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以急性神经精神障碍为表现的类风湿性软脑膜炎。

Rheumatoid leptomeningitis presenting with an acute neuropsychiatric disorder.

作者信息

Lubomski Michal, Sy Joanne, Buckland Michael, Lee Andie S, Richards Bethan, Thompson Elizabeth, Fulham Michael, Breen Nora, Morris Kirsty, Halmagyi G Michael

机构信息

Department of Neurology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.

School of Medicine, The University of Notre Dame Australia, Sydney, New South Wales, Australia.

出版信息

Pract Neurol. 2019 Feb;19(1):68-71. doi: 10.1136/practneurol-2018-001978. Epub 2018 Aug 10.

DOI:10.1136/practneurol-2018-001978
PMID:30097553
Abstract

Leptomeningitis is a rare central nervous system manifestation of rheumatoid arthritis, generally in patients with established chronic rheumatoid disease. We report a 41-year-old man without previous rheumatoid arthritis or psychiatric disorder who presented with an acute neuropsychiatric disturbance and polyarthralgia. His MR scan of brain showed asymmetric bifrontal leptomeningitis, confirmed on (18F)-fluoro-D-glucose-positron emission tomography. Other investigations showed highly positive serum and cerebrospinal fluid anti-cyclic citrullinated peptide. A leptomeningeal biopsy showed necrotising leptomeningeal inflammation with ill-defined granulomas and lymphoplasmacytic infiltrate without organisms. Prolonged high-dose corticosteroids and then rituximab resulted in recovery. Chronic leptomeningitis can present with an acute neuropsychiatric disorder. We highlight that early rheumatoid disease can, rarely, cause a chronic leptomeningitis, reversible with immunotherapy.

摘要

柔脑膜脑膜炎是类风湿关节炎一种罕见的中枢神经系统表现,通常见于已确诊的慢性类风湿疾病患者。我们报告一名41岁男性,既往无类风湿关节炎或精神疾病史,出现急性神经精神障碍和多关节痛。他的脑部磁共振成像扫描显示不对称性双侧额叶柔脑膜脑膜炎,(18F)-氟代脱氧葡萄糖正电子发射断层扫描证实了这一情况。其他检查显示血清和脑脊液抗环瓜氨酸肽高度阳性。柔脑膜活检显示坏死性柔脑膜炎症,伴有边界不清的肉芽肿和淋巴浆细胞浸润,未发现病原体。长期大剂量使用皮质类固醇,随后使用利妥昔单抗,患者得以康复。慢性柔脑膜脑膜炎可表现为急性神经精神障碍。我们强调,早期类风湿疾病很少可导致慢性柔脑膜脑膜炎,免疫治疗可使其逆转。

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