Friedreich's Ataxia

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Friedreich’s Ataxia

 

a hereditary disease marked by slowly progressing ataxia due to sclerosis of the dorsal and lateral columns of the spinal cord and hypoplasia of the cerebellum and spinal cord. The disease was first described by N. Friedreich in 1861.

Inheritance is of the autosomal or, less commonly, autosomal dominant type. The disease usually becomes manifest between the ages of seven and 13. Besides ataxia, it is attended by disappearance of tendon reflexes, lack of responsiveness to stimulation, dysarthria, muscular hypotonia, bone deformity (kyphoscoliosis of the thoracic segment of the vertebral column, change in shape of the foot—Friedreich’s foot). Friedreich’s ataxia is often associated with congenital heart disease. General supporting treatment and orthopedic correction of the bone defects are prescribed. Prevention is based on genetic counseling.

REFERENCE

Tsuker, M. B. Osnovy nevropatologii detskogo vozrasta. Moscow, 1947.