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Infantile CNS Spongy Degeneration-14 Cases:Clinical Update
Neurol 40:1876-1882, Gascon,G.G.,et al, 1990
See this aricle in Pubmed

Article Abstract
We studied 14 Arab infants with infantile spongy degeneration,13 of whom were products of consanguineous marriages.They presented in infancy with macrocephaly,poor visual behavior or blindness,and axial hypotonia with appendicular spasticity.Brain CT and MRI showed diffuse symmetric leukoencephalopathy,even before neurologic symptoms.There were relatively normal EEGs.The visual evoked responses(P100)were either absent or delayed early in the course.The brainstem auditory evoked responses showed milder abnormalities,with loss of later components before the earlier ones. Deficient aspartoacylase activity in cultured fibroblasts or brain biopsy confirmed the diagnosis in all patients.
 
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aspartocyclase
blindness
brain biopsy
Canavan's disease
CAT scan
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cerebrospinal fluid
consanguinity
cultured skin fibroblasts
degenerative diseases of CNS
developmental milestones
developmental milestones,loss of
developmental retardation
evoked potentials
feeding disorder
genetic neurologic disorders
head circumference
hypotonia
leukoencephalopathy
leukoencephalopathy,differential diagnosis
macrocephaly
MRI
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nystagmus
quadriplegia
review article
seizure
spastic diplegia
spongy degeneration of brain
visual evoked response

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