Definition

Friedreich ataxia (FRDA or FA) is an inherited, degenerative nervous system disorder that results in muscle weakness and inability to coordinate voluntary muscle movements.

Description

Onset of FDRA is usually in childhood or early adolescence. The disorder is characterized by unsteady gait, slurred speech, absent knee and ankle jerks, Babinski responses, loss of position and vibrations senses, leg muscle weakness, loss of leg muscle mass, scoliosis, foot deformities, and heart disease. FRDA is a slowly progressive condition associated with a shortened life span, most often due to complications of heart disease.

FRDA is named for Nikolaus Friedreich, the German doctor who first described the condition in 1863. The most common form of the disorder, found in about three–quarters of patients, is referred to as...