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.
It is largely inherited as an autosomal recessive condition, with occasional sporadic cases 1.
- enlarged cauda equina nerve roots / cranial nerves 1
- 1. Maki DD, Yousem DM, Corcoran C et-al. MR imaging of Dejerine-Sottas disease. AJNR Am J Neuroradiol. 1999;20 (3): 378-80. AJNR Am J Neuroradiol (full text) - Pubmed citation
- 2. Gabreëls-festen A. Dejerine-Sottas syndrome grown to maturity: overview of genetic and morphological heterogeneity and follow-up of 25 patients. J. Anat. 2002;200 (4): 341-56. J. Anat. (link) - Free text at pubmed - Pubmed citation