Neuro Path - Dz Flashcards
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
Multiple sclerosis
what does MS histology look like?
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
csf for MS
mild elevated protein, mod pleocytosis, increased IgG, oligoclonal IgG bands
synchronous bilateral optic neuritis and spinal cord demyelination
csf - neutrophils and increased open pressure, turbid
antibody to aquaporins
tx - plasmapheresis
neuromyelitis optic or devic dz
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
acute disseminated encephalomyelitis
fulminant syndrome of cns demyelination
ya or children
recent uri infection
FATAL
acute necrotizing hemorrhagic encephalomyelitis aka AH leukoencephalittis of weston hurst
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
central pontine myelinolysis
rapid correction of hyponatremia
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
alzheimer dz
alzheimer dz histo
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
AB40
accompanies AD
congo red stain
cerebral amyloid angiopathy
truncal rigidity, abn speech, ocular disturbances, dementia
5th-7th decade, men, fatal
widespread neuronal loss
gllobose neurofibrillary tangles, 4R TAU
progressive supranuclear palsy
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
pick dz
pick cell - swollen
pick bodies
from vasculitis - improves w tx
progressive cog disorder assoc w vascular injury
strategic infarcts - embolic: hippocampus, dorsomedial thalamus, cingulate gyrus of frontal cortex
vascular dementia
diminished face expression, stooped posture, slow vol movement, festinating gait, rigidity, pill rolling
parkinsonism
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
parkinson dz
hallucinations and frontal signs
depigmentation of S.Nigra and L. Ceruleus w preservation of cortex, hippocampus and amygdala
dementia w lewy bodies
sporadic dz w cytoplasmic inclusion sof a-synuclein in oligodendrocytes
- striatonigral circuit - parkinsomism
- olivopontocerebellar circuit - ataxia
- autonomic ns - autonomic dysfxn orthos
multiple system atrophy
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
huntingtons
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
friedreich ataxia
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
ataxia telangiectasia
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
amyotrophic lateral sclerosis
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
tay sachs
mitochondria encephalopathy
frataxin gene
children
freidreich ataxia
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
melas
myoclonic epilepsy and ragged red fibers
mut in tRNA, much overlap with meals
myoclonus, seizure disorder, myopathy, ataxia
MERRF
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
kearn sayre syndrome
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
leigh syndrome
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
b12 def
acute, psychotic symptoms or ophthalmoplegia - reverses with thiamine
histo: foci hemorrhage and necrosis in mammillary bodies and in wall of 3/4th ventricle
thiamine def, B1
wernicke
irreversible, memory disturbances and confabulation
if you have cardiac failure as well then
korsakoff syndrome
thaimine def, b1
then it is beriberi
chronic alcoholism causes…
but also gastric disorders including carcinoma, gastritis or persistent vomitting
b1 def
what areas are most affected by hypoglycemia
pyramidal neurons of sommer’s sectors of hippocampus, Purkinje cells of cerebellum
pseudolaminar necrosis - hypoglycemia for large pyramidal neurons of cerebral cortex
Alzheimer type II cell sin cortex, bg, subcortical gray matter regions
hepatic encep
layers III and V or cerebral cortex, sommer sector, Purkinje cells
bilateral necrosis of globus pallidi
brain turns very red
carbon monoxide
attaches the retina, degen of retinal ganglion cells
selective bilateral putaminal necrosis
methanol
breaks down to formate
truncal ataxia, unsteady gait, nystagmus
atrophy and loss of granule cells in anterior vermis
proliferation of adjacent astrocytes = bergman gliosis
ethanol
radiation can induce what tumors?
Histo: large areas of coag necrosis
vessels: thickened walls w intramural fibrin like material
sarcomas, gliomas, meningiomas
acetylcholine is increased and decreased in what movement disorders
increased - parkinsons
decreased - alzheimers, huntingtons
