Maskery Mark Peter, Cooper Paul N, Pace Adrian
Department of Neurology, Salford Royal NHS Foundation Trust, Salford, UK.
Pract Neurol. 2018 Jun;18(3):246-249. doi: 10.1136/practneurol-2017-001794. Epub 2018 Jan 23.
Sarcoidosis is an idiopathic multisystem granulomatous disorder of unknown cause. Nervous system involvement (central and/or peripheral) is uncommon, developing in 5%-10%. The presenting symptoms are variable, reflecting the level of involvement, and frequently fluctuate and progress. Diagnosing neurosarcoidosis in people with previously confirmed systemic disease may be relatively straightforward, but diagnosing primary neurosarcoidosis is challenging. Managing neurosarcoidosis is primarily consensus based; corticosteroid is its mainstay, alongside corticosteroid-sparing agents and emerging novel therapies. We describe a 39-year-old woman who presented with cranial neuropathy. Serial imaging, cerebrospinal fluid sampling and tissue biopsy gave a diagnosis of probable neurosarcoidosis. Her clinical course was complicated by intracerebral haemorrhage following intravenous corticosteroids for neurological relapse. This is a very rare complication of neurosarcoidosis; we discuss its possible causes and suggest ways to reduce its risk.
结节病是一种病因不明的特发性多系统肉芽肿性疾病。神经系统受累(中枢和/或外周)并不常见,发生率为5%-10%。其表现症状多样,反映了受累程度,且常常波动和进展。对于先前已确诊全身性疾病的患者,诊断神经结节病可能相对直接,但诊断原发性神经结节病具有挑战性。神经结节病的管理主要基于共识;皮质类固醇是其主要治疗方法,同时还有皮质类固醇节省剂和新兴的新型疗法。我们描述了一名39岁患有颅神经病变的女性。系列影像学检查、脑脊液采样和组织活检得出可能为神经结节病的诊断。她的临床病程因神经系统复发静脉注射皮质类固醇后发生脑出血而复杂化。这是神经结节病一种非常罕见的并发症;我们讨论了其可能的病因并提出降低其风险的方法。