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.

中文翻译：

---

系统性红斑狼疮的急性重度感觉神经节病

感觉神经节病（或神经元病）是一种罕见的神经病亚组，其特征是感觉神经元受累于背根神经节。虽然比中枢神经系统受累少得多，但系统性红斑狼疮 (SLE) 患者可发生周围神经系统受累 (PNS)，最常见的是慢性长度依赖性对称性感觉运动轴索多发性神经病，作为该疾病的晚期并发症。与 Sjogren 综合征不同，SLE 相关的感觉神经节病极为罕见，通常表现为慢性隐匿性。我们报告了一名 24 岁男性，患有 SLE 相关的感觉神经节病，表现出异常的急性和严重的致残临床病程，对免疫抑制疗法有良好的反应。及时识别这种罕见的关联和早期靶向免疫抑制可预防严重的神经系统后遗症并保护患者的行走能力。我们展示了有关患者神经功能障碍演变和治疗反应的视频，有助于了解 SLE 中 PNS 自然病程的当前知识。