Kamitsukasa I, Yamada T, Tokumaru Y, Hirayama K
Department of Neurology, School of Medicine, Chiba University.
Rinsho Shinkeigaku. 1995 Mar;35(3):231-6.
To clarify the factors determining the amelioration of Meige's syndrome, changes of involuntary movements (IVMs) and functional disability, we examined 60 patients with Meige's syndrome during 5 years after the onset. On average, they showed gradual worsening of IVMs for approximately 2.1 years, then the IVMs ameliorated slowly. In many patients, blepharospasm appeared as the first symptom. Subsequent IVMs were seen in vicinity of the muscles of orbicularis oculi. Phasic involuntary contractions changed to tonic ones in some patients. Asynchrony of the IVMs in various facial or neck muscles may be originated from extensive pathological changes and high excitability in the brainstem. The factors determining the amelioration of functional disability are: (1) younger onset, (2) shorter duration from the onset to the period showing the worst symptoms, (3) mild IVMs when the symptoms were the worst, (4) shorter duration from the onset to the beginning of therapy, (5) synchrony of the IVMs between the muscles of orbicularis oculi and other muscles. Methylphenidate, trihexyphenidyl, and ceruletide showed a higher efficiency for IVMs than the other drugs. The drug therapy in Meige's syndrome should be started as early as possible.
为了阐明决定Meige综合征改善的因素、不自主运动（IVM）的变化和功能残疾情况，我们对60例Meige综合征患者在发病后的5年里进行了检查。平均而言，他们的IVM在大约2.1年的时间里逐渐恶化，然后IVM缓慢改善。在许多患者中，睑痉挛是首发症状。随后在眼轮匝肌附近出现IVM。一些患者的阶段性不自主收缩转变为强直性收缩。面部或颈部不同肌肉的IVM不同步可能源于脑干广泛的病理变化和高兴奋性。决定功能残疾改善的因素有：（1）发病年龄较轻，（2）从发病到出现最严重症状的持续时间较短，（3）症状最严重时IVM较轻，（4）从发病到开始治疗的持续时间较短，（5）眼轮匝肌与其他肌肉之间IVM同步。哌醋甲酯、苯海索和缩胆囊素对IVM的疗效高于其他药物。Meige综合征的药物治疗应尽早开始。
