Published December 29, 2024 | Version v1
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LA MALADIE DE THEVENARD : A PROPOS DUNE OBSERVATION

  • 1. Service de Pediatrie CHU de Fes Exploration de Neuropediatrie.

Description

Thevenard disease is a hereditary sensory neuropathy characterized by a progressive distal neurotrophic syndrome. This condition, first described by Denny-Brown as hereditary sensory radicular neuropathy (HSRN), follows an autosomal dominant inheritance pattern. Diagnostic delays and significant comorbidities often render this disease debilitating.We present the case of a patient under pediatric neurology follow-up since 2018 for the management of sensory polyneuropathy, accompanied by pressure ulcers and foot arthropathies. Functionally, the patient was able to walk without antalgic gait. Electroneuromyography revealed sensory polyneuropathy affecting all four limbs. A multidisciplinary approach involving medical and orthopedic treatments was implemented, addressing delayed wound healing and incorporating sensorimotor rehabilitation.Thevenard disease is a rare familial ulcero-mutilating acropathy that affects both sexes and requires comprehensive care across various specialties.

 

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