Pathology Review Ch 28 CNS Prt 3

22 Questions | By Chachelly | Last updated: Feb 22, 2013 | Total Attempts: 356

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1.

A 37-year-old man who is HIV-1-positive has had increasing memory problems for the past year. He is depressed. During the past 3 months, he has had increasing problems with motor function and is now unable to stand or walk. For the past 3 days, he has had fever, cough, and dyspnea. A bronchoalveolar lavage shows cysts of Pneumocystis jiroveci. MRI of the brain shows diffuse cerebral atrophy; no focal lesions are identified. On microscopic examination of the brain, which of the following findings is most likely to be present?
- A.
  
  Plaques of demyelination in periventricular white matter
- B.
  
  Neocortical senile plaques and neurofibrillary tangles
- C.
  
  Multiple lacunar infarcts in basal ganglia
- D.
  
  Spongiform change involving cerebellum and neocortex
- E.
  
  White matter microglial nodules with multinucleate cells
2.

A term infant is born to a 32-year-old woman whose pregnancy was uncomplicated. A newborn physical examination shows a small lower lumbar skin dimple with a protruding tuft of hair. A radiograph shows that the underlying L4 vertebra has lack of closure of the posterior arches. What is the most likely diagnosis?
- A.
  
  Dandy-Walker malformation
- B.
  
  Spina bifida occulta
- C.
  
  Tuberous sclerosis
- D.
  
  Arnold-Chiari malformation
- E.
  
  Meningomyelocele
3.

A 5-year-old boy has been irritable for the past 2 days and has complained of an earache for the past 5 days. On physical examination, he has a temperature of 39.1°C. Laboratory examination of the CSF from a lumbar puncture shows numerous neutrophils, slightly increased protein level, and decreased glucose concentration. On Gram staining of the CSF, which of the following is most likely to be seen microscopically?
- A.
  
  No organisms
- B.
  
  Gram-positive cocci
- C.
  
  Gram-negative diplococci
- D.
  
  Short Gram-positive rods
- E.
  
  Short Gram-positive rods
4.

A 55-year-old man who had been healthy all his life now has progressive, symmetric muscular weakness. Two years ago, he noted weakness in the area of the head and neck, which caused difficulty with speech, eye movements, and swallowing. In the past year, the weakness in the upper and lower extremities has increased, and he can no longer stand, walk, or feed himself. His mental function remains intact. What is the most likely diagnosis?
- A.
  
  Amyotrophic lateral sclerosis
- B.
  
  Huntington disease
- C.
  
  Multiple sclerosis
- D.
  
  Guillain-Barré syndrome
- E.
  
  Parkinson disease
5.

A neonate is born prematurely at 28 weeks gestation to a 22-year-old primigravida. The infant is initially stable, and a newborn physical examination shows no abnormalities. The infant becomes severely hypoxemic 24 hours later, and seizure activity is observed. There is poor neurologic development during infancy. CT scan of the head shows symmetrically enlarged cerebral ventricles at 8 months of age. Which of the following perinatal complications most likely produced these findings?
- A.
  
  Germinal matrix hemorrhage
- B.
  
  Down syndrome
- C.
  
  Kernicterus
- D.
  
  Congenital cytomegalovirus infection
- E.
  
  Medulloblastoma
6.

A 79-year-old woman was driving her automobile when she had a sudden, severe headache. She drove to a service station, stopped the vehicle, and slumped over the wheel. She was taken to the emergency department, where she remained comatose and died 6 hours later. The gross appearance of the brain at autopsy is shown in the figure. What is the most likely diagnosis?
- A.
  
  Hemorrhage in a glioblastoma multiforme
- B.
  
  Thromboembolization with cerebral infarction
- C.
  
  Tearing of the bridging veins
- D.
  
  Rupture of a berry aneurysm
- E.
  
  Hyaline arteriolosclerosis with hemorrhage
7.

A study is conducted to identify causes of neuronal loss in patients 18 to 90 years old who died in the hospital from a natural manner of death and who had autopsies performed. Histologic sections are taken from multiple areas of the brain in each patient. The sections are analyzed for the appearance of red, shrunken neurons, decreased numbers of neurons, or absent neurons. A subset of patients is identified in which the hippocampal pyramidal cells, the cerebellar Purkinje cells, and the superior parasagittal neocortical pyramidal cells are affected. The medical records of these patients are reviewed to determine what risk factors for neuronal loss were present before death. What condition is most likely to be the major cause of neuronal loss in this subset of patients?
- A.
  
  Autoimmunity
- B.
  
  Chemotherapy
- C.
  
  Diabetes mellitus
- D.
  
  HIV infection
- E.
  
  Global hypoxia
- F.
  
  Lead ingestion
- G.
  
  Poor nutrition
8.

An 83-year-old woman slips in the bathtub in her home and falls backward, striking her head. She is taken to the emergency department, where examination shows a 3-cm, reddish, slightly swollen area over the occiput. She is arousable but somnolent. There are no motor or sensory deficits. There is no papilledema. CT scan of the head is performed. Acute hemorrhage in which of the following locations is most likely to be seen?
- A.
  
  Basis pontis
- B.
  
  Cerebral ventricle
- C.
  
  Epidural space
- D.
  
  Inferior frontal lobe
- E.
  
  Putamen
- F.
  
  Sella turcica
9.

A 36-year-old woman is involved in a motor vehicle accident. She is not wearing a safety belt and is ejected from the vehicle. When paramedics arrive, her vital signs are stable with pulse 80/min, respirations 17/min, and blood pressure 125/80 mm Hg. On examination in the emergency department, she has multiple contusions and abrasions over the skin of her head, torso, and extremities. There is no papilledema, there is no decerebrate posturing, and she has no spontaneous movements. She is not conscious. CT scan of the head shows no intracranial hemorrhage or edema and no skull fractures. She remains in a persistent vegetative state. What lesion is most likely to be present in this patient?
- A.
  
  Acute hydrocephalus
- B.
  
  Cerebellar tonsillar herniation
- C.
  
  Diffuse axonal injury
- D.
  
  Hypoxic neuronal damage
- E.
  
  Multiple cortical contusions
- F.
  
  Myelinolysis
10.

A 4-year-old girl developed clumsiness and difficulty ambulating over 6 months. On physical examination, she showed difficulty with balance while walking, dysarthria, poor hand coordination, absent deep tendon reflexes, and a bilateral Babinski sign. Light touch and vibratory sensation were greatly diminished. There was no muscular weakness. Over the next 5 years, she developed congestive heart failure from hypertrophic cardiomyopathy. She also had hyperglycemia. At autopsy, there was increased perinuclear iron deposition within cardiac myocytes. Which of the following genetic abnormalities with trinucleotide repeat expansions was most likely present in this patient?
- A.
  
  CAG repeats in the huntingtin gene
- B.
  
  CAG repeats in the spinocerebellar ataxia 7 gene
- C.
  
  CGG repeats in the FMR1 gene
- D.
  
  CTG repeats in the dystrophila myotonia-protein kinase gene
- E.
  
  GAA repeats in the frataxin gene
11.

A 63-year-old man had increasing irritability over 3 years. He spent a lot of time wandering about his neighborhood, complaining to the neighbors about everything. He had no memory loss and was always able to find his way home. The neighbors were pleased when he developed aphasia. On physical examination, there were no motor or sensory deficits and no gait disturbances or tremor. MRI of the brain showed bilateral marked temporal and frontal lobe gyral atrophy. He died of pneumonia 1 year later. At autopsy, the frontal cortex microscopically shows extensive neuronal loss, and some remaining neurons show intracytoplasmic, faintly eosinophilic, rounded inclusions that stain immunohistochemically for tau protein. What is the most likely diagnosis?
- A.
  
  Alzheimer disease
- B.
  
  Huntington disease
- C.
  
  Leigh disease
- D.
  
  Multiple system atrophy
- E.
  
  Parkinson disease
- F.
  
  Pick disease
- G.
  
  Vascular dementia
12.

A 20-year-old, mentally retarded woman saw the physician because she had flank pain for 1 week. Physical examination showed right costovertebral angle tenderness. Patches of leathery-appearing (shagreen patches) and hypopigmented (ash-leaf patches) skin were scattered over her body. There was a subungual nodule on her right index finger. Abdominal CT scan showed bilateral renal cysts and tumor masses. MRI of the brain showed subependymal nodules and 1- to 4-cm cortical foci with loss of the gray-white distinction. CT scan of the chest showed a 3-cm mass involving the interventricular septum. Two years later, she has sudden, severe headache. MRI now shows a nodule obstructing the cerebral aqueduct. Neurosurgery is performed, and a subependymal giant-cell astrocytoma is removed. What is the most likely diagnosis?
- A.
  
  Down syndrome
- B.
  
  Krabbe disease
- C.
  
  Neurofibromatosis type 1
- D.
  
  Neurofibromatosis type 2
- E.
  
  Tuberous sclerosis
- F.
  
  Von Hippel–Lindau disease
13.

A 40-year-old man, who rarely had headaches, now has been experiencing headaches for the past 6 months. He comes to the physician because of a seizure that occurred 1 day ago. On physical examination, there are no remarkable findings. MRI of the brain shows a solitary, circumscribed 3-cm mass in the right parietal centrum semiovale. The mass has small cysts and areas of calcification and hemorrhage. Neurosurgery is performed, and the mass is removed. Microscopically, the mass consists of sheets of cells with round nuclei that have granular chromatin. The cells have a moderate amount of clear cytoplasm. These cells mark with GFAP by immunohistochemical staining. The patient receives adjuvant radiation and chemotherapy, and there is no recurrence. Which of the following neoplasms was most likely present in this patient?
- A.
  
  Astrocytoma
- B.
  
  Diffuse large B-cell lymphoma
- C.
  
  Germ cell tumor
- D.
  
  Glioblastoma multiforme
- E.
  
  Medulloblastoma
- F.
  
  Metastatic renal cell carcinoma
- G.
  
  Oligodendroglioma
14.

A 46-year-old woman has had increasing weakness and loss of sensation in the lower extremities for the past 5 months. She comes to the physician because she has been unable to walk without assistance for the past week. On physical examination, there is 4/5 motor strength in the right lower extremity and 3/5 motor strength in the left lower extremity. There is bilateral loss of sensation to light touch from the lateral midthigh distally. MRI of the spine shows a 1 × 4 cm lesion in the filum terminale. The mass is removed. Microscopically, the mass is composed of cuboidal cells around papillary cores in a myxoid background. Which of the following was most likely present in this patient?
- A.
  
  Choroid plexus papilloma
- B.
  
  Ependymoma
- C.
  
  Meningioma
- D.
  
  Metastatic transitional cell carcinoma
- E.
  
  Neurofibroma
- F.
  
  Pilocytic astrocytoma
- G.
  
  Schwannoma
15.

A 52-year-old woman has had malaise for the past 6 months. On physical examination, there are no remarkable findings. Laboratory studies show hemoglobin, 9.3 g/dL; hematocrit, 27.9%; platelet count, 549,000/mm3; and WBC count, 282,000/mm3 with 64% segmented neutrophils, 10% bands, 3% metamyelocytes, 2% myelocytes, 2% lymphocytes, 1% monocytes, 9% eosinophils, and 9% basophils. She undergoes chemotherapy. Two months later, she develops neurologic deficits with ataxia, motor weakness in the right arm, difficulty swallowing, and sensory changes in the left leg. MRI of the brain shows irregular areas of increased attenuation in white matter of the cerebral hemispheres and the cerebellum. A stereotaxic biopsy specimen shows perivascular chronic inflammation, marked gliosis, large reactive astrocytes with bizarre nuclei, and intranuclear inclusions within oligodendroglia. What infection most likely caused these findings?
- A.
  
  Cytomegalovirus
- B.
  
  Herpes simplex virus
- C.
  
  JC papovavirus
- D.
  
  Rabies virus
- E.
  
  Rubeola virus
- F.
  
  West Nile virus
16.

A 39-year-old man who has been HIV-positive for at least 8 years has received no antiretroviral therapy. He has had left-sided weakness for the past month. He sees the physician 1 day after experiencing a generalized seizure. On physical examination, he is afebrile. There is 4/5 motor strength in the left upper extremity. CT scan of the head shows no intracranial hemorrhage, but there is a midline shift. MRI of the brain shows a 4-cm mass in the region of the putamen near the right internal capsule, a 3-cm mass in the right centrum semiovale, and a 1-cm mass near the splenium of the corpus callosum. These masses are circumscribed and solid. CSF from a lumbar puncture shows an elevated protein concentration and a normal glucose level. Cytologic examination shows large cells with large nuclei and scant cytoplasm that mark with CD19, but not with GFAP or cytokeratin. What is the most likely diagnosis?
- A.
  
  Cytomegalovirus encephalitis
- B.
  
  Glioblastoma multiforme
- C.
  
  Kaposi sarcoma
- D.
  
  Large B-cell lymphoma
- E.
  
  Progressive multifocal leukoencephalopathy
- F.
  
  Toxoplasmosis
17.

A 60-year-old woman had problems related to movement for 5 years. Physical examination showed cogwheel rigidity of limbs and a festinating gait, which she had difficulty initiating. Her face was expressionless. She was given levodopa, and her condition improved. Two years later, she had difficulty performing activities of daily living and showed marked cognitive decline. She died of aspiration pneumonia. Autopsy findings include mild cerebral atrophy and loss of substantia nigra pigmentation. Microscopically, there is loss of pigmented neurons, and the remaining substantia nigra neurons and cortical neurons show spheroidal, intraneuronal, cytoplasmic, and eosinophilic inclusions. Immunohistochemical staining for which of the following proteins is most likely to be positive in these inclusions?
- A.
  
  α-Synuclein
- B.
  
  Amyloid precursor protein
- C.
  
  Apolipoprotein E
- D.
  
  Huntingtin
- E.
  
  Presenilin
- F.
  
  Tau protein
18.

A 70-year-old woman comes to the physician because of an episode 2 days earlier during which she lost consciousness for several minutes. Afterward, she had difficulty speaking clearly and had paresthesias in the lower right arm that persisted for several minutes. On physical examination, there is 4/5 motor strength in the right upper extremity and decreased sensation to pinprick on the ulnar aspect of the lower right arm and hand. There are bilateral carotid bruits. A lumbar puncture is performed with normal opening pressure. Laboratory studies on 10 mL of clear, colorless CSF show two mononuclear WBCs/mm3, no RBCs, protein concentration of 40 mg/dL, and glucose concentration of 70 mg/dL. The serum glucose concentration is 95 mg/dL. CT scan of the head shows no intracranial hemorrhage, but there is a slight midline shift; MRI of the brain shows an ill-defined area of edema near the left internal capsule. Which of the following laboratory findings is most suggestive of the risk factor for this patient's disease?
- A.
  
  Positive antiphospholipid antibody test result
- B.
  
  Blood culture positive for Streptococcus pneumoniae
- C.
  
  Elevated serum concentration of very long chain fatty acids
- D.
  
  Hyperammonemia
- E.
  
  Hypercholesterolemia
- F.
  
  Oligoclonal bands on CSF electrophoresis
- G.
  
  Positive ANA test result
- H.
  
  Positive serologic test result for syphilis
19.

A 15-year-old girl is brought to the physician because she has had progressive difficulty speaking during the past 6 months. She becomes dizzy and falls frequently. She complains of headache and facial and neck pain. During the past month, she has had decreasing bladder and bowel control. On physical examination, there is loss of pain and temperature sensation over the nape of the neck, shoulders, and upper arms, but vibration and position sensation are preserved. She has muscle wasting in the lower neck and shoulders. MRI of the spinal cord shows cervical and thoracic enlargement with a CSF collection dilating the central canal. MRI of the brain shows gross findings similar to those shown in the figure. Which of the following is the most likely diagnosis?
- A.
  
  Arnold-Chiari II malformation
- B.
  
  Cerebral palsy
- C.
  
  Dandy-Walker malformation
- D.
  
  Holoprosencephaly
- E.
  
  Multiple sclerosis
- F.
  
  Polymicrogyria
20.

An infant was born at 36 weeks' gestation to a 22-year-old primigravida. A fetal screening ultrasound study at 18 weeks showed a single large cerebral ventricle and fused thalami. On physical examination at birth, the infant was small for gestational age and had multiple anomalies, including postaxial polydactyly of hands and feet, cyclopia, microcephaly, cleft lip and palate, and rocker-bottom feet. The infant died 1 hour after birth. Which of the following CNS abnormalities best explains these findings?
- A.
  
  Anencephaly
- B.
  
  Arnold-Chiari II malformation
- C.
  
  Dandy-Walker malformation
- D.
  
  Holoprosencephaly
- E.
  
  Periventricular leukomalacia
- F.
  
  Subdural hematoma
21.

An 86-year-old man has become progressively unable to live independently for the past 10 years, and he now requires assistance with bathing, dressing, toileting, feeding, and transfers in and out of chairs and bed. On physical examination, he has no motor or sensory deficits. He cannot give the current date or state where he is. Six months later, he suddenly becomes comatose and dies. At autopsy, there is a large superficial left parietal lobe hemorrhage. Histologic examination of the brain shows numerous neocortical neuritic plaques and neurofibrillary tangles. The peripheral cerebral arteries and the core of each plaque stain positively with Congo red. Which of the following mechanisms is most likely responsible for his disease?
- A.
  
  Aggregation of Aβ peptide
- B.
  
  Conformational change in the prion protein (PrP)
- C.
  
  Mutations in the tau gene
- D.
  
  Mutations in the frataxin gene
- E.
  
  Dopamine deficiency
- F.
  
  Expansion of polyglutamine repeats
22.

A 55-year-old man has experienced headaches for the first time in his life beginning 2 months ago. He comes to the emergency department following a generalized tonic-clonic seizure. MR imaging of his brain shows an ill-defined 2 cm mass in the right posterior parietal cortex. A stereotaxic biopsy of the mass microscopically shows pleomorphic cells positive for glial fibrillary acidic protein (GFAP). Molecular analysis shows abnormalities of p53 and platelet-derived growth factor-alpha (PDGF-α). He receives treatment for this lesion. Which of the following neoplasms is he most likely to develop in the future?
- A.
  
  Diffuse large B cell lymphoma
- B.
  
  Glioblastoma
- C.
  
  Hemangioblastoma
- D.
  
  Medulloblastoma
- E.
  
  Pilocytic astrocytoma