脱髄・髄鞘障害性疾患 - UMIN

1 (major dense line) (intraperiod line) 1a (oligodendrocyte: OLs) (Schwann cell: SCs) OLs SCs SCs (basal lamina) OLs OLs (neural stem cell) (bipolar progenitor) (polydendrocyte) pro-OLs OLs OLs 1c OLs halo 1b (intrafascicular) OLs (satellite) (perineuronal) OLs OLs OLs 1c OLs 2',3'-Cyclic-nucleotide 3'-phosphodiesterase (CNPase) proteolipid protein (PLP) Satellite OLs OLs SCs SCs SCs SCs SCs S100 c c luxol fast blue (LFB)

2 Myelin basic protein (MBP), CNPase, PLP Bodian amyloid precursor protein ( APP) (myelin pallor) Table 1 (demyelination) dysmyelination (leukodystrophy) Table 1 Demyelinating diseaseAutoimmune and suspected autoimmune (demyelinating diseases)MS and variants, ADEM, Rabies post-vaccinal encephalomyelitis, Acute hemorrhagic leukoencephalitisViralPML, SSPE, HIV encephalopathy, HIV vacuolar myelopathy, HTLV type I- and II-associated myelopathy (tropical spastic paraplegia)GeneticALD, AMN, MLD (sulphatide lipidoses), GCL (Krabbe disease, galactosylceramide lipidosis), PMD and related PLP1 mutations, Spongiform leukodystrophy (Canavan disease), Dysmyelinogenetic leukodystrophy (Alexander disease), Membranous lipodystrophy (Nasu-Hakola disease), Neuroaxonal leukodystrophy, Leukoencephalopathy with vanishing white matter, MCL, etcNutritional/metabolicMarchiafava-Bign ami disease, Vitamin B12 deficiency (subacute combined degeneration)

3 , Osmotic demyelination syndrome, Hereditary coproporphyriaToxicHexachlorophene intoxication, Solvent vapour leukoencephalopathy, Leukoencephalopathy associated with combined antimitotic medication and radiotherapy, Chmotherapeutic agents( 5-FU, amphotericin B, etc), Carbon monoxide poisoning, Heroin leukoencephalopathyDisorders with varied and overlapping pathogenesisProlonged cerebral edema, Hypoxic-ischemic leukoencephalopathy, Cerebrospinal fluid exchange, Multifocal necrotizing leukoencephalopathy, Focal demyelination associated with compression in trigeminal neuralgia, Leukoencephalopathy in patients with AIDS on antiretroviral therapy, Small vessel ischemia, Vasculitis/arteritis, Congophilic angiopathy with leukoencephalopathy, Solitary focal demyelination associated with paraneoplastic syndromeADEM, acute disseminated encephalomyelitis; AIDS, acquired immunodeficiency syndrome; ALD, adrenoleukodystrophy; AMN, adrenomyeloneuropathy; CADASIL, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

4 GCL, globoid cell leukocystrophies; HIV, human immunodeficiency virus; HTLV, human T-cell lymphotropic virus; MCL, Megalencephalic cystic leukoencephalopathy; MLD, metachromatic leukodystrophies; MS, multiple sclerosis; PMD, Pelizaeus-Merzbacher disease; PML, progressive multifocal leukoencephalopathy; SSPE, subacute sclerosing pan-encephalitis. multiple sclerosis: MS MS 3a mm cm (demyelinating plaque) H&E 2a, 2e OLs 2b, 2f 2c, 2g Sudan oil red O (spheroid)

5 2f APP (tumefactive MS) a b c de f g h (tumefactive MS) (a, e) , b, f (ACs) (c) Creutzfeldt cell (d) (g) oligodendrocyte lymphocyte ACs emperipolesis (h) KB (a), bodian (b), H&E (c, d), MBP (e), neurofilaments (f), CD68 (h), glial fibrillary acidic protein (GFAP) (i) (atypical mitotic figure) Creutzfeldt astrocyte 2d glioma OLs (emperipolesis) 2h OLs shadow plaque 3h, 3i 3b-3g, 3j, 3k (Bal s concentric sclerosis) a b cd e f gh i j k 3 (e, j, k) (d, f, g) (b, c) shadow plaque (h) (i) H&E (b), GFAP (c), KB (d, f, h), bodian (e, i, j), MBP (g), neurofilaments (k) (neuromyelitis optica.)

6 NMO) 3a a b c d e f g h i j KB (a) (b-g) (h-j) aquaporin (AQP) 1 AQP4 H&E (b), KB (c), bodian (d), GFAP (e, h), AQP1 (f, i), AQP4 (g, j) SCs OLs 4b-g NMO (NMO-IgG) (aquaporin 4; AQP4) NMO AQP4 (astrocytopathy) glial fibrillary acidic protein (GFAP) 4e AQP4 GFAP 4g (acute disseminated encephalomyelitis: ADEM) EB HA HB SLE T (experimental allergic encephalomyelitis) (acute hemorrhagic leukoencephalitis.

7 AHL) ADEM 5 a b c de f g h PML KB (a), H&E (b-d), agnoprotein (e), VP1 (f), PML in situ hybridization (g), (h): (progressive multifocal leukoencephalopathy: PML)HIV HTLV-1 papova virus (JC virus) OLs HIV HAART PML-IRIS (immune reconstitution inflammatory syndrome: OLs JC virus agnoprotein VP1 in situ hybridization (Leukodystrophy) Table 2 Table 2 - (orthochromatic)

8 OLs ACs Leukoencephalopathy with vanishing white matter (sudanophilic) Megalencephalic leukoencephalopathy with subcortical cysts (OLD or SLD) OLD (SLD) with calcification OLD (SLD) with other specific features OLD (SLD) with pigmented cells OLD (SLD) with meningeal angiomatosis OLD (SLD) with no other specific features OLD (SLD) not otherwise specified (Alexander disease) (fibrinoid leukodystrophy: FLD) 90% GFAP 2 (Rosenthal) 6 (Canavan disease)aspartoacylase N-acetyl-aspartate Alzheimer II 6 HE 7 LFB-PAS Krabbe disease, globoid leukodystrophy (galactocerebrosidase.)

9 GALC) GALC (psychosine) OLs PAS 7 10-100nm SCs 8 (a, d) (b) (f, g) H&E (a), Holzer (b), Bodian (c, e), LFB-HE (d), (f), Hirsch-Pfeiffer (g) (metachromatic leukodystrophy)a b cd ef g (aryl sulfatase) A (sulfatide) SCs PAS LFB Hirsch-Pfeiffer (cresyl violet) (toluidine blue) 8 (adrenoleukodystrophy: ALD)X adrenoleukodystrophy protein (ALDP) X ALDP (very long chain fatty acid.

10 VLCFA) VLCFA PAS OLs Leidig PA S OLs (Pelizaeus-Merzbacher disease; PMD) PLP Connatal Classic (myelin islet) OLs (Cockayne's syndrome)DNA (ERCC6, ERCC8, etc) 10-20 PMD