Acute severe sensory ganglionopathy in systemic lupus erythematous
| Autor: | Stela Lefter, Brian McNamara, Bernadette Monaghan, Michael J. Regan |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty business.industry Systemic lupus medicine.medical_treatment Central nervous system Sensory system Immunosuppression Disease Dermatology Natural history 03 medical and health sciences 030104 developmental biology 0302 clinical medicine medicine.anatomical_structure Neurology Dorsal root ganglion Peripheral nervous system Pediatrics Perinatology and Child Health medicine Neurology (clinical) business 030217 neurology & neurosurgery Genetics (clinical) |
| Zdroj: | Neuromuscular Disorders. 30:701-706 |
| ISSN: | 0960-8966 |
| Popis: | Sensory ganglionopathies (or neuronopathies) are a rare subgroup of neuropathies characterized by involvement of sensory neurons in the dorsal root ganglion. Although much less common than central nervous system involvement, patients with systemic lupus erythematous (SLE) can develop peripheral nervous system involvement (PNS) and most commonly a chronic length dependent symmetric sensorimotor axonal polyneuropathy as a late complication of the disease. Unlike in Sjogren's syndrome, SLE-associated sensory ganglionopathy is extremely rare and usually manifests in a chronic insidious fashion. We report a 24-year-old man with SLE-associated sensory ganglionopathy manifesting an unusually acute and severe disabling clinical course with a good response to immunosuppressive therapies. Timely recognition of this rare association and early targeted immunosuppression prevented severe neurological sequelae and preserved patient's ambulation. We demonstrate videos on the evolution of patient's neurological impairment and response to treatment, contributing to the current knowledge of the natural history of PNS involvement in SLE. |
| Databáze: | OpenAIRE |
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