spongy degeneration (Canavan disease)

• onset at 2 - 9 months

• megalencephaly
  • (Alexander disease only other degenerative neurological disease in infants with big head!)

• blindness +/- deafness
• hypotonia --> spasticity

• Dx: brain bx
• centrum semiovale most severely affected

© 2002, C.E. Kahn, Jr.
2002-04-18