Hemifacial Spasm

Basics

  • Characterized by repetitive, involuntary contractions of the face (supplied by the Facial Nerve- Cranial Nerve VII)
  • Normally starts with the muscles around the eye and then progresses to the cheek and lips
  • Brief twitches but increase in duration as the disease progresses
  • Often caused by a abnormal blood vessel compressing the facial nerve inside the skull
  • Treatment consist of medication and surgery in refractory cases

Symptoms and Signs

  • Involuntary muscle contractions on one side of the face
  • Contractions may be initiated by voluntary movement of the muscles of facial expression
  • Muscle weakness in the face
  • Normally painless

Demographics

  • 8 (men) to 15 (women) out of 100,000
  • Affects Women > Men, normally in the 50s and 60s
  • slightly more common on the left than the right

Diagnosis

  • EMG
  • MRI / CT
  • Clinical exam

Treatment

  • Medications: Carbamazepine (Tegretol), baclofen, gabapentin (Neurontin), and Phenytoin (Dilantin)
  • Injection of Botox– works for about 5 months and then the injections need to be repeated
  • Surgery: Microvascular decompression (aka MVD) with insertion of a pledget (like a piece of felt) between the nerve and the abnormal blood vessel- the definitive treatment in medically refractory cases

Prognosis

  • According to several studies, 2/3 of patients will achieve some relief with medication but the traditional thinking still views oral medications as being generally ineffective
  • Botox injections- typically provide relief for 5months
  • Surgery offers the definitive treatment of hemifacial spasm caused by an aberrant blood vessel: 86% of patients are spasm free at 10 years

Differential Diagnosis

  • Basilar artery aneurysm
  • Acoustic nerve tumor
  • Meningioma
  • Multiple sclerosis
  • Bell’s palsy- can actually later result in hemifacial spasm

References

  • Adam’s and Victor Neurology
  • Handbook of Neurosurgery: Greenberg
  • Harper, RL et al. “Microvascular decompression for hemifacial spasm: long term results of 114 operations” Journal of Neurosurgery