Disease Information for Neuropathy, hypertrophic interstitial

Dejerine Sottas Disease; Hereditary Motor Sensory Neuropathy Type III, HSMN Type III; Hypertrophic Interstitial Neuritis; Hypertrophic Interstitial Neuropathy; Hypertrophic Interstitial Radiculoneuropathy;

Onion-Bulb Neuropathy; Dejerine-Sottas disease is an inherited neurological disorder that progressively affects mobility; Peripheral nerves become enlarged or thickened leading to muscle weakness; Progress of the disorder is irregular and often accompanied by pain, weakness, numbness, and a tingling, prickling or burning sensation in the legs; Many people with Dejerine-Sottas disease continue to lead active lives; Most neurologists now consider this disorder to be one of 5 types of hereditary motor sensory neuropathy (HMSN) which simply means genetically transmitted disorder of the nerves associated with movement; Dejerine-Sottas disease is one of several that comprise Type III and in which the protective sheath around the long nerves breaks down (demyelination) for unknown reasons exposing and endangering the nerve; The nerves are enlarged due to an accumulation of connective tissue that may present in the form of "onion-bulbs"; ----------- [NORD 2005] ------Also AKA Gombault’s degeneration or demyelination; ombault"s neuritis; Gombault’s syndrome; Neuritis hypertrophica, progressive and hypertrophic interstitial neuritis of infants, hypertrophic interstitial neuritis, hypertrophic interstitial polyneuritis, hypertrophic interstitial neuropathy, hypertrophic neuritis syndrome, nitrite interstitially hypertrophique progressive (French), progressive hypertrophic interstitial neuritis, segmental demyelination; A slowly progressive hereditary form of hypertrophic neuritis characterised by motor and sensory disturbance in the extremities commencing in childhood or adolescence, with numbness, paresthesia, cramps, and lancinating pain in the extremities, followed by muscle weakness and clawing of the hands and deformities of the feet; Affected muscles show decreased responsiveness to electric stimulation; The nerve roots are thickened and show characteristic «onion bulb» histological changes (Schwann-cell hypertrophy); Ocular complications include myosis, nystagmus, anisocoria, papillotonia, and optic atrophy; The disturbance is believed to be transmitted as an autosomal dominant trait

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