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Alexander's syndrome (William Stuart Alexander)
Also known as:
Alexander’s disease
Associated persons:
William Stuart Alexander
Description:
A rare familial disease with degenerative changes of the central nervous system in children. Occurs in an infantile and a juvenile type. Main characteristics of the infantile type are a rapidly progressive course, megaencaphaly, psychomotor retardation, and seizures. Most children die during the preschool years; a few survive into the second decade. The juvenile type has a later onset and is marked by a protracted course and signs of bulbar palsy, ataxia and, sometimes, mental retardation, occurring without seizures. Prevalent in males. Inheritance is autosomal recessive.
Bibliography:
- W. S. Alexander:
Progressive fibrinoid degeneration of fibrillary astrocytes associated with mental retardation in a hydrocephalic infant.
Brain, Oxford, 1949, 72: 373-381.
- I. Rapin:
Alexander disease. In: L. P. Rowland, editor: Merritt’s textbook of neurology.
7th edition; Philadelphia, Lea & Febiger, 1984, pp. 441-442.
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