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Behr's syndrome I

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A hereditary familial syndrome of ocular and neurologic disorders affecting both sexes. Onset in infancy with disturbed vision, disturbed coordination with ataxia, urinary sphincter weakness, and mental deficiency. Other characteristics include optic atrophy (prevalent in males), nystagmus, scotoma, progressive temporal nerve atrophy, bilateral retrobulbar neuritis, increased tendon reflexes, Babinski sign, and incoordination. Inheritance is autosomal recessive.


  • C. Behr:
    Die komplizierte, hereditär-familiäre Optikusatrophie des Kindesalters: ein bisher nicht beschriebener Symptomkompleks.
    Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1909, 47: 138-160.

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