Dejerine–Sottas disease
| Dejerine–Sottas syndrome | |
|---|---|
| Other names | Charcot–Marie–Tooth disease type 3, Dejerine–Sottas syndrome, hereditary motor and sensory polyneuropathy type III |
| MRI compatible with Dejerine-Sottas type spinal nerve enlargement | |
| Specialty | Neurology |
| Symptoms | Weakness, reduced muscle tone, loss of sensation in extremities |
| Usual onset | Infancy or early childhood |
| Causes | Genetic mutations |
| Diagnostic method | Medical 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.