关键词: blepharospasm cervical dystonia meige syndrome oromandibular dystonia upper motor neuron signs

来  源:   DOI:10.7759/cureus.60101   PDF(Pubmed)

Abstract:
Meige syndrome (MS) is a cranial dystonia that involves blepharospasm and oromandibular dystonia. It can also evolve to include other adjacent muscle groups in the cervical region. It typically presents in middle-aged females, and while the disorder is relatively uncommon, its exact prevalence varies. Diagnosis is typically made with a thorough history and physical and workup to rule out other causes. Treatment options include medical management with gamma-aminobutyric acid (GABA) antagonists, dopamine antagonists, and anticholinergics for short-term management. Long-term treatment options are Botox and deep brain stimulation. This case report presents a 56-year-old female with a complex presentation of MS; the patient\'s symptoms progressed from isolated blepharospasms to involve orofacial and cervical musculature. A distinctive aspect of this case was the simultaneous presence of upper motor neuron (UMN) signs in the patient alongside acute to subacute compression fractures of the superior endplate of C7 and T3, as revealed by cervical spine imaging. Treatment with clonazepam led to significant symptomatic improvement, highlighting the importance of a multimodal approach in managing MS. This case underscores the need for careful clinical evaluation, collaboration with movement disorder specialists, and ongoing research efforts to enhance understanding and treatment of MS.
摘要:
Meige综合征(MS)是一种颅骨肌张力障碍,涉及眼睑痉挛和口下颌肌张力障碍。它还可以演变为包括宫颈区域中的其他相邻肌肉群。它通常出现在中年女性身上,虽然这种疾病相对不常见,它的确切流行程度各不相同。诊断通常是通过全面的病史和体格检查来排除其他原因。治疗选择包括γ-氨基丁酸(GABA)拮抗剂的医疗管理,多巴胺拮抗剂,和抗胆碱能药物用于短期管理。长期治疗选择是肉毒杆菌毒素和深部脑刺激。该病例报告介绍了一名56岁的女性,患有复杂的MS;患者的症状从孤立的眼睑痉挛发展到涉及口面部和颈部肌肉组织。这种情况的一个显着方面是患者同时存在上运动神经元(UMN)体征,同时伴有C7和T3的上终板的急性至亚急性压缩性骨折,如颈椎影像学所揭示的。氯硝西泮治疗可显著改善症状,强调多模式方法在管理MS中的重要性。这个案例强调了需要仔细的临床评估,与运动障碍专家合作,以及正在进行的研究工作,以增进对MS的理解和治疗。
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