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Concept information

Preferred term

Behr syndrome  

Definition

  • Behr syndrome is characterized by the association of early-onset optic atrophy with spinocerebellar degeneration resulting in ataxia, pyramidal signs, peripheral neuropathy and developmental delay.Although it is an autosomal recessive disorder, heterozygotes may still manifest much attenuated symptoms. (Wikipedia)

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URI

http://data.loterre.fr/ark:/67375/VH8-ZWPG9CNS-H

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