Acute severe sensory ganglionopathy in systemic lupus erythematous

Abstract

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.