Pathology
Pathologically, Friedreich's ataxia shows:
- characteristically small spinal cord, especially in the thoracic region
- marked degeneration in the posterior columns and lateral columns, especially the corticospinal and posterior spinocerebellar tracts
- dorsal roots and peripheral nerves shrunken in advanced cases
- Purkinje cell loss and atrophy of the dentate nucleus in the cerebellum
- corticobulbar tract and cerebrum relatively unaffected
- peripheral nerves show segmental demyelination
- heart may show enlargement due to thickening of the muscle and a diffuse fibrosis
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