Friedreich ataxia (spinocerebellar ataxia) 4

General: Etiology unknown, either au 151i89b tosomal recessive or dominant; progressive; incapacitating by age 20 years; death from secondary diseases or cardiac failure; prevalent in males.

Ocular: Nystagmus; optic atrophy; there is a form of Friedreich ataxia associated with congenital glaucoma.

Clinical: Kyphoscoliosis; tremor; dysmetria; asynergia; slow ataxic speech; paresthesias; Babinski sign; headache; retarded growth; mental retardation; polyuria; polydipsia; deformity of feet (onset in first year of life); clumsy gait and difficult to turn arms, head, and trunk; deafness.

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