Dejerine-Sottas Disease/Type 3 HMSN

Wikis > Paediatrics > Neurological and Neuromotor Disorders > Dejerine-Sottas Disease/Type 3 HMSN

Dejerine-Sottas Disease/Type 3 HMSN

Autosomal recessive: Characterised by hypertrophic interstitial neuropathy and is pathologically similar to CMT. Begins in infancy. Motor milestones are delayed and sensation is impaired – reflexes absent. Develop pes cavus and drop foot deformities. Spinal deformities  usually confined to wheelchair.

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