Pathology Review Ch 28 CNS Prt 3

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  • 1/22 Questions
    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? - ProProfs

    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?

    • Hemorrhage in a glioblastoma multiforme
    • Thromboembolization with cerebral infarction
    • Tearing of the bridging veins
    • Rupture of a berry aneurysm
    • Hyaline arteriolosclerosis with hemorrhage
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Pathology Review Ch 28 CNS Prt 3 - Quiz
About This Quiz

This quiz, titled 'Pathology Review ch 28 CNS prt 3', delves into various central nervous system pathologies through clinical scenarios. It assesses knowledge in diagnosing CNS conditions, relevant to medical students and professionals preparing for board exams.

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  • 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?

    • Dandy-Walker malformation

    • Spina bifida occulta

    • Tuberous sclerosis

    • Arnold-Chiari malformation

    • Meningomyelocele

    Correct Answer
    A. Spina bifida occulta
    Explanation
    (B) Spina bifida occulta is the mildest form of neural tube defect. It is characterized by defective closure of the vertebral
    arches with intact meninges and spinal cord. A radiograph may show its presence in 20% of the population. Dandy-Walker
    malformation is detected by ultrasound evidence of a cyst in the fourth ventricle and agenesis of the cerebellar vermis.
    Tuberous sclerosis is a rare disease that causes firm hamartomatous “tubers” in the cortex. In an Arnold-Chiari
    malformation, there is a small posterior fossa and extension of the cerebellum into the foramen magnum and a lumbar
    meningomyelocele. Meningomyeloceles are open neural tube defects.
    BP7 821–822BP8 872PBD7 1354PBD8 1284

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  • 3. 

    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?

    • Amyotrophic lateral sclerosis

    • Huntington disease

    • Multiple sclerosis

    • Guillain-Barré syndrome

    • Parkinson disease

    Correct Answer
    A. Amyotrophic lateral sclerosis
    Explanation
    (A) The progressive and symmetric nature of this patient's disease is a classic feature of amyotrophic lateral sclerosis
    (ALS). The muscles show a denervation type of grouped atrophy from loss of upper motor neurons. The “bulbar” form of
    ALS affects mainly cranial nerve nuclei and has a more aggressive course. Huntington disease causes abnormal
    movements, not weakness, and there can be associated dementia over time. Mental function is preserved in ALS. The
    demyelinating plaques of multiple sclerosis can produce various motor signs and symptoms over time, but symmetry is not
    a feature of this disease. Guillain-Barré syndrome usually occurs after a viral infection and manifests as a rapidly
    progressive ascending motor weakness. Parkinson disease is characterized by rigidity and involuntary movements, not by
    muscular weakness.
    BP7 846BP8 896PBD7 1396–1397PBD8 1324–1325

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  • 4. 

    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?

    • Aggregation of Aβ peptide

    • Conformational change in the prion protein (PrP)

    • Mutations in the tau gene

    • Mutations in the frataxin gene

    • Dopamine deficiency

    • Expansion of polyglutamine repeats

    Correct Answer
    A. Aggregation of Aβ peptide
    Explanation
    (A) This patient had Alzheimer disease (AD) complicated by cerebral amyloid angiopathy and terminal hemorrhagic
    stroke. Formation and aggregation of the Aβ peptide is now considered central to the pathogenesis of AD. Aβ peptide is
    derived from abnormal processing of amyloid precursor protein (APP). When APP, a transmembrane protein, is cleaved by
    α-secretase within the Aβ sequence, followed by γ-secretases, a soluble nontoxic fragment is formed. Cleavage of the Aβ
    sequence by β-secretase and then γ-secretase gives rise to Aβ peptides that aggregate and form the amyloid cores that
    elicit a microglial and astrocytic response to form neuritic plaques. Conformational change in prion protein leads to
    Creutzfeldt-Jakob disease, a rapidly progressive dementia with spongiform encephalopathy but not neuritic plaques or
    amyloid deposition. Although abnormally phosphorylated forms of tau protein are found in neurofibrillary tangles seen in AD, there is no mutation of the tau gene, and the tangles are not considered primary in the pathogenesis of AD. Mutation
    of the tau gene leads to frontotemporal dementia with parkinsonism. Mutations in the frataxin gene lead to Friedreich
    ataxia, an autosomal recessive progressive illness, generally beginning in the first decade of life. Loss of dopaminergic
    neurons with deficiency of dopamine is central to the pathogenesis of Parkinson disease. Expansion of polyglutamine
    repeats owing to CAG trinucleotide repeat-expansion underlies Huntington disease.
    BP7 841–843BP8 891–893PBD7 1386–1389PBD8 1313–1317

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  • 5. 
    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? - ProProfs

    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?

    • Arnold-Chiari II malformation

    • Cerebral palsy

    • Dandy-Walker malformation

    • Holoprosencephaly

    • Multiple sclerosis

    • Polymicrogyria

    Correct Answer
    A. Arnold-Chiari II malformation
    Explanation
    (A) This patient has an Arnold-Chiari II malformation, which results in a small posterior fossa, misshapen midline
    cerebellum with downward displacement of the vermis, and tenting of the tectal plate. MRI in this case is characteristic of
    hydromyelia. Cerebral palsy is a general term describing nonprogressive motor deficits that are present from birth. Dandy-
    Walker malformation includes hypoplasia or absence of the cerebellar vermis and cystic enlargement of the posterior
    fossa. Holoprosencephaly is a severe malformation with total (alobar) or incomplete (semilobar) separation of the cerebral
    hemispheres in brain development. Multiple sclerosis results in white matter plaques of demyelination, not malformation.
    Polymicrogyria is characterized by numerous, small, irregularly formed gyral contours.
    BP7 823BP8 872PBD7 1355–1356PBD8 1285–1286

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  • 6. 

    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?

    • α-Synuclein

    • Amyloid precursor protein

    • Apolipoprotein E

    • Huntingtin

    • Presenilin

    • Tau protein

    Correct Answer
    A. α-Synuclein
    Explanation
    (A) This patient has findings of dementia with Lewy bodies (DLB), a form of dementia with clinical features similar to
    those of Alzheimer disease and idiopathic Parkinson disease. Mutations in the gene for α-synuclein have been linked to
    idiopathic Parkinson disease, and Lewy bodies can be found in the substantia nigra neurons, but the clinical dementia and
    cortical Lewy bodies point to DLB. Amyloid precursor protein is encoded by a gene on chromosome 21 (perhaps
    explaining early Alzheimer disease in trisomy 21) and is processed to form the Aβ amyloid of neuritic plaques in Alzheimer
    disease. The e4 allele of apolipoprotein E can bind Aβ and increase the risk of Alzheimer disease. Huntingtin is the protein
    product of the HD gene in Huntington disease. Presenilin 1 and 2 mutations can increase production of Aβ and increase
    the risk of early-onset Alzheimer disease. Tau protein is found in neurofibrillary tangles of Alzheimer disease and in Pick
    bodies of Pick disease.
    BP7 844BP8 891, 895PBD7 1386, 1392PBD8 1321–1322

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  • 7. 

    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?

    • Alzheimer disease

    • Huntington disease

    • Leigh disease

    • Multiple system atrophy

    • Parkinson disease

    • Pick disease

    • Vascular dementia

    Correct Answer
    A. Pick disease
    Explanation
    (F) Pick disease has clinical features similar to the features of Alzheimer disease, but initially it causes less memory
    loss and more behavioral changes. The “knifelike” gyral atrophy of frontal and temporal lobes and relative sparing of
    parietal and occipital lobes are characteristic of Pick disease. Pick bodies are seen in remaining neurons, but the neuritic
    plaques and neurofibrillary tangles seen in Alzheimer disease are not increased. Huntington disease affects mainly the
    caudate nuclei and basal ganglia; onset occurs in middle age, and choreiform movements are common. Leigh disease is a mitochondrial encephalomyopathy that can cause muscular weakness and neurologic deterioration beginning at a young
    age. Multiple system atrophy (MSA) has features that overlap those of striatonigral degeneration, olivopontocerebellar
    atrophy, and Shy-Drager syndrome; most patients with MSA exhibit symptoms similar to those of Parkinson disease. MSA
    is characterized microscopically by the appearance of glial cytoplasmic inclusions. In Parkinson disease, loss of pigmented
    neurons in the substantia nigra leads to movement problems. Vascular dementia, or multi-infarct dementia, can have
    clinical features that mimic those of Alzheimer disease, but there are multiple small infarcts that collectively produce
    dementia, and the neurologic decline occurs in a stepwise fashion.
    BP7 843–844BP8 891PBD7 1390PBD8 1318

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  • 8. 

    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?

    • No organisms

    • Gram-positive cocci

    • Gram-negative diplococci

    • Short Gram-positive rods

    • Short Gram-positive rods

    Correct Answer
    A. Gram-positive cocci
    Explanation
    (B) This patient has acute bacterial meningitis. In his age group, the most common etiologic organism is now
    Streptococcus pneumoniae, a gram-positive coccus. The number of CNS infections with Haemophilus influenzae, a gramnegative
    bacillus, in this age group has decreased because of widespread immunization. Pneumococci also are likely to
    be seen in an adult. The gram-negative diplococci of Neisseria meningitidis are seen in young adults. The short, grampositive
    rods of Listeria monocytogenes appear sporadically or in epidemics caused by food contamination. The gramnegative
    bacilli of Escherichia coli are most often seen in neonates. With these clinical and CSF features, it would be
    unusual if bacteria were not present in the CSF.
    BP7 825–827BP8 874–875PBD7 1369–1370PBD8 1299–1300

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  • 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?

    • Acute hydrocephalus

    • Cerebellar tonsillar herniation

    • Diffuse axonal injury

    • Hypoxic neuronal damage

    • Multiple cortical contusions

    • Myelinolysis

    Correct Answer
    A. Diffuse axonal injury
    Explanation
    (C) Angular acceleration with torsional forces applied to white matter tracts can cause stretching and tearing of axons,
    followed by axonal swelling or “retraction balls” within hours. Acute hydrocephalus requires a blockage of CSF flow, and
    there is no mass lesion, hemorrhage, or brain swelling in this case. Herniation requires brain swelling or a large
    hemorrhage or a mass that enlarges rapidly. Hypoxic injury is most likely to affect neurons of the hippocampus, Purkinje
    cells, and cortical neurons in watershed areas first; the patient's vital signs suggest that this did not occur. Contusions
    represent areas of superficial cortical hemorrhage. Central pontine myelinolysis can occur from rapid correction of
    hyponatremia.
    BP7 819–821BP8 869PBD7 1358–1359PBD8 1288

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  • 10. 

    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?

    • Autoimmunity

    • Chemotherapy

    • Diabetes mellitus

    • HIV infection

    • Global hypoxia

    • Lead ingestion

    • Poor nutrition

    Correct Answer
    A. Global hypoxia
    Explanation
    (E) The neurons that are most sensitive to anoxia reside in the hippocampus, along with the Purkinje cells and the
    larger neocortical neurons. In addition, the first areas in the neocortex to be affected are the “watershed” areas between
    the three major cerebral circulations, including the watershed located superiorly between the anterior cerebral and middle
    cerebral circulations, as in this study. “Red” shrunken neurons, especially in the areas mentioned, are typically seen in the
    early stages of global hypoxia, as may occur in a severe hypotensive episode. Focal hypoxia leads to infarcts.
    Autoimmunity can lead to vasculitis and subsequent hypoxemia. Chemotherapy tends to damage actively dividing cells
    more severely. Diabetes mellitus causes atherosclerosis, which can lead to hypoxemia. HIV infection can produce an
    encephalitis, and there can be opportunistic infections of the CNS. Lead poisoning leads to encephalopathy, not ischemia.
    Poor nutrition with a deficiency of thiamine (vitamin B1) can lead to Wernicke disease, which affects the mamillary bodies
    and periaqueductal gray matter most severely.
    BP7 813–814BP8 863–865PBD7 1350–1351PBD8 1291–1292

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  • 11. 

    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?

    • Cytomegalovirus encephalitis

    • Glioblastoma multiforme

    • Kaposi sarcoma

    • Large B-cell lymphoma

    • Progressive multifocal leukoencephalopathy

    • Toxoplasmosis

    Correct Answer
    A. Large B-cell lymphoma
    Explanation
    (D) Non-Hodgkin lymphomas, including large B-cell lymphomas, are uncommon in the brain, but they may be seen in
    immunocompromised patients, particularly patients with AIDS. They tend to be multifocal. Cytomegalovirus infection is
    common in AIDS, but it is unlikely to produce mass lesions. A glioblastoma multiforme can be a large infiltrative and
    destructive mass, but the cells are typically GFAP positive and CD19 negative. Kaposi sarcoma can occur in association
    with HIV infection, but CNS involvement is rare, and the cells are CD34 positive. Progressive multifocal
    leukoencephalopathy produces granular white matter lesions with large, bizarre oligodendrocytes infected with JC
    papovavirus. Toxoplasmosis can produce multiple mass lesions, but they are chronic abscesses that are filled with necrotic material and surrounded by gliosis; Toxoplasma pseudocysts may be found in the abscesses.
    BP7 835BP8 885PBD7 1408PBD8 1337

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  • 12. 

    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?

    • Diffuse large B cell lymphoma

    • Glioblastoma

    • Hemangioblastoma

    • Medulloblastoma

    • Pilocytic astrocytoma

    Correct Answer
    A. Glioblastoma
    Explanation
    (B) He initially had an infiltrating astrocytoma that is the most common primary brain neoplasm in adults, typically
    arising in a cerebral hemisphere. Lower grade astrocytomas in adults may have a more indolent course. Some of these
    patients go on to develop a high-grade glioma known as “secondary” glioblastoma. Most glioblastomas arise de novo
    (“primary glioblastoma”) and have MDM2 mutations. Regardless of origin, glioblastomas are aggressive and have a poor
    prognosis. Diffuse large B cell lymphoma is the most common type of “primary” CNS lymphoma (without evidence for
    disease elsewhere); some arise in immunocompromised patients. Hemangioblastomas are uncommon neoplasms arising
    in the cerebellum, often with von Hippel-Lindau disease, and associated with polycythemia. Medulloblastomas and
    pilocytic astrocytomas are usually childhood brain tumors arising in the posterior fossa.
    BP7 832–833BP8 882PBD7 1401–1403PBD8 1330–1332

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  • 13. 

    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?

    • Plaques of demyelination in periventricular white matter

    • Neocortical senile plaques and neurofibrillary tangles

    • Multiple lacunar infarcts in basal ganglia

    • Spongiform change involving cerebellum and neocortex

    • White matter microglial nodules with multinucleate cells

    Correct Answer
    A. White matter microglial nodules with multinucleate cells
    Explanation
    (E) This patient has AIDS dementia complex late in the course of HIV infection. HIV-1 produces an encephalitis that is
    characterized by a collection of reactive microglial cells (microglial nodules). HIV-1-infected mononuclear cells, particularly
    macrophages, can fuse to form multinucleate cells, which are seen in association with microglial nodules. Plaques of
    demyelination are typical of multiple sclerosis. Senile plaques and tangles are typical of Alzheimer disease and are unlikely
    to manifest at this patient's age. Lacunar infarcts are small, cavitary infarcts that result from arteriolosclerosis of the deep
    penetrating arteries and arterioles. Such arteriolar lesions occur in individuals with long-standing hypertension and are
    unlikely to be found in a 37-year-old man. Spongiform change suggests Creutzfeldt-Jakob disease, a rapidly progressive
    dementia unrelated to HIV infection.
    BP7 829–830BP8 877–878PBD7 1375–1376PBD8 1305

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  • 14. 

    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?

    • Anencephaly

    • Arnold-Chiari II malformation

    • Dandy-Walker malformation

    • Holoprosencephaly

    • Periventricular leukomalacia

    • Subdural hematoma

    Correct Answer
    A. Holoprosencephaly
    Explanation
    (D) Holoprosencephaly is a midline defect in which there is absent (alobar) or partial (semilobar) cerebral hemispheric
    development. It can occur in trisomy 13, as in this case, with other midline defects. It also may be seen in cases of
    maternal diabetes mellitus. Anencephaly is the absence of a fetal cranial vault, which leads to absence of most of the
    brain. Arnold-Chiari II malformation results in a small posterior fossa, a misshapen midline cerebellum with downward
    displacement of the vermis, and tenting of the tectal plate. Dandy-Walker malformation is characterized by aplasia or
    hypoplasia of the cerebellar vermis, cystic enlargement of the fourth ventricle, and hydrocephalus. Periventricular
    leukomalacia is a form of perinatal injury that is caused by hypoxic-ischemic events or infections. A subdural hematoma
    results from trauma.
    BP7 823BP8 872PBD7 1355PBD8 1285

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  • 15. 

    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?

    • Positive antiphospholipid antibody test result

    • Blood culture positive for Streptococcus pneumoniae

    • Elevated serum concentration of very long chain fatty acids

    • Hyperammonemia

    • Hypercholesterolemia

    • Oligoclonal bands on CSF electrophoresis

    • Positive ANA test result

    • Positive serologic test result for syphilis

    Correct Answer
    A. Hypercholesterolemia
    Explanation
    (E) This patient has findings consistent with an acute cerebral infarction from obstruction of blood flow causing focal
    cerebral ischemia. After 2 days, there would be some cerebral softening and edema with ischemia of neurons, but little
    else. Hypercholesterolemia is a risk factor for atherosclerosis, which is the major cause of thrombotic cerebral arterial
    occlusions. The antiphospholipid syndrome can produce thrombotic and embolic disease, but an embolic “stroke” is
    typically hemorrhagic, and antiphospholipid syndrome is uncommon at this age. A positive blood culture suggests sepsis
    with the possibility of meningitis or cerebral abscess formation, but abscesses typically have ring enhancement on CT
    scans. Elevated serum levels of very long chain fatty acids are present in patients with adrenoleukodystrophy, a rare
    disorder that leads to myelin loss at an early age. Hyperammonemia occurs in hepatic encephalopathy with liver failure; it
    produces Alzheimer type II gliosis, but no focal or gross lesions. Oligoclonal bands are characteristic of multiple sclerosis,
    which typically has an onset in youth or middle age, with formation of plaques of demyelination in white matter. The ANA
    test result is positive in many autoimmune diseases and may be accompanied by vasculitis, which can produce scattered
    areas of ischemic injury, resulting in a clinical picture of encephalopathy. Neurosyphilis is now rare; it does not produce
    focal ischemic lesions.
    BP7 814–815BP8 863–865PBD7 1361–1365PBD8 1292–1295

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  • 16. 

    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?

    • CAG repeats in the huntingtin gene

    • CAG repeats in the spinocerebellar ataxia 7 gene

    • CGG repeats in the FMR1 gene

    • CTG repeats in the dystrophila myotonia-protein kinase gene

    • GAA repeats in the frataxin gene

    Correct Answer
    A. GAA repeats in the frataxin gene
    Explanation
    (E) She had Friedreich ataxia, an autosomal recessive progressive illness that most often has an onset in the first
    decade of life. The frataxin gene encodes for a protein involved in iron regulation in cells. The other options listed have no
    cardiac involvement. Mutations of the huntingtin gene are seen with Huntington disease marked by choreoathetosis
    beginning in young to middle-aged adults. There are several forms of spinocerebellar ataxia. Increased tandem repeats in
    the FMR1 gene account for cases of fragile X syndrome characterized by mental retardation. The dystrophila myotonia–
    protein kinase gene is abnormal in cases of myotonic dystrophy with muscular weakness and dementia.
    BP8 410, 896PBD8 1323

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  • 17. 

    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?

    • Cytomegalovirus

    • Herpes simplex virus

    • JC papovavirus

    • Rabies virus

    • Rubeola virus

    • West Nile virus

    Correct Answer
    A. JC papovavirus
    Explanation
    (C) This patient has progressive multifocal leukoencephalopathy, which is caused by the JC papovavirus and occurs in
    immunocompromised individuals, including individuals with AIDS. The patient was treated for chronic myelogenous
    leukemia. Cytomegalovirus infection also complicates the course of immunocompromised patients, but it causes large
    intranuclear inclusions, most often in endothelial cells. Herpes simplex virus is uncommon, even in immunocompromised
    patients, and it most often produces a hemorrhagic encephalitis in temporal lobes. Rabies virus produces CNS excitability
    with convulsions, meningismus, and hydrophobia. Subacute sclerosing panencephalitis is a rare complication of measles
    (rubeola) virus infection and leads to progressive mental decline, spasticity, and seizures. West Nile virus, similar to many
    arboviruses, can cause a meningoencephalitis.
    BP7 830BP8 878–879PBD7 1376–1377PBD8 1305–1306

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  • 18. 

    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?

    • Choroid plexus papilloma

    • Ependymoma

    • Meningioma

    • Metastatic transitional cell carcinoma

    • Neurofibroma

    • Pilocytic astrocytoma

    • Schwannoma

    Correct Answer
    A. Ependymoma
    Explanation
    (B) This patient had the myxopapillary variant of ependymoma, which is more common in adults than in children. The
    tumors that arise in the ventricles (usually the fourth ventricle) are more common in the first 2 decades of life. Choroid
    plexus papillomas are rare tumors that arise in the cerebral ventricles. Meningiomas most often arise in the cranial cavity
    and have plump, pink, round to spindle-shaped cells, often with psammoma bodies. Metastases to the spinal cord are
    uncommon. Neurofibromas are more likely to arise in peripheral nerves, although in neurofibromatosis type 1, they can
    arise in many sites. A pilocytic astrocytoma is a common pediatric primary intracranial neoplasm; it arises in the posterior
    fossa. A schwannoma most often occurs in cranial nerve VIII, although neurofibromatosis may be associated with multiple
    schwannomas.
    BP7 834BP8 884PBD7 1405PBD8 1334–1335

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  • 19. 

    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?

    • Astrocytoma

    • Diffuse large B-cell lymphoma

    • Germ cell tumor

    • Glioblastoma multiforme

    • Medulloblastoma

    • Metastatic renal cell carcinoma

    • Oligodendroglioma

    Correct Answer
    A. Oligodendroglioma
    Explanation
    (G) Oligodendrogliomas tend to have a better prognosis than most other glial neoplasms. Astrocytomas tend to be less
    circumscribed. Diffuse large B-cell lymphomas can occur in association with AIDS; they are negative for glial fibrillary
    acidic protein (GFAP), but positive for CD19 and CD20. Intracranial germ cell tumors are most likely to arise in the pineal
    body. Glioblastomas are highly aggressive, infiltrative gliomas. Medulloblastomas are posterior fossa tumors that occur in
    children. Renal cell carcinoma has cells with clear cytoplasm, but it is not positive for GFAP and often produces multiple
    metastases. In rare cases, removal of a solitary metastasis of renal cell carcinoma can be beneficial.
    BP7 833–834BP8 883–884PBD7 1404PBD8 1333–1334

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  • 20. 

    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?  

    • Basis pontis

    • Cerebral ventricle

    • Epidural space

    • Inferior frontal lobe

    • Putamen

    • Sella turcica

    Correct Answer
    A. Inferior frontal lobe
    Explanation
    (D) This patient has the classic “contrecoup” type of injury, in which the moving head strikes an object, and the force is
    transmitted to the opposite side of the head. A fall backward is most likely to produce contusions to the inferior frontal
    lobes, temporal tips, and inferior temporal lobes. A blow to a stationary head is more likely to produce a “coup” injury
    directly adjacent to the site of the blow. Hemorrhage into the pons is typical of a Duret hemorrhage, seen in medial
    temporal lobe herniation. Hemorrhage into the cerebral ventricles may occur in premature infants from germinal matrix
    hemorrhage; in adults, it is uncommon, but may occur with dissection of blood from an intraparenchymal lesion. A blow to
    the side of the head is more likely to lacerate the middle meningeal artery and produce an epidural hematoma. Putaminal
    hemorrhage is most likely to occur in hypertension. Subarachnoid hemorrhage at the base of the brain may dissect into the
    sella.
    BP7 819–821BP8 869PBD7 1357–1358PBD8 1287–1288

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  • 21. 

    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?

    • Germinal matrix hemorrhage

    • Down syndrome

    • Kernicterus

    • Congenital cytomegalovirus infection

    • Medulloblastoma

    Correct Answer
    A. Germinal matrix hemorrhage
    Explanation
    (A) Germinal matrix hemorrhage is the most common cause of intraventricular hemorrhage in premature infants. The
    germinal matrix, composed of highly vascularized tissue with primitive cells, is most prominent between 22 and 30 weeks
    of gestation. Hemorrhages within this area readily occur with common neonatal problems such as hypoxemia, hypercarbia,
    acidosis, and changes in blood pressure. Hemorrhages in the germinal matrix can extend into the cerebral ventricles and
    from there into the subarachnoid space. Smaller hemorrhages can resolve without sequelae. With larger hemorrhages,
    organization of the blood in the aqueduct of Sylvius or the fourth ventricle or foramina of Luschka may obstruct the flow of
    CSF, producing hydrocephalus. Infants with Down syndrome may have vascular malformations that can bleed into the
    parenchyma. The bilirubin staining of kernicterus does not result in scarring. Cytomegalovirus infection can cause
    considerable necrosis of the brain parenchyma, but not hemorrhage. Medulloblastomas are tumors that occur in children
    (not typically in infants) that could cause obstruction of CSF flow.
    BP7 824BP8 873PBD7 1356PBD8 1286

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  • 22. 

    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?

    • Down syndrome

    • Krabbe disease

    • Neurofibromatosis type 1

    • Neurofibromatosis type 2

    • Tuberous sclerosis

    • Von Hippel–Lindau disease

    Correct Answer
    A. Tuberous sclerosis
    Explanation
    (E) Tuberous sclerosis is one of the phakomatoses—rare inherited disorders in which hamartomas and neoplasms
    develop throughout the body, along with cutaneous abnormalities. Patients with tuberous sclerosis have cortical tubers,
    which are hamartomas of neuronal and glial tissue; other characteristic findings include renal angiomyolipomas, renal
    cysts, subungual fibromas, and cardiac rhabdomyomas. In Down syndrome (trisomy 21), patients may develop acute
    leukemia, but not brain neoplasms, and individuals who survive to middle age develop Alzheimer disease. Krabbe disease
    is a leukodystrophy that results in deficiency of galactocerebroside β-galactosidase and an onset of neurologic
    deterioration in infancy. Neurofibromatosis type 1 is characterized by deforming cutaneous and visceral neurofibromas,
    cutaneous café-au-lait spots, and neurofibrosarcomas. In neurofibromatosis type 2, acoustic schwannomas, meningiomas,
    gliomas, and ependymomas are present. Von Hippel–Lindau disease is characterized by hemangioblastomas in the
    cerebellum, retina, and spinal cord, and by pheochromocytomas.
    BP7 821BP8 901PBD7 1413PBD8 1342–1343

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  • Mar 16, 2023
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