Essential | Age of onset before 25 years |
---|---|
Progressive ataxia of gait and limbs | |
Absent knee and ankle jerks | |
Axonal picture on neurophysiology | |
Dysarthria (if after five years from onset) | |
Additional (present in over 66%) | Scoliosis |
Pyramidal weakness in lower limbs | |
Absent reflexes in arms | |
Large fibre sensory loss on examination | |
Abnormal ECG | |
Others (less than 50%) | Nystagmus |
Optic atrophy | |
Deafness | |
Distal amyotrophy | |
Pes cavus | |
Diabetes |