非酒精因素导致的原发性胼胝体变性

Primary corpus callosum degeneration due to non-alcohol factors

摘要:

原发性胼胝体变性以胼胝体脱髓鞘和中心性坏死为主要病理特征，临床表现主要为精神异常、意识障碍、癫痫发作等。其病因目前尚不完全明确，常见的诱因为长期大量的酒精摄入。临床上非酒精中毒性因素导致的原发性胼胝体变性非常罕见。为提高临床医师对本病的认识，减少误诊漏诊。该文报道了1例非酒精因素导致的原发性胼胝体变性病例，该例患者以头晕为首发症状，亚急性起病，后出现发作性意识丧失、四肢僵直、二便失禁。颅脑MRI示：胼胝体肿胀，可见多发斑片状，T1WI稍低、T2WI高信号、T2-FLAIR呈稍高信号、DWI可见高信号，累及膝部、体部及压部，呈双侧对称分布。给予激素、口服维生素B1及丙戊酸钠抗癫痫治疗。治疗后病情明显好转，神志清，精神状态明显好改善，言语较前清晰，行走不稳好转。复查MRI示：双侧大脑白质和胼胝体病变范围较前缩小，异常信号较前减低。

Abstract:

Primary corpus callosum degeneration has the main pathological features of demyelination and central necrosis of the corpus callosum， with the main clinical manifestations of mental abnormalities， disturbance of consciousness， and seizure. The etiology of this disease remains unclear， and long-term heavy alcohol intake is a common predisposing factor. Primary corpus callosum degeneration due to non-alcohol factors is rare in clinical practice. In order to improve the awareness of this disease among clinicians and reduce misdiagnosis and missed diagnosis， this article reports a case of primary corpus callosum degeneration due to non-alcohol factors. This patient had dizziness as the initial symptom and experienced subacute disease onset， followed by the appearance of paroxysmal loss of consciousness， limb rigidity， and urinary and fecal incontinence. Cranial MRI showed corpus callosum swelling and multiple patchy lesion， with slight hypointensity on T1WI， hyperintensity on T2WI， slight hyperintensity on T2-FLAIR， and hyperintensity on DWI， with involvement of the genu， body， and splenium of the corpus callosum and symmetric distribution at both sides. The patient was given antiepileptic therapy with hormone， vitamin B1， and sodium valproate and was significantly improved after treatment， with marked improvements in consciousness， mental state， speech， and ataxia. MRI reexamination showed significant reductions in the extent of lesions and abnormal signals in white matter and corpus callosum at both sides.