1.B. For questions 1–7 see V&A pp.291–292.

2.E.

3.G.

4.C.

5.F.

6.D.

7.A.

8.E. V&A pp.345–346.The change from the tonic to the clonic phase results from diencenphalic inhibition of the firing cortex.

9.D. V&A p.291.

10.D. For questions 10–14 see V&A pp.31–32.

11.D.

12.B.

13.E.

14.A.

15.E. V&A p.198.Toradol, a nonsteroidal anti-inflammatory drug (NSAID), is not helpful in relieving the pain of trigeminal neuralgia.

16.B. V&A pp.258–260.Venous pulsations are absent in 10 to 15% of normal individuals.The congested capillaries are derived from the short ciliary arteries.Unilateral edema can occur with optic nerve tumors.Visual acuity is usually normal in papilledema.Answer B is correct.

17.E. V&A p.198.

19.E. V&A pp.277, 292.

20.E. V&A pp.757–758.Headache occurs in more than half the cases.Confusion, coma, and death usually result if the patient is untreated.The inflammatory exudate occurs mainly in the basal meninges and frequently invades the underlying brain by spreading via pial vessels.The cerebrospinal fluid (CSF) protein is always elevated to 100 to 200 mg/dL or higher.

21.C. V&A pp.968–969.The CSF protein is slightly increased in about 40% of patients with multiple sclerosis (MS).A concentration of >100 mg/dL is rare.If the ratio of CSF IgG/serum IgG to CSF albumin/serum albumin is more than 1.7, the diagnosis of MS is probable.This ratio is known as the IgG index.

22.D. V&A pp.1537–1540, 1542.The majority of patients with myasthenia gravis harboring a thymoma are older (50–60 years) and male.The disease is 2 to 3 times more common in women than men in patients < 40 years of age.A decrease in muscle action potential with nerve stimulation at 3 Hz (a decrementing reponse) is seen.Certain aminoglycoside antibiotics can impair transmitter release by inhibiting calcium ion fluxes at the neuromuscular junction.

23.E. V&A pp.1042–1045; Mer pp.1002–1003.Menkes’ (kinky hair) syndrome is a rare sex-linked disease characterized by severe copper deficiency.The other disorders have point mutations or deletions of mitochondrial DNA as part of their pathogenesis.

24.E. V&A pp.875–876.

25.C. V&A p.456.Discrete, bilateral lesions in the hippocampus and dorsomedial thalamus impair memory and learning out of proportion to other cognitive functions.Stereotactic lesions of the amygdala and mammillary bodies have failed to produce these symptoms.

26.C. Y p.2168.The gene (CCM1) responsible for familial cavernous malformations has been mapped to 7q11.2–q21.In addition, CCM2 (7p13–15) and CCM3 (3q25.2–27) have been identified in patients with cavernous malformations.

27.C. V&A p.287.Diabetic third nerve palsy is usually painful.

28.A. For questions 28–36 see V&A pp.1277–1282.

29.B.

30.C.

31.E.

32.D.

33.D.

34.B.

35.B.

36.A.

38.C. V&A p.352.Infantile seizures or spasms usually begin before 6 months of age and are characterized by sudden flexor or extensor spasms of the head, trunk, and limbs and an electroencephalogram (EEG) picture of bilateral high-voltage, slow-wave activity (hypsarrythmia).Lip smacking and generalized tonic-clonic activity are not features.

39.B. For questions 39–42 see V&A pp.1547–1548.These are all features of the

40.B. Eaton-Lambert syndrome.

41.B.

42.B.

43.C. Mer p.448.

44.C. For questions 44–50 see Mer p.448.

45.A.

45.C.

46.B.

47.D.

48.D.

49.B.

50.A. V&A p.1365.

51.C. V&A pp.1503–1504.Frontal balding occurs in both men and women afflicted with myotonic dystrophy.Lens opacities are found by slit lamp in 90% of patients.The inheritance is autosomal dominant, and the defective gene segregates on chromosone 19.Myotonia may precede weakness by several years.Answer C is correct.

52.A. V&A p.1218.Subacute combined deficiency of the cord occurs from failure to transfer cobalamin (vitamin B12) across the interstitial mucosa because of lack of intrinsic factor.

53.A. V&A p.1122.

54.A. V&A pp.494, 511.The lesion described in A would render the patient blind in the right half of the visual field.Visual information reaches only the right occipital lobe but cannot be transferred to Wernicke’s area across the callosum.Thus the ability to read aloud and to understand the written word is lost, but the ability to understand the spoken language, speak, write, dictate, and converse is retained.

55.C. V&A p.849.Deviation of the eyes away from the lesion occurs in brainstem syndromes, for example, the medial midpontine syndrome (occlusion of the paramedian branch of the midbasilar artery).Answers B, D, and E would cause deviation of the eyes to the left.

56.E. V&A p.357.Of the antiepileptic drugs listed, phenobarbital has the longest half-life of 96 ± 12 hours, followed by ethosuximide, 40 ± 6 hours; phenytoin 24 ± 12 hours; carbamazepine 12 ± 4 hours; and valproate 8 ± 2 hours.

59.B. and forearms in amyotrophic lateral sclerosis (ALS), diffuse hyperreflexia is

60.A. seen, with absence of sensory change.

61.D. V&A p.345.Increased glycine levels have been found in neurons in seizure foci.

62.E. V&A p.1222.Although microbiologic assay is the most accurate way to measure serum cobalamin levels, the serum level is not a measure of total body cobalamin.High serum concentrations of cobalamin metabolites (methyl-malonic acid and homocysteine) are the most reliable indicators of an intracellular cobalamin deficiency.

63.D. V&A p.1302.In radiation myelopathy, sensory changes usually precede the weakness.

64.A. V&A p.1362.

65.B. For questions 65–70 see V&A pp.1361–1362.Fibrillation potentials last from

66.A. 1 to 5 ms, may take the form of positive sharp waves, and are seen 10 to 25 days

67.B. after the death of an axon.Fasciculation potentials have three to five phases.

68.C. Both can be seen in poliomyelitis.

69.D.

70.A.

71.B. V&A p.1364.

72.A. V&A p.1537.Normal pupillary response to light and accommodation, together with extraocular and orbicularis oculi muscle weakness, is highly suggestive of myasthenia gravis.

73.E. V&A p.1433.

74.C. V&A pp.760–762.Neurologic involvement in sarcoidosis occurs in 5% of cases.A granulomatous inflammatory response most prevalent at the base of the brain is seen.Visual disturbances (due to lesions in and around the optic nerves and chiasm) and polydipsia, polyuria, somnolence, or obesity (due to involvement of the pituitary and hypothalamus) are the usual features.The facial nerve is the most common cranial nerve involved.

75.A. V&A pp.421–423.The nocturnal sleep of a narcoleptic is often reduced, but frequent naps are taken during the day; hence the total number of hours spent sleeping is similar to a normal individual.The other responses are associated with narcolepsy.

77.A. V&A p.288.Upgaze or downgaze is usually more limited than lateral gaze.These deficits are caused by an inflammatory infiltration of the inferior and medial recti, leading to contractures of these muscles.

78.A. V&A p.1455.The spasm is thought to be caused by nerve root compression and segmental demyelination, which leads to impulses conducted in one motor fiber being transmitted to neighboring fibers (ephaptic transmission).

79.A. V&A p.762.Although all of the options are seen in active neurocarcoidosis, the diagnosis is made on the basis of answer A.

80.D. For questions 80–84 see Mer pp.727, 893.The IgG antibody in patients with

81.E. Eaton-Lambert syndrome (associated with small-cell carcinoma of the lung)

82.D. reacts with presynaptic voltage-gated calcium channels.The Moersch-Woltman

83.B. syndrome is characterized by involuntary muscle rigidity and spasms, and

84.C. 60% of patients have autoantibodies to glutamic acid decarboxylase.Underlying tumors are often found.Most cases of paraneoplastic sensory neuropathy are associated with small-cell carcinoma of the lung or lymphoma, and an antinuclear antibody (anti-Hu) is found in 70% of these patients.Paraneoplastic opsoclonus in adults is associated with breast cancer and an antineuronal antibody (anti-Ri).

85.D. For questions 85–88 see V&A pp. 845–847.

86.B.

87.C.

88.A.

89.E. V&A p.79.

90.D. Mer p.446.

91.D. V&A p.1548.In this syndrome, the presynaptic vesicles are normal, antibodies to the acetylcholine receptor are not present, and the extent of receptor surface is actually increased.There is, however, a defect in the release of acetylcholine quanta from the nerve terminals.

92.C. V&A p.705.Iris harmartomas (Lisch nodules) are seen in neurofibromatosis type 1.

93.B. V&A p.485.The syndrome consists of finger agnosia, left-right confusion, acalculia, and agraphia.

94.E. V&A pp.72–73, G&G p.131.The rate-limiting step is tyrosine hydroxylase (converts L-tyrosine to L-hydroxyphenylalanine [L-dopa]).

96.D. that may release dopamine from striatal neurons.Artane is an anticholinergic

97.C. agent.Bromocriptine is an ergot derivative.Eldepryl is a monoamine oxidase B

98.B. inhibitor and slows progression of disability.Sinemet combines L-dopa

99.D. with a dopa decarboxylase inhibitor.

100.C. V&A p.500.

101.A. Mer p.624, V&A pp.1396–1398.The progressive sensorimotor polyneuropathy is generally (but not universally) thought to be metabolic in origin.

102.C. V&A p.1544.The weakness of a cholinergic crisis is unaffected by Tensilon (edrophonium).

103.C. V&A p.985.The MELAS syndrome is a mitochondrial disease associated with a maternal inheritance.

104.C. For questions 104–107 see Y 4889–4890.

105.A.

106.A.

107.B.

108.C. For questions 108–113 see V&A pp.405–407.Although most dreams occur in

109.B. rapid eye movement (REM) sleep, they can occur in non–REM (NREM) sleep.

110.A. Adult somnambulism, K complexes, and sleep spindles all occur in NREM

111.B. sleep (the latter two in stage 2).Glucose metabolism in the brain is

112.B. increased in REM and decreased in NREM sleep in comparison to the waking

113.A. state.

114.B. For questions 114–117 see V&A pp.1513–1516.Glycogen storage disease type II

115.A. results from acid maltase (alpha-1,4-glucosidase) deficiency and has three

116.A. forms: childhood (Pompe’s disease), childhood, and adult forms.Glycogen

117.D. accumulates in lysosomes throughout the body.Glycogen storage disease type V (McArdle’s disease) results from myophosphorylase deficiency.Glycogen cannot be converted to glucose-6-phosphate, and the blood lactate does not rise after ischemic exercise.Both types are autosomal recessive.Rarely, type V may be autosomal dominant.

118.B. V&A pp.1026–1030.Wilson’s disease is characterized by an increased urinary copper excretion, low serum copper levels, and low ceruloplasmin levels.The computed tomographic (CT) scan sometimes shows hypodense areas in the lenticular nuclei.

119.A. V&A p.1193.Microscopically, destruction of the medullated sheaths with relative sparing of the axis cylinders and preservation of nerve cells in the pons is seen.An inflammatory response is absent.

121.A. those with Marfan’s syndrome have a tall, thin frame and arachnodactyly.

122.A. Patients with homocystinuria (cystathione synthase deficiency) also show evidence of mental retardation and are prone to strokes.

123.D. V&A pp.486–487.

124.E. Mer p.446.

125.A. V&A p.1078.Skull films may reveal “tramline calcification” is present in the parieto-occipital cortical substance, not the vessels.

126.C. HndbkNS 541.

127.E. V&A pp.1417–1419.The autonomic nervous system is usually not involved in Charcot-Marie-Tooth disease.

128.E. V&A p.719.

129.D. V&A p.758.The protein is elevated in tuberculous meningitis; usually 100 to 200 mg/dL.

130.A. V&A pp.844–845.Ipsilateral Horner’s syndrome is seen in the posteroinferior cerebellar artery (PICA) occlusion syndrome.

131.A. V&A p.405.Delta waves are prevalent in stage 3 and 4 sleep.Desynchronization of the EEG occurs in REM sleep, and somnambulism occurs almost exclusively in stage 4 sleep.

132.B. For questions 132–141 see V&A pp.1494–1500, 1503–1504.Duchenne and

133.A. Becker muscular dystrophies are X-linked recessive disorders characterized by

134.E. the absence of the gene product dytrophin in the former and the presence of a

135.B. structurally abnormal form of the product in the latter.Weakness and pseudo-

136.C. hypertrophy of certain muscles (notably the calf) occur.The onset is later and

137.D. the course more benign in the Becker type.Myotonic dystrophy is the most

138.E. common adult form of muscular dystrophy and is characterized by an autosomal

139.D. dominant inheritance, with the defective gene localized to chromosome 19q.

140.E. Features include dystrophic changes in nonmuscular tissues (e.g., lens opacities)

141.E. and a characteristic facies.Landouzy-Dejerine dystrophy is usually transmitted by autosomal dominant inheritance, and the abnormal gene has been localized to chromosome 4.Congenital absence of a pectoral, brachioradialis, or biceps femoris muscle occasionally occurs.Characteristics of Emery-Dreifuss dystrophy, a benign X-linked dystrophy, include contractures of the elbow flexors, neck extensors, and posterior calf muscles.

142.B. V&A p.61.

143.C. V&A pp.814–817.

144.B. Y4 p.2842.

146.B. medial cord lesions, abnormalities of radially innervated C8 muscles are

147.C. seen with the former, but not with the latter.Low-amplitude action poten

148.E. tials in the deltoid and biceps are seen in upper trunk lesions.Median

149.A. sensory responses from the index and middle finger are abnormal, and motor conduction velocities of the hand muscles are normal in middle trunk lesions.Lateral cord lesions cause weakness of the muscles supplied by the musculocutaneous nerve and the lateral root of the median nerve (innervates the forearm muscles).The intrinsic hand muscles innervated by the medial root of the median nerve are spared.

150.A. V&A pp.957–958.Several studies indicate that a person migrating from a high-risk to a low-risk zone of MS before age 15 will develop a risk that is similar to the low-risk zone.If the migration takes place after age 15, the risk is similar to that of natives of the high-risk zone.

151.E. V&A p.1274.

152.D. V&A pp.506, 512–513.Transcortical motor and sensory aphasias are manifested by preserved repetition.

153.E. V&A p.423.

154.A. V&A pp.786–787.

155.E. V&A p.723.Garcin’s (hemibasal) syndrome has been reported with chondromas or chondrosarcomas of the clivus.

156.C. V&A pp.319–320.Early in Meniere’s disease deafness affects mainly the low tones and fluctuates in severity.Later in the disease high tones are affected.

157.E. V&A pp.1547–1548.Men are more often affected than women (5:1).

158.A. V&A p.1465.Type I (red) muscle fibers are richer in oxidative enzymes, poorer in glycolytic enzymes, contain more mitochondria and myoglobin, fire more tonically, have slower rates of contraction and relaxation, and are less fatiguable than type II (white) fibers.

159.B. V&A p.837.

160.E. V&A p.1397.Mononeuropathy multiplex of diabetes is classically asymmetric.In practice, however, a confluence of multiple mononeuropathies may lead to a symmetric picture.

161.C. For questions 161–165 see V&A pp.356–360.

162.B.

163.D or E.

164.A.

165.E.

167.B. V&A p.189.Cluster headaches typically are recurrent for 6 to 12 weeks in a unilateral orbital location.The male to female ratio is 4.5:1 to 6.7:1.Lacrimation, rhinorrhea, flushing of the face, and other such parasympathetic-type responses often accompany the headache.

168.B. V&A p.1281.The anticholinesterase effect of organophosphate poisoning results in increased salivation.

169.B. V&A p.1125.Decreased glutamic acid decarboxylase (hence -γ aminobutyric acid [GABA]) and choline acetyltransferase (hence acetylcholine) have been found in the striatum and lateral pallidum in Huntington’s disease.Also reported has been increased norepinephrine and somatostatin in the striatum.An excess of dopamine or an increased sensitivity of striatal dopamine receptors has been postulated in the pathogenesis of Huntington’s disease.

170.C. V&A pp.490–492.Prosopagnosia refers to the inability to identify a familiar face while retaining the ability to identify its features.

171.C. Carp p.424.

172.D. HndbkNS p.545.

173.D. V&A p.996.Deficiency of hexoaminidase A characterizes Tay-Sachs disease.

174.A. V&A pp.1110–1111.Corticospinal and corticosensory functions, visual acuity, and visual fields are relatively preserved throughout the course of Alzheimer’s disease.

175.B. V&A pp.1380–1387.Neither conventional dose nor high-dose steroids have been shown to be helpful in the treatment of Guillain-Barré syndrome.

176.D. V&A pp.294–295.The pathway from the lateral horn cells at C8 to T3 to the superior cervical ganglion constitutes the second-order neuron (pregan-glionic).Response C constitutes the first-order neuron (central), and response E constitutes the third-order neuron (postganglionic) in the sympathetic innervation of the pupil.

177.C. V&A p.775.

178.D. V&A pp.810–811.Subacute sclerosing panencephalitis (SSPE), characterized by a progressive mental decline with seizures, myoclonus, and ataxia, mainly affects children and adolescents.The lesions are found in the cerebral cortex and white matter.Eosinophilic inclusions are found in the cytoplasm and nuclei of neurons and glial cells.Elevated gamma globulin in the CSF is typical.

180.C. V&A p.967.Schilder’s disease is a demyelinating illness of children and young adults that has several features in common with chronic relapsing MS.

181.A. Moore p.1045.The cricothyroid, supplied by the external laryngeal nerve, is the only intrinsic laryngeal muscle not supplied by the recurrent laryngeal nerve.

182.A. V&A p.1206.In Korsakoff’s psychosis, retentive memory is impaired out of proportion to other cognitive functions in an otherwise alert patient.

183.E. V&A pp.1159–1161.Mental retardation is not a feature of the spinal muscular atrophy of infancy and childhood.

184.B. G&G p.457.

185.C. For questions 185–189 see V&A pp.1155–1157, 1325.Despite the muscle

186.D. weakness and atrophy seen in ALS, hyperreflexia and mild lower extremity

187.C. spasticity are characteristic.

188.A.

189.C.

190.F. For questions 190–195 see Mer pp.902–909.

191.C.

192.E.

193.D.

194.B.

195.A.

196.A. V&A pp.1206–1207.Defects in learning and memory out of proportion to other cognitive functions is a feature of Korsakoff’s psychosis, not Wernicke’s encephalopathy.

197.C. V&A pp.483–487.Parietal lobe lesions are characterized by loss of position sense, impaired ability to localize touch and pain stimuli (atopognosia), astereognosis, and impairment of two-point discrimination.Perception of pain, touch, pressure, vibratory, and thermal stimuli is relatively intact.

198.C. V&A p.269.A lesion in the right inferior occipitotemporal region sparing the optic radiation and striate cortex causes the purest form of achromatopsia.

199.C. V&A pp.298–299.Horner’s syndrome can be confirmed by the failure of the miotic pupil to dilate in response to 2 to 10% cocaine drops.If the later application of the adrenergic mydriatic hydroxyamphetamine has no effect, then the lesion localizes to the third-order neuron (p ostganglionic part).

201.C. V&A p.417.

202.D. V&A p.425.

203.B. V&A p.494.

204.C. For questions 203–208 see V&A pp.505–514.Conduction aphasia is similar

205.A. to Wernicke’s aphasia in that there is a fluent paraphasic speech with

206.F. impaired repetition.In contrast to patients with Wernicke’s aphasia, however,

207.B. those with conduction aphasia have little or no difficulty in comprehension.

208.D. The transcortical aphasias are characterized by good repetition.208.E.

209.C. For questions 209–215 see V&A pp.1482–1487.Both idiopathic polymyositis

210.D. (PM) and dermatomyositis (DM) are more common in women.About 9% of

211.B. patients with PM and 15% of those with DM have an underlying carcinoma.

212.A. Single-fiber necrosis is seen in PM, whereas a perifascicular muscle fiber

213.B. degeneration and atrophy are seen in DM.IgG, IgM, complement, and mem-

214.A. brane attack complexes are deposited in the small vessels in DM, whereas in

215.D. PM the endomysial inflammatory exudate contains a large number of T cells and few B cells.Both disorders are readily responsive to corticosteroids and other immunosuppressants.