Syndrome of Andermann

The syndrome of Andermann is a genetic disease meeting almost exclusively only with the Quebec, in the population of French origin, and more particularly in the population of Saguenay and the Lake St-Jean. This astonishing distribution is due to a effect founder.

This disease is a neurodégénérative disease which appears during childhood by a Neuropathie severe sensitive and driving leading to a hypotonia, a reduction in the reflexes and a Amyotrophie. Almost two thirds of the patients are carrying a sterility partial and complete of the callous body. The backwardness is often important and there exist demonstrations pseudo psychotics.

The Middle Age of walk in the children reached is 4 years with a loss of locomotion towards the 14 years age. The lifespan is reduced with an Middle Age of death at 33 years.

Sources

Nicolas Dupre, Heidi C Howard, Guy has Roller, Hereditary Motor and Sensory Neuropathy with Agenesis off the Corpus Callosum in GeneTests: Medical Genetics Information Resource (database online). Copyright, University off Washington, Seattle. 1993-2006

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