The Many Faces of Hemifacial Spasm: Differential Diagnosis of Unilateral Facial Spasms

ArticleinMovement Disorders 26(9):1582-92 · August 2011with235 Reads
DOI: 10.1002/mds.23692 · Source: PubMed
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.
    • "It can present concurrently with HFS in a syndrome called tic convulsif. Studies have shown that HFS can follow Bell's palsy, which is facial paralysis from dysfunctional facial nerve caused by brain tumor, stroke, myasthenia gravis, and Lyme disease [34]. HFS has also been reported to occur as a result of facial nerve demyelination in multiple sclerosis patients. "
    [Show abstract] [Hide abstract] ABSTRACT: Hemifacial spasm (HFS) is characterized by involuntary unilateral contractions of the muscles innervated by the ipsilateral facial nerve, usually starting around the eyes before progressing inferiorly to the cheek, mouth, and neck. Its prevalence is 9.8 per 100,000 persons with an average age of onset of 44 years. The accepted pathophysiology of HFS suggests that it is a disease process of the nerve root entry zone of the facial nerve. HFS can be divided into two types: primary and secondary. Primary HFS is triggered by vascular compression whereas secondary HFS comprises all other causes of facial nerve damage. Clinical examination and imaging modalities such as electromyography (EMG) and magnetic resonance imaging (MRI) are useful to differentiate HFS from other facial movement disorders and for intraoperative planning. The standard medical management for HFS is botulinum neurotoxin (BoNT) injections, which provides low-risk but limited symptomatic relief. The only curative treatment for HFS is microvascular decompression (MVD), a surgical intervention that provides lasting symptomatic relief by reducing compression of the facial nerve root. With a low rate of complications such as hearing loss, MVD remains the treatment of choice for HFS patients as intraoperative technique and monitoring continue to improve.
    Full-text · Article · Oct 2014
    • "study, all of the patients were diagnosed as primary HFS, but to the best of our knowledge, there is no report analyzing the postural abnormalities in patients with HFS. HFS usually start as ''twitching'' of the lower eyelid, followed by the involvement of the other periorbital, facial, perioral, and platysma muscles, often leading to social embarrassment and interference with vision from involuntary eye closure [1]. The cause of gait and balance problems detected by clinical scale TBGT in our patients would be attributed to involuntary eye closure, but the further posturographic analyses showed that these patients have more complicated postural abnormalities. "
    [Show abstract] [Hide abstract] ABSTRACT: Hemifacial spasm (HFS) is defined as an involuntary, irregular clonic, or tonic movement of muscles innervated by the ipsilateral seventh cranial nerve. It is reported that the coexistence of non-motor- and motor-related symptoms can be seen in patients with HFS. Postural disturbances were investigated in some movement disorders; however, postural abnormalities due to HFS had not been reported before. In this study, we aimed to investigate the postural abnormalities in patients with HFS. In this cross-sectional, controlled study, Tinetti Balance and Gait Test (TBGT) scores and static posturography were performed on fifteen patients with HFS and fifteen healthy age- and sex-matched controls. The total TBGT score and TBGT-balance score were found to be significantly lower in the patient group than in the control group (p values were, respectively, 0.046 and 0.011). The ratio of the patients with high risk of falling was 40 %, and the difference was found to be significantly higher in the patient group (p value = 0.008). In Fourier analyses, a significant difference was found in the medium to high frequencies (F5-6) when the posturographic evaluation was performed on a solid ground with closed eyes, head rotated to right, and head rotated to the left positions (p values were, respectively, 0.045 and 0.007). The stability index of the HFS group was significantly higher than the control group when tested on the neutral, head right, and head left positions (p values were, respectively, 0.004, 0.049, and 0.003). In conclusion, our study showed that the patients with HFS have more balance and falling problems than the controls, which can be both clinically and posturographically determined.
    Full-text · Article · Sep 2014
    • "Hemifacial spasm (HFS) due to vascular loop compression of the seventh cranial nerve at the root exit zone at the cerebellopontine angle has been well documented [1]. HFS is characterized by unilateral clonic twitching, initially affecting the orbicularis oculi muscle then progressing to the paranasal and perioral muscles [2]. "
    [Show abstract] [Hide abstract] ABSTRACT: We report an unusual case of facial pain and swelling caused by compression of the facial and vestibulocochlear cranial nerves due to the tortuous course of a branch of the posterior inferior cerebellar artery. Although anterior inferior cerebellar artery compression has been well documented in the literature, compression caused by the posterior inferior cerebellar artery is rare. This case provided a diagnostic dilemma, requiring expertise from a number of specialties, and proved to be a learning point to clinicians from a variety of backgrounds. We describe the case in detail and discuss the differential diagnoses. A 57-year-old Caucasian woman with a background of mild connective tissue disease presented to our rheumatologist with intermittent left-sided facial pain and swelling, accompanied by hearing loss in her left ear. An autoimmune screen was negative and a Schirmer's test was normal. Her erythrocyte sedimentation rate was 6mm/h (normal range: 1 to 20mm/h) and her immunoglobulin G and A levels were mildly elevated. A vascular loop protocol magnetic resonance imaging scan showed a loop of her posterior inferior cerebellar artery taking a long course around the seventh and eighth cranial nerves into the meatus and back, resulting in compression of her seventh and eighth cranial nerves. Our patient underwent microvascular decompression, after which her symptoms completely resolved. Hemifacial spasm is characterized by unilateral clonic twitching, although our patient presented with more unusual symptoms of pain and swelling. Onset of symptoms is mostly in middle age and women are more commonly affected. Differential diagnoses include trigeminal neuralgia, temporomandibular joint dysfunction, salivary gland pathology and migrainous headache. Botulinum toxin injection is recognized as an effective treatment option for primary hemifacial spasm. Microvascular decompression is a relatively safe procedure with a high success rate. Although a rare pathology, posterior inferior cerebellar artery compression causing facial pain, swelling and hearing loss should be considered as a differential diagnosis in similar cases.
    Full-text · Article · Mar 2014
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