Question 1

A 25-year-old man falls while skateboarding and strikes the left side of his head against a concrete retaining wall. On physical examination only a minor scalp abrasion is present at the site of the impact, with minimal bleeding that stops in a few minutes. He is initially alert following this accident, but then became unconscious 30 minutes later. A head CT scan reveals a convex, lens-shaped area of hemorrhage centered over the left parietal region. These events are most likely to be associated with damage to which of the following parts of the intracranial vasculature?

A Bridging veins

B Cavernous sinus

C Great vein of Galen

D Inferior cerebellar artery

E Middle meningeal artery

F Ophthalmic branch of external carotid artery

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Question 2

A 70-year-old man has a 2 day history of worsening generalized headache and increasing obtundation. He now complains of stiffness in his neck. On physical examination vital signs include T 38.7 C, pulse 85/minute, respirations 23/minute, and blood pressure 130/85 mm Hg. A CBC reveals a WBC count of 16,850/microliter. Serum electrolytes include a sodium of 145 mmol/L, potassium 4.3 mmol/L, chloride 103 mmol/L, CO2 26 mmol/L, urea nitrogen 18 mg/dL, and glucose 88 mg/dL. A lumbar puncture yields cloudy cerebrospinal fluid with a glucose of 32 mg/dL, protein 146 mg/dL, and cell count of 3800 WBCs (95% PMNs and 5 % mononuclears) and 122 RBCs. He receives antibiotic therapy and improves. Which of the following long-term complications is most likely to develop from this man's current disease?

A Cerebral infarction

B Cerebellar tonsillar herniation

C Encephalitis

D Hydrocephalus

E Subdural hematoma

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Question 3

A 68-year-old woman has been placed in a nursing home by her son because she can no longer be cared for at home. She has difficulty keeping her room in order. She misplaces articles of clothing and sometimes dresses herself in an odd fashion. These problems have gotten progressively worse over the past 6 years. She has been continually wandering away from the house and getting lost in the neighborhood over the past 6 months. She took early retirement as an accountant because she was having trouble keeping her clients accounts in order. There is no history of trauma. She has no history of seizures. Which set of histopathologic findings is most typical for her underlying disease process?

A Atrophy of caudate nucleus and gliosis

B Wallerian degeneration and gliosis

C Substantia nigra depigmentation and loss of neurons

D Grouped atrophy of muscle with anterior horn cell loss

E Neurofibrillary tangles and senile neuritic plaques

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Question 4

A 73-year-old man is undergoing a radical prostatectomy for prostatic adenocarcinoma. During this procedure the anesthesiologist reports a drop in blood pressure with hypotension that persists for 30 minutes despite intervention with pressor agents. Which of the following pathologic lesions is most likely to occur in his brain as a consequence of this prolonged hypotensive episode?

A Hydranencephaly

B Linear parasagittal infarction

C Lacunar infarcts of basal ganglia

D Parietal lobe hemorrhagic infarct

E Anterior pituitary necrosis

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Question 5

A 28-year-old man undergoes induction chemotherapy for acute myelogenous leukemia. A week later he becomes severely pancytopenic, with a WBC count of 1320/microliter, Hgb 7.9 g/dL, and platelet count of 72,000/microliter. He becomes comatose a day later. A CT scan of the head reveals a right parietal hemorrhage. He dies a day later. At autopsy, he is found to have a right middle cerebral arterial thrombosis with right parietal hemorrhagic infarction. Which of the following infectious agents is most likely to cause these findings?

A Herpes simplex virus

B Toxoplasma gondii

C Human immunodeficiency virus

D Cytomegalovirus

E Aspergillus niger

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Question 6

A 29-year-old G2 P1 woman gives birth following an uncomplicated pregnancy at 29 weeks gestation to a girl infant. Her prior pregnancy resulted in a normal term birth. She continued to smoke 1 pack of cigarettes per day during the current pregnancy. Following the delivery, the baby receives surfactant therapy and does not develop respiratory distress from hyaline membrane disease. However, it is noted that the baby has a seizure during the next day of life. Which of the following conditions did this baby most likely develop?

A Intraventricular hemorrhage

B Intracerebellar hemorrhage

C Subdural hematoma

D Subgaleal hemorrhage

E Epidural hemorrhage

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Question 7

Following an episode of severe head trauma incurred in a motorcycle accident, an 18-year-old woman is noted to have decerebrate posturing. Funduscopic examination reveals marked bilateral papilledema. A CT scan of the head reveals marked diffuse cerebral edema with effacement of lateral ventricles. This edema results from an increase in sodium and water content. It is most likely to be severest in which of the following parts of the brain?

A Gray matter

B Meninges

C White matter

D Dura

E Ependyma

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Question 8

A 31-year-old woman has noted difficulty with writing for the past month. She has difficulty controlling her hand movements, and the writing is nearly illegible. A neurological examination reveals decreased strength in the right upper extremity and decreased sensation over the left lower extremity. She has no decrease in mentation, and there is no reported seizure activity. A lumbar puncture is performed, and the CSF contains elevated levels of IgG, some mononuclear cells, and oligoclonal bands on gel electrophoresis. Which of the following pathologic findings in the CNS is she most likely to have?

A Loss of pigmented neurons in the substantia nigra

B Perivascular lymphocytes with demyelinated axons in white matter

C Increased neurofibrillary tangles and neuritic plaques in the neocortex

D Periventricular lymphoid aggregates with cells marking with CD19

E Foci of multinucleated cells and macrophages in grey and white matter

F Gliosis with atrophy of caudate nuclei

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Question 9

A 23-year-old primigravida has noted minimal fetal movement, and a fetal ultrasound is performed at 17 weeks gestation. The ultrasound shows an intact fetal cranial vault, mild ventriculomegaly, intact cerebral hemispheres, a "lemon sign" (due to flattened frontal bones), a "banana sign" (due to effacement of the cisterna magna), a normal foramen magnum, and head measurements that are small for gestational age. Which of the following fetal congenital abnormalities is most likely to be present in this case?

A Meningomyelocele

B Encephalocele

C Arnold-Chiari II malformation

D Lissencephaly

E Alobar holoprosencephaly

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Question 10

A 28-year-old G3 P2 woman has had an uncomplicated pregnancy. A screening ultrasound is performed at 16 weeks gestation, and the findings prompt performance of maternal serum alpha-fetoprotein test, which is elevated. Which of the following abnormalities of the CNS is most likely to be present in this fetus?

A Holoprosencephaly

B Metachromatic leukodystrophy

C Encephalocele

D Germinal matrix hemorrhage

E Spina bifida occulta

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Question 11

A 40-year-old man has had decreased mentation with confusion for the past 6 weeks. On physical examination he exhibits incoordination and reduced movement in his right arm. MR imaging of the brain shows 0.5 to 1.5 cm lesions in cerebral hemispheres in white matter and at the grey-white junction that suggest demyelination. A stereotatic biopsy is performed, and immunohistochemical staining of the tissue reveals JC papovavirus in oligodendrocytes. Which of the following abnormal laboratory test findings is this patient most likely to have?

A Oligoclonal bands in CSF

B Hemoglobin AIC of 9.8%

C CD4 lymphocyte count of 90/microliter

D Serum sodium of 109 mmol/L

E HDL cholesterol of 12 mg/dL

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Question 12

A 50-year-old African-American man has had headaches for the past month. On physical examination his blood pressure is 182/108 mm Hg. He refuses to take any medications. He is admitted to the hospital after suddenly losing consciousness 2 months later. When he is aroused, he cannot speak and he cannot move his right arm or his right leg. Which of the following intracranial pathologic abnormalities is most likely to be present?

A Middle cerebral artery embolus

B Subfrontal meningioma

C Cerebral venous thrombosis

D Central pontine myelinolysis

E Hemorrhage into the putamen

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Question 13

A clinical study is performed to assess neurologic abnormalities associated with chronic alcohol abuse in adults patients from 30 to 70 years of age. The physical examination findings are analyzed. Which of the following neurologic findings are these patients most likely to exhibit?

A Choreiform movements

B Nystagmus

C Truncal and lower limb ataxia

D Tremor at rest that diminishes or disappears with movement

E Short-term memory loss

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Question 14

A previously healthy 31-year-old woman experiences a severe headache and loses consciousness within an hour. She is taken to the hospital where an emergent head CT scan reveals extensive subarachnoid hemorrhage at the base of the brain. She is afebrile. A lumbar puncture yields cerebrospinal fluid with many red blood cells, but no white blood cells. The CSF protein is slightly increased, but the glucose is normal. Which of the following is the most likely diagnosis?

A Acute bacterial meningitis

B Ruptured berry aneurysm

C Progressive multifocal leukoencephalitis

D Tay-Sachs disease

E Parkinson disease

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Question 15

Several members of a large family are affected by the onset of decreasing mental function and motor coordination when they reach middle age. Their movements are marked by choreoathetosis. Genetic testing reveals increased trinucleotide CAG repeats. Which of the following structures is most likely to appear grossly abnormal at autopsy of these affected persons?

A Caudate nucleus

B Midbrain

C Temporal lobe

D Locus ceruleus

E Dorsal root ganglion

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Question 16

A 22-year-old man suffers a deep laceration to the forearm. On physical examination there is loss of sensation over the lateral palm, including the thumb and first two fingers of his left hand. Which of the following pathologic changes will most likely result distally in the forearm structure that was injured to produce his physical finding?

A Wallerian degeneration

B Fibrinoid necrosis

C Coagulative necrosis

D Segmental demyelination

E Chronic inflammation

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Question 17

A 50-year-old man has been imbibing martinis (shaken, not stirred) for several hours while at the blackjack table. He wanders off, and several minutes later is found down. Paramedics arrive, and discover a bruise on his posterior occiput, but no other signs of trauma. He is transported to the hospital in stable condition, with vital signs showing temperature 36.9 C., pulse 81/minute, respirations 20/minute, and blood pressure 115/80 mm Hg. On arrival, his blood ethanol is 330 mg/dL. He becomes progressively obtunded. His right pupil is 8 mm and the left 4 mm. A head CT scan reveals a collection of blood in the right subdural region. Damage to which of the following intracranial vascular structures has most likely resulted in these findings?

A Middle meningeal artery

B Cavernous sinus

C Middle cerebral artery

D Dural bridging vein

E Great vein of Galen

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Question 18

A 13-month-old child was born at term, with no apparent anomalies at birth. The child has exhibited irritability, vomiting, and loss of appetite for the past 2 months. On physical examination there is macrocephaly with impaired extraocular movements, hypertonia of the lower extremities, and generalized hyperreflexia. A head CT scan reveals bilateral symmetrical ventriculomegaly. Which of the following pathologic abnormalities is most likely to produce these findings?

A Haemophilus influenzae meningitis

B Forking of the aqueduct of Sylvius

C Epidural hematoma

D Ruptured berry aneurysm

E Glioblastoma multiforme

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Question 19

A 48-year-old woman has the sudden onset of a severe headache. On physical examination there are no localizing neurologic signs, but she is minimally responsive. A cerebral angiogram demonstrates marked narrowing of cerebral artery branches near the base of the brain, consistent with vasospasm, but no intraparenchymal hemorrhage is present. Laboratory studies show a serum urea nitrogen of 50 mg/dL. An ultrasound scan of the abdomen shows bilaterally enlarged cystic kidneys. Which of the following is the most likely diagnosis?

A Bacterial meningitis

B Severe atherosclerosis

C Malignant hypertension

D Cerebral edema

E Subarachnoid hemorrhage

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Question 20

A 41-year-old woman has had a worsening headache for the past week, along with a fever and increasing obtundation. On physical examination her temperature is 38.2 C. A head CT scan reveals a solitary 3 cm diameter lesion with ring enhancement located in the right parietal lobe. A stereotactic biopsy is performed and a frozen section shows granulation tissue with adjacent collagenization, gliosis, and edema. Which of the following is the most likely diagnosis?

A Chronic brain abscess

B Aspergillosis

C Progressive multifocal leukocencephalopathy

D Toxoplasmosis

E Rabies virus infection

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Question 21

A 66-year-old man is finding that he has more difficulty moving about for the past year. He is annoyed by a tremor in his hands, but the tremor goes away when he performs routine tasks using his hands. His friends remark that he seems more sullen and doesn't smile at them, but only stares with a fixed expression on his face. He has not suffered any loss of mental ability. Which of the following diseases is he most likely to have?

A Amyotrophic lateral sclerosis (ALS)

B Alzheimer disease

C Parkinson disease

D Niemann-Pick disease

E Tuberous sclerosis

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Question 22

A 72-year-old woman has a five year history of worsening mental functioning with trouble remembering things. She has no problems with movement. MR imaging of the brain shows symmetrically increased size of the lateral ventricles along with cerebral cortical atrophy in a mainly frontal and parietal distribution. A lumbar puncture reveals a normal opening pressure, and analysis of the clear, colorless cerebrospinal fluid reveals a glucose and protein which are in normal ranges. Cell count on the CSF shows 3 WBCs (all lymphocytes) and 1 RBC. A funduscopic examination is normal. Which of the following abnormalities is most likely to be associated with her underlying disease process?

A Loss of gamma aminobutyric acid

B Presence of the e4 allele of apolipoprotein E

C Increased numbers of Lewy bodies

D Perivascular mononuclear inflammation

E Loss of Betz cells

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Question 23

A 26-year-old previously healthy woman has the sudden onset of mental confusion. She has a seizure and is brought to the hospital. Her vital signs show temperature 37 C, pulse 89/minute, respirations 22/minute, and blood pressure 100/60 mm Hg. A lumbar puncture reveals a normal opening pressure, and clear, colorless cerebrospinal fluid is obtained with 1 RBC and 20 WBC's (all lymphocytes), with normal glucose and protein. An MRI scan of the brain reveals swelling of the right temporal lobe with hemorrhagic areas. Which of the following infectious agents is the most likely cause for these findings?

A Herpes simplex virus

B Influenza virus

C Mycobacterium tuberculosis

D Hemophilus influenzae

E Neisseria meningitidis

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Question 24

A 30-year-old G2 P1 woman delivers a stillborn male infant at 28 weeks gestation. Her previous pregnancy resulted in a normal term birth. At autopsy, the cerebrum of the fetus demonstrates extensive diffuse periventricular areas of necrosis. Infection in utero with which of the following organisms is most likely to have caused these findings?

A Taenia solium

B Cytomegalovirus

C Poliovirus

D Candida albicans

E Treponema pallidum

F Group B streptococcus

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Question 25

A 73-year-old man has exhibited problems remembering things for the past 7 months, and he is noted by his immediate family to confabulate. He dies as a consequence of a hepatocellular carcinoma. At autopsy, his brain demonstrates bilaterally small mamillary bodies that show brown discoloration. Microscopically, there is gliosis and vascular proliferation and hemosiderin deposition in the mamillary bodies and periaqueductal gray matter. Which of the following is the most likely diagnosis?

A Multiple sclerosis

B Parkinson disease

C Amyotrophic lateral sclerosis

D Wernicke-Korsakoff syndrome

E Huntington disease

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Question 26

A 50-year-old man was involved in a vehicular accident in which he was not wearing any restraint and struck his head against the windshield of his van. He did not lose consciousness at that time or at any point thereafter. Physical examination showed a minor contusion to his forehead. However, a month later he began complaining of headaches, becoming irritable and acting strangely. Which of the following intracranial vascular abnormalities most likely developed in this man?

A Epidural hematoma

B Chronic subdural hematoma

C Cerebral contusions

D Subarachnoid hemorrhage

E Intracerebral hematoma

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Question 27

A previously healthy 42-year-old former major league baseball player developed progressive, symmetric muscular weakness of his upper extremities over the course of 3 years. Then he developed difficulty speaking and swallowing. He did not have myalgias or arthralgias. He remained afebrile. His mental function never became diminished. Which of the following is the most likely diagnosis?

A Amyotrophic lateral sclerosis

B von Recklinghausen disease

C Multiple sclerosis

D Werdnig-Hoffman disease

E Guillain-Barré syndrome

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Question 28

A 20-year-old man has recently been inducted into the army. Several weeks into basic training, he experiences a severe headache for an entire day. He had been healthy prior to this, noting only a mild sore throat the prior day. He goes to the base physician, who records vital signs of T 39.2 C, P 90/minute, RR 19/minute, and BP 110/70 mm Hg. A lumbar puncture is performed and examination of the cerebrospinal fluid shows 2 RBC's/microliter, 34,000 WBC's/microliter, glucose of 20 mg/dl (serum 75 mg/dL), and CSF protein of 105 mg/dL. Which of the following additional laboratory tests on the CSF would be the most helpful in reaching a diagnosis in this case?

A Cryptococcal antigen

B Acid fast stain

C India ink stain

D Serology for Herpes simplex

E Gram stain

F VDRL

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Question 29

A 77-year-old man has had a step-wise loss of mental function for the past 8 years, but his condition has remained stable for the past year. He can no longer care for himself. On physical examination he has decreased motor strength in his right upper extremity. He is able to ambulate and his gait is normal. He cannot remember any of 3 objects after 3 minutes. A chest x-ray shows cardiomegaly. Laboratory studies show a serum glucose of 95 mg/dL, cholesterol 192 mg/dL, creatinine 1.0 mg/dL, total thyroxine 9.5 microgm/dL, vitamin B12 481 pg/mL, and hemoglobin 13.9 g/dL. Which of the following is the most likely diagnosis?

A Alzheimer disease

B Diffuse Lewy body disease

C Huntington disease

D Parkinson disease

E Vascular dementia

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Question 30

A 45-year-old man has had a severe headache for a week. Physical examination reveals papilledema on the right. A head CT scan reveals a marked right to left midline shift. An MRI scan demonstrates a 6 cm enhancing mass lesion in the right parietal region with marked surrounding edema. He develops a dilated pupil on the right and soon thereafter loses consciousness and dies. At autopsy, which of the following lesions is most likely to be found?

A Superior sagittal sinus thrombosis

B Right cerebellar hemispheric hemorrhage

C Pontine hemorrhages

D Thrombosis of the posterior cerebral artery

E Diffuse subarachnoid hemorrhage

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Question 31

A 43-year-old woman develops a progressive, ascending motor weakness over the past 4 days. She is afebrile. She is hospitalized and requires intubation with mechanical ventilation. A lumbar puncture is performed with normal opening pressure. The CSF obtained is clear and colorless with glucose of 65 mg/dL (serum 100 mg/dL), protein 95 mg/dL, and cell count of 5/microliter, all lymphocytes. She gradually recovers over the next month. Which of the following conditions most likely preceded the onset of her neurologic disease?

A Ketoacidosis

B Viral pneumonia

C Staphylococcus aureus septicemia

D Systemic lupus erythematosus

E Vitamin B12 deficiency

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Question 32

A 50-year-old woman suddenly lost consciousness. On examination in the emergency department, her temperature is 37 C, pulse 79/minute, respirations 18/minute, and blood pressure 160/95 mm Hg. A head CT scan shows a 4 cm area of bright attenuation in the left basal ganglia region. There is effacement of the lateral ventricles and a shift of the midline to the right. Through which of the following mechanisms is death in the patient most likely to occur?

A Cerebellar tonsillar herniation

B Intraventricular hemorrhage

C Widespread metastases

D Septicemia

E Extensive watershed infarction

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Question 33

The mother of a 5-year-old girl realizes that her child has spent all of Saturday in bed. The girl is listless and not arousable, so her mother takes the child to the emergency room. The examining physician notes a temperature of 38.8 C and nuchal rigidity. A lumbar puncture yields slightly cloudy CSF with a protein of 61 mg/dL and glucose 19 mg/dL (serum glucose 83 mg/dL). A culture of CSF is most likely to yield which of the following organisms?

A Hemophilus infuenzae

B Cryptococcus neoformans

C Aspergillus fumigatus

D Mycobacterium tuberculosis

E Cytomegalovirus

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Question 34

A 45-year-old woman noticed tinnitus in her left ear which progressed over 5 weeks to hearing loss in that ear. On physical examination she is found to have a marked decrease in hearing on the left, with Rinne test indicating air conduction better than bone conduction. The other cranial nerves I - VII and IX - XII are intact. A brain MRI scan revealed a solitary, fairly discrete, 3 cm mass located in the region of the left cerebellopontine angle. Which of the following statements is most appropriate to tell the patient regarding these findings?

A You are unlikely to survive for more than a year

B Remissions and exacerbations are likely to occur in coming years

C Other family members should undergo MR imaging of the brain

D The lesion can be resected with a good prognosis

E A test for HIV-1 is likely to be positive

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Question 35

A 65-year-old man has had general paresis with increasing loss of higher mental functions for the past 3 years. On physical examination he walks with a widened gait. Laboratory studies show that a VDRL is positive on cerebrospinal fluid obtained by lumbar puncture. The CSF protein and glucose are normal, and there is 1 mononuclear cell present. Which of the following pathologic findings is most likely to be present in his spinal cord?

A Anterior horn cell loss

B Hemorrhage

C Plaques of demyelination

D Vacuolar myelopathy

E Atrophy of dorsal columns

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Question 36

A 53-year-old woman has had transient ischemic attacks (TIAs) for 3 years. She then has the sudden onset of a left hemiparesis. Four months later, an MRI scan of the brain shows a 4 cm diameter cystic area in the right frontal-parietal region. Which of the following underlying conditions is she most likely to have?

A Occlusive coronary atherosclerosis

B Chronic meningitis

C Alzheimer disease

D Glioblastoma multiforme

E Cerebral arterial vasculitis

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Question 37

A 50-year-old man has a history of falling multiple times over the past 4 years. Today he fell and on examination is noted to have a 2 cm contusion on the scalp at his occiput. Funduscopic examination shows no papilledema. A lumbar puncture is performed and laboratory studies on the CSF show 1 mononuclear cell/microliter, 1 RBC/microliter, protein 33 mg/dL, and glucose 60 mg/dL (serum glucose 80 mg/dL). His blood ethanol is 0.29 gm% (290 mg/dL). In which of the following intracranial locations are hemorrhages most likely to be found in this man?

A Basal ganglia

B Gyrus rectus

C Pons

D Thalamus

E Ventricle

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Question 38

A 54-year-old woman has noted changes in sensation in her legs for the past 5 months. On physical examination she has a distal, symmetric, primarily sensory polyneuropathy. She also has a non-healing ulceration on the ball of her left foot. She had a myocardial infarction last year but recovered and is doing well following angioplasty. Which of the following laboratory test findings would you most likely expect to be present in this woman?

A CSF protein of 110 mg/dL

B Positive herpes simplex virus serology

C Blood lead of 50 micrograms/dL

D Chromosome analysis with a 47, XX, +21 karyotype

E Serum glucose of 195 mg/dL

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Question 39

A 17-year-old primigravida has a screening fetal ultrasound performed at 19 weeks gestation. Major internal thoracic and abdominal organs are identified, with no major abnormalities. However, an abnormality is detected, but it is considered to have minimal significance for the fetus. Laboratory studies show that the maternal serum alpha-fetoprotein is not elevated. Which of the following defects is most likely to be present in this fetus?

A Meningocele

B Anencephaly

C Spina bifida occulta

D Meningomyelocele

E Encephalocele

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Question 40

A 50-year-old man is noted by his wife to have undergone personality changes over the last year. In the past, he was noted to be obsessive-compulsive, but he became slovenly and now does not appear to take an interest in his work. He has become more forgetful. On physical examination he has frontal release signs and memory loss. He appears unconcerned about his illness. MR imaging of the brain is performed and shows a 3 cm diameter left frontal lobe mass with areas of calcification. Which of the following diagnoses is most likely to be made on microscopic examination of this mass?

A Thrombosed berry aneurysm

B Oligodendroglioma

C Meningioma

D Schwannoma

E Organizing abscess

F Remote infarct

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Question 41

A 53-year-old previously healthy man has had a rapid decline in mental function over the past 4 months. On physical examination he exhibits profound dementia along with myoclonus. He is afebrile. A cerebral electroencephalogram shows periodic biphasic synchronous sharp-wave complexes that are superimposed upon a slow background rhythm. He dies from bronchopneumonia. At autopsy, his brain appears grossly normal, but a spongiform encephalopathy is seen microscopically in a section of the cerebral cortex (which was put in concentrated formic acid for 1 hour prior to processing). Which of the following is the most likely diagnosis?

A Alzheimer disease

B Creutzfeldt-Jakob disease

C HIV encephalopathy

D Herpes viral encephalitis

E Lead poisoning

F Leigh disease

G Progressive multifocal leukoencephalopathy

H Rabies

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Question 42

A 10-month-old infant is noted to be failing to reach developmental milestones. On physical examination there is a prominent 2 cm lumbar meningomyelocele. An MRI scan of the brain demonstrates downward extension of the cerebellar vermis and displacement of the medulla from a small posterior fossa into the foramen magnum. There is tenting of the tectum of the midbrain. The cerebral ventricles are enlarged. The spinal cord has findings of hydromyelia. Which of the following is the most likely diagnosis?

A Dandy-Walker malformation

B Viral meningoencephalitis

C Arnold-Chiari malformation

D Maternal folate deficiency

E Werdnig-Hoffman disease

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Question 43

A 46-year-old woman has experienced severe headaches, worsening over the past two months. She has noted difficulty moving her left arm during the past day. On physical examination papilledema is noted, worse on the right. A lumbar puncture is performed, and the CSF reveals a protein of 190 mg/dL, glucose of 68 mg/dL (serum glucose is 100 mg/dL), and a cell count showing 5 WBCs/microliter (4 mononuclears and 1 PMN) and 6 RBCs/microliter. Through which of the following mechanisms are these findings most likely to be produced?

A Blockage of lymphatics by metastatic tumor

B Overproduction of cerebrospinal fluid

C A storage disease with neuronal loss

D Hyperosmolar coma with diabetes mellitus

E Edema around a glioblastoma multiforme

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Question 44

A 22-year-old man has recently emigrated from Mexico City. He has the sudden onset of a seizure disorder while working as a chef in a restaurant. On physical examination he is afebrile. No papilledema is noted. MR imaging of the brain reveals a 2 cm rounded cyst in the right temporal lobe cortex and another 1.5 cm cyst in the subarachnoid space over the left parietal lobe. Both lesions are non-enhancing. A lumbar puncture yields colorless CSF under normal pressure. The CSF protein and glucose are normal, and there are 5 WBCs/microliter (4 monos, 1 PMN). Which of the following conditions most likely to cause these findings?

A Metastatic adenocarcinoma

B HIV encephalopathy

C Left atrial mural thrombosis

D Cysticercosis

E Hypertension

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Question 45

A 29-year-old woman notes the appearance of an expanding ring-like rash on her forearm over the past week. As this subsides, 3 weeks later she develops a stiff neck, left facial droop, and chest pain. Which of the following is most likely to have initiated this woman's sequence of problems?

A The bite of a deer tick

B Mercury poisoning

C Taking a cephalosporin

D Sexual intercourse

E Systemic lupus erythematosus

F Snorting cocaine

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Question 46

A 61-year-old man has had a chronic cough for 6 years as a result of smoking 2 packs of cigarettes per day for 45 years. He has noted the onset of headaches over the past 2 weeks. His physician on neurologic exam can find no localizing signs. MR imaging of the brain reveals a solitary 3.5 cm lesion that is located at the grey-white junction in the posterior left frontal lobe. There is no ring enhancement. A stereotactic biopsy of this lesion is performed. Which of the following microscopic appearances is most likely to be present in this biopsy?

A An organizing abscess

B Viral inclusions

C A plaque of demyelination

D Neuronal loss with gliosis

E Metastatic carcinoma

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Question 47

A 48-year-old man presents to the emergency room with sudden onset of a severe headache. He is afebrile. He is found to have papilledema on funduscopy. A head CT scan shows a 6 cm mass in the right cerebral hemisphere with a midline shift. He dies before further workup can be performed. At autopsy there are recent hemorrhages in the pons. No other hemorrhages are found in the central nervous system. He does not have hemorrhages elsewhere in the body. Which of the following pathologic findings is most likely to be present at autopsy?

A Severe thrombocytopenia

B Gram negative rods in cerebrospinal fluid

C Fracture of the right parietal bone

D Loss of pigmented neurons in the substantia nigra

E Cerebral edema with uncal herniation

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Question 48

A 47-year-old man has had the new onset of headaches for the past 4 months. The headaches are associated with dull pain and seem diffuse, but they are becoming more frequent and prolonged. On physical examination he has no focal neurologic deficits. His memory is intact. MR imaging reveals enlargement of the lateral ventricles. There is a 4 cm homogenous, well-circumscribed mass within the fourth ventricle. Which of the following is the most likely diagnosis?

A Astrocytoma

B Choroid plexus papilloma

C Ependymoma

D Meningioma

E Metastatic bronchogenic carcinoma

F Schwannoma

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Question 49

A 36-year-old woman has noted increasing numbers of disfiguring nodular masses involving the skin of her trunk and extremities. She has experienced frequent headaches over the past month. On physical examination these 0.5 to 2 cm subcutaneous masses are firm and non-tender. MR imaging of the brain shows an ill-defined 4 cm mass of the right parietal lobe. An abdominal CT scan shows a 3 cm mass involving the left adrenal gland. Laboratory studies show increased urinary free catecholamines. No other family members are affected by these problems. A mutation involving which of the following genes is most likely to be present in this woman?

A APC

B CFTR

C K-RAS

D NF-1

E N-MYC

F TP53

G RB

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Question 50

A A 70-year-old man has had increasing difficulty with movement, starting with his feet and ascending to involve legs, trunk, and now arms, over the past 10 days. On physical examination there are variable sensory changes noted. He is afebrile. He does not lose consciousness and remains mentally alert. He becomes ventilator dependent a week after the onset of this illness. A lumbar puncture is performed and the CSF demonstrates a protein of 86 mg/dL, glucose 63 mg/dL (serum 89 mg/dL), and only 3 mononuclear cells/microliter. He gradually recovers over the ensuing 4 weeks. Which of the following conditions most likely preceded the onset of this man's illness?

A Exposure to a toxin

B Recent viral infection

C Severe hypotension

D Bacterial septicemia

E Radiation therapy

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Question 51

A 33-year-old HIV-positive woman has had increasing inability to think clearly, with forgetfulness, over the past 3 weeks. She now has trouble doing everyday tasks. She has no history of seizures, headaches, nausea, vomiting, fever, chills or diarrhea. On examination she is oriented to time, place and date. She is indifferent to her surroundings. She is unable to perform calculations and has difficulty in word finding. She is afebrile. MR imaging of the brain shows an irregular ring-enhancing lesion in the white matter of the left frontal lobe. Her CD4 count is 90/microliter. Which of the following is the most likely diagnosis?

A Infarction

B Toxoplasmosis

C Contusion

D Astrocytoma

E Cysticercosis

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Question 52

A 28-year-old man swerves to avoid an oncoming vehicle while riding his motorcycle. He falls and rolls along the pavement for 100 m. On physical examination his vital signs include temperature 37 C, pulse 78/minute, respirations 20/minute, and blood pressure 120/80 mm Hg. He has multiple contusions and abrasions involving the skin of his torso and extremities, but none on his head because he was wearing a helmet. He is unconscious. There is no decerebrate posturing. A head CT scan shows no intracranial hemorrhage or edema and no skull fractures. He remains in a persistent vegetative state. Which of the following pathologic findings is most likely to be present?

A Central pontine myelinolysis

B Cerebral venous sinus thrombosis

C Demyelination

D Diffuse axonal injury

E Meningoencephalitis

F Neuronal loss

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Question 53

A 58-year-old man has an episode of hemoptysis. Over the next week, he becomes progressively obtunded. He has a blood pressure of 110/70 mm Hg, pulse 90/minute, respirations 25/minute, and temperature 36.8 C. A chest radiograph on admission reveals a right hilar lung mass. Serum chemistries reveal a sodium of 108 mmol/L, potassium 3.9 mmol/L, chloride 85 mmol/L, and CO2 19 mmol/L. He is given intravenous saline, and the serum sodium is 135 mmol/L the next day. He then becomes comatose and dies. At autopsy, which of the following lesions is most likely to be found in his brain?

A Metastases to the cerebral hemispheres

B Central pontine myelinolysis

C Multiple watershed infarctions

D Hemorrhages in the basal ganglia

E Periventricular plaques of demyelination

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Question 54

A 36-year-old man notices loss of sensation at the site of a mongoose bite he incurred 6 weeks ago while out working in a field on a trip to a game preserve in Madras. On examination he is afebrile. Paresthesias are present only at the site of the bite. Over the next 4 days he develops convulsions, then flaccid paralysis and coma. Which of the following microscopic pathologic abnormalities is most likely to be present in this patient?

A Spinal cord anterior horn neuronal loss with gliosis

B Meningitis with gelatinous intraventricular exudates

C Hippocampal neuronal eosinophilic intracytoplasmic inclusions

D Cortical nodules with neutrophils

E Widespread spongiform encephalopathy

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Question 55

An 18-month old girl has exhibited poor psychomotor development since birth, along with seizures. On examination her muscle strength and tone are poor. Brain MR imaging shows multifocal abnormalities with loss of tissue in periventricular regions of midbrain, pons, thalamus, and hypothalamus. Laboratory studies show plasma lactate of 4.2 mmol/L. Which of the following is the most likely diagnosis?

A Leigh disease

B Cytomegalovirus infection

C Wernicke disease

D Herpes simplex encephalitis

E Metachromatic leukodystrophy

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