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Spongy degeneration

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spongy degeneration

(Science: radiology) (canavan disease)

dysmyelinating disease, autosomal recessive, onset at 2 - 9 months, megalencephaly, (alexander disease only other degenerative neurological disease in infants with big head!), blindness with or without deafness, hypotonia may lead to spasticity, diagnosis: brain biopsy, centrum semiovale most severely affected