Rheumatology and Clinical Immunology Unit, Internal Medicine Department, Faculty of Medicine, Cairo University, Giza, Egypt.
Department of Medicine, Rochester General Hospital, 1425 Portland Ave, Rochester, NY, 14621, USA.
Rheumatol Int. 2017 Nov;37(11):1917-1930. doi: 10.1007/s00296-017-3773-7. Epub 2017 Jul 13.
The first cases of systemic lupus erythematosus (SLE) with effects on the nervous system were reported more than 100 years ago. Cerebellar involvement and obstructive hydrocephalus are rarely encountered in patients with SLE. We report two patients with SLE who developed cerebellar ataxia. The first patient presented with significant headache at time of SLE diagnosis and negative brain imaging studies on initial evaluation. The headache recurred with findings of cerebellar ataxia and obstructive hydrocephalus. The patient's symptoms markedly improved with shunt insertion and immunosuppressive therapies. The second patient, known to have SLE, presented with crossed hemiplegia and cerebellar symptoms. Imaging studies showed cerebellar atrophy as well as thalamic and brainstem lesions. The patient had partial improvement with immunosuppression. To understand the impact of SLE on the cerebellum and in hydrocephalus formation, we reviewed the literature and case reports over the last 50 years. Clinical presentations, laboratory results, imaging results, treatment and outcomes, for reported cases found in the literature, were summarized and discussed along with our conclusions. We emphasize the importance of history, examination and close monitoring of patients with SLE presenting with headache or any new neurological symptoms.
系统性红斑狼疮(SLE)对神经系统影响的首例病例早在 100 多年前就有报道。小脑受累和阻塞性脑积水在 SLE 患者中很少见。我们报告了两例 SLE 患者发生小脑性共济失调。第一例患者在 SLE 诊断时出现严重头痛,初次评估时脑部影像学检查未见异常。头痛复发伴小脑共济失调和阻塞性脑积水。患者的症状在分流植入和免疫抑制治疗后显著改善。第二位患者已知患有 SLE,表现为交叉性偏瘫和小脑症状。影像学检查显示小脑萎缩以及丘脑和脑干病变。患者经免疫抑制治疗后部分缓解。为了了解 SLE 对小脑和脑积水形成的影响,我们回顾了过去 50 年的文献和病例报告。总结并讨论了文献中报道的病例的临床表现、实验室结果、影像学结果、治疗和预后。我们强调了对出现头痛或任何新的神经系统症状的 SLE 患者进行病史、检查和密切监测的重要性。