非酒精中毒性亚急性原发性胼胝体变性1例报道并文献复习

Non-alcoholic subacute primary dege-neration of corpus callosum: A case report and literature review

摘要:

原发性胼胝体变性是一种罕见的以胼胝体受累为主的脱髓鞘和继发坏死的病变，其病因及发病机制尚不明确。该病主要见于中年男性，通常与长期大量酗酒或营养不良相关，其缺乏特异的诊断标准，容易漏诊及误诊。该文报道了1例非酒精中毒引起的亚急性原发性胼胝体变性患者。该例患者亚急性起病，有糖尿病病史，无饮酒史，主要临床表现为肢体乏力、进行性认知功能下降、行为异常及轻度意识障碍。颅脑磁共振成像示：胼胝体呈膨胀性改变，其内可见多发片状异常信号灶；液体抑制反转恢复序列呈高信号，弥散加权成像序列呈高信号，表观弥散系数序列呈低信号；以上病灶呈双侧对称性分布，累及胼胝体膝部、体部及压部。在给予补充大剂量维生素B₁、维生素B₁₂，控制血糖后，患者症状明显好转，神志清醒，言语较前流利，认知功能较前明显好转，肢体肌力基本正常。复查颅脑磁共振成像示：胼胝体对称性异常信号灶较前明显减少。该文旨在通过报道1例非酒精中毒性原发性胼胝体变性的病例，并复习该类疾病相关的国内外文献，探讨MBD的病因、发病机制、影像学表现、临床特点、诊断、鉴别诊断及治疗方法的研究进展。

Abstract:

Primary dege-neration of corpus callosum is a rare disease of demyelination and secondary necrosis with the involvement of the corpus callosum， and its etiology and pathogenesis remain unclear. The disease is mainly observed in middle-aged male individuals and is often associated with long-term alcoholism or malnutrition， and a lack of specific diagnostic criteria may easily cause missed diagnosis and misdiagnosis. This article reports a patient with subacute primary dege-neration of corpus callosum due to non-alcoholic etiology. The patient had subacute onset with a history of diabetes， without a history of drinking and had the main clinical manifestations of limb weakness， progressive cognitive decline， behavioral abnormalities， and mild disturbance of consciousness. Cranial magnetic resonance imaging showed that the corpus callosum presented with swelling changes and multiple patchy abnormal signal lesions， with hyperintensity on fluid attenuated inversion recovery sequence， hyperintensity on diffusion weighted imaging， and hypointensity on apparent dispersion coefficient imaging， and these lesions were symmetrically distributed at both sides， involving the genu， body， and splenium of the corpus callosum. The patient's symptoms were significantly improved after supplementation of high-dose vitamin B₁ and vitamin B₁₂ and blood glucose control， with remarkable improvements in consciousness， speech， cognitive function， and muscle strength of extremities. Cranial magnetic resonance imaging reexamination showed a significant reduction in the symmetrical abnormal signal lesions in the corpus callosum. By reporting a case of MBD due to non-alcoholic etiology and further reviewing related articles in China and globally， this article summarizes the research advances in the etiology， pathogenesis， imaging findings， clinical features， diagnosis， differential diagnosis and treatment methods of MBD.