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Definition

An autosomal recessive syndrome characterized by optic atrophy beginning in early childhood associated with ataxia, pyramidal signs, spasticity, mental retardation, and posterior column sensory loss. The ataxia, spasticity, and muscle contractures, mainly of the hip adductors, hamstrings, and soleus, are progressive and become more prominent in the second decade.

Acronym

BEHRS

Synonyms

Infantile hereditary optic atrophy with neurologic abnormalities
Optic atrophy, infantile hereditary, with neurologic abnormalities

Keywords

Neurodegeneration