Dejerine–Sottas disease

The Dejerine -Sottas disease is one of the hereditary motor and sensory neuropathies, a group of inherited diseases of the peripheral nerves. It is referred to as HMSN type III and was named after Joseph Jules Dejerine the neurologist (1849-1917) and Jules Sottas (1866-1943) named.

The disease can be inherited in an autosomal dominant and recessive. The clinical picture corresponding to the hereditary sensory-motor neuropathy type I, the disease starts earlier and is stronger, however, in the expression.

In Dejerine -Sottas disease can in the cerebrospinal fluid, the protein concentration to up to 2000 mg / L may be increased, however, the various types HMSN can not be distinguished due to the Liqourdiagnostik.