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Neural disease - Coggle Diagram
Neural disease
Multiple sclerosis
Autoimmune disease
gene
SNPs in IL2R and IL7R
MHC II、DR15 and DQ6 haplotype
HLA-DRB1
affected
Th17(聚集 leukocytes)
CD4+ TH1(分泌 IFNγ 活化 macrophages)
Gross
Diffuse / multiple regions
may invade optic nerve
Brown plaque along the occipital horn of the lateral ventricle
Micro
LFB(Luxol fast blue)stain
target : lipoproteins of myelin sheath
Normal : blue / Abn: white
MS plaques(sharp border)around 4th ventricle
Pathology
T cell attack myelin sheath
white matter demyelination
T cell attack β-synuclein of neuron
white & grey matter demyelination
related disease
Leukodystrophy Pelizaeus-Merzbacher disease
Congenital myelin
diseases
X-linked recessive mutation
Proteolipid protein 1(PLP1) dysfunction
No normal myelin sheath
Periventricular leukomalacia
seen in ELBW infants
OPC dead or can't differentiate to OL
No remyelination
white matter dead
Difference w/ hypoxia
can see dead glial cell in hypoxia
Alzheimer’s Disease(AD)
features
Cortical atrophy
hydrocephalus ex vacuo
Micro
Aβ Plaques
extracellular
amyloid core
complement、 proinflammatory
cytokines、 apolipoproteins
dystrophic
neurite
congo-red positive
40 & 42
42 diffuse
tau protein stain
pos. = yellow
hyperphosphorylated tau protein
microtubular associated protein
MAP2
ubiquitin
Ghost tangle / Tombstone tangle
Pathology
Neurofibrillary tangles
Tau
Neuritic plaques
Aβ(mainly) and tau protein
Neocortex 與 limbic cortex atrophy
risk factor
ε4
Acute disseminated encephalomyelitis(ADEM)
Onset
child or youth
Fast
after vac. or viral infection
feature
monophasic
Diffuse
Tx
Pulse steroid
Prion disease
neurodegenerative disease
CJD
Pathology
PrPsc aggregation
Vacuolation of neurons and glia
neuron degeneration
liquefactive necrosis
Stain
Cargo red
PAS
Three distinct causes
sporadic CJD
PRNP mutation
β sheet form easily aggregate
PrPSc seed
recruit β sheet form & unfolded PrP
same mechanism but have protective eff.
codon129
BSE-contaminated food or kuru
v-CJD
PRNP gene mutation
codon 178
aspartate substitution for asparagine
No spongiform pathology
But 失眠、ataxia、自律神經障礙等
Mutation
FTD & ALS
FTD、ALS 表現各佔一半:C9orf72 突變
hexanucleotide GGGGCC repeat expansion
傾向 FTD 表現:Tau(MAPT=microtubule-associated protein tau)
傾向 ALS 表現:SOD1。
Pick’s disease
3R tau
Ch 17 FTDP-17T
Parkinson’s disease(PD)
feature
pallor substantia nigra
pathology
α-synuclein gene mutation