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Neuropathology Flashcards

1
Q

What is fluid collection within the extravascular space?

A

Cerebral Edema

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2
Q

What is excessive CSF accumulation that expands the ventricular system and increases ICP?

A

Hydrocephalus

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3
Q

What is the increase in CSF due to loss of brain parenchyma called?

A

Ex Vacuo

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4
Q

What type of herniation is associated with one-sided cerebral mass effect and what does is compress?

A

Subfalcine Herniation (cingulate gyrus compression against falx cerebri; Anterior Cerebral a.

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5
Q

What type of herniation causes compression of the medial aspect of the temporal lobe against the tentorium and what type of hemorrhage can it cause?

A

Transtentorial (Uncal) Herniation; Duret hemorrhage (midbrain and upper pons; tearing of upper branches of basilar a.)

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6
Q

What herniation is a lumbar puncture contraindicated for?

A

Tonsillar Herniation (cerebellar tonsils herniate downward through foramen magnum)

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7
Q

What is protrusions of brain/meninges through openings in the skull?

A

Encephalocele (Cranium Bifidum)

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8
Q

What is malformation of the anterior end of the neural tube?

A

Anancephaly (absence of brain and skull; “frog-like” appearance)

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9
Q

What does excess cells exiting the proliferating pool too early cause?

A

Microcephaly

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10
Q

What does too few cells exiting during early divisions cause?

A

Megalencephaly

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11
Q

What is the complete absence of migration from the ventricles?

A

Subependymal (Periventricular) Heterotopia

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12
Q

What is partial migration to the neocortex?

A

Lissencephaly (no normal cortical layers formed)

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13
Q

What is it called when there is disruption during the late stage of migration and cortical organization?

A

Polymicrogyria (cobblestone appearance)

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14
Q

What is the absence of the forebrain division into two hemispheres?

A

Holoprosencephaly

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15
Q

What is the congenital failure of development of the cerebellar vermis?

A

Dandy-Walker Malformation

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16
Q

What posterior malformation is associated with meningomyelocele and syringomyelia?

A

Arnold-Chiari Malformation

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17
Q

What is dilatation of the spinal canal by CSF?

A

Hydromyelia

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18
Q

What is a fluid-filled cavity (syrinx) within the spinal cord causing cystic degeneration called?

A

Syringomyelia

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19
Q

What are racoon eyes and battle’s sign associated with?

A

Basal Skull Fractures

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20
Q

What is an incomplete fracture of the skull called?

A

Greenstick Fracture

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21
Q

What is dementia pugilistica associated with?

A

Repetitive concussions

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22
Q

What are plaque jaune (old injuries showing retracted brownish appearance at gyral crests) associated with?

A

Contusions

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23
Q

What cerebrovascular injury is associated with a lucid period often preceding neurological signs?

A

Epidural Hematoma

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24
Q

What is generalized reduction of cerebral perfusion?

A

Global Cerebral Ischemia

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25
Q

What is associated with bilateral hippocampal damage resulting in anterograde amnesia and less severe retrograde amnesia?

A

Korsakoff’s Amnesia

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26
Q

What type of focal cerebral ischemia often causes nonhemorrhagic infarcts (pale infarcts)?

A

Thrombotic Stroke (most commonly caused by atherosclerosis)

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27
Q

Where are common sites of thrombotic infarcts?

A

Carotid bifurcation, middle cerebral artery, basilar artery

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28
Q

What type of focal cerebral ischemia often causes hemorrhagic infarcts and what is the reason?

A

Embolic Stroke; due to reperfusion injury

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29
Q

What are common sites of embolic infarcts and where do they originate from?

A

Middle cerebral artery; left side of heart, carotid arteries, paradoxical

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30
Q

What are small dilations at small artery bifurcations and what are they most commonly associated with?

A

Charcot-Bouchard Microaneurysms; hypertension

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31
Q

What are small non-cortical infarcts caused by occlusion of a penetrating branch of a large cerebral artery?

A

Lacunar Infarcts

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32
Q

What causes fibrinoid necrosis/thrombosis of arterioles and capillaries leading to microinfarcts and microhemorrhages?

A

Acute Hypertensive Encephalopathy (due to increased ICP due to elevated diastolic pressure)

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33
Q

What is associated with amyloid fibrils deposited in walls of leptomeningeal and cortical arteries?

A

Cerebral Amyloid Angiopathy (CAA)

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34
Q

What is the most common aneurysm of the brain and where does it occur?

A

Berry Aneurysm (subarachnoid hemorrhage); circle of willis

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35
Q

What CNS infection is associated with headache, fever, and nuchal rigidity?

A

Meningitis

36
Q

What type of meningitis is associated with neutrophils, increased serum protein, and decreased serum glucose in the CSF?

A

Bacterial/Pyogenic Meningitis

37
Q

What is diffuse inflammation of brain tissue?

A

Encephalitis

38
Q

What type of encephalitis is associated with Cowdry type A bodies?

A

HSV-1 (kids and teens)

39
Q

What type of encephalitis is associated with Negri bodies?

A

Rabies virus

40
Q

What disease has a rapid clinical course causing dementia and death within 7 months?

A

Prion Disease

41
Q

What type of prion disease is associated with spongiform transformation of cerebral cortex and caudate, putamen and formation of vacuoles within the neuropil?

A

Creutzfeldt-Jakob Disease

42
Q

What disease is associated with oligoclonal bands?

43
Q

What disease is associated with a postinfectious autoimmune reaction to myelin?

A

Acute Disseminated Encephalomyelitis (ADEM)

44
Q

What disease is associated with a JC Polyoma viral infection?

A

Progressive Multifocal Leukoencephalopathy

45
Q

Progressive Multifocal Leukoencephalopathy is almost always found in what type of individuals?

A

HIV patients

46
Q

This disease is thought to be due to oligodendrocyte injury related to edema induced by sudden changes in osmotic pressure.

A

Central Pontine Myelinolysis

47
Q

This disease may present with rapidly evolving quadriplegia.

A

Central Pontine Myelinolysis

48
Q

What disease has the most extensive injury involving proximal motor nerve roots?

A

Guillain-Barre Syndrome

49
Q

What is the most common leukodystrophy?

A

Metachromatic Leukodystrophy

50
Q

Metachromatic Leukodystrophy involves a deficiency in what enzyme?

A

Arylsulfatase A

51
Q

What disease is associated with Globoid cells?

A

Krabbe Disease

52
Q

What x-linked disease is associated with impaired degradation of long chain FAs?

A

Adrenoleukodystrophy

53
Q

What x-linked disease is associated with mutation in a gene encoding two myelin proteins?

A

Pelizaeus-Merzbacher Disease

54
Q

What disease results in the accumulation of N-acetyl-L-aspartic acid (NAA)?

A

Canavan Disease

55
Q

What are the cardinal symptoms of thiamine deficiency?

A

Polyneuropathy, Edema, Cardiac Failure

56
Q

What is the difference between dry and wet beriberi?

A

Dry: when NM symptoms predominate; Wet: if cardiac failure predominates

57
Q

What is associated with hemorrhagic mamillary bodies?

A

Wernicke’s Encephalopathy (abrupt onset of confusion, abnormal eye movements, ataxia)

58
Q

Pernicious anemia is associated with what deficiency?

A

Vitamin B12

59
Q

Hippocampal neurons are especially sensitive to what metabolic disorder?

A

Hypoglycemia

60
Q

Astrocytes in the cortex and basal ganglia develop swollen pale nuclei in what metabolic disorder?

A

Hepatic Encephalopathy (elevated ammonia)

61
Q

What disorder is associated with hemorrhagic necrosis of the putamen?

A

Methanol Toxicity

62
Q

What disorder is associated with bilateral liquefactive necrosis of the globus pallidus?

A

Carbon Monoxide Toxicity

63
Q

What tumor is associated with Rosenthal fibers and BRAF serine threonine kinase?

A

Pilocytic Astrocytoma

64
Q

What is associated with a butterfly pattern of the frontal lobes and corpus callosum?

A

Gliobastoma Multiforme

65
Q

What tumor has a fried-egg appearance?

A

Oligodendroglioma

66
Q

Chromosome 1p and 19q loss/deletion is associated with what disease and what can it be detected with?

A

Oligodendroglioma; FISH

67
Q

What tumor arises from arachnoidal meningothelial cells?

A

Meningioma

68
Q

What tumor is associated with Psammoma bodies?

A

Meningioma

69
Q

What is the most common type of Meningioma?

A

Sporadic (NF2 22q12 loss)

70
Q

What tumor is associated with pseudorosettes?

71
Q

What tumors are associated with neurofibromatosis type 2?

A

Diffuse Astrocytoma, Meningioma, Ependyoma

72
Q

What tumor is associated with Homer-Wright rosettes?

A

Medullablastoma

73
Q

Associated with mutation on the 17q chromosome.

A

Neurofibromatosis Type 1 (Von Recklinghausen’s Disease)

74
Q

What is associated with mutation of merlin in 22q chromosome?

A

Neurofibromatosis Type 2

75
Q

What is associated with mutation of the VHL gene on chromosome 3p?

A

Von Hippel-Lindau Disease

76
Q

What is associated with mutation of hamartin on 9q or tuberin on 16p?

A

Tuberous Sclerosis

77
Q

What disease is associated with ApoE4?

A

Alzheimer’s Disease

78
Q

What disease is associated with neurofibrillary tangles?

A

Alzheimer’s Disease

79
Q

Pick’s disease is a sub-type of what disorder?

A

Frontotemporal Lobar Degeneration

80
Q

Echolalia is associated with what disease?

A

Pick’s Disease/FLD

81
Q

What disease is associated with Lewy bodies?

A

Parkinson’s Disease

82
Q

This disease is associated with CAG repeats.

A

Huntington’s Disease

83
Q

What is the clinical triad associated with Huntington’s disease?

A

Dementia, Choreiform movements, Family history

84
Q

What is the most common spinocerebellar ataxia?

A

Friedrich Ataxia

85
Q

What disease is associated with GAA expansion causing loss of function of the frataxin protein?

A

Friedrich Ataxia

86
Q

What disease is associated with mutation in the superoxide dismutase gene (SOD1)?

Pathology (3 decks)

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