Hemifacial spasm (HFS) is a peripherally-induced movement disorder characterized by the involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve.
This disease takes two forms: typical and atypical. In typical form, the twitching usually starts in the lower eyelid in orbicularis oculi muscle. As time progresses, it spreads to the whole lid, then to the orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area.
The reverse process of twitching occurs in atypical hemifacial spasm; twitching starts in orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area in the lower face, then progresses up to the orbicularis oculi muscle in the eyelid as time progresses.
The most common form is the typical form, and atypical form is only seen in about 2–3% of patients with hemifacial spasm.
The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons.
This disorder occurs in both men and women, although it affects middle-aged or elderly women more frequently.
Hemifacial spasm is much more common in some Asian populations.
The purpose of a study was to characterize the natural history and clinical outcome in patients with HFS.
The initial visits of all 2,155 patients and the diagnosis of HFS took place between 2001 and 2010. In 1,775 of the patients, compressing vessels were identified on magnetic resonance imaging. Of these, we excluded 1,469 patients (82.8%) who received microvascular decompression, 101 (5.7%) who continued to visit the clinic for botulinum toxin injections, and 9 (0.5%) who died or suffered from other diseases. Ninety-two (5.2%) of the patients were lost to follow-up; the remaining 104 were followed up for 5-42 years (mean 12 years) after the onset of the symptoms of HFS.
The condition was aggravated in 11 (10.6%) of the 104 patients and stationary in 40 (38.5%) for 6-42 years (mean 13 years). Ten (9.6%) improved partially for 7-18 years (mean 11 years). Forty-three (41.3%) were in remission for between 2 months and 23 years (mean 6.4 years) after onset and required no further treatment for 5 months to 13 years (mean 5.7 years) 1).
Tu et al. used resting state functional magnetic resonance imaging with regional homogeneity (ReHo) analysis to investigate changes in spontaneous brain activity of patients with hemifacial spasm HFS and to determine the relationship of these functional changes with clinical features. Thirty patients with HFS and 33 age-, sex-, and education-matched healthy controls were included in this study. Compared with controls, HFS patients had significantly decreased ReHo values in left middle frontal gyrus (MFG), left medial cingulate cortex (MCC), left lingual gyrus, right superior temporal gyrus (STG) and right precuneus; and increased ReHo values in left precentral gyrus, anterior cingulate cortex (ACC), right brainstem, and right cerebellum. Furthermore, the mean ReHo value in brainstem showed a positive correlation with the spasm severity (r = 0.404, p = 0.027), and the mean ReHo value in MFG was inversely related with spasm severity in HFS group (r = -0.398, p = 0.028). This study reveals that HFS is associated with abnormal spontaneous brain activity in brain regions most involved in motor control and blinking movement. The disturbances of spontaneous brain activity reflected by ReHo measurements may provide insights into the neurological pathophysiology of HFS. 2).
Abnormal lateral spread response (LSR) is a typical finding in facial electromyography (EMG) in patients with hemifacial spasm (HFS). Although intraoperative monitoring of LSR has been widely used during microvascular decompression (MVD), the prognostic value of this monitoring is still debated.
Although intraoperative EMG monitoring during Microvascular decompression for hemifacial spasm was beneficial for identifying the offending vessel and suggesting the most appropriate surgical end point, loss of LSR did not always correlate with long-term HFS treatment outcome. Because the HFS cure rate improved over time, revision might be considered for persistent LSR when follow-up has been performed for more than 1 year and the spasm remains despite adequate decompression 3).
Takeda et al. report the successful treatment of a patient with hemifacial spasm due to a tortuous vertebral artery that appeared to have developed to compensate for agenesis of the ipsilateral carotid artery. The 51-year-old man presented with a 1-year history of progressive left hemifacial spasm. His medical history was otherwise unremarkable except for untreated mild hypertension. Magnetic resonance angiography and bone window computed tomography demonstrated congenital agenesis of the left carotid artery and compression of the root exit zone of the left facial nerve by a tortuous left vertebral artery (VA). Microvascular decompression was performed via a left suboccipital craniotomy, and the offending vessel was identified using endoscopy. The vertebral artery was successfully transposed using polytetrafluoroethylene (PTFE) tape and a PTFE ball (Bard PTFE felt, Tempe, Arizona). This is the first report of a patient with hemifacial spasm caused by an ectatic VA associated with agenesis of the ipsilateral carotid artery 4).
A 61-year-old female presented with 4 years history of left-sided hemifacial spasm. Head MRI and angiography indicated left vertebral artery dissecting aneurysm which compressed ipsilateral cranial nerves Ⅶ and Ⅷ. Microvascular decompression was performed. The dissecting aneurysm was pushed apart and the distal part of the parent artery was adhered to the dura on the petrosum. The compressed nerves were totally decompressed. The symptom of facial spasm was completely resolved immediately after surgery and did not recur during 6 months of follow up 5).