FACTSHPI PHYSICAL EXAMIMAGINGA/PNotesMcDonalds CriteriaSchilder’s Disease (Myelinoclastic Diffuse Sclerosis)
FACTS
- idiopathic inflammatory disorder affecting optic nerves, cerebrum, spinal cord
- demographics: 20-40, F > M
- symptoms disseminated in space and time
- pathogenesis: oligodendrocytes damage →loss of myelin sheath
Notes
McDonalds Criteria
# clinical attacks | lesions | Additional criteria to make Diagnosis |
≥ 2 | objective e/o ≥ 2 lesions OR 1 lesion with hx prior attack | None |
≥ 2 | objective e/o 1 lesion | Disseminated in space |
1 | objective e/o 2 lesions | Disseminated in time |
1 | objective e/o 1 lesion | Disseminated in space or wait for further clinical attack |
0 | none | on |
Schilder’s Disease (Myelinoclastic Diffuse Sclerosis)
- mass-like demyelinating lesion that can mimic glioma or abscess radiographically.
- Biopsy may be required when diagnosis uncertain
- MS variant in children with bilateral, large (>2cm) parieto-occipital WM lesions and subacute cognitive/motor decline
- Pathology: destruction of myelin with relative axonal preservation; perivascular lymphocytic infiltration similar to MS.
- Etiology: unclear; likely autoimmune with environmental or infectious trigger in genetically susceptible individuals.
- Differs from MS in its solitary or few large lesions rather than multiple small plaques.
- Acute management: High-dose IV corticosteroids (e.g., methylprednisolone 1 g/day for 3–5 days), followed by oral taper, Plasma exchange for steroid-refractory cases.
- Long-term: Immunomodulatory therapy (interferon beta or other MS-directed agents) in relapsing forms.