DejeRine-SotTas.com
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Title
Dejerine-Sottas
Description
Information about Dejerine-Sottas
Dejerine-Sottas is a disease of the peripheral nervous system caused by defects in the genes for proteins found in the protective insulating myelin sheath around the nerves, resulting in eventual loss or thinning of the sheath and subsequent loss of conductivity in the peripheral nerves. First described in the 1890s by Joseph Jules Dejerine (a student of Jean-Martin Charcot) and Jules Sottas (student of Dejerine), Dejerine-Sottas disease was later reclassified as Charcot-Marie-Tooth disease (CMT) Type 3, a rare subtype, because the two diseases have been linked to different defects in the same genes.
(CMT is named after the three physicians who first described it in 1886 — Jean-Martin Charcot and Pierre Marie of France, and Howard Henry Tooth of the United Kingdom. Although most people have never heard of CMT, it affects some 115,000 Americans.)
As researchers attempt to create a coherent classification scheme for neuromuscular diseases, Dejerine-Sottas has also sometimes come to be known as Hereditary Motor Sensory Neuropathy Type III. Some researchers argue that it is really a variant of CMT Type I instead of its own subtype. (With both the name and classification still a subject of debate, it is perhaps unsurprising that little useful research into the disease has been done.)
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- Alexa: DejeRine-SotTas.com