Np resident lecture-wmdisease2009-slideshareupload

DISEASES OF WHITE MATTER Charles L. White, III, M.D. November 19, 2009

Oligodendroglia ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Oligodendroglia in white matter

Oligodendroglia: Myelin stain

Oligodendroglia: Nodes of Ranvier ,[object Object]

Phenomenon of “selective vulnerability” ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Demyelinating disorders: Substrates of selective vulnerability ,[object Object],[object Object]

Primary demyelination vs. secondary loss of myelin ,[object Object],[object Object],[object Object],[object Object]

Secondary loss of myelin: Amyotrophic lateral sclerosis

Primary demyelinating disorders ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Demyelinating disorders: General features ,[object Object],[object Object],[object Object],[object Object]

Dysmyelinating disorders (leukodystrophies) ,[object Object],[object Object],[object Object],[object Object]

Dysmyelinating disorders (leukodystrophies) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Leukodystrophy: Confluent demyelination

Leukodystrophy: Subcortical “U-fiber” sparing

Leukodystrophies: Pathogenesis Disease Enzyme deficiency Toxic agent Krabbe disease Galactocerebroside β -galactosidase (GALC) Galactosylsphingosine (psychosine) Metachromatic leukodystrophy (MLD) Arylsulfatase A Sulfatides Adrenoleukodystrophy (ALD) ABCD1 VLCFA

Krabbe disease (globoid cell leukodystrophy) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Krabbe disease: “Globoid” cells

Metachromatic leukodystrophy ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Metachromatic leukodystrophy: Peripheral nerve involvement

Adrenoleukodystrophy ,[object Object],[object Object],[object Object],[object Object],[object Object]

Adrenoleukodystrophy: Asymmetrical frontal demyelination

Adrenoleukodystrophy: Adrenal cortical cytoplasmic inclusions

Adrenomyeloneuropathy ,[object Object],[object Object],[object Object],[object Object],[object Object]

Myelinoclastic disorders ,[object Object],[object Object]

Multiple sclerosis: Clinical features ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Multiple sclerosis: Epidemiology ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Multiple sclerosis: Laboratory findings ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Multiple sclerosis: Widely distributed lesions

Multiple sclerosis: Periventricular plaques

Multiple sclerosis: Gross plaque morphology

Multiple sclerosis: active plaque

Multiple sclerosis Active plaque

Multiple sclerosis: Active demyelination Myelin stain (blue) with PAS counterstain CD68 (histiocytes)

Multiple sclerosis: Microscopic plaque morphology H&E Myelin stain CD68 (histiocytes) Axon stain H&E

Multiple sclerosis: Relative axonal preservation Normal white matter Demyelinated plaque

Multiple sclerosis: Pathogenesis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

MS variants ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]

Animal models of MS ,[object Object],[object Object],[object Object],[object Object],[object Object]

Parainfectious disorders ,[object Object],[object Object],[object Object]

Parainfectious disorders ,[object Object],[object Object]

ADEM Small foci of perivenular demyelination

Np resident lecture-wmdisease2009-slideshareupload

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