Suzuki Yasuo, Matsuda Taisuke, Washio Noriaki, Ohtsuka Kenji
Department of Ophthalmology, Sapporo Medical University, School of Medicine, Sapporo, Japan.
Jpn J Ophthalmol. 2005 May-Jun;49(3):220-2. doi: 10.1007/s10384-004-0182-8.
We report an unusual case of Wernicke's encephalopathy presenting with transient upbeat nystagmus that changed to a persistent downbeat nystagmus.
A 27-year-old man presented with upbeat nystagmus. Three months earlier, he had been diagnosed with Wernicke's encephalopathy after fasting for a month.
This diagnosis was supported by his symptoms (ataxia, a confused state). Clinical recovery followed thiamine therapy. His upbeat nystagmus had linear slow phases with average amplitude and frequency (+/-SD) during fixation straight ahead of 2.8 +/- 0.7 degrees and 4.6 +/- 2.2 Hz, respectively. Two months later, the primary position upbeat nystagmus had diminished and downbeat nystagmus (0.9 +/- 0.5 degrees and 3.2 +/- 0.7 Hz on average) for a 20 degrees downward gaze had developed. Then, 8 months later, he showed only downbeat nystagmus, which obeyed Alexander's law. His primary position downbeat nystagmus was completely suppressed by clonazepam, a gamma-aminobutyric acid (GABA) agonist.
Owing to an underlying central vestibular imbalance, even after the recovery of acute neurological symptoms, Wernicke's encephalopathy can be complicated by persistent downbeat nystagmus, which can be treated by a GABA agonist.
我们报告一例罕见的韦尼克脑病,表现为短暂的上跳性眼球震颤,随后转变为持续性下跳性眼球震颤。
一名27岁男性出现上跳性眼球震颤。三个月前,他在禁食一个月后被诊断为韦尼克脑病。
其症状(共济失调、意识模糊状态)支持这一诊断。硫胺素治疗后临床症状有所改善。他的上跳性眼球震颤在向前注视时慢相呈线性,平均幅度和频率(±标准差)分别为2.8±0.7度和4.6±2.2赫兹。两个月后,原部位的上跳性眼球震颤减弱,向下注视20度时出现下跳性眼球震颤(平均为0.9±0.5度和3.2±0.7赫兹)。然后,8个月后,他仅表现为下跳性眼球震颤,符合亚历山大定律。他原部位的下跳性眼球震颤被γ-氨基丁酸(GABA)激动剂氯硝西泮完全抑制。
由于潜在的中枢前庭失衡,即使急性神经症状恢复后,韦尼克脑病仍可能并发持续性下跳性眼球震颤,可通过GABA激动剂进行治疗。