Hemifacial spasm

Synonyms
HFS, Spasmus hemifacialis, Hemifacial spasm
ICD-10
G51.3
ICD-11
8B88.2

Hemifacial spasm is a movement disorder affecting the muscles innervated by the Facial nerve. This results in involuntary, brief or prolonged contractions of the facial muscles.

Epidemiology

The prevalence of hemifacial spasm is approximately 11 per 100,000 inhabitants[^2].

Symptoms

Hemifacial spasm is characterized by progressive, involuntary, irregular, clonic or tonic movements of the muscles innervated by the facial nerve. These symptoms are generally observed on only one side of the face. At the onset of the disease, involuntary spasms frequently occur in the region of the Musculus orbicularis oculi, which then gradually spread to other parts of the affected side of the face. In pronounced cases, the Platysma may also be affected. In most patients, the symptoms persist during sleep. In very rare cases, a "clicking sound" may also occur in the ear, which can be explained by a contraction of the stapedius muscle[^4]. Patients with hemifacial spasm may exhibit a positive Babinski-2 sign.

Etiology

In most cases, the underlying cause of this disease is a neurovascular conflict, which can be treated surgically.

Artery involved in the neurovascular conflict

Relative frequency in %[^1]

PICA

35.4 %

AICA

33.8 %

Vertebrobasilar artery (VBA) with PICA or AICA

20 %

Vertebrobasilar artery alone

3.1 %

In very rare cases, a venous conflict with the facial nerve may also be present[^4].

Endoscopic neurovascular conflict
Neurovascular conflict involving the facial nerve in an endoscopic view with a 45-degree optic. The pontomedullary sulcus is also visible in the illustration.

Imaging

Neurovascular conflicts are often difficult to diagnose definitively on MRI imaging. TOF and the CISS sequence are optimally suited for assessing the root exit zone (REZ) of the facial nerve.

MRI of a neurovascular conflict
Axial MRI TOF sequence of a neurovascular conflict between the PICA and the facial nerve at the root exit zone (REZ), indicated by an orange arrow.

Treatment

Conservative

A conservative treatment option is therapy with botulinum toxin injections.

Surgical

When a neurovascular conflict is present, it can also be treated surgically. This procedure eliminates the neurovascular conflict. This can be achieved, for example, using a piece of autologous muscle[^10], Teflon[^9] [^13], Ivalon[^11] or Goretex[^12].

Video

Microvascular decompression for hemifacial spasm

Operative video of endoscope-assisted microvascular decompression of the facial nerve.

License
CC BY 4.0
Download video 180.93 MB

Complications

Complications of microvascular decompression of the facial nerve include, among others[^6]:

  • Postoperative Cerebrospinal fluid fistula.

  • Granuloma formation caused by the Teflon used, in very rare cases[^5].

  • Hypacusis progressing to Anacusis.

  • Postoperative paresis of the facial nerve. This may occur immediately postoperatively or may be delayed for several days after surgery and is transient in most cases [^8].

Prognosis

85-95 % of patients experience moderate to marked relief of symptoms from local injections of botulinum toxin, which must be repeated every 3 to 4 months. Alternatively, microvascular decompression has a success rate of approximately 85 %[^4]. Intraoperative monitoring of the Lateral Spread Response correlates with postoperative symptom improvement in patients [^7].

Editorial transparency

Authorship & revisions

Who created and updated this article, and which changes are documented.

Article created by
Last edited:
on
Revision history
5 edits · 1 contributor
Author list:
Show revision history 5 entries

5 edits by 1 author

Edit · Alaric Steinmetz ·

No content change recorded

Edit · Alaric Steinmetz · (+585 −0)

No content change recorded

Edit · Alaric Steinmetz · (+0 −583)

No content change recorded

Edit · Alaric Steinmetz ·

No content change recorded

Article created on · Alaric Steinmetz

Image viewer

Image

Open original