Dejerine–Sottas disease

Dejerine–Sottas disease, also known as, Dejerine–Sottas syndrome,[3] hereditary motor and sensory polyneuropathy type III, and Charcot–Marie–Tooth disease type 3, is a hereditary neurological disorder characterized by damage to the peripheral nerves, demyelination, and resulting progressive muscle wasting and somatosensory loss.

Progression is slow until the teenage years at which point it may accelerate, resulting in severe disability.

Some patients may never walk and will be reliant on wheelchair use by the end of their first decade, while others may need only a cane, crutches, or similar support through most of their lives, but this is rare.

Other symptoms may include pain in the extremities, curvature of the spine, clawed hands, foot deformities, ataxia, peripheral areflexia, and slow acquisition of motor skills in childhood.

[2] On medical imaging, the peripheral and cranial nerves are enlarged by redundant connective tissue.