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一名成年克罗恩病患者出现暴发性小脑炎并影像学复发

Fulminant cerebellitis with radiological recurrence in an adult patient with Crohn's disease.

作者信息

Flanagan Eoin P, Rabinstein Alejandro A, Kumar Neeraj, Schroeder Kenneth, Kantarci Orhun H

机构信息

Department of Neurology, Mayo Clinic, Rochester, MN, USA.

Gastroenterology, Mayo Clinic, Rochester, MN, USA.

出版信息

J Neurol Sci. 2014 Jan 15;336(1-2):247-50. doi: 10.1016/j.jns.2013.09.017. Epub 2013 Sep 20.

Abstract

There are few reports of cerebellitis in adults and reports of recurrent cerebellitis are extremely rare. This report highlights both the fulminant course that may follow cerebellitis and a potentially important association with Crohn's disease. A 41-year-old man presented with headache and cerebellar dysfunction a month after ileo-colonic resection for Crohn's disease. MRI demonstrated T2-signal abnormalities, pial enhancement and cerebellar enlargement. His course was complicated by obstructive hydrocephalus, intraventricular hemorrhage associated with external ventricular drain placement and left transverse sinus venous thrombosis. Treatment with high-dose intravenous steroids and anti-coagulation resulted in improvement. Asymptomatic radiological worsening 13 months after initial presentation occurred during a relapse of Crohn's disease. He was treated with intravenous steroids and maintenance azathioprine for his Crohn's and suspected immune mediated cerebellitis. At last follow-up 4 years after initial presentation his clinical status remained unchanged and radiological changes had stabilized. Adult onset cerebellitis may be fulminant and recurrent. Early immunotherapy in acute relapsing cerebellitis may speed recovery and prevent recurrence. Onset and radiological relapse of cerebellitis during Crohn's disease flare-ups as in our case possibly suggests a shared autoimmune pathogenesis.

摘要

关于成人小脑炎的报道较少,而复发性小脑炎的报道极为罕见。本报告强调了小脑炎可能随之而来的暴发性病程以及与克罗恩病潜在的重要关联。一名41岁男性在因克罗恩病行回结肠切除术后1个月出现头痛和小脑功能障碍。磁共振成像(MRI)显示T2信号异常、软脑膜强化和小脑肿大。他的病程因梗阻性脑积水、与外置脑室引流管相关的脑室内出血和左侧横窦静脉血栓形成而复杂化。大剂量静脉注射类固醇和抗凝治疗后病情有所改善。初次就诊13个月后,在克罗恩病复发期间出现无症状的影像学恶化。他接受了静脉注射类固醇治疗,并使用硫唑嘌呤维持治疗克罗恩病和疑似免疫介导的小脑炎。初次就诊4年后的最后一次随访时,他的临床状况保持不变,影像学改变已稳定。成人起病的小脑炎可能是暴发性和复发性的。急性复发性小脑炎的早期免疫治疗可能会加速恢复并预防复发。正如我们病例中所示,在克罗恩病发作期间小脑炎的起病和影像学复发可能提示存在共同的自身免疫发病机制。

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