背景:Wernekinck连动综合征(WCS)是一种极为罕见的中脑综合征,选择性地破坏小脑上脚梗和中央被盖束的收缩,通常表现为双侧小脑共济失调,构音障碍,和核间眼肌麻痹。Wernekinck连合综合征的腭肌阵挛症是不常见的,并且由于双侧下橄榄核的肥大性变性而发生晚期现象。
方法:一名WCS患者,从12月开始住院,2023年,被选为这项研究,和综合征的临床表现,成像特征,根据文献对其病因进行回顾性分析。一名68岁的右撇子东亚男子出现头晕,含糊不清的讲话,吞咽和行走困难,和有节奏的软腭收缩。他有几个缺血性脑血管病的危险因素(年龄,性别,血脂异常,高血压和吸烟史)。脑磁共振成像显示,在中脑导水管前方的副中中脑膜被盖周围的尾中脑,DWI强度高,ADC强度低。
结果:他被诊断为继发于尾旁正中脑梗塞的Wernekinck连合综合征(WCS)。他开始接受双重抗血小板治疗(阿司匹林和氯吡格雷)和强化他汀类药物治疗。血压和血糖也被调整。他的症状迅速改善,经过7天的治疗,他走路平稳,说话清晰。
结论:众所周知,由于双侧下橄榄核肥大性变性,腭肌阵挛症是一种晚期现象。然而,我们的病例表明,在WCS的早期阶段可能会发生pal肌阵鸣。
BACKGROUND: Wernekinck commissure syndrome (WCS) is an extremely rare midbrain syndrome, which selectively destroys the decussation of the superior cerebellar peduncle and the central tegmental tract, which commonly presents with bilateral cerebellar ataxia, dysarthria, and internuclear ophthalmoplegia. Palatal myoclonus in Wernekinck commissure syndrome is uncommon and often occurs as a late phenomenon due to hypertrophic degeneration of bilateral inferior olivary nuclei.
METHODS: A patient with WCS, admitted to our hospital from December 2023, was chosen for this study, and the syndrome\'s clinical manifestations, imaging features, and etiology were retrospectively analyzed based on the literature. A 68-year-old right-handed East Asian man presented with dizziness, slurred speech, difficulty with swallowing and walking, and rhythmic contractions of the soft palate. He had several risk factors for ischemic cerebrovascular diseases (age, sex, dyslipidemia, hypertension and smoking history). Brain magnetic resonance imaging showed hyperintensity of DWI and hypointensity of ADC at the caudal midbrain which was around the paramedian mesencephalic tegmentum anterior to the aqueduct of midbrain.
RESULTS: He was diagnosed with Wernekinck commissure syndrome (WCS) secondary to caudal paramedian midbrain infarction. He was started on dual antiplatelet therapy (aspirin and clopidogrel) and intensive statin therapy. Blood pressure and glucose were also adjusted. His symptoms improved rapidly, and he walked steadily and speak clearly after 7 days of treatment.
CONCLUSIONS: Palatal myoclonus is known to occur as a late phenomenon due to hypertrophic degeneration of bilateral inferior olivary nuclei. However, Our case suggests that palatal myoclonus can occur in the early stages in WCS.