DSA 24 CNS Pathology Flashcards

1
Q

elongated eosinophilia within astrocytic process in response to injury

A

rosenthal fibers

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2
Q

contain alphabeta-crystallin and hsp27

A

rosenthal fibers

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3
Q

lamellated polyglucosan bodies

A

corpora amylacea

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4
Q

increases with advancing age and represent degenerative change; injury response

A

corpora amylacea

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5
Q

enlarged nucleus with intranuclear glycogen and pale chromatin; response to injury

A

alzehimer type II astrocyte

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6
Q

occur in the setting of hyperammonemia

A

Alzheimer type II astrocyte

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7
Q

central chromatolysis

A

axonal reaction/regeneration/degeneration

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8
Q

compresses the ACA

A

subfalcine herniation

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9
Q

subfalcine herniation

A

compresses ACA; results in contralateral paralysis of lower limb and loss of sensation

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10
Q

presses on PCA

A

transtentorial (uncinate, mesial tmeporal herniation)

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11
Q

transtentorial herniation

A

CN III compromised, contralateral hemianopsia and macular sparing

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12
Q

Duret hemorrhage

A

due to substantial herniation of the transtentorial causing downward displacement of the brainstem

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13
Q

compresses the medulla and compromises cardiac/respiratory centers

A

tonsillar herniation

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14
Q

hydrocephalus due to extensive tissue loss; compensatory expansion of the entire CSF compartment

A

ex vacuo hydrocephalus

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15
Q

malformation of the anterior neural tube

A

anencephaly

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16
Q

no forebrain and an open calvarium

A

anencephaly

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17
Q

increased AFP, polyhydramnios

A

anencephaly

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18
Q

associated with maternal type II diabetes; maternal folate supplementation decreases risk

A

anencephaly

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19
Q

malformed CNS diverticulum extending through a defect in the cranium

A

encephalocele

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20
Q

What are the AFP levels in encephalocele?

A

normal

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21
Q

complete absence of gyri

A

Lissencephaly

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22
Q

abnormal clusters of neurons in inappropriate locations along normal migratory routes

A

neuronal heterotopias

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23
Q

commonly associated with epilepsy

A

neuronal heterotopias

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24
Q

what are the mutations commonly associated with neuronal heterotopias?

A

cytoskeletal (filamin A) or microtubule associated proteins can be causal

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25
Q

What are the mutations associated with Holoprosencephaly?

A

Trisomy 13 and sonic hedgehog gene mutation

26
Q

failure of the left and right hemispheres to separate resulting in midline facial abnormalities

A

holoprosencephaly

27
Q

associated with low-lying cerebellar tonsils extending into the vertebral canal

A

Chiari I

28
Q

significant herniation of the cerebellar tonsils and vermis through foramen magnum with aqueductal stenosis and hydrocephalus

A

chiari II

29
Q

associated with lumbosacral myelomeningocele

A

chiari II

30
Q

agenesis of cerebellar vermis with cystic enlargement of 4th ventricle

A

dandy-walker malformation

31
Q

syrinx

A

fluid-filled neuroglial cavity within the spinal cord; syringomyelia

32
Q

spinal cord ependymal-lined canal expansion in the cervical cord

A

hydromyelia

33
Q

intraparenchymal hemorrhage in infants

A

occurs within the germinal matrix between the thalamus and caudate nucleus and extends into the ventricles

34
Q

infarcts in infants that occur within the periventricular white matter

A

periventricular leukomalacia

35
Q

ischemic infarcts within the hemispheres

A

multicystic encephalopathy

36
Q

cortical scarring in the deep regions of the sulcus that leads to distortion of gyri

A

ulegyria

37
Q

ischemic neuronal loss and gliosis in the basal ganglia and thalamus associated with aberrant and irregular myelin; occurs in infants

A

status marmoratus

38
Q

coupe and contrecoup

A

gyral crests are most susceptible to contusion at the site of impact (coupe) or the point in the cranium opposite site of impact (contrecoup)

39
Q

plaque jaune

A

brain hemorrhage and edema as a result of a contusion and eventually resolves as a depressed, yellow-brown glial scar extending to the pial surface

40
Q

Where do intraparenchymal hemorrhages in adults usually occur?

A

basal ganglia and internal capusle

41
Q

Charcot-Bouchard aneurysm

A

occurs in the small vessels of the basal ganglia; lenticulostriate vessels ; causes intraparenchymal hemorrhage

42
Q

Damage to what blood vessel is common with an ischemic stroke due to thrombosis?

A

MCA

43
Q

What does damage to MCA cause?

A

motor cortex (upper limb and face), sensory cortex (upper limb and fact), temporal lobe (Wernicke area) and frontal lobe (Broca area)

44
Q

Most common site of intracerebral hemorrhage?

A

basal ganglia

45
Q

when maintained on mechanical ventilation on someone who is brain dead it results in the brain autolyzing

A

respiratory brain

46
Q

Notch3 receptor mutation

A

CADASIL

47
Q

low flow without arteriovenous shunting

A

cavernous hemangiomas

48
Q

tangles of abnormally tortuous and misshapen vessels, shunting arterial blood directly into the venous circulation

A

arteriovenous malformation

49
Q

associated with Foix_Alajouanine disease

A

venous angiomas; venous antiomatous malformation typically seen in the lumbosacral region

50
Q

less than 15 mm cystic infarcts

A

lacunar infarct

51
Q

accompanied by lipid_laden macrophages and surrounding gliosis

A

lacunar infarct

52
Q

small vessel rupture that eventually resorbs and leaves residual hemosiderin_laden macrophages and associated gliosis

A

slit hemorrhage

53
Q

psuedobulbar signs

A

multi_infarct dementia

54
Q

when the pattern of recurrent ischemic injury preferentially involves subcortical white matter with myelin and axonal loss

A

binswanger disease

55
Q

what are the two conditions that are a result of chronic hypertensive injury?

A

multi-infarct dementia, Binswanger disease

56
Q

what is anencephaly? clinical finding?

A

malformation of anterior neural tube → no forebrain. increased levels of AFP

57
Q

what is holoprosencephaly? what mutation is it related to?

A

failure of left and right hemispheres to separate. mutations in SHH signaling pathway

58
Q

clinical presentation of holoprosencephaly?

A

moderate form has cleft lip/palate. severe form results in cyclopia.

59
Q

what is Dandy-Walker associated with?

A

hydrocephalus and spina bifida

60
Q

what is the difference between syringomyelia and hydromyelia?

A

syringomyelia is a cystic cavity within spinal cord. hydromyelia involves the central canal.

61
Q

what is the clinical presentation of syringomyelia?

A

cape-like bilateral loss of pain and temperature sensation in the upper extremities. fine touch sensation is preserved.

62
Q

what is syringomyelia associated with?

A

Chiari malformation, trauma, and tumors