Demyelinating Neuropathology
Demyelinating Neuropathology
What You'll Learn
- The pathology of multiple sclerosis — active vs chronic plaques — and its fulminant variants
- Antibody-mediated demyelination (NMOSD, MOGAD) and how it differs from MS on tissue
- PML and other secondary / metabolic demyelination (osmotic demyelination, B12, Marchiafava-Bignami)
- The classic inherited leukodystrophies and their diagnostic buzzwords
The neuropathology view. Clinical diagnosis and treatment of MS/NMOSD live in the Demyelinating / Neuroimmunology notes; the inherited white-matter diseases are covered in Leukodystrophies.
Plaque stages (know the contrast)
| Plaque | Histology | Stains |
|---|---|---|
| Active | Hypercellular; sheets of foamy macrophages full of myelin debris; perivascular lymphocytes; relative axonal sparing | LFB+ debris in macrophages; CD68+; axons preserved (neurofilament) |
| Chronic active (smoldering) | Gliotic center, rim of iron-laden macrophages/microglia at the expanding edge | Rim macrophages CD68+; iron stain rim+ |
| Chronic inactive | Sharply demarcated, hypocellular, dense astrogliosis, oligodendrocyte and axon loss | LFB pale (myelin lost); GFAP+ gliosis |
| Shadow plaque | Partial remyelination — thin myelin, blurred border | LFB pale-but-present |
Plaque stages (know the contrast)
| Variant | Pathology | Buzzword |
|---|---|---|
| Marburg | Massive, destructive demyelination with axonal loss & edema | Fulminant, often fatal |
| Baló concentric sclerosis | Alternating rings of preserved and lost myelin | “Onion-bulb / tree-ring” on MRI |
| Tumefactive demyelination | Large solitary lesion mimicking tumor; macrophages, Creutzfeldt-Peters cells (reactive astrocytes with fragmented nuclei), open ring enhancement | Don't call it glioma — look for the macrophage sheet |
| ADEM | Perivenular (“sleeves of Virchow-Robin”) demyelination & inflammation; monophasic, post-infectious/vaccinal | Perivenular vs confluent (MS) |
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