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Canavan's disease

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A progressive, degenerative disorder of the central nervous system characterised by spongy changes in the white matter. The clinical picture shows muscle rigidity; muscle hypotonia, especially of the neck, with dropping of the head; macrocephaly, mental and motor retardation, optic atrophy with blindness, and exaggerated reflexes. The head can become enlarged. It develops in infancy, usually at the age of three to nine months. Death usually within 18 months after onset of symptoms.

Both sexes affected. It has a predilection for families of Ashkenazi Jewish origin, but has later been observed also in an Irish-American as well as a German family. The affected individuals tend to have blond hair and light complexion, in contrast to the darker hair and complexion of their normal siblings. Inheritance is autosomal recessive.


  • M. M. Canavan:
    Schilder's encephalitis periaxialis diffusa. Report of a case in a child aged sixteen and one-half months.
    Archives of Neurology and Psychiatry, Chicago, 1931, 25: 299-308.
  • L. van Bogaert, I. Bertrand:
    Sur une idiotie familiale avec dégénérescence spongieuse du névraxe (Note préliminaire).
    Acta neurologica et psychiatrica belgica, 1949, 49: 572-587.

  • L. van Bogaert:
    Familial spongy degeneration of brain (Complementary study of Family R).
    Acta Psychiatrica et Neurologica Scandinavica, Copenhagen, 1963, 39: 107-113.
  • L. van Bogaert, I. Bertrand:
    Spongy Degeneration of the Brain in Infancy.
    Amsterdam, Elsevier North-Holland, 1967.
  • M. Mahloudji, K. Daneshbod, M. Karjoo:
    Familial spongy degeneration of the brain.
    Archives of Neurology, Chicago, 1970, 22: 294-298.
  • R. Matalon, K. Michals, D. Sebesta, M. Deanching, P. Gashkoff, J. Casanova:
    Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with Canavn disease.
    American Journal of Medical Genetics, New York, 1988, 29: 463-471.

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