“…The neuropathology in FRDA is characterized by loss of large sensory neurons in the dorsal root ganglia, atrophy of the large sensory fibers in peripheral nerves, of the posterior columns, spinocerebellar and pyramidal tracts in the spinal cord and of the dentate nucleus in the cerebellum [4]. Patients show progressive gait and limb ataxia, dysarthria, lower limbs areflexia, decreased vibration sense, muscle weakness in the legs, and positive extensor plantar responses [5–8]. Most patients also have hypertrophic cardiomyopathy, and about one third have diabetes or carbohydrate intolerance [9–12].…”