Yadala Sisira, Luo Jin Jun
Department of Neurology, Temple University School of Medicine, 3401 North Broad Street, Suite C525, Philadelphia, PA 19140, USA.
Case Rep Neurol Med. 2013;2013:979383. doi: 10.1155/2013/979383. Epub 2013 Apr 24.
Marchiafava-Bignami disease (MBD) is a rare neurological disorder mostly seen in alcoholic and malnourished patients with a pathognomonic hallmark of corpus callosum demyelination. MBD in nonalcoholics without malnutrition has rarely been reported. We report a case of MBD in a diabetic patient, without alcoholism or malnutrition, caused by a wide range of glycemic level fluctuations. A 38-year-old man presented with sudden onset of alteration in speech and multiple falls in three days. Neurologic examination showed dysarthria, dysmetria, and ataxia but, otherwise, normal cranial nerves, motor and sensory functions, and tendon reflexes. Brain MRI showed symmetric abnormalities in the splenium of the corpus callosum. In addition, demyelination was also observed in bilateral posterior limbs of the internal capsule and brachium ponti. His symptoms significantly improved after stabilization and normalization of his plasma glucose level and administration of multivitamins and corticosteroids. The underlying pathophysiology of the development of MBD in our case is likely to be osmotic stress from a wide range of glycemic fluctuations causing structural and functional disturbance of oligodendrocytes, which may be reversible in its early stage.
马基亚法瓦-比尼亚米病(MBD)是一种罕见的神经系统疾病,多见于酗酒和营养不良的患者,其特征性标志是胼胝体脱髓鞘。非酗酒且无营养不良的患者发生MBD的情况鲜有报道。我们报告一例糖尿病患者发生的MBD,该患者无酗酒或营养不良,病因是血糖水平广泛波动。一名38岁男性在三天内突然出现言语改变和多次跌倒。神经系统检查显示构音障碍、辨距不良和共济失调,但其他方面,颅神经、运动和感觉功能以及腱反射均正常。脑部MRI显示胼胝体压部有对称性异常。此外,在内囊后肢和脑桥臂也观察到脱髓鞘。在其血糖水平稳定和正常化,并给予多种维生素和皮质类固醇后,他的症状明显改善。我们病例中MBD发生的潜在病理生理学可能是血糖广泛波动引起的渗透应激,导致少突胶质细胞的结构和功能紊乱,在早期阶段可能是可逆的。