Dictionary > Dejerine-sottas disease

Dejerine-sottas disease

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.


You will also like...

Evolution of Life - Ancient Earth
Evolution of Life – Ancient Earth

Autotrophs flourished, absorbing carbon and light. Soon after, primitive life forms that could assimilate oxygen thrived..

green leaf - close up view
Leaves

Leaves are the major photosynthetic organ of a plant. Apart from that, they are also crucial to water movement. In this ..

Lake Wakatipu, New Zealand
New Zealand’s Unique Geographical History

Explore why New Zealand has such unique flora and fauna, and learn why long periods of geographical isolation. This less..

Freshwater Ecology
Freshwater Ecology

Freshwater ecology focuses on the relations of aquatic organisms to their freshwater habitats. There are two forms of co..

genes controlling growth and development
Control of Growth & Development

Control of Growth & Development tutorials look at how the genetic makeup determines the biological processes on a da..

Sensory systems
Sensory Systems

A sensory system is a part of the nervous system consisting of sensory receptors that receive stimuli from the internal ..