What is Dejerine-Sottas syndrome in Charcot-Marie-Tooth (CMT) disease?

Updated: Feb 19, 2019
  • Author: Francisco de Assis Aquino Gondim, MD, MSc, PhD, FAAN; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE  more...
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Answer

Answer

DSS was originally described as a hypertrophic polyneuropathy with onset in infancy or early childhood, distal sensory loss with ataxia, pes cavus with progression towards the proximal limbs, and Argyll-Robertson pupils. Such patients must be tested for mutations in MPZ, PMP22, EGR2, and PRX. Milder and unrecognized hereditary neuropathies in the parents must be considered and investigated.


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