Neurological Diagnosis of Friedreich’s ataxia is described below
Loss of large sensory neurons (diminishing proprioception, balance, coordination and reflexes).
Loss of spinocerebellar tracts (diminishing balance and coordination).
Loss of dentate nucleus of the cerebellum (causing dysarthria, modest eye-movement abnormalities).
Cerebellar cortex and cerebral cortex are spared, cognition is normal.
MRI scans are normal or almost normal in early disease.