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Dejerine-Roussy syndrome <syndrome> A syndrome produced by infarction of the postero-inferior thalamus causing transient hemiparesis, severe loss of superficial and deep sensation with preservation of crude pain in the hypalgic limbs which frequently have vasomotor or trophic disturbances.
Synonym: Dejerine-Roussy syndrome.
(05 Mar 2000)
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.
(05 Mar 2000)
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