Surgical Management of Hemifacial Spasm and Meige Syndrome

This chapter focuses on the surgical management of hemifacial spasm (HFS) and meige syndrome. HFS is a benign, chronic, involuntary movement of one side of the face, characterized by twitching, tonic spasm, and synkinesis of the muscles innervated by the facial nerve. It typically starts with intermittent twitches in the orbicularis oculi muscle. The symptoms usually progress gradually in frequency and severity, and spread downward to the other facial muscles of the affected side. HFS is seen almost exclusively in middle-aged and older patients, with a female predominance. In most patients, the spasm of HFS begins at the lower eyelid, gradually progressing to involve the entire orbicularis oculi muscle and then moving on to the orbicularis oris or perioral muscles. In advanced cases, the platysma and, less frequently, the frontalis muscle of the forehead can also be affected. The occurrence in childhood and adolescence is extremely rare. The symptoms of HFS can be aggravated or induced by emotional stress, psychological tension and/or fatigue. MVD is now considered the treatment of choice for HFS because of its low morbidity and its long-term benefit of symptom relief. The term "Meige syndrome" was used by Paulson to describe spontaneous, involuntary spasm involving the facial muscles. This syndrome is now considered a variant of adult-onset torsion dystonia or idiopathic cranialcervical dystonia, which is considered as a segmental dystonia. There are several recent reports of bilateral pallidal deep brain stimulation (DBS) effectively controlling symptoms in medication refractory Meige syndrome. There are only a few cases of Meige syndrome, either isolated or advanced, that have been treated with bilateral GPi DBS. Some adverse effects that have been reported include worsening of handwriting, typing, balancing, and walking. But these adverse effects were mild and not evident on objective examination.