Ziekenhuis Oost-Limburg, Genk, Belgium.
Top Magn Reson Imaging. 2023 Feb 1;32(1):1-4. doi: 10.1097/RMR.0000000000000301. Epub 2023 Jan 12.
In this case report we describe the case of a 66-year old man with subacute gait difficulties, with a progression to confusion coma with multiple generalised epileptic seizures during the following days. Biochemical analysis showed hyperglycaemia, cerebrospinal fluid (CSF) testing showed a mild lymphocytic pleocytosis and an elevated protein and lactate. Broad-spectrum antibiotics and antiviral therapy where initiated. However, all other CSF testing remained negative. Magnetic resonance imaging of the brain showed remarkably symmetric hyperintense T2 white matter lesions most noticable in the corpus callosum. The lesion pattern was suggestive of a metabolic or toxic encephalopathy, the preponderance for the corpus callosum was furthermore suggestive for Marchiafava-Bignami disease (MDB), as was the clinical course since admission of the patient. A high dose IV substitution of vitamin B1, B6 and B12 was started and antibiotic and antiviral therapy was discontinued. After one day the patient showed progressive regaining of consciousness and he returned to premorbid functioning in a matter of 1-2 weeks. MRI of the brain after 1 week showed notable improvement of the white matter lesions. At routine follow-up two weeks later he presented with icterus and a diagnosis of Epstein-Barr virus (EBV) hepatitis was made, lymph node biopsies showed an EBV positive diffuse large cell B-cell lymphoma (DLCBL). MDB is mostly associated with severe alcoholism, with malnourishment being the second leading cause, however there are case reports describing MDB in patients with chronically poorly controlled diabetes mellitus. We hypothesize that his condition may have been precipitated by his poorly controlled diabetes mellitus. However it is also possible that weight loss (probably related to the DLCBL diagnosis) might have contributed to a state of malnourishment and therefore played a role in the aetiology as well.
在本病例报告中，我们描述了一位 66 岁男性的病例，他出现亚急性步态困难，并在接下来的几天内进展为意识混乱昏迷，伴有多次全身性癫痫发作。生化分析显示高血糖，脑脊液（CSF）检测显示轻度淋巴细胞增多症以及蛋白和乳酸升高。广谱抗生素和抗病毒治疗开始。然而，所有其他 CSF 检测均为阴性。脑部磁共振成像显示明显对称的高信号 T2 白质病变，在胼胝体中最为明显。病变模式提示代谢或中毒性脑病，胼胝体的优势进一步提示 Marchiafava-Bignami 病（MDB），因为患者入院后的临床过程也是如此。开始大剂量静脉注射维生素 B1、B6 和 B12 替代治疗，并停止抗生素和抗病毒治疗。一天后，患者表现出意识逐渐恢复，在 1-2 周内恢复到发病前的功能。1 周后的脑部磁共振成像显示白质病变明显改善。两周后常规随访时，他出现黄疸，并被诊断为 EBV 肝炎，淋巴结活检显示 EBV 阳性弥漫性大 B 细胞淋巴瘤（DLCBL）。MDB 主要与严重的酗酒有关，营养不良是第二大原因，但也有病例报告描述了 MDB 发生在慢性控制不佳的糖尿病患者中。我们假设他的病情可能是由他未得到控制的糖尿病引起的。然而，体重减轻（可能与 DLCBL 诊断有关）也可能导致营养不良状态，因此也可能在病因中起作用。
