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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.