Tsai Chun-Yi, Huang Po-Kai, Huang Poyin
Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Department of Pediatrics, E-Da Dachang Hospital Department of Neurology, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan Program in Translational Medicine, Kaohsiung Medical University and Academia Sinica Department of Neurology, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
Medicine (Baltimore). 2018 Feb;97(8):e9878. doi: 10.1097/MD.0000000000009878.
Marchiafava-Bignami disease (MBD) is a rare disease characterized by demyelination of the corpus callosum. It is most commonly seen in patients with chronic alcoholism. The clinical diagnosis of MBD can be difficult due to its nonspecific manifestation. Central pontine myelinolysis (CPM) occurs mostly as a complication of severe and prolonged hyponatremia, especially when corrected too rapidly. However, CPM can be associated with chronic alcoholism and its clinical presentation can be heterogeneous. Because both MBD and CPM can have fatal outcomes, early recognition and treatment can result in a better prognosis. We present a very rare case of simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis in a patient with chronic alcoholism who was diagnosed unexpectedly using brain magnetic resonance imaging and improved after proper treatment.
We presented a case of a 39-year-old patient who visited the hospital with general weakness and an altered neurologic condition after a week of vomiting.
The patient was diagnosed with simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis using brain magnetic resonance imaging.
Administration of a high dose of thiamine.
The neurologic signs improved after a week of thiamine administration.
This case suggests that Marchiafava-Bignami disease and central pontine myelinolysis might have a common pathogenesis, and brain magnetic resonance imaging is of crucial importance in chronic alcoholic patients presenting with nonspecific neurological deterioration. The appropriate administration of thiamine may prevent poor outcomes.
马奇亚法瓦-比尼亚米病（MBD）是一种罕见疾病，其特征为胼胝体脱髓鞘。最常见于慢性酒精中毒患者。由于其临床表现不具特异性，MBD的临床诊断可能存在困难。中枢性桥脑髓鞘溶解症（CPM）大多作为严重且长期低钠血症的并发症出现，尤其是在纠正过快时。然而，CPM可能与慢性酒精中毒有关，其临床表现可能多种多样。由于MBD和CPM都可能导致致命后果，早期识别和治疗可带来更好的预后。我们报告了一例非常罕见的病例，一名慢性酒精中毒患者同时发生急性马奇亚法瓦-比尼亚米病和中枢性桥脑髓鞘溶解症，通过脑部磁共振成像意外确诊，并在经过适当治疗后病情好转。
我们报告了一例39岁患者，在呕吐一周后因全身无力和神经状态改变前来就诊。
通过脑部磁共振成像，该患者被诊断为同时患有急性马奇亚法瓦-比尼亚米病和中枢性桥脑髓鞘溶解症。
给予高剂量硫胺素。
在给予硫胺素一周后，神经体征有所改善。
该病例表明，马奇亚法瓦-比尼亚米病和中枢性桥脑髓鞘溶解症可能具有共同的发病机制，脑部磁共振成像对于出现非特异性神经功能恶化的慢性酒精中毒患者至关重要。适当给予硫胺素可预防不良后果。
