RARE DISEASE
Hemifacial spasm
Hemifacial spasm
Hemifacial spasm
Synonyms: Facial hemispasm, Focal myoclonus of face
Synonyms: Facial hemispasm, Focal myoclonus of face
Synonyms: Facial hemispasm, Focal myoclonus of face
Drug discovery
1
drug
With orphan designation
Overview
Hemifacial spasm (HFS) is a movement disorder characterized by unilateral, involuntary contractions of facial muscles, typically beginning in the eyelid and progressing to the cheek and mouth [1][6][10]. Most cases stem from neurovascular compression of the facial nerve, though tumors or nerve injuries may cause secondary HFS [1][6]. Diagnosis relies on clinical evaluation and MRI to identify vascular compression [1][10]. First-line treatment involves botulinum toxin injections for symptomatic relief, while microvascular decompression surgery offers long-term resolution in eligible patients [1][8][13].
Categories: rare genetic diseases, rare neurological diseases
Research Papers
1,510 drug discovery papers about Hemifacial spasm, with 1 first-in-class and 5 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
1,510 drug discovery papers about Hemifacial spasm, with 1 first-in-class and 5 next-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-06-02 | Eye spasm/Eye twitching: Mg Supplementation and Stress-Reduction in Treating Eyelid Myokymia, Psychosomatic of Anxiety: of Eye Twitching Among High-Stress, Hemifacial Spasm,Blepharospasm
Eyelid twitching and involuntary facial muscle spasms have become common neuromuscular disorders due to stress, anxiety, sleeplessness, prolonged computer usage, exhaustion, and other external factors. The purpose of this review is to discuss various neurophysiological, psychosomatic, environmental, and medical aspects of eye twitching disorders such as eyelid myokymia, hemifacial spasm, and blepharospasm in highly stressed people. Human research demonstrates that chronic stress along with dysfunction in the autonomic nervous system plays an important role in neuromuscular hyperactivity and ocular muscle spasms. Magnesium is discussed in this review as an important nutrient for nerve signaling, muscle relaxation, and neurotransmitter function. Therefore, magnesium intake in combination with stress management methods like meditation, yoga, and sleep may help alleviate the symptoms of eyelid twitches. Neurological complications like hemifacial spasm and blepharospasm generally require the intervention of drugs, neurological procedures like botulinum toxin injection therapy, anticonvulsants, and microvascular decompression surgery. The review also touches upon the effects of prolonged muscular spasm within the eye muscles on emotions, occupation, and quality of life from a psychosocial perspective. While previous human-based studies have shed light on various clinical aspects of the subject, there remain certain issues like small sample size, variation in therapeutic protocols, and absence of longitudinal studies that underscore the need for further clinical research.
2026-04-27 | Hemifacial spasm: a nodal, stretch-mediated paradigm beyond neurovascular compression.
Hemifacial spasm (HFS) has traditionally been interpreted as a neurovascular compression syndrome, in which arterial contact at the root exit zone induces focal demyelination and hyperexcitability of the facial nerve. Although this framework underpins the clinical success of microvascular decompression (MVD), multiple consistent observations remain difficult to reconcile with a purely compression-based mechanism, including the high prevalence of neurovascular contact in asymptomatic individuals, the rapid and reproducible reversibility of symptoms after surgery, and the limited and inconsistent morphological evidence for demyelination. This review integrates clinical, electrophysiological, anatomical, and biomechanical findings to re-examine the pathophysiology of HFS, with particular focus on the transitional zone at the central-peripheral myelin interface. Structural discontinuity and elastic mismatch at the transitional zone increase susceptibility to mechanical perturbation, identifying the transitional node of Ranvier as a critical site of vulnerability. I propose a revised framework-Stretched Cranial Nerve Syndrome (SCNS)-in which HFS reflects mechanically induced, reversible nodal dysfunction arising from chronic stretch and altered neurovascular geometry. This framework provides a coherent and internally consistent explanation for observations insufficiently accounted for by compression-based models, offering a more integrative mechanistic understanding of HFS.
2026-01-28 | Predictors of Postoperative Complications After Microvascular Decompression for Trigeminal Neuralgia and Hemifacial Spasm: A Retrospective Cohort Study.
Microvascular decompression (MVD) is a definitive treatment for trigeminal neuralgia and hemifacial spasm, but a subset of patients experiences postoperative complications. Identifying risk factors for these complications could improve perioperative management and patient counseling. We retrospectively analyzed 453 patients who underwent MVD for classic trigeminal neuralgia or hemifacial spasm from January 2020 to March 2024 at the First Affiliated Hospital of Anhui Medical University. Univariate analyses compared patients with versus without complications. Multivariate logistic regression was performed to identify independent predictors. Model performance was assessed with bootstrapped internal validation (1000 resamples), concordance index (C-index/area under ROC curve), and calibration analysis. A nomogram was constructed based on the final model. In multivariate logistic analysis, elevated preoperative glucose (adjusted OR = 2.63 per 1 mmol/L increase, 95% CI: 1.96-3.66, P<0.001) and lower albumin (OR = 0.61 per 1 g/L, 95% CI: 0.51-0.71, P<0.001) emerged as strong independent predictors of complications. Incision type was not significant after adjustment (OR = 1.12, P=0.913), suggesting its crude effect was confounded by glucose and albumin. The final model showed excellent discrimination (bootstrap-corrected C-index = 0.904) and good calibration (calibration slope ≈ 0.97, intercept -0.05). In patients undergoing MVD for trigeminal neuralgia or hemifacial spasm, elevated preoperative blood glucose and low serum albumin are significant predictors of postoperative complications. Future studies should validate this model externally and explore interventions (strict glucose management and nutritional support) to reduce complications in this patient population.
2025-11-21 | Impact of Ultrasound-guided Superficial Cervical Plexus Block on Early Postoperative Recovery in Patients Undergoing Microvascular Decompression: A Randomized Controlled Trial.
Cervical plexus block (CPB) provides postoperative analgesia and reduces postoperative nausea and vomiting (PONV), which are important for improving the quality of recovery after head and neck surgical procedures. This randomized controlled trial investigated the effect of superficial CPB on early postoperative quality of recovery in patients undergoing microvascular decompression (MVD). Patients undergoing MVD for trigeminal neuralgia and hemifacial spasm were randomly assigned to receive superficial CPB or not (n = 30 per group). Ultrasound-guided superficial CPB was performed with 10 cc of 0.5% ropivacaine before surgery. The primary outcome measure was the Korean version of the 15-item Quality of Recovery (QoR-15K) score on postoperative day 1. The secondary outcome measures included postoperative pain scores, the incidence of PONV, and perioperative inflammatory cytokine levels. The QoR-15K score on postoperative day 1 did not show a significant difference (94.5 [68.0-116.0] vs. 80.5 [51.5-100.8], P = 0.077) between the 2 groups. Perioperative QoR-15K scores also showed no significant time × group effect (P = 0.324). Postoperative pain scores did not differ between the 2 groups. The incidence of PONV was significantly reduced in the superficial CPB group at 6 (P = 0.001) and 12 (P < 0.001) hours postoperatively. Perioperative inflammatory cytokine levels showed no differences between the 2 groups. Superficial CPB did not improve early postoperative QoR in patients undergoing MVD. However, it reduced the incidence of PONV during the early postoperative period, which could potentially enhance patient comfort and satisfaction.
2025-11-13 | INVESTIGATION OF THE RELATIONSHIP BETWEEN VITAMIN B₁₂ LEVEL, NEUROTRANSMITTER IMBALANCE, AND THE CLINICAL SEVERITY OF HEMIFACIAL SPASM
The article is devoted to the study of metabolic and neurochemical factors influencing the pathogenesis of hemifacial spasm (HFS).The levels of vitamin B₁₂, cortisol, and dopamine were determined in patients with HFS and compared with those in a control group. Significant correlations were established between decreased vitamin B₁₂ levels, hyperactivity of the hypothalamic–pituitary–adrenal axis, and disturbances in neurotransmitter processes. The findings confirm the involvement of vitamin B₁₂ deficiency in the disruption of neurovascular interactions, which contributes to the development of chronic pathological spasm of the facial muscles and defines directions for comprehensive patient rehabilitation. Keywords: Hemifacial Spasm, Vitamin B₁₂, Dopamine, Cortisol, Neurotransmitter Imbalance, Metabolic Markers, Neurovascular Dysfunction, Rehabilitation
proteins
2026-07-29 | Comparison of botulinum toxin type A administered at different dilutions in patients with clonic hemifacial spasm
Background: This study aims to compare the motor, functional, and psychosocial clinical changes and post-procedural injection pain following administration of the same total dose of botulinum toxin type A (BoNT-A) diluted with 2 mL versus 1 mL of physiological saline (PS) in patients with hemifacial spasm (HFS). Materials and Methods: This prospective study included patients with HFS, older than 18 years, who had either not previously received BoNT-A or in whom at least six months had elapsed since the last administration. Data from 21 patients who completed both application periods were analyzed. In the first application, BoNT-A was administered diluted with 2 mL of PS (0.9% NaCl), and in the subsequent period, the same total dose was diluted with 1 mL of PS. Patients were evaluated one month after each application using the Hemifacial Spasm Score; procedural pain was assessed with a 0-10 Visual Analog Scale (VAS). Changes from baseline were calculated and the dilution methods compared using the Wilcoxon signed-rank test (<em>p</em> < 0.05). Results: The mean age was 51.62 ± 13.00 years. One month after both applications, a significant decrease from baseline was observed in the severity and frequency of contractions, disease-related complaints, functional difficulties, and all psychosocial parameters. When the changes produced by the two methods were compared, no significant difference was found in the main motor parameters; however, improvement in difficulty reading, depressive mood, avoidance of eye contact, and feeling of embarrassment was greater with the 1 mL application (<em>p</em> = 0.010, <em>p</em> = 0.002, <em>p</em> = 0.007, and <em>p</em> = 0.003, respectively). The post-procedural VAS score was 2.43 ± 2.20 with 2 mL and 0.29 ± 0.78 with 1 mL, with significantly lower injection pain in the 1 mL application (<em>p</em> < 0.001). No adverse effects were observed. Conclusion: Administration of the same total BoNT-A dose diluted with either 1 mL or 2 mL of PS provided marked improvement in the motor symptoms of HFS, with no significant difference between the two dilutions in the main motor efficacy parameters. The 1 mL application was associated with lower injection pain and greater improvement in some functional-psychosocial parameters.
2026-03-30 | Adverse effects of botulinum therapy in facial and neck muscles for aesthetic and neurological indications and recommendations for their prevention. Possibilities of using ultrasound navigation
Objective. To evaluate the effectiveness of ultrasound navigation to minimize adverse effects during botulinum therapy in facial muscles for aesthetic and neurological reasons. Material and methods. The analysis has been carried out on the frequency and nature of adverse effects during injections of botulinum toxin type A into the upper, middle and lower thirds of the face in patients with neurological diseases (blepharospasm, hemifacial spasm, bruxism, migraine, etc.) and for aesthetic reasons (correction of hyperkinetic facial folds). The effectiveness of ultrasound imaging methods, a comparative analysis of injection techniques, and the evaluation of the effectiveness of the drug Relatox have been assessed. Results. The overall incidence of adverse effects with the standard botulinum toxin type A injection technique is 16-18%, and decreases to 5-10% when using ultrasound navigation. In the upper third of the face, the most common complications are ptosis (15%), diplopia (8%), and dry eye (6.5%). The use of injection monitoring reduces the risk of ptosis from 25-30% to 10-15% (p<0.001) due to precise penetration into the pretarsal part of the m. orbicularis oculi. In the middle and lower third of the face, the use of ultrasound navigation minimizes the development of smile asymmetry, dysphagia, and paradoxical bulging of the masticatory muscle. The drug Relatox demonstrates high efficacy (87-92%) in the correction of migraines, facial pain and hyperkinetic wrinkles with a frequency of 3-5%. Conclusion. Ultrasound navigation significantly improves the accuracy of botulinum toxin type A administration, reduces the risk of developing adverse effects, and improves treatment outcomes. The integration of the ultrasound method into clinical practice is recommended for neurological and aesthetic procedures.
2026-02-26 | Beyond Improvement of Motor Symptoms: Central Effects of Botulinum Toxin on Anxiety and Depression in Focal Dystonia, Hemifacial Spasm, and Blepharospasm.
Cervical dystonia (CD), blepharospasm (BSP), and idiopathic hemifacial spasm (HFS) are focal hyperkinetic movement disorders with distinct underlying mechanisms. While CD and BSP involve central network dysfunctions within the basal ganglia-thalamo-cortical and cerebellar circuits, HFS primarily results from peripheral facial nerve hyperexcitability. Still, people living with all three conditions often struggle with mood issues like depression and anxiety, which can originate from both the burden of illness and changes in brain biology. We studied 61 patients (CD, n = 30; BSP, n = 9; HFS, n = 22) and assessed depression and anxiety before and three weeks after botulinum neurotoxin type A (BoNT-A) therapy, considering injection site and dose. BoNT-A significantly reduced depressive and anxiety symptoms across all groups, regardless of disease type, dose, or glabellar injection. These psychiatric improvements were not associated with the degree of motor symptom reduction, suggesting a partially independent mechanism of mood modulation. Our findings indicate that BoNT-A's mood benefits may extend beyond local motor effects, possibly involving broader sensorimotor-limbic interactions. These results highlight the therapeutic potential of BoNT-A for addressing non-motor symptoms in both dystonic and non-dystonic hyperkinetic disorders. Future studies employing imaging and neurophysiological methods are necessary to explain the neural pathways underlying these effects.
2026-01-05 | Efficacy of botulinum toxin treatment in older adults with hemifacial spasm: A retrospective pilot study using the Hemifacial Spasm Severity Score
Background: Hemifacial spasm (HFS) is a chronic and disabling movement disorder commonly affecting older adults. Although botulinum toxin (BoNT) is an established treatment, standardized and multidimensional assessments of severity remain limited. Aim: This study evaluates the therapeutic efficacy of the HFS Severity Score (HFSS), a newly developed scale incorporating five symptom domains, and compares its responsiveness with that of the Jankovic Rating Scale (JRS). Methods: This retrospective study included 18 patients aged 65 years or older who were treated with 10 units of BoNT. HFSS and JRS were assessed at baseline and 4 weeks post-injection by two blinded neurosurgeons. Inter-rater reliability (intraclass correlation coefficient [ICC]) and internal consistency (Cronbach’s α) were calculated. Pre–post differences were evaluated using the Wilcoxon signed-rank test. Convergent validity was examined using Spearman correlation between baseline HFSS and JRS. Responsiveness was assessed using change–score correlations and standardized response means (SRMs). Scatter plots were generated to visualize relationships between the scales. Results: Mean HFSS improved significantly from 9.39 to 3.72 (p<0.001), with all subdomains showing improvement. Baseline HFSS and JRS demonstrated a strong correlation (ρ = 0.82, p<0.001), while ΔHFSS and ΔJRS showed a weaker association (ρ = 0.32), reflecting a floor effect in post-treatment JRS scores. SRM scores indicated high responsiveness for HFSS (1.73) and JRS (1.54), while the ICC (0.91) and Cronbach’s α (0.85) confirmed excellent reliability. No adverse events were observed. Conclusion: BoNT treatment is effective and well-tolerated in older adults with HFS. HFSS is a reliable, multidimensional, and highly responsive tool that complements existing scales and enhances clinical assessment. Relevance for Patients: Older adults with HFS may achieve symptom relief with BoNT. HFSS provides a comprehensive metric that can enhance personalized care and reduce reliance on medication.
2025-12-29 | EFFECT OF LOW-DOSE BOTULINUM TOXIN ON HEMIFACIAL SPASM SEVERITY
Background: Hemifacial spasm is a condition in which there are unilateral contractions of facial muscles. One of the most effective forms of treatment is botulinum toxin injections. However, this therapy only temporarily relieves symptoms, so repeated injections are necessary. This may place a significant economic strain on patients. Due to this, this study aims to see if low-dose botulinum toxin reduces the severity of hemifacial spasm. Objective: To find out whether there is an effect of low-dose botulinum toxin on the severity of hemifacial spasm. Methods: This study is a paired comparison test with a retrospective cohort design. Primary (HFS Score) and secondary (medical record) data was collected from hemifacial spasm patients at Siloam Hospitals Lippo Village Building B. Results: Data was collected from the 36 patients that fulfilled the inclusion and exclusion criteria. All patients were treated with 10 U or less of botulinum toxin. Results show that for HFS Clinical there was a decrease from a mean of 13,03 ± 2,077 to 6,44 ± 2,311 (p < 0,001), whereas HFS Subjective decreased from a mean of 401,53 ± 229,748 to 95,42 ± 151,607 (p < 0,001). Both components have a p-value < 0,001, making it statistically significant. Conclusion: Low-dose botulinum toxin affects the severity of hemifacial spasm in patients at Siloam Hospitals Lippo Village Building B.
cell therapies
2026-07-19 | Management of Slowly Progressive Facial Weakness in Patients With Benign Tumors of the Facial Nerve.
Benign facial nerve tumors have unique presentations granting distinct diagnostic and management implications. Unlike acute-onset facial paralysis, the gradual, intermittent course complicates diagnosis, treatment strategy, and timing, especially when considering potential reanimation. This study presents our institutional experience and proposes approaches to management and evaluation of patients with slowly progressive facial weakness due to benign tumors. A retrospective review included patients between August 2009 and April 2026 with slowly progressive facial weakness or hemifacial spasm due to a benign facial nerve tumor. Demographics, facial palsy history, tumor characteristics, treatment strategies, reanimation procedures, and outcomes were analyzed. Fifteen patients met inclusion criteria with a mean age of onset of 45 years. Sixty-seven percent were initially misdiagnosed. Forty-seven percent presented with synkinesis. The most common tumor type was facial nerve schwannoma (60%). Forty percent underwent complete resection, 27% received radiation, and 27% are under observation. Six patients underwent dynamic facial reanimation: proactively (before tumor extirpation) in three cases, with one undergoing both proactive and concomitant reanimation during extirpation, concomitantly with extirpation in one, and following radiation in one. All achieved recovery of motion on average 3.7 months (range 2-7) later. Two patients are currently planned for reanimation. Benign facial nerve tumors require individualized management based on functional trajectory, tumor characteristics, and patient preference. Treatment options range from observation and radiation to surgical extirpation, but due to the slowly progressive and partial nature of the facial paralysis, the reanimation strategy is guided not only by mimetic musculature viability but also by patient preference on timing. In patients with progressive weakness and anticipated nerve sacrifice during extirpation, proactive reanimation using nerve transfers, cross facial nerve grafts, and free functional muscle transfer should be considered early to establish reinnervation pathways before the denervation window closes, avoiding irreversible facial paralysis.
2024-02-19 | Giant Petrous Segment Aneurysm Causing Facial Palsy: Case Report
Objectives: Co-occurance of aneurysm causing facial palsy by compression and fibrous dysplasia. Background: The aim of this study is to acknowledge this condition due to its rare frequency. Methods: We report a case of a 54 year old patient presenting with sporadic right hemifacial spasms, lasting up to a minute. In 2023 it evolved with facial palsy House Brackmann V and episodes of headache. Imaging investigation showed a giant petrous segment aneurysm on the right internal carotid artery, with signs of aneurysm thrombosis, compression of the internal acoustic meatus and fibrous dysplasia of the temporal bone as well as erosion in the petrous apex. In our case embolization with coils was proposed.
2022-11-22 | Autologous bone fragments for skull reconstruction after microvascular decompression.
Various methods are used to reconstruct the skull after microvascular decompression, giving their own advantages and disadvantages. The objective of this study was to evaluate the efficacy of using autologous bone fragments for skull reconstruction after microvascular decompression. The clinical and follow-up data of 145 patients who underwent microvascular decompression and skull reconstruction using autologous bone fragments in our hospital from September 2020 to September 2021 were retrospectively analyzed. Three patients (2.06%) had delayed wound healing after surgery and were discharged after wound cleaning. No patient developed postoperative cerebrospinal fluid leakage, incisional dehiscence, or intracranial infection. Eighty-five (58.62%) patients underwent follow-up cranial computed tomography at 1 year postoperatively, showed excellent skull reconstruction. And, the longer the follow-up period, the more satisfactory the cranial repair. Two patients underwent re-operation for recurrence of hemifacial spasm, and intraoperative observation revealed that the initial skull defect was filled with new skull bone. The use of autologous bone fragments for skull reconstruction after microvascular decompression is safe and feasible, with few postoperative wound complications and excellent long-term repair results.
2001-07-01 | Fusiform aneurysm of the vertebral artery presenting hemifacial spasm treated by intravascular embolization
BACKGROUND A rare case of fusiform vertebral artery aneurysm causing hemifacial spasm was successfully treated by intravascular embolization. CASE DESCRIPTION A 53-year-old man presented with left hemifacial spasm persisting for 2 years. No other clinical symptoms were observed. Vertebral angiography revealed a fusiform aneurysm of the left vertebral artery, and thin-slice spoiled gradient-recalled acquisition magnetic resonance imaging demonstrated the aneurysm compressing the root exit zone of the left facial nerve. The parent artery was occluded together with the aneurysm by intravascular embolization with Guglielmi detachable coils (GDCs). The patient is free of left hemifacial spasm without any complication. CONCLUSION Hemifacial spasm caused by aneurysms, especially fusiform aneurysms, is quite rare but can be treated by parent artery occlusion and coiling the aneurysm with GDCs.
1988-10-01 | Transtympanic surgery for hemifacial spasm?an electrophysiological study
Producing deliberate damage to the facial nerve in the middle ear is an established method of treating idiopathic hemifacial spasm. However, no previous electrophysiological studies have investigated the effect of this operation on facial nerve function. Eleven patients undergoing transtympanic facial nerve needling were investigated with electromyography. Following operation reduction in the abnormal discharges and synkinesis typical of hemifacial spasm was seen in the majority of patients. The success of this type of operation, although temporary in some patients, together with other neurophysiological studies conflicts with the hypothesis that this disorder is due to ephaptic transmission caused by vascular compression of the facial nerve in the cerebello-pontine angle.
antibodies
2026-03-28 | Neurovascular Mechanisms and Clinical Imaging Correlates in Hemifacial Spasm: A Prospective Observational Study with Surgical Outcome Evaluation
This prospective observational study evaluated the neurovascular mechanisms and imaging correlates of hemifacial spasm (HFS), with surgical outcome assessment following microvascular decompression (MVD). A total of 1,670 patients with primary HFS were enrolled and underwent clinical, radiological, and surgical evaluation over 24 months. Clinical severity was assessed using the Jankovic Rating Scale (JRS) and 36-Item Short Form Health Survey (SF-36) quality-of-life measures. High-resolution magnetic resonance imaging (MRI) (Fast Imaging Employing Steady-State Acquisition [FIESTA] and Constructive Interference in Steady State [CISS] sequences) identified neurovascular compression (NVC) in 94.7% of cases, predominantly involving the anterior inferior cerebellar artery (AICA) (51.2%). Compression at the root exit zone (REZ) was observed in 87.5%. Imaging severity correlated significantly with clinical severity (r = 0.52, p < 0.001) and quality-of-life impairment (r = –0.44, p < 0.001). Among 964 patients undergoing MVD, 56.1% achieved complete relief, and 31.6% had partial improvement. Severe compression predicted better outcomes (74.2% relief rate). Longer symptom duration (>5 years) was associated with reduced surgical benefit (p = 0.008). Recurrence occurred in 11.1% overall, most frequently among patients with venous or multiple vessel compression. Logistic regression failed to identify age, sex, or imaging score alone as significant predictors of poor outcome. However, subgroup analysis confirmed that shorter duration, high imaging severity, and REZ involvement improved surgical prognosis. Health-related quality of life (HRQoL) improvements were greatest among patients with complete symptom relief. These findings support the utility of neuroimaging in diagnosis, prognostication, and surgical planning, emphasizing the importance of early intervention and anatomical characterization of NVC.
2025-12-25 | Phenomenology in Craniofacial Movement Disorders: A Narrative Review
Craniofacial or cranial movement disorders are commonly seen in clinical practice. However, they are usually underdiagnosed or even misdiagnosed, leading to significant impairment in the quality of life of patients affected by these disorders. They are traditionally defined as movement disorders involving the face, jaw, tongue, and palate; but, it is possible to include the eye and ear based on the literature available. The current study aims to generally describe the phenomenology related to these abnormal movements for the general neurologist and practitioner. The most frequent are hemifacial spasm, blepharospasm, and myokymia. Other less commonly discussed are rabbit syndrome, apraxia of the eyelid opening/closing, tardive dyskinesia, chorea, tics, myorhythmia, dystonia, and tremor. Also, there are locations for the development of movement dis-orders, such as the ears (ear dyskinesia and ear myoclonus), chin (geniospasm), and tongue (lingual dystonia and lingual myoclonus) that are rarely discussed. Craniofacial movements are also seen in patients with autoimmune conditions, and the different features of these movements led to new terms like dystonic or dyskinetic stereotypies due to the dynamic change of the phenomenology. Another fact is that up to ten percent of patients with functional movement disorder will have craniofacial movement disorders.
2025-09-01 | Factors associated with persistent abnormal muscle responses in microvascular decompression for hemifacial spasm and prognostic analysis.
An abnormal muscle response (AMR) is an important electrophysiological indicator for the diagnosis, treatment, and prognosis of hemifacial spasm (HFS). The purpose of this study was to analyze the factors associated with and to establish a predictive model for the persistence of AMRs during microvascular decompression (MVD), while also evaluating the relationship between the disappearance of AMRs and delayed recovery. In this retrospective study, authors collected clinical data from patients with HFS who underwent MVD at The First Affiliated Hospital of Dalian Medical University between August 2019 and August 2024. Factors associated with the persistence of AMRs were analyzed, and a predictive model for their persistence was developed. The results showed the disappearance of AMRs at a rate of 78.3% among the 157 patients included in the study. Factors influencing the persistence of AMRs included disease duration, preoperative symptom severity, carbamazepine use, number of responsible vessels, preoperative AMR amplitude, cerebellar retraction depth, and degree of responsible vessel displacement. The predictive model achieved an area under the curve of 0.931, indicating high accuracy. Follow-up data revealed that the persistence of AMRs was associated with recovery rates at 3 months postoperatively (p < 0.01). Risk factors for the persistence of AMRs can be used to predict the probability of persistent intraoperative AMRs. An intraoperative AMR that persists despite having a low preoperative predicted probability may indicate inadequate facial nerve decompression or undetected offending vessels, requiring either additional surgical exploration or transposition of the offending vessels. Conversely, when an AMR persists in the context of a high preoperative predicted probability, the MVD procedure can be safely terminated after confirming adequate decompression to minimize surgical complications. Patients with persistent AMRs may experience delayed recovery, with symptom relief potentially taking 3 months. If symptoms persist without improvement for 6 months to a year, a second surgery can be considered.
2023-09-16 | Blocking insulin-like growth factor 1 receptor signaling pathway inhibits neuromuscular junction regeneration after botulinum toxin-A treatment
Botulinum toxin-A (BTX) administration into muscle is an established treatment for conditions with excessive muscle contraction. However, botulinum therapy has short-term effectiveness, and high-dose injection of BTX could induce neutralizing antibodies against BTX. Therefore, prolonging its effects could be beneficial in a clinical situation. Insulin-like growth factor-1 receptor (IGF1R) and its ligands, insulin-like growth factor (IGF) -I and II, regulate the physiological and pathological processes of the nervous system. It has been suggested that IGF1R is involved in the process after BTX administration, but the specific regeneration mechanism remains unclear. Therefore, this study aimed to determine how inhibition of IGF1R signaling pathway affects BTX-induced muscle paralysis. The results showed that anti-IGF1R antibody administration inhibited the recovery from BTX-induced neurogenic paralysis, and the synaptic components at the neuromuscular junction (NMJ), mainly post-synaptic components, were significantly affected by the antibody. In addition, the wet weight or frequency distribution of the cross-sectional area of the muscle fibers was regulated by IGF1R, and sequential antibody administration following BTX treatment increased the number of Pax7+-satellite cells in the gastrocnemius (GC) muscle, independent of NMJ recovery. Moreover, BTX treatment upregulated mammalian target of rapamycin (mTOR)/S6 kinase signaling pathway, HDAC4, Myog, Fbxo32/MAFbx/Atrogin-1 pathway, and transcription of synaptic components, but not autophagy. Finally, IGF1R inhibition affected only mTOR/S6 kinase translational signaling in the GC muscle. In conclusion, the IGF1R signaling pathway is critical for NMJ regeneration via specific translational signals. IGF1R inhibition could be highly beneficial in clinical practice by decreasing the number of injections and total dose of BTX due to the prolonged duration of the effect.
2015-10-01 | Facial Weakness, Otalgia, and Hemifacial Spasm: A Novel Neurological Syndrome in a Case-Series of 3 Patients With Rheumatic Disease.
Bell palsy occurs in different rheumatic diseases, causes hemifacial weakness, and targets the motor branch of the 7th cranial nerve. Severe, persistent, and refractory otalgia having features of neuropathic pain (ie, burning and allodynic) does not characteristically occur with Bell palsy. Whereas aberrant regeneration of the 7th cranial nerve occurring after a Bell palsy may lead to a variety of clinical findings, hemifacial spasm only rarely occurs. We identified in 3 rheumatic disease patients (2 with Sjögren syndrome, 1 with rheumatoid arthritis) a previously unreported neurological syndrome of facial weakness, otalgia with neuropathic pain features, and hemifacial spasm. We characterized symptoms, examination findings, and response to therapy. All 3 patients experienced vertigo, as well as severe otalgia which persisted after mild facial weakness had completely resolved within 1 to 4 weeks. The allodynic nature of otalgia was striking. Two patients were rendered homebound, as even the barest graze of outdoor breezes caused intolerable ear pain. Patients developed hemifacial spasm either at the time of or within 3 months of facial weakness. Two patients had a polyphasic course, with recurrent episodes of facial weakness and increased otalgia. In all cases, otalgia and hemifacial spasm were unresponsive to neuropathic pain regimens, but responded in 1 case to intravenous immunoglobulin therapy. No patients had vesicles or varicella zoster virus in spinal-fluid studies. We have defined a novel neurological syndrome in 3 rheumatic disease patients, characterized by facial weakness, otalgia, and hemifacial spasm. As described in infectious disorders, the combination of otalgia, facial weakness, and 8th cranial nerve deficits suggests damage to the geniculate ganglia (ie, the sensory ganglia of the 7th cranial nerve), with contiguous involvement of other cranial nerves causing facial weakness and vertigo. However, the relapsing nature and association with hemifacial spasm constitute a unique part of this neurological syndrome.
small molecules
2026-06-02 | Eye spasm/Eye twitching: Mg Supplementation and Stress-Reduction in Treating Eyelid Myokymia, Psychosomatic of Anxiety: of Eye Twitching Among High-Stress, Hemifacial Spasm,Blepharospasm
Eyelid twitching and involuntary facial muscle spasms have become common neuromuscular disorders due to stress, anxiety, sleeplessness, prolonged computer usage, exhaustion, and other external factors. The purpose of this review is to discuss various neurophysiological, psychosomatic, environmental, and medical aspects of eye twitching disorders such as eyelid myokymia, hemifacial spasm, and blepharospasm in highly stressed people. Human research demonstrates that chronic stress along with dysfunction in the autonomic nervous system plays an important role in neuromuscular hyperactivity and ocular muscle spasms. Magnesium is discussed in this review as an important nutrient for nerve signaling, muscle relaxation, and neurotransmitter function. Therefore, magnesium intake in combination with stress management methods like meditation, yoga, and sleep may help alleviate the symptoms of eyelid twitches. Neurological complications like hemifacial spasm and blepharospasm generally require the intervention of drugs, neurological procedures like botulinum toxin injection therapy, anticonvulsants, and microvascular decompression surgery. The review also touches upon the effects of prolonged muscular spasm within the eye muscles on emotions, occupation, and quality of life from a psychosocial perspective. While previous human-based studies have shed light on various clinical aspects of the subject, there remain certain issues like small sample size, variation in therapeutic protocols, and absence of longitudinal studies that underscore the need for further clinical research.
2026-04-27 | Hemifacial spasm: a nodal, stretch-mediated paradigm beyond neurovascular compression.
Hemifacial spasm (HFS) has traditionally been interpreted as a neurovascular compression syndrome, in which arterial contact at the root exit zone induces focal demyelination and hyperexcitability of the facial nerve. Although this framework underpins the clinical success of microvascular decompression (MVD), multiple consistent observations remain difficult to reconcile with a purely compression-based mechanism, including the high prevalence of neurovascular contact in asymptomatic individuals, the rapid and reproducible reversibility of symptoms after surgery, and the limited and inconsistent morphological evidence for demyelination. This review integrates clinical, electrophysiological, anatomical, and biomechanical findings to re-examine the pathophysiology of HFS, with particular focus on the transitional zone at the central-peripheral myelin interface. Structural discontinuity and elastic mismatch at the transitional zone increase susceptibility to mechanical perturbation, identifying the transitional node of Ranvier as a critical site of vulnerability. I propose a revised framework-Stretched Cranial Nerve Syndrome (SCNS)-in which HFS reflects mechanically induced, reversible nodal dysfunction arising from chronic stretch and altered neurovascular geometry. This framework provides a coherent and internally consistent explanation for observations insufficiently accounted for by compression-based models, offering a more integrative mechanistic understanding of HFS.
2026-01-28 | Predictors of Postoperative Complications After Microvascular Decompression for Trigeminal Neuralgia and Hemifacial Spasm: A Retrospective Cohort Study.
Microvascular decompression (MVD) is a definitive treatment for trigeminal neuralgia and hemifacial spasm, but a subset of patients experiences postoperative complications. Identifying risk factors for these complications could improve perioperative management and patient counseling. We retrospectively analyzed 453 patients who underwent MVD for classic trigeminal neuralgia or hemifacial spasm from January 2020 to March 2024 at the First Affiliated Hospital of Anhui Medical University. Univariate analyses compared patients with versus without complications. Multivariate logistic regression was performed to identify independent predictors. Model performance was assessed with bootstrapped internal validation (1000 resamples), concordance index (C-index/area under ROC curve), and calibration analysis. A nomogram was constructed based on the final model. In multivariate logistic analysis, elevated preoperative glucose (adjusted OR = 2.63 per 1 mmol/L increase, 95% CI: 1.96-3.66, P<0.001) and lower albumin (OR = 0.61 per 1 g/L, 95% CI: 0.51-0.71, P<0.001) emerged as strong independent predictors of complications. Incision type was not significant after adjustment (OR = 1.12, P=0.913), suggesting its crude effect was confounded by glucose and albumin. The final model showed excellent discrimination (bootstrap-corrected C-index = 0.904) and good calibration (calibration slope ≈ 0.97, intercept -0.05). In patients undergoing MVD for trigeminal neuralgia or hemifacial spasm, elevated preoperative blood glucose and low serum albumin are significant predictors of postoperative complications. Future studies should validate this model externally and explore interventions (strict glucose management and nutritional support) to reduce complications in this patient population.
2025-11-21 | Impact of Ultrasound-guided Superficial Cervical Plexus Block on Early Postoperative Recovery in Patients Undergoing Microvascular Decompression: A Randomized Controlled Trial.
Cervical plexus block (CPB) provides postoperative analgesia and reduces postoperative nausea and vomiting (PONV), which are important for improving the quality of recovery after head and neck surgical procedures. This randomized controlled trial investigated the effect of superficial CPB on early postoperative quality of recovery in patients undergoing microvascular decompression (MVD). Patients undergoing MVD for trigeminal neuralgia and hemifacial spasm were randomly assigned to receive superficial CPB or not (n = 30 per group). Ultrasound-guided superficial CPB was performed with 10 cc of 0.5% ropivacaine before surgery. The primary outcome measure was the Korean version of the 15-item Quality of Recovery (QoR-15K) score on postoperative day 1. The secondary outcome measures included postoperative pain scores, the incidence of PONV, and perioperative inflammatory cytokine levels. The QoR-15K score on postoperative day 1 did not show a significant difference (94.5 [68.0-116.0] vs. 80.5 [51.5-100.8], P = 0.077) between the 2 groups. Perioperative QoR-15K scores also showed no significant time × group effect (P = 0.324). Postoperative pain scores did not differ between the 2 groups. The incidence of PONV was significantly reduced in the superficial CPB group at 6 (P = 0.001) and 12 (P < 0.001) hours postoperatively. Perioperative inflammatory cytokine levels showed no differences between the 2 groups. Superficial CPB did not improve early postoperative QoR in patients undergoing MVD. However, it reduced the incidence of PONV during the early postoperative period, which could potentially enhance patient comfort and satisfaction.
2025-11-13 | INVESTIGATION OF THE RELATIONSHIP BETWEEN VITAMIN B₁₂ LEVEL, NEUROTRANSMITTER IMBALANCE, AND THE CLINICAL SEVERITY OF HEMIFACIAL SPASM
The article is devoted to the study of metabolic and neurochemical factors influencing the pathogenesis of hemifacial spasm (HFS).The levels of vitamin B₁₂, cortisol, and dopamine were determined in patients with HFS and compared with those in a control group. Significant correlations were established between decreased vitamin B₁₂ levels, hyperactivity of the hypothalamic–pituitary–adrenal axis, and disturbances in neurotransmitter processes. The findings confirm the involvement of vitamin B₁₂ deficiency in the disruption of neurovascular interactions, which contributes to the development of chronic pathological spasm of the facial muscles and defines directions for comprehensive patient rehabilitation. Keywords: Hemifacial Spasm, Vitamin B₁₂, Dopamine, Cortisol, Neurotransmitter Imbalance, Metabolic Markers, Neurovascular Dysfunction, Rehabilitation
proteins
2026-07-29 | Comparison of botulinum toxin type A administered at different dilutions in patients with clonic hemifacial spasm
Background: This study aims to compare the motor, functional, and psychosocial clinical changes and post-procedural injection pain following administration of the same total dose of botulinum toxin type A (BoNT-A) diluted with 2 mL versus 1 mL of physiological saline (PS) in patients with hemifacial spasm (HFS). Materials and Methods: This prospective study included patients with HFS, older than 18 years, who had either not previously received BoNT-A or in whom at least six months had elapsed since the last administration. Data from 21 patients who completed both application periods were analyzed. In the first application, BoNT-A was administered diluted with 2 mL of PS (0.9% NaCl), and in the subsequent period, the same total dose was diluted with 1 mL of PS. Patients were evaluated one month after each application using the Hemifacial Spasm Score; procedural pain was assessed with a 0-10 Visual Analog Scale (VAS). Changes from baseline were calculated and the dilution methods compared using the Wilcoxon signed-rank test (<em>p</em> < 0.05). Results: The mean age was 51.62 ± 13.00 years. One month after both applications, a significant decrease from baseline was observed in the severity and frequency of contractions, disease-related complaints, functional difficulties, and all psychosocial parameters. When the changes produced by the two methods were compared, no significant difference was found in the main motor parameters; however, improvement in difficulty reading, depressive mood, avoidance of eye contact, and feeling of embarrassment was greater with the 1 mL application (<em>p</em> = 0.010, <em>p</em> = 0.002, <em>p</em> = 0.007, and <em>p</em> = 0.003, respectively). The post-procedural VAS score was 2.43 ± 2.20 with 2 mL and 0.29 ± 0.78 with 1 mL, with significantly lower injection pain in the 1 mL application (<em>p</em> < 0.001). No adverse effects were observed. Conclusion: Administration of the same total BoNT-A dose diluted with either 1 mL or 2 mL of PS provided marked improvement in the motor symptoms of HFS, with no significant difference between the two dilutions in the main motor efficacy parameters. The 1 mL application was associated with lower injection pain and greater improvement in some functional-psychosocial parameters.
2026-03-30 | Adverse effects of botulinum therapy in facial and neck muscles for aesthetic and neurological indications and recommendations for their prevention. Possibilities of using ultrasound navigation
Objective. To evaluate the effectiveness of ultrasound navigation to minimize adverse effects during botulinum therapy in facial muscles for aesthetic and neurological reasons. Material and methods. The analysis has been carried out on the frequency and nature of adverse effects during injections of botulinum toxin type A into the upper, middle and lower thirds of the face in patients with neurological diseases (blepharospasm, hemifacial spasm, bruxism, migraine, etc.) and for aesthetic reasons (correction of hyperkinetic facial folds). The effectiveness of ultrasound imaging methods, a comparative analysis of injection techniques, and the evaluation of the effectiveness of the drug Relatox have been assessed. Results. The overall incidence of adverse effects with the standard botulinum toxin type A injection technique is 16-18%, and decreases to 5-10% when using ultrasound navigation. In the upper third of the face, the most common complications are ptosis (15%), diplopia (8%), and dry eye (6.5%). The use of injection monitoring reduces the risk of ptosis from 25-30% to 10-15% (p<0.001) due to precise penetration into the pretarsal part of the m. orbicularis oculi. In the middle and lower third of the face, the use of ultrasound navigation minimizes the development of smile asymmetry, dysphagia, and paradoxical bulging of the masticatory muscle. The drug Relatox demonstrates high efficacy (87-92%) in the correction of migraines, facial pain and hyperkinetic wrinkles with a frequency of 3-5%. Conclusion. Ultrasound navigation significantly improves the accuracy of botulinum toxin type A administration, reduces the risk of developing adverse effects, and improves treatment outcomes. The integration of the ultrasound method into clinical practice is recommended for neurological and aesthetic procedures.
2026-02-26 | Beyond Improvement of Motor Symptoms: Central Effects of Botulinum Toxin on Anxiety and Depression in Focal Dystonia, Hemifacial Spasm, and Blepharospasm.
Cervical dystonia (CD), blepharospasm (BSP), and idiopathic hemifacial spasm (HFS) are focal hyperkinetic movement disorders with distinct underlying mechanisms. While CD and BSP involve central network dysfunctions within the basal ganglia-thalamo-cortical and cerebellar circuits, HFS primarily results from peripheral facial nerve hyperexcitability. Still, people living with all three conditions often struggle with mood issues like depression and anxiety, which can originate from both the burden of illness and changes in brain biology. We studied 61 patients (CD, n = 30; BSP, n = 9; HFS, n = 22) and assessed depression and anxiety before and three weeks after botulinum neurotoxin type A (BoNT-A) therapy, considering injection site and dose. BoNT-A significantly reduced depressive and anxiety symptoms across all groups, regardless of disease type, dose, or glabellar injection. These psychiatric improvements were not associated with the degree of motor symptom reduction, suggesting a partially independent mechanism of mood modulation. Our findings indicate that BoNT-A's mood benefits may extend beyond local motor effects, possibly involving broader sensorimotor-limbic interactions. These results highlight the therapeutic potential of BoNT-A for addressing non-motor symptoms in both dystonic and non-dystonic hyperkinetic disorders. Future studies employing imaging and neurophysiological methods are necessary to explain the neural pathways underlying these effects.
2026-01-05 | Efficacy of botulinum toxin treatment in older adults with hemifacial spasm: A retrospective pilot study using the Hemifacial Spasm Severity Score
Background: Hemifacial spasm (HFS) is a chronic and disabling movement disorder commonly affecting older adults. Although botulinum toxin (BoNT) is an established treatment, standardized and multidimensional assessments of severity remain limited. Aim: This study evaluates the therapeutic efficacy of the HFS Severity Score (HFSS), a newly developed scale incorporating five symptom domains, and compares its responsiveness with that of the Jankovic Rating Scale (JRS). Methods: This retrospective study included 18 patients aged 65 years or older who were treated with 10 units of BoNT. HFSS and JRS were assessed at baseline and 4 weeks post-injection by two blinded neurosurgeons. Inter-rater reliability (intraclass correlation coefficient [ICC]) and internal consistency (Cronbach’s α) were calculated. Pre–post differences were evaluated using the Wilcoxon signed-rank test. Convergent validity was examined using Spearman correlation between baseline HFSS and JRS. Responsiveness was assessed using change–score correlations and standardized response means (SRMs). Scatter plots were generated to visualize relationships between the scales. Results: Mean HFSS improved significantly from 9.39 to 3.72 (p<0.001), with all subdomains showing improvement. Baseline HFSS and JRS demonstrated a strong correlation (ρ = 0.82, p<0.001), while ΔHFSS and ΔJRS showed a weaker association (ρ = 0.32), reflecting a floor effect in post-treatment JRS scores. SRM scores indicated high responsiveness for HFSS (1.73) and JRS (1.54), while the ICC (0.91) and Cronbach’s α (0.85) confirmed excellent reliability. No adverse events were observed. Conclusion: BoNT treatment is effective and well-tolerated in older adults with HFS. HFSS is a reliable, multidimensional, and highly responsive tool that complements existing scales and enhances clinical assessment. Relevance for Patients: Older adults with HFS may achieve symptom relief with BoNT. HFSS provides a comprehensive metric that can enhance personalized care and reduce reliance on medication.
2025-12-29 | EFFECT OF LOW-DOSE BOTULINUM TOXIN ON HEMIFACIAL SPASM SEVERITY
Background: Hemifacial spasm is a condition in which there are unilateral contractions of facial muscles. One of the most effective forms of treatment is botulinum toxin injections. However, this therapy only temporarily relieves symptoms, so repeated injections are necessary. This may place a significant economic strain on patients. Due to this, this study aims to see if low-dose botulinum toxin reduces the severity of hemifacial spasm. Objective: To find out whether there is an effect of low-dose botulinum toxin on the severity of hemifacial spasm. Methods: This study is a paired comparison test with a retrospective cohort design. Primary (HFS Score) and secondary (medical record) data was collected from hemifacial spasm patients at Siloam Hospitals Lippo Village Building B. Results: Data was collected from the 36 patients that fulfilled the inclusion and exclusion criteria. All patients were treated with 10 U or less of botulinum toxin. Results show that for HFS Clinical there was a decrease from a mean of 13,03 ± 2,077 to 6,44 ± 2,311 (p < 0,001), whereas HFS Subjective decreased from a mean of 401,53 ± 229,748 to 95,42 ± 151,607 (p < 0,001). Both components have a p-value < 0,001, making it statistically significant. Conclusion: Low-dose botulinum toxin affects the severity of hemifacial spasm in patients at Siloam Hospitals Lippo Village Building B.
cell therapies
2026-07-19 | Management of Slowly Progressive Facial Weakness in Patients With Benign Tumors of the Facial Nerve.
Benign facial nerve tumors have unique presentations granting distinct diagnostic and management implications. Unlike acute-onset facial paralysis, the gradual, intermittent course complicates diagnosis, treatment strategy, and timing, especially when considering potential reanimation. This study presents our institutional experience and proposes approaches to management and evaluation of patients with slowly progressive facial weakness due to benign tumors. A retrospective review included patients between August 2009 and April 2026 with slowly progressive facial weakness or hemifacial spasm due to a benign facial nerve tumor. Demographics, facial palsy history, tumor characteristics, treatment strategies, reanimation procedures, and outcomes were analyzed. Fifteen patients met inclusion criteria with a mean age of onset of 45 years. Sixty-seven percent were initially misdiagnosed. Forty-seven percent presented with synkinesis. The most common tumor type was facial nerve schwannoma (60%). Forty percent underwent complete resection, 27% received radiation, and 27% are under observation. Six patients underwent dynamic facial reanimation: proactively (before tumor extirpation) in three cases, with one undergoing both proactive and concomitant reanimation during extirpation, concomitantly with extirpation in one, and following radiation in one. All achieved recovery of motion on average 3.7 months (range 2-7) later. Two patients are currently planned for reanimation. Benign facial nerve tumors require individualized management based on functional trajectory, tumor characteristics, and patient preference. Treatment options range from observation and radiation to surgical extirpation, but due to the slowly progressive and partial nature of the facial paralysis, the reanimation strategy is guided not only by mimetic musculature viability but also by patient preference on timing. In patients with progressive weakness and anticipated nerve sacrifice during extirpation, proactive reanimation using nerve transfers, cross facial nerve grafts, and free functional muscle transfer should be considered early to establish reinnervation pathways before the denervation window closes, avoiding irreversible facial paralysis.
2024-02-19 | Giant Petrous Segment Aneurysm Causing Facial Palsy: Case Report
Objectives: Co-occurance of aneurysm causing facial palsy by compression and fibrous dysplasia. Background: The aim of this study is to acknowledge this condition due to its rare frequency. Methods: We report a case of a 54 year old patient presenting with sporadic right hemifacial spasms, lasting up to a minute. In 2023 it evolved with facial palsy House Brackmann V and episodes of headache. Imaging investigation showed a giant petrous segment aneurysm on the right internal carotid artery, with signs of aneurysm thrombosis, compression of the internal acoustic meatus and fibrous dysplasia of the temporal bone as well as erosion in the petrous apex. In our case embolization with coils was proposed.
2022-11-22 | Autologous bone fragments for skull reconstruction after microvascular decompression.
Various methods are used to reconstruct the skull after microvascular decompression, giving their own advantages and disadvantages. The objective of this study was to evaluate the efficacy of using autologous bone fragments for skull reconstruction after microvascular decompression. The clinical and follow-up data of 145 patients who underwent microvascular decompression and skull reconstruction using autologous bone fragments in our hospital from September 2020 to September 2021 were retrospectively analyzed. Three patients (2.06%) had delayed wound healing after surgery and were discharged after wound cleaning. No patient developed postoperative cerebrospinal fluid leakage, incisional dehiscence, or intracranial infection. Eighty-five (58.62%) patients underwent follow-up cranial computed tomography at 1 year postoperatively, showed excellent skull reconstruction. And, the longer the follow-up period, the more satisfactory the cranial repair. Two patients underwent re-operation for recurrence of hemifacial spasm, and intraoperative observation revealed that the initial skull defect was filled with new skull bone. The use of autologous bone fragments for skull reconstruction after microvascular decompression is safe and feasible, with few postoperative wound complications and excellent long-term repair results.
2001-07-01 | Fusiform aneurysm of the vertebral artery presenting hemifacial spasm treated by intravascular embolization
BACKGROUND A rare case of fusiform vertebral artery aneurysm causing hemifacial spasm was successfully treated by intravascular embolization. CASE DESCRIPTION A 53-year-old man presented with left hemifacial spasm persisting for 2 years. No other clinical symptoms were observed. Vertebral angiography revealed a fusiform aneurysm of the left vertebral artery, and thin-slice spoiled gradient-recalled acquisition magnetic resonance imaging demonstrated the aneurysm compressing the root exit zone of the left facial nerve. The parent artery was occluded together with the aneurysm by intravascular embolization with Guglielmi detachable coils (GDCs). The patient is free of left hemifacial spasm without any complication. CONCLUSION Hemifacial spasm caused by aneurysms, especially fusiform aneurysms, is quite rare but can be treated by parent artery occlusion and coiling the aneurysm with GDCs.
1988-10-01 | Transtympanic surgery for hemifacial spasm?an electrophysiological study
Producing deliberate damage to the facial nerve in the middle ear is an established method of treating idiopathic hemifacial spasm. However, no previous electrophysiological studies have investigated the effect of this operation on facial nerve function. Eleven patients undergoing transtympanic facial nerve needling were investigated with electromyography. Following operation reduction in the abnormal discharges and synkinesis typical of hemifacial spasm was seen in the majority of patients. The success of this type of operation, although temporary in some patients, together with other neurophysiological studies conflicts with the hypothesis that this disorder is due to ephaptic transmission caused by vascular compression of the facial nerve in the cerebello-pontine angle.
antibodies
2026-03-28 | Neurovascular Mechanisms and Clinical Imaging Correlates in Hemifacial Spasm: A Prospective Observational Study with Surgical Outcome Evaluation
This prospective observational study evaluated the neurovascular mechanisms and imaging correlates of hemifacial spasm (HFS), with surgical outcome assessment following microvascular decompression (MVD). A total of 1,670 patients with primary HFS were enrolled and underwent clinical, radiological, and surgical evaluation over 24 months. Clinical severity was assessed using the Jankovic Rating Scale (JRS) and 36-Item Short Form Health Survey (SF-36) quality-of-life measures. High-resolution magnetic resonance imaging (MRI) (Fast Imaging Employing Steady-State Acquisition [FIESTA] and Constructive Interference in Steady State [CISS] sequences) identified neurovascular compression (NVC) in 94.7% of cases, predominantly involving the anterior inferior cerebellar artery (AICA) (51.2%). Compression at the root exit zone (REZ) was observed in 87.5%. Imaging severity correlated significantly with clinical severity (r = 0.52, p < 0.001) and quality-of-life impairment (r = –0.44, p < 0.001). Among 964 patients undergoing MVD, 56.1% achieved complete relief, and 31.6% had partial improvement. Severe compression predicted better outcomes (74.2% relief rate). Longer symptom duration (>5 years) was associated with reduced surgical benefit (p = 0.008). Recurrence occurred in 11.1% overall, most frequently among patients with venous or multiple vessel compression. Logistic regression failed to identify age, sex, or imaging score alone as significant predictors of poor outcome. However, subgroup analysis confirmed that shorter duration, high imaging severity, and REZ involvement improved surgical prognosis. Health-related quality of life (HRQoL) improvements were greatest among patients with complete symptom relief. These findings support the utility of neuroimaging in diagnosis, prognostication, and surgical planning, emphasizing the importance of early intervention and anatomical characterization of NVC.
2025-12-25 | Phenomenology in Craniofacial Movement Disorders: A Narrative Review
Craniofacial or cranial movement disorders are commonly seen in clinical practice. However, they are usually underdiagnosed or even misdiagnosed, leading to significant impairment in the quality of life of patients affected by these disorders. They are traditionally defined as movement disorders involving the face, jaw, tongue, and palate; but, it is possible to include the eye and ear based on the literature available. The current study aims to generally describe the phenomenology related to these abnormal movements for the general neurologist and practitioner. The most frequent are hemifacial spasm, blepharospasm, and myokymia. Other less commonly discussed are rabbit syndrome, apraxia of the eyelid opening/closing, tardive dyskinesia, chorea, tics, myorhythmia, dystonia, and tremor. Also, there are locations for the development of movement dis-orders, such as the ears (ear dyskinesia and ear myoclonus), chin (geniospasm), and tongue (lingual dystonia and lingual myoclonus) that are rarely discussed. Craniofacial movements are also seen in patients with autoimmune conditions, and the different features of these movements led to new terms like dystonic or dyskinetic stereotypies due to the dynamic change of the phenomenology. Another fact is that up to ten percent of patients with functional movement disorder will have craniofacial movement disorders.
2025-09-01 | Factors associated with persistent abnormal muscle responses in microvascular decompression for hemifacial spasm and prognostic analysis.
An abnormal muscle response (AMR) is an important electrophysiological indicator for the diagnosis, treatment, and prognosis of hemifacial spasm (HFS). The purpose of this study was to analyze the factors associated with and to establish a predictive model for the persistence of AMRs during microvascular decompression (MVD), while also evaluating the relationship between the disappearance of AMRs and delayed recovery. In this retrospective study, authors collected clinical data from patients with HFS who underwent MVD at The First Affiliated Hospital of Dalian Medical University between August 2019 and August 2024. Factors associated with the persistence of AMRs were analyzed, and a predictive model for their persistence was developed. The results showed the disappearance of AMRs at a rate of 78.3% among the 157 patients included in the study. Factors influencing the persistence of AMRs included disease duration, preoperative symptom severity, carbamazepine use, number of responsible vessels, preoperative AMR amplitude, cerebellar retraction depth, and degree of responsible vessel displacement. The predictive model achieved an area under the curve of 0.931, indicating high accuracy. Follow-up data revealed that the persistence of AMRs was associated with recovery rates at 3 months postoperatively (p < 0.01). Risk factors for the persistence of AMRs can be used to predict the probability of persistent intraoperative AMRs. An intraoperative AMR that persists despite having a low preoperative predicted probability may indicate inadequate facial nerve decompression or undetected offending vessels, requiring either additional surgical exploration or transposition of the offending vessels. Conversely, when an AMR persists in the context of a high preoperative predicted probability, the MVD procedure can be safely terminated after confirming adequate decompression to minimize surgical complications. Patients with persistent AMRs may experience delayed recovery, with symptom relief potentially taking 3 months. If symptoms persist without improvement for 6 months to a year, a second surgery can be considered.
2023-09-16 | Blocking insulin-like growth factor 1 receptor signaling pathway inhibits neuromuscular junction regeneration after botulinum toxin-A treatment
Botulinum toxin-A (BTX) administration into muscle is an established treatment for conditions with excessive muscle contraction. However, botulinum therapy has short-term effectiveness, and high-dose injection of BTX could induce neutralizing antibodies against BTX. Therefore, prolonging its effects could be beneficial in a clinical situation. Insulin-like growth factor-1 receptor (IGF1R) and its ligands, insulin-like growth factor (IGF) -I and II, regulate the physiological and pathological processes of the nervous system. It has been suggested that IGF1R is involved in the process after BTX administration, but the specific regeneration mechanism remains unclear. Therefore, this study aimed to determine how inhibition of IGF1R signaling pathway affects BTX-induced muscle paralysis. The results showed that anti-IGF1R antibody administration inhibited the recovery from BTX-induced neurogenic paralysis, and the synaptic components at the neuromuscular junction (NMJ), mainly post-synaptic components, were significantly affected by the antibody. In addition, the wet weight or frequency distribution of the cross-sectional area of the muscle fibers was regulated by IGF1R, and sequential antibody administration following BTX treatment increased the number of Pax7+-satellite cells in the gastrocnemius (GC) muscle, independent of NMJ recovery. Moreover, BTX treatment upregulated mammalian target of rapamycin (mTOR)/S6 kinase signaling pathway, HDAC4, Myog, Fbxo32/MAFbx/Atrogin-1 pathway, and transcription of synaptic components, but not autophagy. Finally, IGF1R inhibition affected only mTOR/S6 kinase translational signaling in the GC muscle. In conclusion, the IGF1R signaling pathway is critical for NMJ regeneration via specific translational signals. IGF1R inhibition could be highly beneficial in clinical practice by decreasing the number of injections and total dose of BTX due to the prolonged duration of the effect.
2015-10-01 | Facial Weakness, Otalgia, and Hemifacial Spasm: A Novel Neurological Syndrome in a Case-Series of 3 Patients With Rheumatic Disease.
Bell palsy occurs in different rheumatic diseases, causes hemifacial weakness, and targets the motor branch of the 7th cranial nerve. Severe, persistent, and refractory otalgia having features of neuropathic pain (ie, burning and allodynic) does not characteristically occur with Bell palsy. Whereas aberrant regeneration of the 7th cranial nerve occurring after a Bell palsy may lead to a variety of clinical findings, hemifacial spasm only rarely occurs. We identified in 3 rheumatic disease patients (2 with Sjögren syndrome, 1 with rheumatoid arthritis) a previously unreported neurological syndrome of facial weakness, otalgia with neuropathic pain features, and hemifacial spasm. We characterized symptoms, examination findings, and response to therapy. All 3 patients experienced vertigo, as well as severe otalgia which persisted after mild facial weakness had completely resolved within 1 to 4 weeks. The allodynic nature of otalgia was striking. Two patients were rendered homebound, as even the barest graze of outdoor breezes caused intolerable ear pain. Patients developed hemifacial spasm either at the time of or within 3 months of facial weakness. Two patients had a polyphasic course, with recurrent episodes of facial weakness and increased otalgia. In all cases, otalgia and hemifacial spasm were unresponsive to neuropathic pain regimens, but responded in 1 case to intravenous immunoglobulin therapy. No patients had vesicles or varicella zoster virus in spinal-fluid studies. We have defined a novel neurological syndrome in 3 rheumatic disease patients, characterized by facial weakness, otalgia, and hemifacial spasm. As described in infectious disorders, the combination of otalgia, facial weakness, and 8th cranial nerve deficits suggests damage to the geniculate ganglia (ie, the sensory ganglia of the 7th cranial nerve), with contiguous involvement of other cranial nerves causing facial weakness and vertigo. However, the relapsing nature and association with hemifacial spasm constitute a unique part of this neurological syndrome.
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Drug Discovery Landscape
1 orphan drug designation for Hemifacial spasm.
1 orphan drug designation for Hemifacial spasm.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
incobotulinumtoxinA | proteins | FDA | 2022-11-28 | — | Merz Pharmaceuticals, LLC |
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