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Year 2 > Neuro Path - Dz > Flashcards

Neuro Path - Dz Flashcards

1
Q

unilateral visual impairment (optic neuritis), ataxia, nystagmus, motor and sensory impairment

multiple well-circumscribed, depressed, glassy, gray tan irregularly shaped plaques, sclerosis,
episodes separated by time and space
-relapsing and remitting episodes of variable duration

women mc, genetic and mc in family 1st-degree relative (DR2, IL2 and 7)
chronic inflammation by cd4, th1 and 17

A

Multiple sclerosis

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2
Q

what does MS histology look like?

A

active plaques - ongoing myelin breakdown, axons preserved, oligo depleted

  • macrophages +PAS
  • perivascular inflammatory infiltrate at outer edge

inactive plaques - quiescent, no inflamm
shadow plaques - border bw NL and white matter

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3
Q

csf for MS

A

mild elevated protein, mod pleocytosis, increased IgG, oligoclonal IgG bands

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4
Q

synchronous bilateral optic neuritis and spinal cord demyelination
csf - neutrophils and increased open pressure, turbid
antibody to aquaporins
tx - plasmapheresis

A

neuromyelitis optic or devic dz

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5
Q

perivenous, monophasic demyelinating dz
follows viral infection 1 wk - then get ha, tired, coma
myelin loss w relative preservation of axons, gray discoloration around white matter vessels
early - pmn then mononuclear
most recover

A

acute disseminated encephalomyelitis

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6
Q

fulminant syndrome of cns demyelination
ya or children
recent uri infection
FATAL

A

acute necrotizing hemorrhagic encephalomyelitis aka AH leukoencephalittis of weston hurst

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7
Q

acute paralysis, dysphagia, dysarthria, diplopia, loss of consciousness

loss of myelin in symmetric pattern involving basis pontis and portion of pontine tegmentum
no evidence of inflammation
severe electrolyte or osmolar imbalance

A

central pontine myelinolysis

rapid correction of hyponatremia

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8
Q

AD
impairment of intellectual fxn w alteration in mood and behavior
incidence rises w aging, doubles every 5yts
sporadic
assoc w down syndrome
global cortical atrophy - frontal temporal and parietal lobes -> hydrocephalus ex vacuo
AB peptides from APP
# of tangles correlates w degree of dementia

A

alzheimer dz

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9
Q

alzheimer dz histo

A

neuritic plaques - dilated tortuous neuritic processes around amyloid core in hippocampus, amygdala, neocortex
- stain congo red AB (from APP)

diffuse plaques w no core amyloid (AB42)

neurofib tangles - in cytoplasm of neurons that displace
- (pyramidal) or encircle nucleus (round nucleus)
bielschowsky stain (silver)
- ghost or tombstone tangles
- # tangles correlate with degree

tau - hyperphosphorylated, axonal microtubule assoc protein - map and ubiquitin

granulovauolar degen
- small clear intraneuronal cytoplasmic vacuoles w argyrophilic granules, abundant

hirano bodies

  • elongated glassy eosinophilic bodies
  • paracrystalline arrays of beaded filaments, actin major component
  • hippocampal pyramidal cells
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10
Q

AB40
accompanies AD
congo red stain

A

cerebral amyloid angiopathy

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11
Q

truncal rigidity, abn speech, ocular disturbances, dementia

5th-7th decade, men, fatal
widespread neuronal loss
gllobose neurofibrillary tangles, 4R TAU

A

progressive supranuclear palsy

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12
Q

rare, progressive dementia
sporadic
early onset - alt personality (Frontal) and language (temporal)
asymmetric atrophy of frontal and temporal with sparing of posterior 2.3 superior temporal gyrus
knife edge thin gyri
swollen cells and cytoplasmic filaments inclusions that basophilic, strongly stain w silver

A

pick dz

pick cell - swollen
pick bodies

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13
Q

from vasculitis - improves w tx
progressive cog disorder assoc w vascular injury
strategic infarcts - embolic: hippocampus, dorsomedial thalamus, cingulate gyrus of frontal cortex

A

vascular dementia

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14
Q

diminished face expression, stooped posture, slow vol movement, festinating gait, rigidity, pill rolling

A

parkinsonism

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15
Q

progressive L dopa responsive signs
AD
a-synuclein, ch 4 LRRK2
juvenile autosomal recessive Parkin
pallor of substantia nigra and locus ceruleus
lewy bodies - cytoplasmic, eosinophilic, dense core w place halo - a synuclein, cholingergic cells of basal nucleus o myenert

A

parkinson dz

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16
Q

hallucinations and frontal signs

depigmentation of S.Nigra and L. Ceruleus w preservation of cortex, hippocampus and amygdala

A

dementia w lewy bodies

17
Q

sporadic dz w cytoplasmic inclusion sof a-synuclein in oligodendrocytes

  • striatonigral circuit - parkinsomism
  • olivopontocerebellar circuit - ataxia
  • autonomic ns - autonomic dysfxn orthos
A

multiple system atrophy

18
Q

decrease in body of corpus callosum, head of caudate nucleus, globus pallidus
AD
movement disorder and dementia from degen of spiny striatal neurons
chorea
polyglutamine trinucleotide repeat, ch 4 GOF huntingtin protein, CAG repeats
anticipation

A

huntingtons

19
Q

AR
GAA repeat, ch 9, frataxin
1st decade of life w gait ataxia, hand clumsiness, dysarthria
res cavus, kyphoscoliosis, loss of pain, temp, light tough
joint position and vibratory sense impaired
cardiac arrhythmias and chf, cardiac dz, pulm infections
DM

A

friedreich ataxia

20
Q

AR
childhood
telangiectasias, death in the second decade- lymphoid neoplasms, gliomas, carcinomas, immunodef (recurrent sinopulm infections)
ATM gene, ch 11
sensitive to x-ray, fails to remove cells w DNA damage

A

ataxia telangiectasia

21
Q

lower and upper motor neurons
5th decade, AD if familial
SOD1, ch 21 GOF

anterior roots sc are thin, precentral gyrus atrophic, decrease in anterior horn neurons
PAS+ cytoplasmic inclusions = bunina bodies

skeletal m w neurogenic atrophy
loss of UMN
asym weak of hands, dropping objects, cramp and spasticity of arms and legs, fasciculations, resp infections
progressive bulbar palsy - degen of lower brainstem cranial motor nuclei occurs early and progresses rapidly, deglutination and phonation diff

A

amyotrophic lateral sclerosis

22
Q

Hexa gene, hexosaminidase A enzyme, ch 15
build-up of GM2 gangliosides
normal birth, 1yo diagnosis and death 2-3yo
cherry-red spots in maculae

A

tay sachs

23
Q

mitochondria encephalopathy
frataxin gene
children

A

freidreich ataxia

24
Q

mitochondrial encephalomyopathy, lactic acidosis, stroke-like episodes
mc mut - tRNAs
mc neuro syndrome caused by mito abnormalities

m weakness, stroke like episodes that do not correspond well to specific vascular territories

A

melas

25
Q

myoclonic epilepsy and ragged red fibers

mut in tRNA, much overlap with meals
myoclonus, seizure disorder, myopathy, ataxia

A

MERRF

26
Q

large mito dna deletion/rearrangement

sx: cerebellar ataxia, ophthalmoplegia plus, pigmentary retinopathy, cardiac conduction defects

histo: spongiform change in gray and white
neuronal loss, most evident in cerebellum

A

kearn sayre syndrome

27
Q

subacute necrotizing encephalopathy
early childhood: lactic acidemia, seizures, hypotonia,

histo: spongiform appearance, vascular proliferation
- periventricular gray matter of midbrain, tegmentum of pons and perivent region of thalamus and hypothalamus

A

leigh syndrome

28
Q

anemia
numbness, tingling and slt ataxia in LE
rapid progress = spastic weakness of LE
complete paraplegia (once here, replacement can’t improve)

histo: swell of myelin layers -> vacuoles, axons of both the ascending tracts of posterior columns and descending pyramidal tracts
- subacute combined degeneration of SPC

A

b12 def

29
Q

acute, psychotic symptoms or ophthalmoplegia - reverses with thiamine

histo: foci hemorrhage and necrosis in mammillary bodies and in wall of 3/4th ventricle

A

thiamine def, B1

wernicke

30
Q

irreversible, memory disturbances and confabulation

if you have cardiac failure as well then

A

korsakoff syndrome

thaimine def, b1

then it is beriberi

31
Q

chronic alcoholism causes…

but also gastric disorders including carcinoma, gastritis or persistent vomitting

A

b1 def

32
Q

what areas are most affected by hypoglycemia

A

pyramidal neurons of sommer’s sectors of hippocampus, Purkinje cells of cerebellum

pseudolaminar necrosis - hypoglycemia for large pyramidal neurons of cerebral cortex

33
Q

Alzheimer type II cell sin cortex, bg, subcortical gray matter regions

A

hepatic encep

34
Q

layers III and V or cerebral cortex, sommer sector, Purkinje cells
bilateral necrosis of globus pallidi
brain turns very red

A

carbon monoxide

35
Q

attaches the retina, degen of retinal ganglion cells

selective bilateral putaminal necrosis

A

methanol

breaks down to formate

36
Q

truncal ataxia, unsteady gait, nystagmus
atrophy and loss of granule cells in anterior vermis
proliferation of adjacent astrocytes = bergman gliosis

A

ethanol

37
Q

radiation can induce what tumors?

Histo: large areas of coag necrosis
vessels: thickened walls w intramural fibrin like material

A

sarcomas, gliomas, meningiomas

38
Q

acetylcholine is increased and decreased in what movement disorders

A

increased - parkinsons

decreased - alzheimers, huntingtons

Year 2 (10 decks)

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