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Neuro > Neuro path > Flashcards

Neuro path Flashcards

1
Q

perifasicular atrophy suggests…

A

dermatomyositis

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2
Q

type II muscle fiber atrophy without type I muscle fiber atrophy suggegsts…

A

prolonged steroids or disuse atrophy

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3
Q

What antibodies are associated with Gottron’s papules and heliotrope rash? They are antibodies against helicase implicated in nucleosome remodeling

A

anti-Mi2 abs

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4
Q

Anti-Jo1 abs are known to be against histidyl-tRNA synthetase, and is associated with what ssx?

A

ILD

nonerosive arthritis

mechanic’s hands

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5
Q

What antibodies are against transcriptional regulators and a/w parneoplastic and juvenile forms of dermatomyositis?

A

Anti-P155 and P140 abs

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6
Q

What will show endomysial inflammatory cell infiltrates and lymphocytes invading non-necrotic tissues?

A

Polymyositis

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7
Q

What is the MC inflammatory myopathy in pts >65yo? What will the biopsy of muscle show?

A

Inclusion Body myositis

rimmed vacuoles

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8
Q

What is the leading cause of rx-induced myopathy?

A

statins

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9
Q

Myosin deficient myopathy especially with corticosteroid treatment could be considered…

A

ICU myopathy

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10
Q

What is the mechanism of X-linked MD?

A

LOF mutation in dystrophin gene

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11
Q

expansions of CTG triplet repeats on DMPK gene are found in what disease?

A

myotonic dystrophy

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12
Q

multisystem disorder involving skeletal muscle weakness, cataracts, endocrinopathies, and cardiomyopathy

Dx?

A

myotonic dystrophy

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13
Q

What is the morphologic hallmark of axonal neuropathies?

A

Wallerian degeneration and reduction in signal strength

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14
Q

neuronopathies - destruction of neurons and secondarily axons (eg herpes zoster, toxins like platinum) will affect what part of a nerve?

A

equally likely to affect proximally and distally

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15
Q

if a disease is affecting nerve roots and peripheral nerves in a diffuse and symmetric pattern, we would call this

A

polyradiculoneuropathy

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16
Q

What will the lab draw of a pt’s CSF that has GBS?

A

increased protein with normal WBC

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17
Q

How long does a person have to have symptoms of Chronic Inflammatory Demyelinating Polyradiculoneuropathy for it to be diagnosed?

A

persisting for 2 or more months

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18
Q

What leads to the ‘onion bulb’ look seen in Chronic Inflammatory Demyelinating Polyradiculoneuropathy?

A

Schwann cell axon junction is target - causing recurrent demyelination and remyelination creating multiple layers around an axon

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19
Q

Which form of Hansen’s disease will have no organisms found in the acid fast blood smear?

A

tuberculoid leprosy

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20
Q

What is paraproteinemic neuropathy associated with? What is it?

A

monoclonal gammopathy

distal sensorimotor polyneuropathy - mild, slow, progresses symmetrically

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21
Q

What disease has autoabs against AChR and can sometimes be associated with thymomas?

A

Myasthenia Gravis

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22
Q

Weakness is worse with exertion in what myopathy?

A

myasthenia gravis

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23
Q

What syndrome blocks ACh release by inhibiting presynaptic Calcium channels?

A

Lambert-Eaton Syndrome

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24
Q

Developmental malformations of the CNS are usually due to chromosomal abnormality or single gene defect that result in…

A

midline, bilateral, symmetric deficit without gliosis

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25
Q

Disruptions, unlike malformations, are more likely to be…

A

focal, asymmetric, and gliosis associatied

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26
Q

What is the MC of congenital CNS abnormalities? What does this most often present as?

A

neural tube defect

MC in spinal cord and lumbosacral region

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27
Q

What are two known risk factors for neural tube defects?

A

folate deficiency

MTHFR mutation

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28
Q

What is the worst form of spina bifida?

A

myelomeningocele - meninges and neural tissue projection, motor and sensory dysfunction, loss bowel and bladder, L4/L5 commonly, increased risk infection

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29
Q

What is a protrusion of the brain through the skull, usually in the occipital region called? What is it due to and what is it associated with?

A

Encephalocele

ischemic destruction a/w craniofacial abnormalities

30
Q

Anencephaly is what?

A

absence of brain/calvarium - incompatible with life

31
Q

What is cerebrovasculosa?

A

If the brain initially protrudes through a defect in the calvarium, it will gradually get destroyed. The small, vascular mass left is this

32
Q

What is the definition of megaencephaly?

A

brain weight >1800 grams or 3 SD above the mean

33
Q

What is Lissencephaly?

gene mut?

ssx?

A

smooth brain

due to abnormal migration during development, no cortical gyri

microcephaly

Type-1 is mutation in LIS-1 gene

severe impairment and seizures

34
Q

During 4-6 weeks of gestation, if there is incomplete separation of cerebral hemispheres across the midline, this is called…

A

holoprosencephaly

35
Q

What does holoprosencephaly present with?

A

midline facial abnormalities

proboscis - trunk protrusion above single eye

single nostril, cleft lip and palate

close set eyes - hypotelorism

Diabetes inspidis is common

a/w trisomy 13 and 18

36
Q

What is the mutation a/w holoprosencephaly?

A

SHH type protein

37
Q

Agenesis of the corpus callosum will result in…

A

misshapen lateral ventricles, usually psychomotor retardation

38
Q

What is the failed opening of foramina of Luschka and Magendie called? What is this?

A

Dandy-Walker Malformation

agenesis of vermis; instead has large cystic dilation of fourth ventricle lined by ependyma

results in obstruction of CSF flow from fourth ventricle

39
Q

Obstruction of CSF flow out of the fourth ventricle, as caused by Dandy-walker Malformation, results in…

A

non-communicating hydrocephalus

40
Q

Which Chiari Malformation is more common? When does it present?

A

Chiari Type I

noticed in adolescence or adulthood

41
Q

Which Chiari Malformation is associated with a neural tube defect?

A

Chiari Type II (Arnold-Chiari)

usually a/w myelomeningocele

42
Q

Which Chiari malformation has a decreased volume in the posterior fossa, leading to herniation of cerebellar tonsils and dorsal cerebellum into spinal canal through the foramen magnum?

A

Chiari Type I

43
Q

What happens in Chiari Type II malformation?

A

downward displacement of the vermis through foramen magnum

hydrocephalus

pressure atrophy of displaced brain tissue (w partial or complete paralysis)

may have hydromyelia or syringomyelia

44
Q

fluid filled cleft-like cavity lined by glial tissue in inner portion of spinal cord

A

syringomyelia

45
Q

hydromyelia can be a feature of what two other malformations?

A

dandy-walker malformation

chiari type II

46
Q

hypoxic-ischemic encephalopathy is generally due to decreased oxygen or blood in the perinatal period. In premature infants this will affect… while in term infants it affects…

A

premature - affect white matter more severely, role in interventricular hemorrhage

term - affects cerebral cortex and deep nuclei

47
Q

What is periventricular leukomalacia? What does it look like?

A

ischemic white matter infarts affecting sick premies with heart defects

chalky yellow plaques

cystic lesions can develop - multicystic encephalopathy

48
Q

What all goes into the category of cerebral palsy?

A

any one of many nonprogressive neuro disorders in infancy or early childhood permanently affect body movement

brain damage present at birth but may not be clinically apparent

49
Q

What is the syndrome associated with medulloblastoma or glioblastoma caused by mutation in APC or mismatch repair gene?

A

Turcot syndrome

50
Q

What is MC characterized by bilateral Schwannomas of vestibulocochlear nerves

A

neurofibromatosis

51
Q

To dx a CNS tumor, what is the study of choice?

A

contrast MRI

52
Q

What are three main types of gliomas?

A

astrocytomas

oligodendrogliomas

ependymomas

53
Q

What glioma is found mostly in children and young adults, is relatively benign, and grossly may appear cystic with a mural nodule and is well circumscribed. What does it look like microscopically?

A

pilocystic astrocytoma

micro: biphasic pattern, has rosenthal fibers (eosinophilic granular bodies)

54
Q

grossly, this tumor is ill-defined and has a blurred gray white junction

microscopically, it is GFAP + and has increased cellularity

A

diffuse (infiltrating) astrocytoma

55
Q

What kind of astrocytom has bright eosinophilic cell bodies with stout processes?

A

gemistocytic astrocytoma

56
Q

This astrocytoma is mitotically active, infiltrating, and give the pt a pretty poor prognosis.

What is a key word in describing its gross appearance?

A

anaplastic astrocytoma

fleshy

57
Q

What is the MC primary brain tumor? What causes it to arise?

A

glioblastoma

arises de novo

58
Q

What will I see upon imaging of a glioblastoma?

A

enhancing lesion in rim or ring pattern

often crosses midline

59
Q

microscopically, what will I see in a glioblastoma?

A

necrosis - serpentine or pseudopalisading of tumor nuclei around edges

vascular or endothelial cell proliferation forming tufts of vessels - glomeruloid bodies

60
Q

Pt 30-40 yo, affects cerebral hemisphere white matter

well-circumscribed, gelatinous gray masses, cysts, focal hemorrhage, or calcifications

A

oligodendroglioma

61
Q

micro of oligodendroglioma will show what?

A

fried-egg appearance of monotonous tumor cells with clear halo of cytoplasm

tendency to calcify

anastomosing capillary network

62
Q

what unbalanced translocation gives a more favorable prognosis in an adult with an oligodendroglioma?

A

t(1:19)

63
Q

Where is an ependymoma likely located in a child v adult?

A
  • child
    • near fourth ventricle, unfavorable prognosis
  • adult
    • lumbosacral spinal cord, favorable prognosis
64
Q

tumor cells forming rosettes with canals and perivascular pseudorosettes are associated with what gliomas?

A

ependymomas

65
Q

What will occur if the lesion in the posterior fossa ends up destroying the fourth ventricle?

A

hydrocephalus

66
Q

a medulloblastoma is exclusively where and in what age of pt?

A

exclusively in cerebellum, midline in kids 5-10, lateral in adults

67
Q

if a medulloblastoma disseminates through the CSF and mets to cauda equina, this is called…

A

“drop metastasis”

68
Q

A meningioma is from what cells? How does it cause damage to the brain?

A

from arachnoid cells/meningothelial cells

compresses underlying brain without invading it

69
Q

micro of meningioma will have what?

A

arachnoid like cells

transitional - tumor cells in whorls with hyalinzed and calcified centers - psammoma bodies

70
Q

loss of entire chromosome of 22 or of 22q could cause

A

meningioma

71
Q

What is the MC CNS neoplasm in immunocompromised pt?

A

primary CNS lymphoma

likely large B cell NHL

72
Q

If a tumor was to met to the CNS, it would likely be from..

A

lung, breast, or skin (melanoma)

Neuro (9 decks)

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