Pelizaeus-Merzbacher syndrome
slowly progressive dysmyelinating disease
- diffuse, symmetrical demyelination
- cerebrum
- cerebellum
- brainstem
- spinal cord
- "islands" of partially demyelinated fibers
- CT may be normal
- two types:
- X-linked recessive (presents in infancy)
- autosomal dominant (preadulthood)
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© 2002, C.E. Kahn, Jr.
2002-04-18