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Flashcards in XXIII - The Nervous System Deck (209)
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181

A malformation characterized by reduction in the number of gyri, agyria in extreme cases

Lissencephaly(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1257

182

Infarcts in the supratentorial periventricular white matter in premature infants presenting with chalky yellow plaques consisting of discrete regions of white matter necrosis and calcification

Perventricular leukomalacia(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1259

183

Most common site of contusions in the brain

Frontal and temporal lobes(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1260

184

Vascular injury presenting with slowly evolving neurologic symptoms, often with a delay from the time of injury(most often within 48 hours of injury) Grossly, it appears as a collection of freshly clotted blood along the brain surface, without extension into the depths of sulci.

Subdural hematoma(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1262

185

After a vehicular crash, patient presented with quadriplegia and respiratory distress. Spinal cord injury was suspected. This can be due to damage to what level of vertebra?

Above C4(TOPNOTCH)

186

CNS cells most sensitive to ischemia

Neurons(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1264

187

Clinicopathologic syndrome arising in the setting of malignant hypertension, characterized by diffuse cerebral dysfunction, including headaches, confusion, voming, and convulsion, and sometimes leading to coma

Acute hypertensive encephalopathy(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1268

188

Risk factor most commonly associated with deep brain parenchymal hemorrhages.

Hypertension(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1268

189

Most common cause of bacterial meningitis in adolescents and young adults.

Neisseria meningitidis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1272

190

Typical CSF findings in brain abscess

Increased WBC, increased protein, normal glucose(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1273

191

Most serious complications of chronic TB meningitis

Arachnoid fibrosis(producing hydrocephalus) and Obliterative endarteritis(causing arterial occlusion and infarction)(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1274

192

Morphology: perivascular accumulation or lymphocytes and/or neutrophils, multiple foci of necrosis of gray and white matter, single-cell neuronal necrosis with phagocytosis of debris, microglial nodules

Viral Encephalitis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1275

193

It produces brain abscess, most ofen in the cerebral cortex and deep gray nuclei, central necrosis, petechial hemorrhages surrounded by acute and chronic inflammation, macrophage infiltration, and vascular proliferation; CT and MRI studies may show multiple ring enhancing lesions.

Toxoplasmosis of the CNS(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1280

194

A rapidly progressive encephalitis, cerebral malaria, is the complication with the highest mortality caused by what etiologic agent?

Plasmodium falciparum(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 1280

195

HSV-1 infection has predilection to what part of the brain?

Temporal lobe(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1281

196

This group of degenerative disorders is caused by "spreading" of misfolded proteins, allowing a pathogenic protein to acquire many of the characteristics of an infectious agent. Accumulation of PrPSC in neural tissue seems to the cause of pathologic changes in these diseases.

Prion diseases(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1281

197

The most common prion disease that manifest clinically as a rapidly progressive dementia.

Creutzfeldt-Jakob Disease (TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1283

198

Pathologic hallmarks of Alzheimer disease

Plaques and tangles(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1288

199

The fundamental abnormality in this disease is the accumulation of A-beta and tau in specific brain regions.

Alzheimers Disease(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1288

200

Syndrome caused by deletions in mitochondrial DNA, characterized by ophthalmoplegia, pigmentary degeneratation of the retina, and complete heart block

Kearns-Sayre syndrome(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1245

201

Mitochondrial disoder caused by mutations in either mtDNA or the nuclear genome causing subacute necrotizing encephalopathy

Leigh syndrome(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 1245

202

A 12 year old student presents with lethargy, fever, and headaches. These progress to irritability, anxiety, and confusion, with difficulty in swallowing. He refuses any drink being offered. A few days later, he slips into a coma, and dies. On history, he was bitten by a stray dog 4 months ago. At autopsy, where would the characteristic infected neurons with cytoplasmic inclusions most likely be found? (A) frontal and parietal cerebral cortex (B) hippocampus and cerebellum (C) globus pallidus and medulla (D) pineal gland and pituitary

hippocampus and cerebellum (TOPNOTCH) Robbins Pathologic Basis of Disease 8th ed

203

A 19 year old male presents with fever and changes in mood and behavior. His neck is supple, and shows no lateralizing signs, or cranial nerve defects. Memory is impaired. A lumbar tap is performed with a slight lymphocytosis and protein elevation. Bacterial cultures were negative. On PCR, herpes simplex virus I was detected. THe histologic changes of necrotizing inflammation and Cowdry Type A bodies would most likely be found in (A) temporal lobes (B) occipital lobes (C) cerebellar vermis (D) parietal lobes

temporal lobes (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 876

204

A 78 year old woman in a nursing home is found dead in her bed. She has had a 5 year history of progressive disorientation, memory loss, and alteration of mood and behavior. In her final year, she was mute and could not perform activities of daily living without assistance. Her relatives requested an autopsy, and her brain showed cortical atrophy with widening of the sulci. Histology showed multiple plaques and neurofibrillary tangles. The plaques are collections of neuritic processes with a central core of extracellular (A) amyloid (B) tau protein (C) glycogen (D) sphingomyelin

amyloid (Alzheimer disease) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 893

205

A 45 year old actor begins to experience hand tremors, and eventually develops a shuffling gait, a stooped posture, and diminished facial expressions. His mental faculties are intact. What is the expected histologic change in his brain? (A) loss of pigmented catecholaminergic neurons in his midbrain (B) loss of neurons in the striatum (C) foci of hemorrhage and necrosis in the mamillary bodies (D) multiple Lewy bodies in his cerebral cortex

loss of pigmented catecholaminergic neurons in his midbrain (pallor of substantia nigra in Parkinsonism) (B - Huntington disease; C - Wernicke encephalopathy; D - Lewy body dementia/disease) (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 893-894

206

A 30 year old saleslady has untreated hypertension. While at work, her colleagues heard her complain of an excruciating headache then lose consciousness a few seconds later. She was pronounced dead at the ER. An autopsy is performed, which showed massive subarachnoid hemorrhage, and a ruptured aneurysm at the right anterior cerebral artery. Her kidneys were markedly enlarged, with multiple 3-4 cm diameter cysts. Which of the following is accurate? (A) the aneurysm is neoplastic (B) the aneurysm was likely present at birth (C) her kidney disease is heritable, in an autosomal dominant fashion (D) her kidney disease more commonly manifests in early childhood

her kidney disease is heritable, in an autosomal dominant fashion (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 569-570, 866-867

207

A 26 year old backpacker presents with seizures. On imaging, there are four 1-2 cm cysts in his cerebral cortex. He undergoes surgery, where two were removed. Histopathology showed an ovoid structure with a tegument, underlying smooth muscle, three oval suckers, all surrounded by an intense inflammmatory infiltrate of plasma cells, lymphocytes, and eosinophils. The surrounding brain tissue is gliotic. The patient acquired this infection by ingesting (A) raw pork (B) raw beef (C) ova from cat litter (D) raw freshwater crabs

raw pork (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 880

208

A 33 year old woman has been having intermittent headache for 3 years. She develops a seizure. Imaging studies show a 4 cm well circumscribed intracranial mass at the convexity of the right cerebral cortex, compressing the adjacent brain. The tumor is surgically excised, and histopathology showed cells in syncytial whorls, some in fascicles. There are occasional concentric calcifications interspersed. There is no significant mitosis, necrosis, pleomorphism, or brain invasion. Her tumor is (A) a metastasis from a thyroid primary (B) a primary lymphoma (C) a glioma (D) a meningioma

a meningioma (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 886

209

A 40 year old lawyer presents with seizures and loss of consciousness. On imaging, there is a contrast-enhancing, intracranial mass straddling the corpus callosum, with a "butterfly" appearance. An open biopsy is performed, which showed a densely cellular tumor of with astrocytic cell processes, foci of necrosis with surrounding pseudo-palisading of tumor cell nuclei, vascular proliferation, and numerous mitoses. His tumor (A) is a glioma (B) is poorly circumscribed (C) confers a dire prognosis (D) all of the above

all of the above (TOPNOTCH)Robbins Basic Pathology, 8th ed., p 882