METHODS: Clinical and laboratory records of 12 consecutive patients with WE admitted to our hospital during the past 11 years were reviewed. The WE diagnosis was confirmed based on low blood VB1 levels and the presence of at least one of the classical triad.
RESULTS: Ophthalmoplegia and nystagmus were recorded in 75% and 50% of the patients, respectively. Eleven of 12 patients presented with consciousness disturbance/memory loss. All patients experienced gait disturbances. Eight of the 12 patients exhibited MRI abnormalities at typical sites (the dorsal midbrain [n = 7], medial thalamus [n = 6], mammillary bodies [n = 5], and dorsal pons [n = 5]). Of the 12 patients, six showed abnormalities at atypical sites (the splenium of the corpus callosum [n = 4], fornix [n = 3], cerebral cortex [n = 2], cerebellar vermis [n = 2], and dorsal medulla [n = 1]). Patients with positive MRI abnormalities had significantly lower blood VB1 levels than those without abnormalities (9.5 vs. 16.0 ng/mL).
CONCLUSIONS: In cases of confirmed WE with low blood VB1 levels, the corpus callosum, fornix, and cerebral cortex were more frequently involved than in previous studies. MRI abnormalities at both typical and atypical sites were correlated with low blood VB1 levels in WE, suggesting that lower blood VB1 levels are associated with more severe brain damage in patients with WE.
方法:回顾了过去11年来我院收治的12例WE患者的临床和实验室记录。WE诊断是基于低血液VB1水平和至少一个经典三联征的存在而确认的。
结果:记录了75%和50%的患者眼肌麻痹和眼球震颤,分别。12例患者中有11例出现意识障碍/记忆丧失。所有患者均出现步态障碍。12例患者中有8例在典型部位(背侧中脑[n=7],丘脑内侧[n=6],乳头体[n=5],和背桥[n=5])。在12名患者中,六个在非典型部位显示异常(call体的脾[n=4],穹窿[n=3],大脑皮层[n=2],小脑疣[n=2],和背髓质[n=1])。MRI异常阳性的患者血液VB1水平明显低于无异常的患者(9.5vs.16.0ng/mL)。
结论:在证实WE具有低血VB1水平的病例中,call体,穹窿,与以前的研究相比,大脑皮层的参与频率更高。典型和非典型部位的MRI异常与WE中的低血液VB1水平相关,提示较低的血液VB1水平与WE患者更严重的脑损伤相关。