Hemifacial spasm

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The hemifacial spasm (or hemispasmus facialis ) is a unilateral ( hemi ) occurring involuntary cramping ( spasm ) of the facial muscles. The cause is usually vascular / nerve contact, a so-called microvascular compression syndrome .

Epidemiology

The disease usually occurs after the age of 60. Predisposing are female sex (male to female = 1: 2) and longstanding high blood pressure . The prevalence for men and women is 7 and 14 / 100,000 people, respectively.

Symptoms

Spasms of the muscles controlled by the facial nerve develop. These can be painful. The symptoms often begin around the eye ( orbicularis oculi muscle ) and spread over the entire half of the face (up to the platysma ) over months or years . A facial paralysis is very rare and indicates another underlying medical condition.

Pathogenesis

In more than 90% of cases, the cause is a compression of the facial nerve by a blood vessel in the posterior fossa, i.e. near the origin of the nerve before it enters the temporal bone. The inferior cerebellar artery posterior (PICA) is most frequently involved in approx. 40% and the anterior cerebellar inferior artery (AICA) in approx. 20% of cases. These are usually lengthened by longstanding high blood pressure and then increasingly meander until they press against the nerves. The pulsation of the vessel then causes local damage to the myelin sheaths . As a result of this loss of electrical insulation, electrical excitations can jump between the nerve fibers and thereby trigger cramping of the facial muscles.

The cause of the hemifacial spasm is identical to that of trigeminal neuralgia , except that a different cranial nerve is affected.

Rare other causes are vascular malformations ( angioma ) or tumors or multiple sclerosis .

Diagnosis

Magnetic resonance imaging (MRI) is part of the investigation . An electrophysiological examination should also be carried out ( electromyography , neurography ).

This means that other differential diagnoses have to be ruled out, in particular tumors of the posterior fossa (e.g. acoustic neuroma ) and multiple sclerosis. Therefore, a CSF puncture can be useful in individual cases.

therapy

The therapy of choice is the injection of botulinum toxin into the affected muscles. This is only necessary every 3–5 months and has an effectiveness of> 80% for very good success or> 90% for relief. Loss of effectiveness can occur, usually due to the formation of antibodies against botulinum toxin.

Antiepileptic drugs are also effective, similar to the effect in trigeminal neuralgia. Most of the experience is available for carbamazepine (more often loss of effect indicated in the course). But many newer anti-epileptic drugs have also been used successfully in individual cases ( gabapentin , pregabalin , topiramate , levetiracetam , zonisamide ).

Microvascular decompression (Jannetta operation) can be used as a causal therapy . The success rate is around 80%. Since this is a complicated operation in the posterior fossa , this remains the last resort. It should be considered if treatment with medication is ineffective or tolerated.

literature

  • Andreas Hufschmidt et al .: Neurology compact. 5th ed., Thieme 2009.
  • Andreas Schulze-Bonhage, Andreas Ferbert : Spasmus facialis: Current aspects of operative and drug therapy . In: Dtsch Arztebl 2000; 97 (47): A-3184.
  • O. Daniele et al .: Gabapentin in the treatment of hemifacial spasm . In: Acta Neurol Scand . 2001 Aug; 104 (2): 110-2.
  • D. Deleu: Levetiracetam in the treatment of idiopathic hemifacial spasm . In: Neurology . 2004 Jun 8; 62 (11): 2134-5.
  • PP. Urban: Pregabalin as add-on treatment to botulinum toxin in idiopathic hemifacial spasm . In: Neurology . 2006 Jun 13; 66 (11): 1781.
  • H. Alonso-Navarro et al .: Topiramate as treatment for hemifacial spasm . In: Clin Neuropharmacol . 2007 Sep-Oct; 30 (5): 308-9.
  • A. Siniscalchi: Idiopathic hemifacial spasm responsive to zonisamide: a case report . In: Clin Neuropharmacol . 2009 Jul-Aug; 32 (4): 230-1.

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