Dejerine Sottas disease

Dr Henry Knipe and Dr Yuranga Weerakkody et al.

Dejerine-Sottas disease (also sometimes known as hereditary motor and sensory neuropathy type III or hypertrophic interstitial polyneuritis) is a rare hereditary motor and sensory neuropathy (HMSN).

The disease is characterised by an early-onset demyelinating neuropathy, and usually manifests as gradual progression of distal weakness, sensory loss, and areflexia in the legs. As the disorder progresses, involvement extends to the upper extremities, and palpable peripheral nerves become evident.

It tends that presents with distal extremity motor and sensory symptoms as well as palpable peripheral nerves. Cranial nerve involvement is reportedly seen in ~15% of cases.

Genetics

It is largely inherited as an autosomal recessive condition, with occasional sporadic cases 1.

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Article information

rID: 16789
Synonyms or Alternate Spellings:
  • Dejerine-Sottas syndrome
  • Hereditary motor and sensory neuropathy type III
  • HMSN III
  • HMSN type III
  • Dejerine-Sottas syndrome (DSS)
  • Dejerine-Sottas disease

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