Dejerine–Sottas disease

Dejerine–Sottas syndrome
Other namesCharcot–Marie–Tooth disease type 3, Dejerine–Sottas syndrome, and hereditary motor and sensory polyneuropathy type III.
MRI compatible with Dejerine-Sottas type spinal nerve enlargement
SpecialtyNeurology 
SymptomsWeakness, reduced muscle tone, loss of sensation in extremities.
Usual onsetInfancy or early childhood.
CausesGenetic mutations.
Diagnostic methodMedical imaging.

Dejerine–Sottas disease, also known as, Dejerine–Sottas syndrome, 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. The condition is caused by mutations in various genes and currently has no known cure.

The disorder is named for Joseph Jules Dejerine and Jules Sottas, French neurologists who first described it.

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