Alexander's syndrome (William Stewart Alexander)
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.
- 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. Niels Holm-Nielsen, editor:
• Klinisk Ordbog. Munksgaard, Copenhagen, 1991.
- Barry G. Firkin and Judith A. Whitworth:
Dictionary of Medical Eponyms.
The Parthenon Publishing Group. 1989. New edition in 2002.
- Sergio I. Magalini, Sabina C. Magalini, Giovanni de Francisi:
Dictionary of Medical Syndromes. J. B. Lippincott Company, Philadelphia. 1990.
- Stanley Jablonski:
Jablonski’s Dictionary of Syndromes & Eponymic diseases.
Krieger Publishing Company, Malabar, Florida, 1991.