非酒精性Wernicke脑病的临床和MRI表现

Clinical characteristics and MRI features of nonalcoholic Wernicke encephalopathy

摘要:

目的：分析非酒精性Wernicke脑病（non alcoholic Wernicke encephalopathy，nWE） 的临床表现和MRI特征。方法：回顾性分析10例nWE脑病的临床和影像学资料并文献复习。结果：10例患者中仅2例临床表现为典型三联征。6例患者出现急性WE的典型MRI特征，即在T2WI和T2液体衰减反转恢复序列像上对称的丘脑内侧、中脑导水管周围、第四脑室底出现高信号病灶；其中3例前庭内侧核、2例面神经核及舌下神经核、1例乳头体出现异常信号。增强扫描可见相应部位的强化，其中导水管周围灰质强化呈“Ω”状，具有特征性。1例患者出现皮层异常信号，最终死亡。1例动态观察提示双侧丘脑内侧病灶出现少量出血。磁共振扩散加权成像不能发现更多的异常部位。1例患者行3D氢质子磁共振波谱检查，其丘脑内侧、导水管周围氮-乙酰天门冬氨酸/肌酸比值下降，可见乳酸峰。所有患者均未出现乳头体及小脑萎缩。结论：MRI是评价nWE的敏感方法，丘脑内侧、第四脑室底、脑干神经核团是特异性损害部位，合并皮层损害者预后不良。

Abstract:

Objective：To investigate the clinical and magnetic resonance imaging（MRI） characteristics of nonalcoholic Wernicke encephalopathy（nWE）. Methods：We conducted a review on clinical characteristics and MRI features of 10 nWE cases. Results：Among 10 patients with varying degrees of central nervous system symptoms，only 2 had a typical triad. On MRI，6 out of 10 patients had evidences of symmetric lesions in the medial thalami，periaqueductal area and periventricular gray matter located anteriorly to the fourth ventricle；among the 6 patients，signal-intensity alterations were appeared in the medial vestibular nuclei in 3 patients，in the facial nuclei and the prepositus hypoglossal nuclei in 2 patients，in the mamillary bodies in 1 patient. Lesions were enhanced by differ－ent degrees on enhanced T1WI，periaqueductal gray matter showing characteristic ‘Ω’ shape. One patient with cortical damage died. Dynamic observations suggested that internal lesions of bilateral thalamus developed a little bleeding in 1 patient. Diffusion weighted imaging can’t find more abnormal regions. In 1 patient，3D proton magnetic vesonance spectroscopy showed low levels of N-acetylas－partate/creatine and a lactate peak in the medial thalami and periaqueductal area. In all patients，no atrophy of the mammillary bodies and cerebellar vermis was found. Conclusions：MRI is an effective method to investigate neurologic complications associated with nWE. The medial thalami，periaqueductal area，periventricular gray matter located anteriorly to the fourth ventricle and the cranial nerve nuclei involvement are characteristic findings of nWE. Cortical involvement in patients with nonalcoholic WE may be indicative of a poor prognosis.