Hereditary sensory neuropathy with spastic paraplegia

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
Louisa Kherbaoui-RedouaniJacques Motte

Abstract

We report the case of a 4-year-old boy with hereditary sensory neuropathy manifesting as insensitivity to pain in all four limbs and associated with spastic paraplegia. The patient was referred with multiple injuries to his fingers suggestive of self-mutilation and attributed to psychiatric disturbance. Clinical examination corrected the diagnosis by revealing insensitivity to pain in all four limbs, associated with spastic paraplegia. The histamine test reaction was positive, nerve biopsy and electrophysiological investigations confirmed sensory nerve involvement and the diagnosis of hereditary sensory neuropathy. Classification and treatment of such rare diseases is difficult and a multidisciplinary approach is often necessary.

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Jan 1, 1992·Clinical Neurology and Neurosurgery·G HagemanA R Rozeboom
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