Tenth case of bilateral hemifacial spasm treated by microvascular decompression: Review of the pathophysiology

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Abstract

Background:

Bilateral hemifacial spasm (BHFS) is a rare neurological syndrome whose diagnosis depends on excluding other facial dyskinesias. We present a case of BHFS along with a literature review.

Methods:

A 64-year-old white, hypertense male reported involuntary left hemiface contractions in 2001 (aged 50). In 2007, right hemifacial symptoms appeared, without spasm remission during sleep. Botulinum toxin type A application produced partial temporary improvement. Left microvascular decompression (MVD) was performed in August 2013, followed by right MVD in May 2014, with excellent results. Follow-up in March 2016 showed complete cessation of spasms without medication.

Results:

The literature confirms nine BHFS cases bilaterally treated by MVD, a definitive surgical option with minimal complications. Regarding HFS pathophysiology, ectopic firing and ephaptic transmissions originate in the root exit zone (REZ) of the facial nerve, due to neurovascular compression (NVC), orthodromically stimulate facial muscles and antidromically stimulate the facial nerve nucleus; this hyperexcitation continuously stimulates the facial muscles. These activated muscles can trigger somatosensory afferent skin nerve impulses and neuromuscular spindles from the trigeminal nerve, which, after transiting the Gasser ganglion and trigeminal nucleus, reach the somatosensory medial posterior ventral nucleus of the contralateral thalamus as well as the somatosensory cortical area of the face. Once activated, this area can stimulate the motor and supplementary motor areas (extrapyramidal and basal ganglia system), activating the motoneurons of the facial nerve nucleus and peripherally stimulating the facial muscles.

Conclusions:

We believe that bilateral MVD is the best approach in cases of BHFS.

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Most cited references 107

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Hemifacial spasm: clinical findings and treatment.

J. Jankovic, S Wang (1998)

Hemifacial spasm (HFS) is a peripherally induced movement disorder characterized by involuntary, unilateral, intermittent, irregular, tonic or clonic contractions of muscles innervated by the ipsilateral facial nerve. We reviewed the clinical features and response to different treatments in 158 patients (61% women) with HFS evaluated at our Movement Disorders Clinic. The mean age at onset was 48.5+/-14.1 years (range: 15-87) and the mean duration of symptoms was 11.4+/-8.5 (range: 0.5-53) years. The left side was affected in 56% instances; 5 patients had bilateral HFS. The lower lid was the most common site of the initial involvement followed by cheek and perioral region. Involuntary eye closure which interfered with vision and social embarrassment were the most common complaints. HFS was associated with trigeminal neuralgia in 5.1% of the cases and 5.7% had prior history of Bell's palsy. Although vascular abnormalities, facial nerve injury, and intracranial tumor were responsible for symptoms in some patients, most patients had no apparent etiology. Botulinum toxin type A (BTX-A) injections, used in 110 patients, provided marked to moderate improvement in 95% of patients. Seven of the 25 (28%) patients who had microvascular decompression reported permanent complications and the HFS recurred in 5 (20%). Although occasionally troublesome, HFS is generally a benign disorder that can be treated effectively with either BTX-A or microvascular decompression.

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Microvascular decompression for hemifacial spasm.

E-h Jho, Peter J. Jannetta, M Larkins … (1995)

The authors report the results of 782 microvascular decompression procedures for hemifacial spasm in 703 patients (705 sides), with follow-up study from 1 to 20 years (mean 8 years). Of 648 patients who had not undergone prior intracranial procedures for hemifacial spasm, 65% were women; their mean age was 52 years, and the mean preoperative duration of symptoms was 7 years. The onset of symptoms was typical in 92% and atypical in 8%. An additional 57 patients who had undergone prior microvascular decompression elsewhere were analyzed as a separate group. Patients were followed prospectively with annual questionnaires. Kaplan-Meier methods showed that among patients without prior microvascular decompression elsewhere, 84% had excellent results and 7% had partial success 10 years postoperatively. Subgroup analyses (Cox proportional hazards model) showed that men had better results than women, and patients with typical onset of symptoms had better results than those with atypical onset. Nearly all failures occurred within 24 months of operation; 9% of patients underwent reoperation for recurrent symptoms. Second microvascular decompression procedures were less successful, whether the first procedure was performed at Presbyterian-University Hospital or elsewhere, unless the procedure was performed within 30 days after the first microvascular decompression. Patient age, side and preoperative duration of symptoms, history of Bell's palsy, preoperative presence of facial weakness or synkinesis, and implant material used had no influence on postoperative results. Complications after the first microvascular decompression for hemifacial spasm included ipsilateral deaf ear in 2.6% and ipsilateral permanent, severe facial weakness in 0.9% of patients. Complications were more frequent in reoperated patients. In all, one operative death (0.1%) and two brainstem infarctions (0.3%) occurred. Microvascular decompression is a safe and definitive treatment for hemifacial spasm with proven long-term efficacy.

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The many faces of hemifacial spasm: differential diagnosis of unilateral facial spasms.

Joseph Jankovic, Toby Yaltho (2011)

Hemifacial spasm is defined as unilateral, involuntary, irregular clonic or tonic movement of muscles innervated by the seventh cranial nerve. Most frequently attributed to vascular loop compression at the root exit zone of the facial nerve, there are many other etiologies of unilateral facial movements that must be considered in the differential diagnosis of hemifacial spasm. The primary purpose of this review is to draw attention to the marked heterogeneity of unilateral facial spasms and to focus on clinical characteristics of mimickers of hemifacial spasm and on atypical presentations of nonvascular cases. In addition to a comprehensive review of the literature on hemifacial spasm, medical records and videos of consecutive patients referred to the Movement Disorders Clinic at Baylor College of Medicine for hemifacial spasm between 2000 and 2010 were reviewed, and videos of illustrative cases were edited. Among 215 patients referred for evaluation of hemifacial spasm, 133 (62%) were classified as primary or idiopathic hemifacial spasm (presumably caused by vascular compression of the ipsilateral facial nerve), and 4 (2%) had hereditary hemifacial spasm. Secondary causes were found in 40 patients (19%) and included Bell's palsy (n=23, 11%), facial nerve injury (n=13, 6%), demyelination (n=2), and brain vascular insults (n=2). There were an additional 38 patients (18%) with hemifacial spasm mimickers classified as psychogenic, tics, dystonia, myoclonus, and hemimasticatory spasm. We concluded that although most cases of hemifacial spasm are idiopathic and probably caused by vascular compression of the facial nerve, other etiologies should be considered in the differential diagnosis, particularly if there are atypical features. Copyright © 2011 Movement Disorder Society.

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Author and article information

Contributors

Warley Carvalho da Silva Martins

Lucas Alverne Freitas de Albuquerque

Gervásio Teles Cardoso de Carvalho

Jules Carlos Dourado

Marcos Dellaretti

Atos Alves de Sousa

Journal

Journal ID (nlm-ta): Surg Neurol Int

Journal ID (iso-abbrev): Surg Neurol Int

Journal ID (publisher-id): SNI

Title: Surgical Neurology International

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 2229-5097

ISSN (Electronic): 2152-7806

Publication date Collection: 2017

Publication date (Electronic): 26 September 2017

Volume: 8

Electronic Location Identifier: 225

Affiliations

[1 ]Department of Neurosurgery, Hospital Santa Casa de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil

[2 ]Faculty of Medical Sciences of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil

[3 ]Department of Neurosurgery, Hospital das Clínicas de Belo Horizonte, Belo Horizonte, Minas Gerais, Brazil

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Article

Publisher ID: SNI-8-225

DOI: 10.4103/sni.sni_95_17

PMC ID: 5629840

SO-VID: 6d6b72f4-4667-4c66-9ba0-e0794817f38b

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Tenth case of bilateral hemifacial spasm treated by microvascular decompression: Review of the pathophysiology

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Abstract

Background:

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International Journal of Applied Technology in Medical Sciences

Most cited references 107

Hemifacial spasm: clinical findings and treatment.

Microvascular decompression for hemifacial spasm.

The many faces of hemifacial spasm: differential diagnosis of unilateral facial spasms.

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