Whittaker Konrad, Guggenberger Konstanze, Venhoff Nils, Doostkam Soroush, Schaefer Hans-Eckart, Fritsch Brita
Department of Neurology and Neuroscience, Medical Center, University of Freiburg, Breisacher Straße 64, D-79106, Freiburg, Germany.
Department of Neuroradiology, University of Freiburg, Breisacher Straße 64, D-79106, Freiburg, Germany.
BMC Neurol. 2018 Oct 3;18(1):161. doi: 10.1186/s12883-018-1163-8.
Crohn's disease (CD) is associated with a variety of extra-intestinal manifestations. Most commonly these involve the eye, skin, joints, coagulation system and liver. Cerebral manifestations of CD have been reported to a far lesser extent. The extensive detrimental impact of neurological symptoms on a patient's quality of life makes an early diagnosis and treatment particularly important. In previous case-reports, diagnosis of cerebral manifestations in CD often relied upon magnetic resonance imaging (MRI) and computed tomography (CT) alone. To our knowledge, only one case-report has documented a histologically confirmed case of cerebral lesions associated with CD so far.
A 39-year-old right-handed woman with a history of CD was referred to our hospital with etiologically unexplained Gadolinium (Gd)-enhancing cortical lesions, triggering epileptic seizures. A CT-scan of the thorax and bronchoalveolar lavage found no signs of sarcoidosis. Lumbar punctures and laboratory testing found no underlying infection or coincidental autoimmune disorders and MRI-scans showed progression of lesion load. Consequently, the patient underwent stereotactic biopsy of a cortical lesion. Histological examination revealed a mixed lympho-histiocytic and tuberculoid granulomatous inflammation surrounding small vessels and no signs for infection. After exclusion of other granulomatous diseases and the typical histological findings we diagnosed a cerebral granulomatosis as a manifestation of CD. The patient was initially started on azathioprine, which had to be switched to corticosteroids and methotrexate because of an azathioprine related pancreatitis. The patient has not suffered any further epileptic seizures to date.
Cerebral manifestation of CD is a possibly underreported entity that may respond well to immunosuppressive treatment. In contrast to earlier reports of cerebral manifestations in CD, our patient showed no coincident gastrointestinal symptoms indicating an activity of CD during the progression of cortical lesion load, suggesting that similar to other extra-intestinal manifestations in CD, the activity of gastrointestinal symptoms does not necessarily reflect the activity of CD associated cerebral vasculitis. Therefore, diagnosis and therapy of cerebral manifestation may be delayed when focusing on gastrointestinal symptoms alone.
克罗恩病（CD）与多种肠外表现相关。最常见的累及眼睛、皮肤、关节、凝血系统和肝脏。CD的脑部表现报道较少。神经症状对患者生活质量有广泛的不利影响，因此早期诊断和治疗尤为重要。在以往的病例报告中，CD脑部表现的诊断通常仅依靠磁共振成像（MRI）和计算机断层扫描（CT）。据我们所知，迄今为止仅有一篇病例报告记录了经组织学证实的与CD相关的脑部病变病例。
一名39岁右利手女性，有CD病史，因病因不明的钆（Gd）增强皮质病变并引发癫痫发作被转诊至我院。胸部CT扫描和支气管肺泡灌洗未发现结节病迹象。腰椎穿刺和实验室检查未发现潜在感染或并发自身免疫性疾病，MRI扫描显示病变负荷进展。因此，患者接受了皮质病变的立体定向活检。组织学检查显示小血管周围有混合性淋巴细胞-组织细胞性和结核样肉芽肿性炎症，未发现感染迹象。排除其他肉芽肿性疾病并结合典型的组织学表现后，我们诊断为脑部肉芽肿病，为CD的一种表现形式。患者最初开始使用硫唑嘌呤治疗，但由于硫唑嘌呤相关的胰腺炎，不得不改用皮质类固醇和甲氨蝶呤。该患者至今未再发生癫痫发作。
CD的脑部表现可能是一种报道不足的疾病，可能对免疫抑制治疗反应良好。与早期关于CD脑部表现的报道不同，我们的患者在皮质病变负荷进展期间未出现提示CD活动的同时存在的胃肠道症状，这表明与CD的其他肠外表现类似，胃肠道症状的活动不一定反映与CD相关的脑血管炎的活动。因此，仅关注胃肠道症状时，脑部表现的诊断和治疗可能会延迟。
