalitis B. Eaton-Lambert syndrome C. Moersch-Woltman (stiff-man) syndrome D. opsoclonus-myoclonus E. sensory neuropathy 80. seen most often in children with neuroblastoma 81. anti-Hu antibodies 82. anti-Ri antibodies 83. autoantibodies to voltage-gated calcium channels 84. autoantibodies to glutamic acid decaboxylase For questions 85 to 88, match the vascular syndrome with the description.Each response may be used once, more than once, or not at all. A. basilar syndrome B. lateral medullary syndrome (vertebral or posteroinferior cerebellar artery [PICA] occlusion) C. lateral superior pontine syndrome (superior cerebellar artery occlusion) D. medial medullary occlusion E. none of the above 85. contralateral hemiparesis sparing the face, contralateral loss of position and vibration sense, ipsilateral paralysis, and atrophy of the tongue 87. ipsilateral cerebellar ataxia, contralateral loss of pain and temperature in the body, partial deafness, and nausea and vomiting 88. bilateral motor weakness in all extremities, bilateral cerebellar ataxia, and diplopia 89. The lesion in hemiballismus is localized to the contralateral A. brachium conjunctivum B. caudate nucleus C. dorsomedial nucleus of the thalamus D. substantia nigra E. subthalamic nucleus 90. The long thoracic nerve innervates the A. latissimus dorsi B. levator scapulae C. rhomboids D. serratus anterior E. teres minor 91. Which of the following is most consistent with the Eaton-Lambert syndrome? A. abnormal presynaptic vesicles B. antibodies to the acetylcholine receptor C. decreased numbers of acetylcholine receptors D. defect in release of acetylcholine quanta E. none of the above 92. The von Hippel-Lindau disease has been associated with all of the following except A. a defect on chromosome 3 B. dominant inheritance C. iris hamartomas D. pancreatic cysts E. renal cell carcinoma 93. Gerstmann’s syndrome classically involves a lesion in the A. dominant frontal lobe B. dominant parietal lobe C. dominant temporal lobe D. nondominant parietal lobe E. nondominant temporal lobe 94. Each of the following is true of dopamine pharmacology except the following: A. Homovanillic acid is a metabolite B. It is derived from phenylalanine C. It is metabolized by monoamine oxidase (MAO). D. The activation of the D2 receptor decreases the release of transmitter at synaptic terminals. E. The rate-limiting step in its synthesis is dopa decarboxylase For questions 95 to 99, match the antiparkinsonian drug with the description.Each response may be used once, more than once, or not at all. A. amantadine B. Artane (trihexyphenidyl) C. bromocriptine D. Eldepryl (selegiline) E. Sinemet (carbidopa-levidopa) 95. contains a dopa decarboxylase inhibitor 96. slows progression of the disease in its early stages 97. stimulates D2 receptors 98. Dryness of the mouth and blurred vision are some of the side effects. 99. inhibits intracerebral metabolic degradation of dopamine 100. Wernicke’s area corresponds most closely to Brodmann’s area(s) A. 17 B. 19 C. 22 D. 41 and 42 E. 44 101. Complications of diabetes generally thought to be vascular in origin include I. ophthalmoplegia II. acute mononeuropathy III. mononeuritis multiplex IV. distal sensorimotor polyneuropathy A. I, II, III B. I, III C. II, IV D. IV E. all of the above A. bradycardia B. diarrhea C. increased strength after the Tensilon test D. miosis E. sweating 103. The genetic transmission of the MELAS syndrome is A. autosomal dominant B. autosomal recessive C. maternal inheritance D. sporadic E. X-linked recessive For questions 104 to 107, match the cord syndrome with the description.Each response may be used once, more than once, or not at all. A. anterior cord syndrome B. Brown-Séquard syndrome C. central cord syndrome D. A and B E. none of the above 104. acute hyperextension 105. flexion injury 106. dissociated sensory loss 107. Among the incomplete syndromes, this has the best prognosis. For questions 108 to 113, match the description with the sleep stage A. REM sleep B. non–rapid eye movement (NREM) sleep C. both D. neither 108. dreaming 109. adult somnambulism 110. desynchronization of the EEG 111. K complexes 112. sleep spindles 113. Glucose metabolism in the brain is increased in comparison to the waking state For questions 114 to 117, match the description with the disease A. glycogen storage disease type II (acid maltase deficiency) B. glycogen storage disease type V (McArdle’s disease) C. both D. neither 114. myophosphorylase deficiency 115. Large amounts of glycogen are deposited in various organs. 116. Three clinical forms are noted 117. X-linked recessive inheritance 118. Wilson’s disease is characterized by I. high urinary copper excretion II. high serum copper III. low ceruloplasmin levels IV. hyperdensity of the globus pallidus and putamen on CT A. I, II, III B. I, III C. II, IV D. IV E. all of the above 119. Each of the following is true of central pontine myelinolysis except A. A marked inflammatory response with destruction of nerve cells in the pons is seen. B. It is associated with rapid correction of hyponatremia C. It is associated with chronic alcoholism. D. Quadriplegia, pseudobulbar palsy, and a locked in syndrome can occur. E. Some patients have no signs or symptoms referable to the pontine lesion. For questions 120 to 122, match the description with the disease A. homocystinuria B. Marfan’s syndrome C. both D. neither 120. arachnodactyly 122. brain infarcts 123. Dressing apraxia is associated with a lesion in the A. dominant frontal lobe B. dominant parietal lobe C. nondominant frontal lobe D. nondominant parietal lobe E. nondominant temporal lobe 124. The axillary nerve innervates the A. coracobrachialis B. rhomboids C. supraspinatus D. teres major E. teres minor 125. All of the following are seen in Sturge-Weber syndrome except A. calcified cortical vessels B. facial nevus contralateral to seizure activity C. hemisensory deficit contralateral to facial nevus D. meningeal venous angiomas E. tramline calcifications outlining the convolution of the parieto-occipital cortex 126. The normal sensory nerve conduction velocity in the median and ulnar nerves is approximately A. 10 meters per second (m/s) B. 25 m/s C. 50 m/s D. 100 m/s E. 150 m/s 127. Each of these statements is true of Charcot-Marie-Tooth disease except A. Autosomal dominance is the usual mode of inheritance B. Distal muscle atrophy is prominent. C. It can affect the upper extremities. D. Steroids have no effect on disease progression. E. The autonomic nervous system is usually involved 128. Cranial nerves that may be affected by a clival chordoma include I. cranial nerve XII II. cranial nerve V III. cranial nerve X IV. cranial nerve II A. I, II, III B. I, III C. II, IV D. IV E. all of the above 129. Which of the following CSF findings is least consistent with tuberculous meningitis? A. glucose of 30 mg/dL B. lymphocytic predomination after 1 week of illness C. opening pressure of 200 mm CSF D. protein of 35 mg% E. white blood cell count (WBC) of 200 cells/mm3 130. The syndrome of PICA occlusion results in all of the following except A. contralateral Horner’s syndrome B. contralateral loss of pain and temperature over the body C. ipsilateral ataxia D. ipsilateral numbness of the limbs E. ipsilateral paralysis of the palate 131. Stage 2 sleep is characterized by A. K complexes B. delta waves C. desynchronization of the EEG D. REM sleep E. somnambulism For questions 132 to 141, match the muscular dystrophy with the description.Each response may be used once, more than once, or not at all. A. Becker’s muscular dystrophy B. Duchenne’s muscular dystrophy C. Emery-Dreifuss muscular dystrophy D. Landouzy-Dejerine (fascioscapulohumeral) dystrophy E. myotonic dystrophy 132. The protein dystrophin is absent. 133. The protein dystrophin is structurally abnormal. 134. the most common adult form of muscular dystrophy 135. Prominent pseudohypertrophy of the calves is seen in Becker and in this type 136. Contractures of the elbow flexors and neck extensors occur early. 137. Abnormal gene is on chromosome 4. 138. Lens opacities are found in 90% of patients. 139. occasionally associated with congenital absence of an involved muscle 140. Masseter atrophy, ptosis, and frontal baldness are characteristic 141. Abnormal gene is on chromosome 19. 142. Monoplegia without muscular atrophy is most often secondary to a lesion in the A. brainstem B. cortex C. internal capsule D. peripheral nerve E. spinal cord 143. The transmissable agent of Creutzfeldt-Jakob disease is inactivated by I. formalin II. autoclaving at 132°C under pressure for 1 hour III. alcohol IV. immersion for 1 hour in bleach A. I, II, III B. I, III C. II, IV D. IV E. all of the above 144. The most common finding on audiography in patients with acoustic neuromas is A. flat loss B. high-frequency loss C. low tone loss D. normal audiogram E. trough-shaped loss For questions 145 to 149, match the brachial plexus lesion with the description.Each response may be used once, more than once, or not at all. A. lateral cord lesion B. lower trunk lesion C. medial cord lesion D. middle trunk lesion E. upper trunk lesion 145. Median sensory responses from the index and middle finger are low in amplitude, but motor conduction velocities of the hand muscles are normal. 147. Ulnar sensory response from the little finger is abnormal; normal responses are seen from the extensor indicis proprius. 148. Action potentials from the deltoid and biceps are of low amplitude 149. Abnormal median sensory responses and denervation are seen in the biceps and flexor carpi radialis; normal response is seen from the abductor pollicis brevis. 150. Persons migrating from a zone with high risk of multiple sclerosis (MS) to one of low risk after age 15 show a risk of developing MS that is A. equal to that of the high-risk zone B. equal to that of the low-risk zone C. intermediate between the two zones D. lower than that of the low-risk zone E. unpredictable 151. Eye findings in botulinism include I. ptosis II. strabismus III. diplopia IV. unreactive pupils A. I, II, III B. I, III C. II, IV D. IV E. all of the above 152. Repetition is least likely to be affected by a A. Broca’s aphasia B. conduction aphasia C. global aphasia D. transcortical sensory aphasia E. Wernicke’s aphasia 153. Which stage of sleep is prominent on EEG at the onset of narcoleptic sleep attacks? A. stage 1 B. stage 2 C. stage 3 D. stage 4 E. REM 154. The most common cause of viral meningitis is A. enterovirus B. human immunodeficiency virus (HIV) C. leptospirosis D. lymphocytic choriomeningitis E. mumps 155. Successive involvement of all cranial nerves on one side has been reported in A. meningitis B. sarcoidosis C. tumors of the brainstem D. tumors of the cavernous sinus E. tumors of the clivus 156. Each of the following is true of Meniere’s disease except A. Distention of the endolymphatic duct occurs. B. Hearing loss is usually unilateral. C. High tone loss occurs early in the disease D. Horizontal nystagmus occurs during an acute attack. E. Low-pitched tinnitus is typical. 157. Each of the following is true of Eaton-Lambert syndrome except A. Autonomic disturbances are seen. B. Fasciculations are not seen. C. It has been associated with carcinoma of the stomach and colon. D. Temporary increase in muscle power may occur during the first few contractions. E. Women are more frequently affected than men. 158. Type I (red) muscle fibers differ from type II (white) fibers in all of the following ways except that they A. are more fatiguable B. fire more tonically C. have a slower contraction and relaxation rates D. have more mitochondria E. have more oxidative enzymes 159. Historically, one of the treatment modalities of Parkinson’s disease was surgical ligation of the A. anterior cerebral artery B. anterior choroidal artery C. middle cerebral artery D. posterior communicating artery E. recurrent artery of Huebner 160. Which of the following is not characteristic of diabetic mononeuritis multiplex? A. Lower extremities are more commonly affected than upper extremities. B. painful neuropathy C. Proximal extremities are more commonly affected than distal extremities. D. Recovery is usual. E. symmetric neuropathy For questions 161 to 165, provide the best match of each antiepileptic drug with the seizure type Each response may be used once, more than once, or not at all. A. adrenocorticotropic hormone (ACTH) B. ethosuximide C. lorazepam D. Tegretol E. valproic acid 161. status epilepticus 162. absence seizures 163. complex partial seizures 164. infantile seizures 165. atypical petit mal syndrome of Lennox-Gastaut 166. Each of the following is true of polymyositis associated with carcinoma except A. Carcinoma affects 9% of patients with polymyositis. B. It is most commonly associated with lung and prostate cancer in men. C. It is usually painful. D. Muscle biopsies show no evidence of tumor cells. E. Proximal muscles are initially affected more than distal ones. 167. Which of the following is least suggestive of cluster headaches? A. associated with lacrimation and rhinorrhea B. bilateral location C. daily occurrence for 2 months D. male predominance E. orbital location 168. Organophosphate poisoning is characterized by all of the following except A. bronchial spasms B. dry mouth C. miosis D. sweating E. vomiting 169. One of the cerebral biochemical defects in Huntington’s disease is A. decreased dopamine B. decreased GABA C. decreased norepinephrine D. decreased somatostatin E. increased acetylcholine 170. Prosopagnosia is associated with lesions of the A. anterior corpus callosum B. bilateral anterorinferior temporal lobes C. bilateral medial temporo-occipital lobes D. occipital poles E. posterior corpus callosum 171. A lesion of the supplementary motor cortex produces A. echolalia B. palilalia C. poverty of spontaneous speech D. receptive aphasia E. no speech abnormalities 172. Lesions of the peroneal nerve produce weakness of the A. abductor hallucis and gastrocnemius B. extensor digitorum longus and brevis and abductor hallucis C. gastrocnemius and flexor hallucis longus D. tibialis anterior and extensor digitorum longus and brevis E. tibialis anterior and flexor digitorum brevis 173. Which of the following is not characteristic of Tay-Sachs disease? A. abnormal startle response B. autosomal recessive inheritance C. cherry-red spots in the retina D. deficiency of sphingomyelinase E. macrocephaly 174. Which of the following deficits is least characteristic of Alzheimer’s disease? A. corticospinal tract dysfunction B. dysnomia C. Korsakoff’s amnesic state D. personality change E. spatial disorientation 175. Each of the following is true of Guillain-Barré syndrome except A. Disturbances of autonomic function are common. B. High-dose steroids form the mainstay of therapy. C. Hypo- or areflexia is characteristic D. The mortality rate is 3%. E. The peak severity is 10 to 14 days after onset in 80% of cases. 176. The second-order neuron in the sympathetic pathway to the pupil arises from the A. ciliary ganglion to the iris B. Edinger-Westphal nucleus to the ciliary ganglion C. hypothalamus to the lateral horn cells at C8 to T3 D. lateral horn cells at C8 to T3 to the superior cervical ganglion E. superior cervical ganglion to the iris 177. The treatment of choice for toxoplasmosis is A. penicillin B. praziquantel C. pyrimethamine and sulfadiazine D. rifampin and nafcillin E. thiabendazole 178. Which of the following is true of subacute sclerosing panencephalitis (SSPE)? A. Intracytoplasmic but not intranuclear inclusions are found B. It is more common in patients <18 years of age C. Lesions are confined to the white matter. D. The EEG shows characteristic periodic 2 to 3 per second waves. E. The CSF protein is normal. 179. The treatment of choice for optic neuritis is A. intrathecal prednisolone B. intravenous methylprednisolone followed by oral prednisone C. oral prednisone only D. oral prednisone followed by intravenous methylprednisolone E. plasmapheresis 180. Schilder’s disease most closely resembles A. Duchenne muscular dytrophy B. Krabbe’s disease C. multiple sclerosis D. trisomy 13 E. tuberous sclerosis 181. The cricothyroid muscle is innervated by the A. external branch of the superior laryngeal nerve B. internal laryngeal branch of the superior laryngeal nerve C. ninth cranial nerve D. recurrent laryngeal nerve E. seventh cranial nerve 182. Korsakoff’s syndrome is best characterized by (a) A. defect in learning and loss of past memories B. global confusional state C. manic-depressive state D. paranoid ideation E. stupor or coma 183. Werdnig-Hoffmann disease is notable for all of the following except A. areflexia B. autosomal recessive inheritance C. hypotonia D. involvement of chromosome 5q E. mental retardation 184. Tricyclic antidepressants I. block norepinephrine uptake II. block oxidative deamination of monoamines III. block serotonin uptake IV. bBind to GABA receptors A. I, II, III B. I, III C. II, IV D. IV E. all of the above questions 185 to 189, match the description with the disease A. amyotrophic lateral sclerosis (ALS) B. cervical spondylosis C. both D. neither 185. lower extremity spasticity 186. hyporeflexia 187. hyperreflexia 188. absence or paucity of sensory symptoms 189. atrophy of the hand muscles For questions 190 to 195, match the vasculitis with the description.Each response may be used once, more than once, or not at all. A. Cogan’s syndrome B. polyarteritis nodosa C. systemic lupus erythematosus D. Takayasu’s syndrome E. temporal arteritis F. Wegener’s granulomatosis 190. antineutrophil cytoplasmic antibodies 191. antinuclear antibodies and malar rash 192. visual loss and claudication with chewing 193. visual loss and loss of peripheral pulses 194. mononeuritis multiplex, kidney involvement, and skin purpura 195. deafness and keratitis 196. Wernicke’s encephalopathy consists of all of the following except A. defect in retentive memory out of proportion to other cognitive functions B. gait ataxia C. gaze palsy D. mental confusion E. nystagmus 197. Which of the following is least suggestive of a parietal lobe lesion? A. astereognosis B. loss of position sense C. loss of temperature sensation D. loss of two-point discrimination E. atopognosia 198. The purest form of achromatopsia is caused by a lesion involving the A. left calcarine cortex B. left superior occipitotemporal region C. right inferior occipitotemporal region D. right occipital cortex and angular gyrus E. right superior calcarine cortex A. first-order Horner’s syndrome B. second-order Horner’s syndrome C. third-order Horner’s syndrome D. first- or second-order Horner’s syndrome E. second- or third-order Horner’s syndrome 200. Somnambulism occurs in which stage of sleep? A. stage 1 B. stage 2 C. stage 4 D. REM E. all of the above 201. The most effective treatment of enuresis is A. clonopin B. clonidine C. haloperidol (Haldol) D. imipramine (Tofranil) E. methylphenidate (Ritalin) 202. In most cases, section of the corpus callosum causes A. apraxia of both hands to command B. apraxia of the left hand to command C. apraxia of the right hand to command D. object agnosia E. no deficit For questions 203 to 208, match the aphasia with the description.Each response may be used once, more than once, or not at all. A. good comprehension, fluent speech, poor repetition B. good comprehension, nonfluent speech, good repetition C. good comprehension, nonfluent speech, poor repetition D. poor comprehension, fluent speech, good repetition E. poor comprehension, fluent speech, poor repetition F. poor comprehension, nonfluent speech, poor repetition 203. Broca’s aphasia 204. conduction aphasia 205. global aphasia 206. transcortical motor aphasia 207. transcortical sensory aphasia 208. Wernicke’s aphasia questions 209 to 215, match the description with the disease A. dermatomyositis B. polymyositis C. both D. neither 209. may be associated with carcinoma 210. Men are more frequently affected than women. 211. Necrosis and phagocytosis of individual muscle fibers are the principal changes. 212. Perifascicular muscle degeneration and atrophy are found 213. Large numbers of T cells are found in the intramuscular inflammatory exudates. 214. Immune complexes are deposited in the walls of arterioles and venules. 215. Corticosteroids have no effect on symptoms.
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