It becomes symptomatic usually at 10-15 years of age, can be earlier or later but almost always before 20 years and most of the patients are usually wheelchair dependent within 10-15 years of disease onset.[1, 6]
Its earliest signs and symptoms vary in onset and speed of progression, but typically include posterior column and peripheral sensory neuropathy, areflexia, progressive limb and gait ataxia (loss of balance and coordination), ascending muscle weakness (begins in