Path 2 Flashcards
ischemic stroke vs TIA vs hemorrhagic stroke
occluded blood vessel –> acute infarct vs transient neuro dysfxn d/t focal brain, spinal cord, retinal ischemia w/o acute infarct; short –> pre/syncope, long –> permanent neuro injury vs ruptured blood vessel –> acute infarct
causes of focal vs global ischemic stroke
cerebral embolism (types), atherosclerosis (sites), sm vessel dz in sm penetrating aa in A/MCA (lacunar infarcts), vasculitis/hypercoag, cryptogenic vs circ failure (Selective neuronal nec, infarcts)
unmodifiable vs modifiable risk factors of stroke
o >55yo
o men > women
o African American > Caucasian
o Fhx of stroke in first degree relatives
o hereditary dyslipidemia, protein C & S or antithrombin deficiency
vs
o Arterial HTN, TIA, cardiac dz, hyperchol, DM, smoking/alc, OCP, obese/inactive
hypoxic-ischemic encephalopathy. sel neuronal vulnerability?
circ/resp failure –> O2 deprivation in brain –> brain injury –> shrunken eos neurons. Hippo/CA1 of pyramidal cell layer/Sommer sector > cortex layers 3-5, cerebellum/Purkinje > thal > BG > brainstem > hypothal > spinal cord
borderzone/watershed infarcts. ant vs post vs internal/subcortical vs external
b/w 2 main nonanastomosing arterial territories from proximal stenosis of cerebral a + severe systemic hypoTN. frontal cortex b/w ACA/MCA vs occipital cortex b/w PCA/MCA vs cigar shaped deep to A/M/PCA territories w/ recurrent a of Heubner, lenticulostriate, ant choroid a territories vs wedge shaped
pathogenesis of hypoxic-ischemic encephalopathy?
gluE synapses on dendritic spines –> pckg gln into vesicles –> exocytosis –> gluE bind to postsynaptic receptor –> influx depolarize neuron, transporters in astrocytes remove gluE from synaptic space
gluE excitotox d/t dysfxn of?
transporters in astrocytes: cardiac arrest –> cerebral blood perfusion dec –> hypoxia –> impaired intake of gluE by astrocytes –> neuronal death when [gluE] = 10umol/L. malfxn of gluE NMDA receptors: influx of Na+ & Ca2+ into cell, efflux of K+ from cell; Ca2+ for synaptic plasticity & mem; too much intracellular Ca2+ –> apop
hemorrhagic stroke/intracerebral hemorrhage: deep/ganglionic vs lobar vs cerebellum vs brainstem
BG, internal capsule, periventriular white matter, thal vs sub/cortical areas crossing 1+ lobes of brain vs cerebellum vs brainstem
cause of deep intracerebral hemorrhage?
HTN –> sometimes into ventricular system –> subfalcine/transtentorial/tonsillar hern –> myelin sheath breaks down –> hemosiderin from RBC lysis –> phag –> cystic activity => encephalomalacia
* Spont rupture of sm penetrating a from A/M/PCA
* Hyaline arteriolosclerosis, Lipohyalinosis, Fibrinoid necrosis
* Charcot-Bouchard aneurysm esp in lenticulostriate
cause 1 of lobar intracerebral hemorrhage: cerebral amyloid angiography
inc age –> amyloid protein deposit in cortical & leptomeningeal tunic media & adventitia of arterioles in occ > front > temp > par.
* Rigid nonbranching apple-green birefringent fibrils w/ Congo red
* APP gene –> amyloid precursor glycoprotein –> amyloid-B pep
* APOE 2/4 > 3 inc risk (also seen in Alzheimer’s)
cause 2 of lobar intracerebral hemorrhage: vascular malformations in young ppl
- Arteriovenous malformations: abnl vessels b/w feeding a & draining v –> direct communication w/o capillaries; mostly MCA
- Cavernous hemangioma: abnl dil vessels w/ back-to-back arrangement & no intervening neural parenchyma in endothel lining of fibrous adventitia of subcortical front-par
- Venous angioma
- Capillary telangiectasia
causes of subarach hemorrhage. sites?
ruptured saccular/berry > fusiform > mycotic aneurysms mostly from ant circ/Circle of Willis; By head trauma –> bleed into subarach space –> ICP.
ACA/ACOM > ICA > MCA > BA > PCOM > PCA
risk factors of subarach hemorrhage: familial vs acquired
- Fhx of intracranial aneurysms
- Acquired: smoking, HTN –> shift tensile forces on arterial wall @ bifurcation –> breakdown of internal inelastic lamina –> degrade ECM –> disorganize sm muscle cells –> disrupt endothelial cells
how to dx subarach hemorrhage? complications?
head CT at basal cisterns; LP for inc opening pressure, inc RBC; xanthochromia d/t bilirubin in CSF; cerebral angiography. rebleeding, delayed cerebral ischemia, intraventricular hemorrhage
Intraventricular hemorrhage –> obstructive hydrocephalus: infants vs adults?
germinal matrix hemorrhage in premies:
Immature germinal matrix vessels, no cerebral autoreg
Dx: cranial US
vs
arteriovenous malformations, cavernous malformations, choroid plexus tumors:
Sudden HA, loss consc, N/V + photophobia + stiff neck, sz
Dx: noncontrast CT
vasc dementia: mild cog impairment vs Binswanger dz vs Cerebral sm vessel dz
elderly w/ mem problems but have nml living skills vs ischemic brain dmg in periventricular white matter w/ hyaline arteriolosclerosis from leptomeningeal border zone vs hyaline arteriosclerosis (from age); lipohyalinosis, microaneurysm (from HTN), amyloid-B deposit (from cerebral amyloid angiopathy)
clin pres of ischemic stroke vs TIA vs hemorrhagic stroke vs subarach hemorrhage
neuro sxs that may slowly improved for mo vs inc risk of cerebral infarct vs sudden HA, loss consc, N/V, delirium, sz, hemiparesis if big enough vs asx; chronic HA, dizzy, facial or eye pain, visual loss, CN palsies
know main diff b/w lysosomal storage dz vs leukodystrophies. but describe leukodystrophies more
- Auto dom, auto rec, X linked
- Dec brain wt b/c dec white matter vol, ventriculomegaly; spares subcortical arcuate U fibers; diffuse pallor, soft, cystic, spongy white matter; diffuse dec/absent myelin staining –> axon degen, loss of oligodendrocytes
Krabbe dz. clin pres: infant vs juvenile/adult. dx?
GALC mutation encoding galactosylceramidase catab galactosylceramide in myelin –> buildup of psychosine, GalCer undergoes acetylation by acid ceramidase –> demyelin & apop oligodendrocytes –> fibrillary astrocytic gliosis –> PAS-pos globoid cell/macs w/ tubular or filamentous inclusions infiltrate. hyperirritability, hyperactive reflexes, spastic, blind/deaf, facial paralysis, sz
vs weak, blind/deaf, intellect disability. GalCer in leuks
metachromatic leukodystrophy. clin pres. dx?
Auto rec ARSA mutation encoding arylsulfatase A breaking cerebroside 3-sulfate in myelin to cerebroside & sulfate –> buildup of cerebroside 3-sulfate in lysosomes of oligodendrocytes & Schwann cells –> disrupt lysosomal system –> dmg & loss myelin in white matter & corticospinal tracts –> PAS macs infiltrate. abnl Gait, motor, speech, intellect. ARSA activity in leuks
adrenoleukodystrophy. clin pres. dx?
ABCD1 encoding ABC transporter in peroxisomal membrane for transporting VLCFA to peroxisomal matrix for B[O] –> buildup of VLCFA in plasma, white matter, spinal cord –> ROS toxic to myelin & adrenal cortex –> inc malondialdehyde & 4-hydroxynonenal –> demyelinate splenium CC, pyramidal tracts, centrum ovale. adrenal insufficiency. high VLCFA C22/26 in plasma
mito encephalopathy
point mutations in mDNA or nDNA for mito components via mito inheritance from females –> oxidative phosphorylation defic –> resp chain fails to make ATP –> affects tissue w/ high energy needs
MELAS vs MERRF vs Leigh syndrome/subacute necrotizing encephalomyopathy w/ clin pres
MT-TL1 point mutation for leu RNA transporter –> multisystemic w/ COX-pos red ragged fiber myopathy, encephalopathy w/ sz/dementia, lactic acidosis, stroke-like episodes; cortical laminar nec, sub/cortical infarct-like lesions. Prodromal sxs: migraines, mental changes; dec consc (lethargy to coma), sz, focal neuro deficit vs MTTK point mutation for lys RNA transporter –> multisystemic red ragged fiber myoclonus, epilepsy, ataxia. Optic n atrophy, sensorineural hearing loss, exer intol, dementia, dil cardiomyopathy, Wolff-Parkinson-White syndrome, short stature vs Auto rec/X linked/mito mutations in 50+ m/nDNA encoding subunits of PDH or resp complexes I/II –> inc blood lac –> nec lesions in grey matter brainstem, BG, cerebellum, spinal cord bil. Loss motor milestones, hypotonia, movement d/o, emesis
acquired metab encephalopathies: thiamine/B1 vs B12 defic
psychotic sxs, ophthalmoplegia
o Wernicke encephalopathy: hemorrhage & nec in mamillary bodies in 3/4th ventricle; w/ Korsakoff syndrome => Wernicke-Korsakoff syndrome
o Cerebellar dysfxn –> ataxia, abnl gait, nystagmus; atrophic sup vermis, wide spaces b/w folia
vs
megaloblastic anemia, subacute combined degen of spinal cord, pernicious anemia (autoimmune dmging parietal cells secreting intrinsic factor)
o Numb/tingly extremities, ataxia, spastic LE, complete paraplegia
hypo vs hyperglycemia
low glu –> similar sxs in hypoxia b/c brain really sensitive to glu vs assoc w/ ketoacidosis, hyperosmolar coma in unctrled DM pts –> dehydration, confusion, stupor, coma
intox: CO vs methanol vs ethanol
bil GP nec; injury to cortical layers 3/5, hippo, cerebellar Purkinje vs blind d/t degen retinal ganglion cells & formate vs cerebellar dysfxn –> ataxia, abnl gait, nystagmus; atrophy & loss of granule cell layer in ant cerebellar vermis; Bergmann gliosis (loss of Purkinje & prolif of astrocytes b/w granular & molecular layers)
what are lysosomes? what is ELS? what are lysosomal enzymes?
catab organelles break down & recycle complex substrates; can be acid hydrolases, nonenzymatic proteins; pH4.5-5; part of endosomal-lysosomal system. early/recycling/late endosomes & lysosomes that interconvert. made in ER & transported to Golgi to add M6P residues on lumen –> endosome matures –> released from M6P & go to lysosomes
what are lysosomal storage dzs?
auto rec/X linked d/o from accumulation of undigested substrates. enzyme activity in dried blood spots, dec lysosomal hydrolase in fibroblasts or leuks; pathogenic mutations in leuks for nonenzymatic lysosomal proteins
ceramide vs cerebroside vs sulfatide vs globoside vs ganglioside
sphingosine + LCFA vs ceramide + 1 sugar vs cerebroside + sulfate vs ceramide + polysacch vs globoside + N-acetylneuraminiciacid
describe GM2 gangliosidosis
loss of fxn auto rec HEXA –> α subunit of β-hexosaminidase –> Tay Sachs; HEXB –> β subunit of β-hexosaminidase –> Sandhoff; GM2A –> GM2 activator protein –> AB variant
histo of Tay Sachs. how to dx?
o Lg, pale neurons; lysosomes w/ gangliosides; cytoplasmic inclusions, whorls
o Destroyed neurons, prolif microglia, accumulation of complex lipids in phags
o Fat stains/oil red O pos
little to no β-hexosaminidase activity, genetic tests
Tay Sachs clin pres: classic infantile vs juvenile vs adult. clin pres of Sandhoff & AB variant?
6-10mo: can’t overturn, sit, crawl; loss vision, stiff/spastic vs Abnl gait, bal, falls, speech vs Progressive, psychosis. indistinguishable from TS
Niemann-Pick dz
SMPD1 mutation –> defic acid sphingomyelinase –> sphingomyelin builds up in reticuloendothelial system
Niemann-Pick dz clin pres: A vs B vs C
hepatosplenomegaly –> lg lymph nodes & spleen w/ Niemann-Pick cells (sphingomyelin laden macs); fail to thrive, recurrent infxns, irritability, progressive loss motor & intellect; die w/in 2-3yo vs hepatosplenomegaly –> lg lymph nodes & spleen w/ Niemann-Pick cells; thrombocytopenia; cough/dyspnea/recurrent infxns, short, delayed puberty vs Hepatosplenomegaly; fail to thrive, hypotonia/spastic, loss developmental milestones, vertical supranuclear gaze, sz
describe Niemann-Pick C dz. how to dx?
auto rec NPC1/2 mutation –> impaired transport LDL from lysosomes to cyto –> unesterified chol builds up in lysosomes. genetic test for NPC1/2
Gaucher dz. dx?
auto rec GBA1 mutation encoding glucocerebrosidase hydrolyzing glucosylceramide into ceramide + glu –> build up of glucosylceramide in lysosomes of reticuloendothelial system. dec glucocerebrosidase activity in peripheral blood leuks or cxed fibroblasts; genetic tests
histo of Gaucher dz. where else can you find it?
Excess glucosylceramide in macs –> lg vacuolated blue ovoid histiocytes in wrinkled paper => Gaucher cells –> expanded red bone marrow
Spleen has lots of Gaucher cells
Liver shows lg foamy Kupffer cells & Gaucher cells in portal tracts
Lung bxs show intraalveolar Gaucher cells
Brain shows perivascular lipid-laden macs, astrogliosis, microglia
Gaucher dz 1 vs 2 vs 3 clin pres
Hepatosplenomegaly –> thrombocytopenia, bone marrow dz –> anemia, skel dz –> bone crises w/ fever & leukocytosis –> osteonec, no primary CNS involved vs Hepatosplenomegaly –> thrombocytopenia; no glucocerebrosidase activity –> neck/trunk rigidity, bulbar signs, oculomotor paralysis vs Hepatosplenomegaly, skel dz; supranuclear gaze, strabismus, myoclonic epi, ataxia
mucopolysaccharidosis
involve glycosaminoglycans; types 1-4, 6-7, 9 involving 11 genes
MPS I/Hurler’s syndrome vs MPS II/Hunter’s syndrome genetics
auto rec IDUA encoding α-L-iduronidase –> buildup of dermatan & heparan sulfate vs X linked IDUA
MPS I clin pres
Coarse facial features, skel dysplasia, short, stiff joints, thoracolumbar kyphosis, genu valgum, thick mitral & aortic valves/mitral & aortic regurg, hepatosplenomegaly, corneal clouding, vision/hearing, intellect
MPS I dx
Excess glycosaminoglycan in urine
Neu/lymphocytes/monocytes in blood smears showing purple red granules in cyto stained w/ Toluine blue; metachromasia
Dec enzyme activity in leuks or cxed fibroblasts
Pathogenic mutations
peripheral nerve tumors w/ clin pres
elong spindle-shaped cells w/ uniform nuclei around nerve fibers & nerve trunks –> soft tissue grey mass, pain, sensory/motor changes, electrodiag studies
schwannoma. mutation?
benign tumor from Schwann cells; most common solitary & sporadic peripheral nerve tumor –> affects spinal roots; cervical, sympathetic, vagus, peroneal, ulnar nerves (Tinel’s sign). Inactivating NF2 mutation –> no merlin –> neurofibromatosis 2 –> bil schwannomas of cranial nerves mostly CN8
histo of schwannomas: Antoni A vs B. both are what?
compact hypercellular, Palisaded nuclei = Verocay bodies vs myxoid hypocellular, Hyalinized stroma containing collagen fibers. S100-immunoreactive –> derived from neural crests
neurofibroma w/ clin pres
elong spindle-shaped Schwann cells w/ wavy hyperchromatic nuclei + wirelike strands of collagen like shredded carrots. skin dermis; painless superficial tumors from cutaneous nerves
mutation of neurofibroma
NF1 mutation –> no neurofibromin –> inc Ras-GTPase activation –> overexpressed tyr kinases/MAPK –> mixed non-neoplastic peripheral nerve components, not well encapsulated
plexiform vs diffuse neurofibroma
Mult nodular growths of fascicles along long seg of major nerve trunk –> extend to minor nerve branches –> look like rosary beads, bag of worms vs Head & neck –> spreads laterally along tissue planes –> plaque-like thickening of skin –> yellow-gray mass on subq tissues
malig peripheral nerve tumors w/ clin pres
fusiform white opaque surface from Schwann cells or pluripotent cells of neural crest –> major nerve trunks like sciatic nerve, brachial/sacral plexus –> hem & nec. lg palpable mass w/ neuro deficits like radicular pain, paresthesia, motor weakness
mutations of malig periph n tumor
NF1 –> no neurofibromin –> Ras-GTPase –> tyr kinase/MAPK
CDKN2A –> no p16 –> overexpressed CDK4&6 –> no retinoblastoma protein
no p14 –> no p53 as tumor suppressor
SUZ12 & EED –> Ras –> MAPK
TP53 –> no p53 as tumor suppressor
histo of malig periph n tumor
resembles fibrosarcoma: hypercellular spindles w/ hyperchromatic nuclei, eos cyto, brisk mitosis activity –> invade surrounding tissue or vasc. 1+ histo differentiation => divergent differentiation –> exhibit rhabdomyosarcoma (malig triton tumor), osteo/chondro/angiosarcoma, adenocarcinoma
neurofibromatosis type 1 mutation. what does neurofibromin nmlly do?
- Auto dom NF1 mutation –> no neurofibromin –> Ras –> MAPK
o neurofibromin pos reg cAMP –> astrocytic growth & differentiation in brain
o Follows 2-hit hypothesis: heterozygous NF1 mutation –> neurofibromas
neurofibromatosis type 1 clin pres/dx
o (6+) Café au lait macules d/t inc epidermal melanin in melanocytes & keratinocytes
o Axillary & inguinal freckles
o (2+) Lisch nodules: hamartomas of iris; don’t affect vision
o (2+ any type or 1 plexiform) Soft cutaneous neurofibromas
o Segmental neurofibromatosis: by somatic mosaicism d/t postzygotic mutation NF1 –> neurofibromas +/- café au lait unilateral w/o crossing midline
o Optic pathway gliomas (low grade pilocytic astrocytomas) w/ proptosis, strabismus, nystagmus; chiasmal gliomas w/ vision loss
o Sphenoid wing dysplasia: no greater wing –> temp lobe hern into orbital cavity –> exophthalmos & facial deformity
o Cog deficits & learning disabilities
neurofibramotosis type 2 mutation
- Auto dom NF2 mutation –> no merlin –> nervous system tumors
o Follows 2-hit hypothesis: heterozygous NF2 mutation –> tumor
o Mosaicism
neurofibramotosis type 2 clin pres
o Bil vestibular schwannomas: intracranial, extra-axial, vestibular part of CN8 –> brainstem compression, hydrocephalus if untxed
Press against cochlear nerve –> hearing loss, tinnitus; vestibular nerve –> dizziness
o Meningioma: cranial & spinal, blind
o Ependymoma: cervical > thoracic > lumbar cord
o Café au lait, astrocytomas of spinal cord
axonal neuropathies. can they regen? electrophys?
dmg to axon –> distal part of axon degen => Wallerian degen –> myelin degen –> phag by macs to remove debris. if 2 cut sections = close to e/o; if not –> painful nodular traumatic neuroma. dec amp b/c low axons but preserved conduction velocity
demyelinating neuropathies
breakdown & loss myelin over few seg => segmental demyelination –> phag by macs –> Schwann cells prolif & remyelinate –> onion bulbs if rpted –> hypertrophic neuropathy. loss saltatory conduction –> dec conduction vel but preserved axons
neuronopathy vs mononeuropathy vs polyneuropathy vs mononeuritis multiplex
destroyed motor neurons or DRG –> secondary degen axon & dendrites –> motor, sensory, autonomic neuropathy –> sensory deficits, ataxia vs affects single nerve d/t trauma, entrapment, infxn vs affects mult sensory & motor nerves in length dependent fashion –> deficits in feet & ascend vs dmgs several nerves in haphazard
plexopathy vs radiculopathy
affects brachial/lumbar/sacral plexus d/t trauma, compression, penetrating wounds, ca –> pain, weak, sensory deficit, loss DTRs at lvl of plexus & distal in ipsi extremity vs affects spinal nerve roots d/t chronic pressure, herniated disc –> pain, loss sensation in dermatome, muscle weakness innervated by that nerve root
vasculitis neuropathy examples vs toxic neuropathy examples
polyarteritis nodosa, granulomatosis w/ polyangiitis, eos granulomatosis w/ polyangiitis, mononeuritis multiplex –> axon degen vs platinum drugs, taxanes, vinca alkaloids, myeloma tx from chemotherapy-induced peripheral neuropathy
neuropathies from physical force: amputation vs compression/entrap neuropathy; electrodiag studies?
hyperplastic rxn to peripheral nerve dmg 1-12mo post amputation –> disorganized bundles of interlacing regen axons in fibrous stroma vs peripheral nerve chronically subjected to inc pressure; carpal tunnel –> paresthesia, ischemic dmg –> de/remyelin; slow motor nerve conduction velocity, prolonged distal motor latencies
neuropathies w/ tumor: brachial plexopathy vs Horner vs obturator neuropathy
lung ca invading brachial plexus –> pain in shoulder, axilla vs lung, br ca invading lower cervical/upper thoracic spinal cord –> ipsi ptosis, miotic but reactive pupil, facial anhidrosis vs pelvic neoplasms, urothelial ca of bladder –> obturator n compression –> paresthesia, sensory loss, pain in medial thigh
paraneoplastic neuropathies = caused by ca but not via direct invasion: paraneoplastic sensory vs sensorimotor vs autonomic neuropathy
autoimmune against tumor ag similar to DRG; SCLC w/ antiHu vs mimics chronic inflamm demyelinating polyneuropathy; SCLC w/ antiHu & antiCV2, lymphoma, br/ov ca vs SCLC w/ antiHu, pancreas, thyroid, rectum, Hodgkin lymphoma
diabetic neuropathy affects peripheral & autonomic nervous systems: Distal symmetric polyneuropathy vs Diabetic autonomic neuropathy vs Diabetic mononeuropathy vs Diabetic polyradiculoneuropathy
loss sensory & motor neurons –> stock & glove deficits vs cardiovasc, gastric, intestinal, urinary, genital, cutaneous sxs vs median n > ulnar n > radial n > common peroneal n (fib head) vs lumbosacral roots, peripheral nerves –> unilateral pain, muscle weakness, proximal leg atrophy
Charcot-Marie-Tooth type 1 v type 2
Duplication PMP22 –> overexpress PMP22 –> intracellular degrad of membrane –> high arched feet, atrophy of calf => stork leg/inverted champagne bottle vs MFN2 mutation –> abnl mito fusion –> distal muscle weakness & atrophy; electrophys studies show dec cmpd muscular AP but nml conduction vel
hereditary sensory & autonomic neuropathies type 1
auto dom SPTLC1 encoding serine palmitoytransferase in sphingolipid biosynthesis –> amyloid deposits in auto & peripheral nervous system –> no pain/temp, distal to prox muscle weakness, impaired sweating
familial amyloid polyneuropathy
Auto dom TTR mutation encoding transthyretin transporting T4 & retinol in serum –> amyloid deposits in auto & peripheral nervous system –> carpal tunnel, sensorimotor polyneuropathy (stock & glove), autonomic neuropathy (orthostatic hypoTN), incont/retention, ED
MS
multifactorial autoimmune dz w/ relapsing-remitting d/o affecting white matter in CNS that’s separated by space & time d/t DRB1*1501
MS: active vs Inactive vs Shadow plaques
myelin breakdown w/ foamy macs vs hypocellular densely gliotic lesions, no macs or oligodendrocytes vs remyelin lesions by surviving oligodendrocytes, no macs
path vs clin pres of MS
little blue stain vs optic neuritis, CN sxs, spinal cord sxs
img vs CSF of MS
T2 showing Dawson fingers vs clear/colorless, inc igG & oligoclonal bands
central pontine myelinolysis = caused by Osmotic demyelination syndrome
osmotic myelinolysis of white matter tracts dmging oligodendrocytes –> cerebral edema –> hyponatremia –> brain loses K+ –> water leaves brain to dec swelling –> hypotonic brain cells –> hyponatremia sxs
Neuromyleitis optica/Devic dz
infectious viral prodrome –> ab-mediated synchronous or sequential optic neuritis & spinal cord inflamm –> targets astrocytes –> vision loss, para/quadriplegia, sensory loss, incont
labs vs CSF of Neuromyleitis optica/Devic dz
AQP4 autoab in serum vs lymphocytic pleiocytosis / inc protein & nml glu; no oligoclonal bands
Acute disseminated encephalomyelitis
infxn or viral vax –> acute multifocal demyelin d/t myelin autoag –> buildup of lipid-laden macs, axonal dmg in white matter of subcortical regions, cerebellum, brainstem, spinal cord –> encephalopathy, sleepy/lethargy/coma
img vs CSF of Acute disseminated encephalomyelitis
T2W shows hyperintensity in white matter vs inc protein, WBC, ig; rare oligoclonal bands
acute hemorrhagic leukoencephalitis
necrotizing vasculitis of sm vessels w/ fibroid nec, perivascular microhemorrhages /in cerebral white matter, perivascular demyelin w/ inflamm cellular infiltrates in children –> more severe sxs of ADEM
