Friedrich’s Ataxia

Friedrich’s Ataxia     Spinocerebellar Degeneration
·       Autosomal recessive; present symptoms late childhood/early adult.·       Ataxic gait, loss of dexterity.·       Pathology-posterior root fibers, sensory fibers of peripheral nerve degenerate. Dorsal root ganglion cell loss.  Central loss also in posterior/lateral columns of spinal cord.

·       Corticospinal fiber loss leads to weakness of the legs, extensor plantar responses.

Evoked sensory SNAP responses small or absent.  Normal motor NCS

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