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Central Nervous System Systemic Lupus Erythematosus (CNS-SLE) Vasculitis Mimicking Lewy Body Dementia: A Case Report Emphasizing the Role of Imaging With an Analysis of 33 Comparable Cases From the Scientific Literature.
Journal of Geriatric Psychiatry and Neurology ( IF 2.6 ) Pub Date : 2020-01-30 , DOI: 10.1177/0891988720901788
Peter Abraham 1 , Ian Neel 2 , Steven Bishay 3 , Daniel D Sewell 1
Affiliation  

INTRODUCTION Neuropsychiatric symptoms occur in 30% to 40% of patients living with systemic lupus erythematosus (SLE). Brain imaging may play a pivotal role in determining the etiology as it did for the case presented here. METHODS A new case of central nervous system (CNS) SLE is presented along with an analysis of 33 comparable cases from the scientific literature. RESULTS A 70-year-old female with subacute cutaneous lupus presented to a university-based geropsychiatry program after 1 year of benign visual hallucinations and several months of shuffling gait, recurrent falls, and forgetfulness. These symptoms were highly suggestive of Lewy body dementia; however, the patient's history of basal ganglia infarct, cognitive testing demonstrating inattention and executive dysfunction, and follow-up brain imaging, which did not reveal acute findings, aligned with cerebral pathology previously attributed to vasculitis and supported the diagnosis of subcortical dementia due to SLE-CNS vasculitis. Oral prednisone 20 mg daily resolved her symptoms. Over the next 19 months, her prednisone was tapered completely and her symptoms did not return. A systematic literature search identified 33 comparable cases. CONCLUSION An analysis of previously published cases suggests that extending the duration of the prednisone taper beyond 1 year may decrease the risk of later occurring neuropsychiatric symptoms in this patient population.

中文翻译:

模仿路易体痴呆的中枢神经系统系统性红斑狼疮 (CNS-SLE) 血管炎:一份强调影像学作用的病例报告,分析了来自科学文献的 33 个可比病例。

引言 30% 到 40% 的系统性红斑狼疮 (SLE) 患者会出现神经精神症状。脑成像可能在确定病因方面发挥关键作用,就像这里介绍的病例一样。方法 介绍了一个新的中枢神经系统 (CNS) SLE 病例以及对来自科学文献的 33 个可比病例的分析。结果 一名患有亚急性皮肤狼疮的 70 岁女性在出现 1 年良性视幻觉和数月的蹒跚步态、反复跌倒和健忘后就诊于大学老年精神病学项目。这些症状高度提示路易体痴呆;然而,患者的基底神经节梗塞病史、认知测试证明注意力不集中和执行功能障碍,以及后续的脑成像,没有显示急性发现,与先前归因于血管炎的脑病理一致,并支持由 SLE-CNS 血管炎引起的皮层下痴呆的诊断。每天口服泼尼松 20 毫克解决了她的症状。在接下来的 19 个月里,她的泼尼松完全减量了,她的症状也没有复发。系统的文献检索确定了 33 个可比较的案例。结论 对先前发表的病例的分析表明,将泼尼松逐渐减量的持续时间延长至 1 年以上,可能会降低该患者群体后期发生神经精神症状的风险。她的泼尼松完全减量,她的症状没有恢复。系统的文献检索确定了 33 个可比较的案例。结论 对先前发表的病例的分析表明,将泼尼松逐渐减量的持续时间延长至 1 年以上,可能会降低该患者群体后期发生神经精神症状的风险。她的泼尼松完全减量,她的症状没有恢复。系统的文献检索确定了 33 个可比较的案例。结论 对先前发表的病例的分析表明,将泼尼松逐渐减量的持续时间延长至 1 年以上,可能会降低该患者群体后期发生神经精神症状的风险。
更新日期:2020-04-12
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