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. 2018 Nov 2;2(4):NS20180060. doi: 10.1042/NS20180060

Table 1. Clinical features of FRDA.

System Pathology Clinical result
Neurological Degeneration of large sensory neurones – proprioception Loss of balance and coordination
Loss of deep tendon reflexes
Degeneration of spinocerebellar tracts (dorsal) Loss of balance and coordination
Degeneration of dentate nucleus of the cerebellum Loss of balance and coordination
Dysarthria (slurred speech)
Eye movement abnormalities (modest)
Degeneration of corticospinal tracts Spasticity, pyramidal weakness
Visual Degeneration of retinal ganglion cells Optic neuropathy
Auditory Degeneration of auditory nerve Auditory neuropathy
Cardiac Hypertrophic cardiomyopathy, with early hypertrophy, later fibrosis ECG abnormalities
Arrhythmias
Progressive heart failure
Endocrine Loss of pancreatic islet cells Diabetes mellitus
Increased insulin resistance Diabetes mellitus
Orthopedic Scoliosis
Pes cavus (fixed plantar foot flexion; high arched feet)

Abbreviation: ECG, electrocardiogram.