See Also: dejerine-Sottas disease(medicine)
Dejerine's disease(medicine)
Sottas, Jules(medicine)
Herbal medicine (botanical medicine, herbology, phytomedicine)(health)
dejerine(medicine)
Dejerine's sign(medicine)
Dejerine's reflex(medicine)
Dejerine-Klumpke(medicine)
Dejerine-Roussy syndrome(medicine)
Dejerine-Lichtheim phenomenon(medicine)

dejerine-Sottas disease (medicine)


Dejerine-Sottas disease


A familial type of demyelinating sensorimotor polyneuropathy that begins in early childhood and is slowly progressive; clinically characterised by foot pain and paresthesias, followed by symmetrical weakness and wasting of the distal limbs; one of the causes of stork legs; patients are wheelchair bound at an early age; peripheral nerves are palpably enlarged and non-tender; pathologically, onion bulb formation is seen in the nerves: whorls of overlapping, intertwined Schwann cell processes that encircle bare axons; usually autosomal recessive inheritance.

Synonym: Dejerine's disease, progressive hypertrophic polyneuropathy.