infantile neuroaxonal dystrophy

infantile neuroaxonal dystrophy

n.

A disorder in infants or young children characterized by slow progressive mental and neurological deterioration

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infantile neuroaxonal dystrophy,

progressive hereditary degenerative encephalopathy transmitted as an autosomal-recessive trait, beginning in infancy with muscular hypotonia and arrest of development in late infancy, followed by dementia, blindness, spasticity, and ataxia. Pathologically it is characterized by widespread focal swellings and degeneration of the axons with scattered globular bodies in the brain. Also called Seitelberger's disease.