An autopsy study is conducted involving patients in the hospital who died from neurologic diseases. It is observed that patients with one underlying disease have brains that are decreased in weight from normal. There is bilateral symmetrical cortical atrophy involving mainly the frontal, parietal, and temporal lobes. There is ex vacuo cerebral ventricular dilation. Cerebral arteries at the base of the brain show no atherosclerosis. The cerebral cortex microscopically shows numerous neuritic plaques along with neurofibrillary tangles and amyloid angiopathy. Which of the following physical examination findings was most likely to have been present in patients with this disease?

A Ataxia

B Bilateral symmetrical muscular weakness

C Blindness

D Choreiform movements

E Cogwheel muscular rigidity

F Memory loss

G Upward gaze palsy

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A 69-year-old man with a history of a remote myocardial infarction suffers a "stroke" and dies. He is found at autopsy to have a 4-cm diameter area of softening in the region of the left middle cerebral artery distribution. Which of the following is the most likely diagnosis?

A Vasculitis

B Thromboembolization

C Venous thrombosis

D Hypertension

E Mycotic aneurysm

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A 50-year-old woman has had right-sided headaches for 5 years, but has noted mild weakness in her left hand for the past week. On physical examination there is 4/5 motor strength of the left forearm, and 5/5 strength elsewhere. A CT scan of the head shows a 7 cm well circumscribed lateral mass compressing the right hemisphere at the frontal-parietal junction. Which of the following neoplasms is this woman most likely to have?

A Medulloblastoma

B Metastatic carcinoma

C Schwannoma

D Glioblastoma multiforme

E Meningioma

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A 65-year-old man has been healthy all his life until he experiences a sudden grand mal seizure. A neurologic exam reveals no focal abnormalities. MR imaging of the brain reveals a large, poorly demarcated mass with central necrosis in the right frontal lobe. Which of the following neoplasms is he most likely to have?

A Glioblastoma multiforme

B Medulloblastoma

C Oligodendroglioma

D Meningioma

E Choroid plexus papilloma

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A 35-year-old man experienced a severe headache accompanied by a perception of strange smells 2 days ago. On physical examination his temperature is 37.1 C and blood pressure 120/80 mm Hg. A CT scan of the head shows a focal 2 cm bright subfrontal lesion. A brain MR angiogram reveals an aneurysmal dilation of the anterior communicating artery. Which of the following complications of this lesion is most likely to occur?

A Epidural hematoma

B Intraparenchymal hematoma

C Intraventricular hemorrhage

D Subarachnoid hemorrhage

E Subdural hematoma

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A 52-year-old man has had chronic respiratory difficulty for 5 years. He requires use of accessory muscles of respiration. He dies from bronchopneumonia. At autopsy, the anterior spinal nerve roots are atrophic in the cervical region, and cervical spinal cord anterior horns show neuronal loss with gliosis. Which of the following is the most likely diagnosis?

A Gullain-Barre syndrome

B Poliomyelitis

C Rabies infection

D Botulism

E Amyotrophic lateral sclerosis

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A 78-year-old man has sudden loss of ability to move his left arm and to speak. On physical examination there is a flaccid paralysis of his left arm. He gradually recovers some function of his left arm over the next 2 months. A CT scan of the head now shows a 3 cm cystic area involving the right internal capsule. Which of the following pathologic changes is most likely to produce this man's findings?

A Gangrenous necrosis

B Liquefactive necrosis

C Coagulative necrosis

D Caseous necrosis

E Fat necrosis

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During a fight at the Beacon Club in Casper, Wyoming, a patron is knocked backwards off a barstool and lands on the back of his head. He remains conscious and continues the fight until someone decides to stop the altercation by buying everyone a round of beers. Which of the following pathologic findings is most likely to be present as a consequence of this injury (not the beers--the fall)?

A Occipital lobe axonal injury

B Subarachnoid hemorrhage

C Inferior frontal lobe contusions

D Anterior pituitary necrosis

E Skull fracture with epidural hematoma

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A 19-year-old woman becomes pregnant for the first time. At 18 weeks gestation there is less fetal movement than normal. A screening ultrasound scan shows a "lemon sign" with concavity of the frontal portions of the fetal brain. Which of the following laboratory test abnormalities will most likely be present?

A Decreased human chorionic gonadotropin

B Hypochromic, microcytic anemia

C Decreased serum folate

D Increased alpha-fetoprotein

E Positive serologic test for syphilis

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A 44-year-old man has developed increasing fatigue with motor and sensory problems along with decreased vision over the past year. On physical examination he has spastic paraparesis, sensory ataxia, and marked paresthesias of the legs. Laboratory studies show Hgb 8.9 g/dL, platelet count 222,200/microliter, and WBC count 8190/microliter. MR imaging of the brain shows optic nerve atrophy. Which of the following diseases is most likely to produce these findings?

A Chronic alcoholism

B Down syndrome

C Pernicious anemia

D Lead poisoning

E Diabetes mellitus, type I

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A 54-year-old woman has experienced headaches for the past week. On physical examination her temperature is 37.6 C, pulse 73/minute, respirations 16/minute, and blood pressure 130/85 mm Hg. She has a lumbar puncture performed. The cerebrospinal fluid (CSF) opening pressure is 220 mm H2O, the CSF protein 60 mg/dl, and the CSF glucose 75 mg/dl (serum glucose 105 mg/dl). A CT scan reveals a 3 cm ring-enhancing mass in the right parietal region. Biopsy of the mass reveals gliosis and fibrosis with necrosis, neutrophils, and lymphocytes. Which of the following is the most likely diagnosis?

A Glioblastoma multiforme

B Herpes simplex type II encephalitis

C Vascular malformation

D Subacute infarction

E Cerebral abscess

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A 38-year-old woman has the abrupt onset of dizziness with nausea and vomiting. She does not have a headache. On physical examination she is afebrile. No motor deficits are noted. She has difficulty with balance. Her nausea and vomiting subside in 3 days, and she has only mild vertigo for 5 more days. Which of the following is the most likely diagnosis?

A Gullain-Barre syndrome

B Cerebral edema

C A schwannoma

D A cerebral abscess

E Vestibulitis

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A 69-year-old man has developed problems with ambulation over the past 8 months. On physical examination he has a festinating gait, cogwheel rigidity of the limbs, poverty of voluntary movement, and a pill rolling type of tremor at rest. His facies appear expressionless, or mask-like. An MRI scan of the brain shows no abnormal findings, other than minimal cerebral atrophy consistent for his age. Which of the following microscopic lesions is he most likely to have?

A Gliosis with neuronal loss in the caudate nucleus

B Loss of anterior horn cells

C Loss of pigmented neurons in the substantia nigra

D Increased neurofibrillary tangles in the hippocampi

E Scattered white matter plaques of demyelination

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A clinical study is performed to determine the prognosis for patients diagnosed with a brain neoplasm. It is observed that one subset of patients has a high rate of long-term survival, over 95% at 10 years. These patients have a low rate of recurrence. Almost all of the patients who had a job were able to return to their job following surgical therapy. Which of the following intracranial neoplasms was this subset of patients most likely to have?

A Schwannoma

B Glioblastoma multiforme

C Astrocytoma

D Medulloblastoma

E Solitary metastasis

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A 35-year-old man with HIV infection has had headaches for the past month. He has noted difficulty with movement of his right arm for the past 2 weeks. On physical examination he is afebrile. There is flaccid paralysis of his right arm. Laboratory studies show an HIV-1 RNA of 5630 copies/mL. Brain MR imaging reveals several periventricular 1 to 3 cm masses. A stereotaxic biopsy is performed and on microscopic examination shows a monoclonal proliferation of immunoblasts. Which of the following is the most likely diagnosis?

A Cysticercosis

B HIV encephalitis

C Non-Hodgkin lymphoma

D Progressive multifocal leukoencephalopathy

E Toxoplasma gondii encephalitis

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A 50-year-old man has had a history of paresthesias, difficulty moving one or more extremities, loss of sensation, and ataxia for over 22 years. These problems would come and go, but he eventually he developed paraplegia and incontinence. Which of the following pathologic abnormalities is most likely to explain the course of his disease?

A Parasagittal meningioma

B Scattered plaques of demyelination

C Shrapnel in spinal cord

D Wernicke disease

E Progressive multifocal leukoencephalopathy

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A 41-year-old woman complains of increasing pain in her right foot for the past year. This pain is making it difficult for her to wear her high-heeled shoes (her closet is full of them). This has been become more bothersome for the past two months. The pain seems to be worse at the end of the day. Upon physical examination, there is severe pain on palpation of the interdigital space between the 2nd and 3rd toes. There is no swelling or erythema of the foot. Motor strength in her lower extremities is normal. Which of the following is the most likely diagnosis?

A Lead poisoning

B Traumatic neuropathy

C Thiamine deficiency

D Diabetes mellitus

E Small cell anaplastic carcinoma

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A clinical study involving patients with traumatic injuries to extremities is conducted. Some of these patients had transection of a peripheral nerve trunk. It is observed that axonal and myelin sheath degeneration occurs in the nerve trunk distal to the site of injury. In many of these patients, motor function is regained in the affected extremity. Which of the following cells is most important in regaining function following nerve injury?

A Macrophage

B Microglial cell

C Neuroblast

D Oligodendrocyte

E Satellite cell

F Schwann cell

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An 82-year-old nursing home patient has had a history of several falls during the past week, but has never lost consciousness and has continued to be active. She then develops increasing mental confusion. A week later she is examined by the physician. She is afebrile and normotensive. There is a mild right hemiparesis. A head CT scan demonstrates left hemispheric compression, effacement of the right lateral ventricle, and midline shift. Which of the following conditions also seen on the CT scan is the most likely diagnosis?

A Epidural hematoma

B Glioblastoma multiforme

C Hypertensive hemorrhage

D Cerebral abscess

E Chronic subdural hematoma

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A 59-year-old woman is unable to move her right arm and has difficulty speaking following sudden loss of consciousness for a hour. She slowly regains function over the next three months. Brain MR imaging now shows a 4 cm non-enhancing cystic area in the left parietal lobe. By which of the following vascular events was this lesion most likely to be produced:

A Embolism from a deep femoral vein

B Thrombosis of the sagittal sinus

C Thrombosis of the left middle cerebral artery

D Vasculitis of the left external carotid artery

E Hematoma formation following trauma

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A 42-year-old previously healthy woman has sudden weakness develop on the left side of her body. She also reports a severe headache. On physical examination there is 3/5 motor strength in left lower and upper extremities, compared with 5/5 strength on the right. A head CT scan reveals a 2.5 cm area of bright attenuation involving the region of the right basal ganglia. Which of the following is the most likely diagnosis?

A Hypertensive hemorrhage

B Herpes simplex encephalitis

C Vascular malformation

D Cysticercosis

E Metastatic renal cell carcinoma

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A 45-year-old woman suddenly experiences the inability to move the left side of her body. On physical examination there is left hemiparesis and facial droop. A cerebral angiogram demonstrates incomplete occlusion of the right middle cerebral artery. Which of the following sites is the most likely source for an embolus to produce her findings?

A Left atrium

B Carotid bifurcation

C Saphenous vein

D Tricuspid valve

E Aortic arch

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An autopsy study of primary brain neoplasms is conducted. It is observed that the cause of death in patients with one type of neoplasm is often related to Duret hemorrhages of the midbrain and/or the pons following medial temporal lobe (uncal) herniation. Which of the following neoplasms is most likely to cause these findings?

A Acoustic schwannoma

B Choroid plexus papilloma

C Glioblastoma multiforme

D Medulloblastoma

E Myxopapillary ependymoma

F Oligodendroglioma

G Pilocytic astrocytoma

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A 24-year-old woman has experienced headaches for the past month. Today she had a generalized seizure lasting for 5 minutes. On physical examination she has no focal neurologic findings. She is afebrile and normotensive. No papilledema is noted. A CT scan of the head shows a bright area of attenuation involving the right temporal lobe. A lumbar puncture is performed. The opening pressure is normal. Laboratory studies on the CSF show 1 WBC and 1 RBC/microliter, glucose 57 mg/dL (serum glucose 76 mg/dL), protein 40 mg/dL, and gram stain negative. Which of the following is the most likely diagnosis?

A Arteriovenous malformation

B Berry aneurysm

C Cysticercosis

D Leigh disease

E Metastatic carcinoma

F Tuberous sclerosis

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A 46-year-old man with a history of intravenous drug use has had an increasing headache over the past day along with fever. Physical examination reveals vital signs with T 39.3 C, P 81/minute, RR 18/minute, and BP 120/70 mm Hg. He has nuchal rigidity. Laboratory studies show that he is HIV positive with a CD4 lymphocyte count of 189/microliter. Which of the following infectious agents is most likely to account for these findings?

A J-C strain of papovavirus

B Mycobacterium tuberculosis

C Staphylococcus aureus

D Herpes simplex virus

E Toxoplasma gondii

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A 79-year-old retired banker has had a long history of transient ischemic attacks. For the past few years, his wife is saying with greater frequency, "Now what did I tell you just a few minutes ago?" However, he can recognize all family members and can talk at length about events of the past. He can still feed and clothe himself. Which of the following pathologic findings is he most likely to have?

A Loss of neurons in the hippocampi

B Numerous cortical senile plaques

C Metastatic carcinoma to cerebrum

D Cerebral vasculitis

E Head trauma

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(82) A 20-year-old primigravida has a screening ultrasound scan performed at 18 weeks gestation. The scan shows an open thoracic neural tube defect. Which of the following abnormal laboratory test findings is most likely to be present?

(83) A 33-year-old HIV positive man has had increasing difficulty performing activities of daily living for the past 4 months. A head CT scan shows multifocal 0.5 to 1 cm white matter lesions. Which of the following abnormal laboratory test findings is most likely to be present?

A Elevated maternal serum alpha-fetoprotein

B Oligoclonal bands on CSF electrophoresis

C Positive serology for herpes simplex virus

D Brain biopsy with amyloid angiopathy

E Brain biopsy with JC papovavirus

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(84) A 66-year-old man has had difficulty with ambulation for the past 4 years. He has difficulty starting to walk. He can walk better while patterning his gait after another person in front of him. During the past year he has had a decrease in higher mental functioning. MR imaging of the brain shows mild atrophy consistent for age but no focal lesions. Which of the following microscopic pathologic abnormalities of the brain is he most likely to have?

(85) A 41-year-old woman has had the onset of a movement disorder during the past year. On examination she demonstrates irregular writhing motion of her upper extremities. Her sister became similarly affected at age 44. Her mother was affected at age 49. Which of the following microscopic pathologic abnormalities of the brain is he most likely to have?

A Caudate nucleus gliosis

B Anterior horn cell loss

C Cortical Lewy bodies

D Cerebral amyloid angiopathy

E Basal ganglia neuronal enlargement

F Temporal lobe Pick bodies

G Centrum semiovale demyelination

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(86) A 37-year-old HIV positive man with a history of intravenous drug use is has exhibited diminished mental capacity and difficulty performing activities of daily living over the past 6 months. He is afebrile. Laboratory studies show a CD4 lymphocyte count of 150/microliter. Which of the following microscopic findings is most likely to be present in his cerebral cortex?

(87) A 23-year-old man undergoes bone marrow transplantation for acute myelogenous leukemia. He has failure of engraftment. He becomes febrile. Laboratory studies show Hgb 6.8 g/dL, platelet count 51,400/microliter, and WBC count 897/microliter. He then becomes comatose. A CT scan reveals several areas from 1 to 4 cm in size in the hemispheres that suggest necrosis with hemorrhage. Which of the following microscopic findings is most likely to be present in his cerebral cortex?

A Neutrophilic exudates

B Perivascular lymphocytes

C Septate, branching hyphae

D Multinucleate cells and macrophages

E Cysts in the cerebrum

F Plaques of demyelination

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(88) A 9-month-old infant boy has been feeding and gaining weight normally since birth, but displays minimal activity and attention. Funduscopic examination reveals an intense red color to the macular region of the retina bilaterally. Death occurs 6 months later. This infant is most likely to have a deficiency of which of the following enzymes?

(89) A 5-year-old child appeared normal at birth and through infancy. During the past year, this chcild has exhibited progressive loss of neurologic function. On physical examination there is no hepatosplenomegaly or lymphadenopathy. A bone marrow biopsy shows no abnormalities. The child dies 3 years later. This child is most likely to have a deficiency of which of the following enzymes?

A Sphingomyelinase

B Hexosaminidase A

C Glucocerebrosidase

D Aryl-sulfatase A

E Alpha-L-iduronidase

F Peroxisomal acyl-CoA synthetase

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(90) A 40-year-old man has a history of mild mental retardation. He now is unable to remember anything and cannot recognize family members. He had a ventricular septal defect repaired earlier in life. On physical examination he has bilateral transverse palmar creases and absent distal flexion creases on 5th digits. He is most likely to have a genetic abnormality with which of the following patterns of inheritance?

(91) A 40-year-old man has developed a psychosis and a movement disorder characterized by choreiform movements over the past 2 years. His father died from a similar disorder at the age of 45. He is most likely to have a genetic abnormality with which of the following patterns of inheritance?

A Autosomal recessive gene

B X-linked recessive gene

C Extra autosome

D Sex chromosome monosomy

E Marker chromosome

F Triple repeat sequence expansion

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(92) A 50-year-old man experiences a sudden grand mal seizure. On examination he has papilledema on the right. A head CT scan shows a 10 cm irregular mass with surrounding edema in the posterior left frontal lobe. Which of the following forms of intracranial hemorrhage is most likely to complicate this man's course?

(93) A 70-year-old man goes to his physician for a check of his health status. On physical examination his vital signs show T 37.1 C, P 69/minute, RR 15/minute, and BP 165/105 mm Hg. There are no other abnormal findings. Which of the following forms of intracranial hemorrhage is most likely to occur in this man?

A Basal ganglia hemorrhage

B Subdural hematoma

C Subarachnoid hemorrhage

D Pontine Duret hemorrhage

E Epidural hematoma

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(94) A 7-year-old child has complained of headaches for the past month. On physical examination there are no abnormalities. A head CT scan shows a brightly enhancing mass in the posterior fossa. MR imaging of the brain shows a 2 cm homogenous, circumscribed mass arising within the 4th ventricle and filling the 4th ventricle, with enlargement of the 3rd ventricle and lateral ventricles. The lesion is excised and the child recovers completely, with no recurrence. Which of the following neoplasms is this child most likely to have?

(95) A 9-year-old child has been noted to walk abnormally for the past month. On examination the child has a widened gait. The child has difficulty with a finger-to-nose test. MR imaging of the brain shows a 4 cm solid mass in the cerebellar vermis. The mass is excised, but there is a recurrence within a year. Which of the following neoplasms is this child most likely to have?

A Astrocytoma

B Choroid plexus papilloma

C Ependymoma

D Medulloblastoma

E Meningioma

F Oligodendroglioma

G Schwannoma

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(96) A 23-year-old welder has noted increasing difficulty gripping his torch and difficulty concentrating on his work for the past 2 months. He has also experienced headaches, dizziness, and abdominal cramping pain during this time. On physical examination he has a distal symmetric peripheral neuropathy. Poisoning with which of the following substances has most likely occurred?

(97) A 6-year-old girl develops visual loss within 3 days of consuming a clear liquid she found in a bottle in her family's garage. On physical examination she has intact motor and sensory function, except for blindness. Poisoning with which of the following substances has most likely occurred?

A Mercury

B Methanol

C Lead

D Iron

E Arsenic

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(98) A 23-year-old primigravida delivers a stillborn fetus at 34 weeks gestation. Autopsy examination of the fetus shows marked hydrops fetalis and organomegaly. There is extensive cerebral necrosis and periventricular calcification. Infection with which of the following organisms is most likely to produce these findings?

(99) A 25-year-old woman has acute onset of confusion followed by a seizure. On physical examination there are no abnormal findings. A CT scan of the head reveals a hemorrhagic lesion in her left temporal lobe. Infection with which of the following organisms is most likely to produce these findings?

A Cytomegalovirus

B Herpes simplex virus

C Human immunodeficiency virus

D Epstein-Barr virus

E JC Papovavirus

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A 14-year-old boy is brought to the emergency room following an accident in which he hit his head against a concrete wall. He was initially unconscious but then "came to". However, in the emergency room, he is comatose, and skull radiograph reveals a linear fracture of the temporal-parietal region on the left. Which of the following is the most likely diagnosis?

A Acute subdural hematoma

B Epidural hematoma

C Ruptured berry aneurysm

D Cerebral edema with uncal herniation

E Contusion of frontal lobes

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Following a mild upper respiratory flu-like illness, a 47-year-old man develops a rapidly ascending paralysis. A week later he is hospitalized and requires intubation with mechanical ventilation. Lumbar puncture yields clear CSF under normal pressure with a slightly elevated protein, but no red blood cells and only 3 mononuclear cells. He gradually improves over the next couple of weeks. Which of the following is the most likely diagnosis?

A Multiple sclerosis

B Amyotrophic lateral sclerosis

C Huntington disease

D Guillain-Barre syndrome

E Werdnig-Hoffman disease

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A 52-year-old man has had headaches and difficulty concentrating for the past 2 months. He then begins to exhibit odd behavior, including shooting his .30-06 rifle at fence posts in his back yard, which the neighbors find disconcerting. He then suffers a grand mal seizure, and is admitted to the hospital. MR imaging of the brain reveals a large mass with extensive necrosis in the left cerebral hemisphere extending across corpus callosum to the right hemisphere. Which of the following neoplasms is he most likely to have?

A Meningioma

B Glioblastoma multiforme

C Medulloblastoma

D Metastatic adenocarcinoma

E Craniopharyngioma

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A 27-year-old man comes to his physician because of recurrent headaches, averaging one episode per week for the past 2 months. He describes a slowly building, dull, aching, band-like pain accompanied by tight neck muscles. The pain lasts for 4 to 10 hours. Exertion does not worsen the headache. On physical examination there are no abnormal findings. Which of the following types of headache is the most likely diagnosis?

A Brain tumor

B Cluster

C Migraine

D Temporomandibular joint dysfunction

E Tension-type

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A 53-year-old woman has experienced recurring headaches for the past 16 years. These headaches always occur on the right, have a pulsatile quality, and are accompanied by nausea, photophobia, tingling in her left arm, and dizziness. The headache increases in intensity over 6 hours and remains for a day, unless she is able to fall asleep. On physical examination there are no abnormal findings. Which of the following drugs is most likely to be useful in treating her headaches?

A Acetylsalicylic acid

B Carbamazepine

C Oxazepam

D Oxycodone

E Phenytoin

F Sumitriptan

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A 68-year-old man has experienced periodic headaches for the past 3 years since retiring from his job. He feels the pain over his temple on the right. On physical examination there is tenderness on palpation over his face anterior to his right ear. During the examination he is noted to be grinding his teeth in response to questions. Which of the following conditions is he most likely to have?

A Faulty dentures

B Meningioma

C Migraine

D Temporal arteritis

E Trigeminal neuralgia

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A 30-year-old man has noted episodic headaches for the past 6 years. The headaches occur over a month, and then do not recur from another 2 or 3 months. The headaches begin with periorbital pain on the left that increases in intensity for 5 minutes, then persists for an hour before diminishing. The pain is accompanied by lacrimation, drooping of the right eyelid, nasal congestion, and nausea. Which of the following forms of headache is he most likely to have?

A Brain tumor

B Cluster

C Migraine

D Temporal arteritis

E Tension

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