The Many Faces of Hemifacial Spasm: Differential Diagnosis of Unilateral Facial Spasms
ABSTRACT 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.
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ABSTRACT: Psychogenic or functional movement disorders (PMDs) pose a challenge in clinical diagnosis. There are several clues, including sudden onset, incongruous symptoms, distractibility, suggestibility, entrainment of symptoms, and lack of response to otherwise effective pharmacological therapies, that help identify the most common psychogenic movements such as tremor, dystonia, and myoclonus. In this manuscript, we review the frequency, distinct clinical features, functional imaging, and neurophysiological tests that can help in the diagnosis of uncommon presentations of PMDs, such as psychogenic parkinsonism, tics, and chorea; facial, palatal, and ocular movements are also reviewed. In addition, we discuss PMDs at the extremes of age and mass psychogenic illness. Psychogenic parkinsonism (PP) is observed in less than 10% of the case series about PMDs, with a female-male ratio of roughly 1:1. Lack of amplitude decrement in repetitive movements and of cogwheel rigidity help to differentiate PP from true parkinsonism. Dopamine transporter imaging with photon emission tomography can also help in the diagnostic process. Psychogenic movements resembling tics are reported in about 5% of PMD patients. Lack of transient suppressibility of abnormal movements helps to differentiate them from organic tics. Psychogenic facial movements can present with hemifacial spasm, blepharospasm, and other movements. Some patients with essential palatal tremor have been shown to be psychogenic. Convergence ocular spasm has demonstrated a high specificity for psychogenic movements. PMDs can also present in the context of mass psychogenic illness or at the extremes of age. Clinical features and ancillary studies are helpful in the diagnosis of patients with uncommon presentations of psychogenic movement disorders.
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ABSTRACT: The purpose of this study was to evaluate the characteristics and surgical outcomes of familial hemifacial spasm (HFS) and to discuss the role of genetic susceptibility. Between 2001 and 2011, 20 familial HFS patients with ten different pedigrees visited our hospital. The data from comprehensive evaluation of these patients, including clinical, radiological and electrophysiological data and surgical outcomes were reviewed to characterize familial HFS and to compare the characteristics between familial HFS and sporadic HFS. According to the family tree, the inheritance pattern was difficult to define clearly using these data. Radiologic findings suggested that the vertebral artery (VA) was a more frequent offender in familial HFS than in sporadic cases (35.0% vs. 10.0%, p<0.001). Chi-square test showed that there were no correlation between VA tortuosity and underlying morbidity such as diabetes or hypertension (p=0.391). Eighteen out of 19 patients who underwent microvascular decompression showed no residual spasm. Other features of familial HFS overlap with sporadic cases. These findings suggest that certain genetic susceptibilities rather than hypertension or diabetes may influence vascular tortuosity and HFS development. In this study, familial HFS seems not so different from sporadic cases. Authors thought familial HFS could have heterogeneous etiology. Further study of familial HFS including clinical, anatomic, genetic, and molecular information may help identify a gene or trait that can provide insight into the mechanisms of sporadic and familial HFS.Journal of Korean Neurosurgical Society 01/2013; 53(1):1-5. DOI:10.3340/jkns.2013.53.1.1 · 0.52 Impact Factor
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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.Acta neurologica Belgica 09/2014; DOI:10.1007/s13760-014-0358-z · 0.60 Impact Factor