Selective Myectomy and Myotomy In Situ for the Management of Refractory Blepharospasm in Meige Syndrome.

BACKGROUND: Meige syndrome is characterized by involuntary blepharospasm and varied subphenotypes of oromandibular tonic-clonic muscle contraction. Despite botulinum toxin (BTx) being the mainstay of treatment for Meige syndrome, a small subset of patients remain refractory to its effects because the disease is a form of functional blindness. An integrative surgical procedure combining selective myectomy and myotomy in situ of eyelid protractors, blepharoptosis correction, and tightening of the lower eyelid laxity was first applied to treat refractory blepharospasm in patients with Meige syndrome. MATERIALS AND METHODS: This study is a retrospective review conducted on 24 patients with refractory Meige syndrome between 2013 and 2020. Besides selective myectomy and myotomy in situ of eyelid protractors, levator plication and lateral tarsoplasty or canthopexy was performed for blepharoptosis correction and lower eyelid tightening, respectively. Patient demographics, associated diseases, medical treatment history, associated surgical procedures, final aesthetic outcomes, and therapeutic effects as reflected by changes in function disability score and Botox (BTx) treatment were thoroughly recorded and analyzed. RESULTS: The mean age of the patients was 65.2 ± 8.9 years. Twenty-one patients (87.5%) received blepharoptosis correction by levator plication with an average of 11.2 ± 2.9 mm in length. Lateral tarsoplasty was performed in 16 patients (66.7%) by pentagonal tarsal resection with an average of 3.9 ± 0.8 mm in width. Five patients (20.8%) received lateral canthopexy. Among the total of 96 operated eyelids, scar revision with fat graft was performed in 3 eyelids (3.1%). The average amount of BTx treatment decreased from 49.2 ± 12.8 U once every 2.7 ± 0.8 months before surgery to 35.4 ± 7.8 U once every 3.8 ± 0.7 months after surgery. Function disability score improved from 76.7 ± 17.5% preoperatively to 15.6 ± 9.9% postoperatively ( P < 0.001). Only 3 upper eyelids (3.1%) received scar revision and fat grafting due to minor postoperative contour depression. All patients expressed high satisfaction with both functional and aesthetic outcomes (Likert scale 4.5 ± 0.5). CONCLUSIONS: Selective myectomy and myotomy in situ of eyelid protractors combining blepharoplasty correction and lower eyelid tightening can produce long-lasting functional and aesthetic results with minimal complication in patients with refractory Meige syndrome.

Botulinum toxin in Meige's syndrome: A video-based case series.

CONTEXT: Despite being the most common cause of cranial dystonia, Meige's syndrome remains a rare clinical entity. Characterized by blepharospasm and orofacial dystonia, patients suffering from Meige's syndrome benefit from the injection of botulinum toxin (BTX). AIMS: As the majority of the studies tend to discuss Meige's syndrome with blepharospasm patients, there is a paucity of case-based studies dealing exclusively with this syndrome. Hence, we intended to characterize and define the evolution of this syndrome and objectively determine the response of the patients suffering from this entity to BTX therapy. MATERIALS AND METHODS: Eight patients with Meige's syndrome who had never been injected with BTX in the past were evaluated at our movement disorder clinic using a structured questionnaire. Videotaping of abnormal movements was done for 5 minutes before the BTX injection and at a 1-month follow-up. All patients received electromyography-guided injection of BTX and the dosage was decided using clinical evaluation. Their demography, clinical features, and treatment response to BTX were analyzed using the "Burke-Fahn-Marsden dystonia rating scale" (BFMDRS) before injection and at a 1-month follow-up. RESULTS: The peak age of symptom onset was 46.4 years with a male: female ratio of 1:1. The average duration of symptoms was 6.43 years. Majority of the patients (6/8) manifested their disease with blepharospasm, including five patients who had clonic blepharospasm. Lingual dystonia (6/8) and pharyngeal involvement (4/8) were commonly noted. Sensory tricks were present in all, with placement of the fingers over eyelids being the commonest trick (7/8). The average BTX dose administered was 51.58 units, and the peak onset of relief was noted at 8.62 days after the injection. The duration of the effect lasted for 82.5 days on an average. Only one patient reported mild weakness of the muscles of mastication following BTX injection. The average BFMDRS improved from the preprocedural score of 25.06 to 13.12 following the BTX injection. CONCLUSIONS: In this series exclusively dealing with Meige's syndrome patients, tongue involvement was found to be very common (6/8, 75%), and the response to the first dose of BTX treatment was found to be excellent without the occurrence of any major side effects.

Marked Improvement of Meige Syndrome in a Japanese Male Patient with Schizophrenia After Switching from Risperidone to Paliperidone: A Case Report.

Meige syndrome is a relatively rare type of oral facial dystonia. The dominant symptoms involve involuntary eye blinking and chin thrusting. Some patients may experience excessive tongue protrusion, squinting, muddled speech, or uncontrollable contraction of the platysma muscle. A 44-year-old Japanese male was suffering from schizophrenia. The initial presentation of his psychosis consisted of auditory hallucinations, delusions of persecution, psychomotor excitement, loosening association, and restlessness. After being prescribed several antipsychotic drugs, risperidone was started and gradually increased to 4 mg/day. The above symptoms were relieved, particularly auditory hallucination and excitement were promptly improved. Persecutory delusion, however persisted, and deteriorated. At one year after the start of this risperidone regimen, he exhibited severe blepharospasm symptoms (increased rate of eye blinking, light sensitivity) and oromandibular symptoms (trismus, jaw pain, dysarthria). He was diagnosed with Meige syndrome. His antipsychotic drug was changed from risperidone to paliperidone. Two months after switching from risperidone to paliperidone, his eye blinking, light sensitivity, jaw pain, and trismus gradually improved, although the dysarthria persisted. Six months after starting paliperidone, his symptoms of Meige syndrome were completely remitted. He has been well without relapse at 12 mg/day of paliperidone. The case suggests that Meige syndrome is relieved by changing from risperidone to paliperidone. The precise mechanism of the relief remains, however, unknown.

Combination therapy for segmental craniocervical dystonia (Meige syndrome) with aripiprazole, trihexyphenidyl, and botulinum toxin: three cases reports.

Segmental craniocervical dystonia is characterized by blephalospasm and oromandibular dystonia and is also called Meige syndrome. The current treatment strategy including botulinum toxin (BTX) injections has not yet attained an acceptable level. We describe a long-term favorable response of a novel combination therapy with aripiprazole (ARP), trihexyphenidyl (THP), and BTX in three patients with segmental craniocervical dystonia. The symptoms of three patients responded promptly to the combination therapy with ARP 3-6 mg daily, THP 2-8 mg daily, and BTX. Although the patients were required to receive a BTX 50-100 IU injection every 3-6 months, their symptoms were kept in a satisfactory condition for up to 2 years without any adverse effects. ARP possesses the potential for dramatically improving dystonia. THP has the possibility to enhance the efficacy of ARP and prolong the effective period of BTX. It may be an important requisite to give all three agents together for a successful treatment. The combination therapy with ARP, THP, and BTX was well-tolerated and useful in controlling the symptoms of segmental craniocervical dystonia, however, the reason why this combination therapy succeeded is unknown. A further long-term follow-up is required to monitor the delayed neurological adverse effects.

Oral methylphenidate for the treatment of refractory facial dystonias.

Oral methylphenidate (Ritalin, Novartis) has been reported to alleviate symptoms of benign essential blepharospasm in an off-label application. This series presents 3 patients with refractory periorbital and facial dystonias, including blepharospasm, apraxia of eyelid opening, and oromandibular dystonia unresponsive to standard treatments who experienced a response to oral methylphenidate therapy. While the mechanisms for facial dystonias have not been elucidated, there is evidence to suggest that they are on the spectrum with Parkinson disease. Given the role of dopamine loss in the pathogenesis of Parkinson, the authors' speculate that methylphenidate may be acting on the pathway directly involved in facial dystonias. To the authors' knowledge, this is the first report of a case of successful treatment of blepharospasm refractory to upper eyelid myectomy with methylphenidate monotherapy.

Effects of onabotulinum toxin type A injections in patients with Meige's syndrome.

BACKGROUND: Meige's syndrome is a type of facial dystonia characterized by the simultaneous occurrence of blepharospasm and oromandibular dystonia. Although botulinum toxin type A (OBTA) injections are the standard treatment, evidence of their effectiveness and safety in this scenario is still lacking. OBJECTIVE: Our research aimed to evaluate the improvement and occurrence of side effects following injections of onabotulinum toxin type A (OBTA) in patients with Meige's syndrome. METHODS: Patients with Meige's syndrome undergoing botulinum toxin injections were enrolled in this study. We assessed dystonia intensity before and 14 days after OBTA injection using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) to measure the response of symptoms in the eyes (blepharospasm) and mouth (oromandibular dystonia). Other variables, such as dosage, side effects, and demographic data, were also recorded. RESULTS: The study included 41 participants, with a mean age of 67.7 years and a female-to-male ratio of 3.5:1. The mean BFMDRS score before the injections was 8.89, and after 14 days, it was 2.88. The most reported side effect was ptosis, with a 7.3% incidence. OBTA significantly reduced dystonia severity (p < 0.0001). The clinical response for the blepharospasm component was superior to the oromandibular dystonia component. CONCLUSION: Our results support that OBTA seems to be an effective and safe therapeutic option for treating Meige's syndrome. The effect of OBTA was more pronounced in the treatment of blepharospasm than in oromandibular dystonia.

ANTECEDENTES: A síndrome de Meige (SM) é caracterizada pela ocorrência concomitante de blefarospasmo e distonia oromandibular. Embora a toxina onabotulínica do tipo A (TBA) seja o tratamento de escolha, há uma falta de evidências sobre sua eficácia e segurança nesse cenário. OBJETIVO: O objetivo do nosso estudo foi avaliar os efeitos obtidos com a aplicação de TBA em pacientes com SM. MéTODOS: Pacientes com SM que realizam aplicação de TBA foram convidados a participar desse estudo. Os participantes foram questionados sobre a intensidade da distonia antes e 14 dias após a injeção de TBA, utilizando a Escala de Distonia de Burke-Fahn-Marsden (EDBFM) para mensurar a resposta obtida em cada segmento. Outras variáveis, como dose, ocorrência de efeitos colaterais e dados demográficos, também foram registradas. RESULTADOS: O estudo contou com 41 participantes (idade média de 67,7; razão de 3,5 pacientes do sexo feminino para cada participante do sexo masculino). O escore médio na EDBFM antes das aplicações de TBA era 8,89, e, após 14 dias, 2,88. O efeito colateral mais reportado foi ptose (7.3%). A TBA foi capaz de reduzir a severidade da distonia (p < 0.0001), principalmente do blefarospasmo. CONCLUSãO: Nossos resultados corroboram que a TBA é uma terapêutica eficaz e segura no tratamento da SM. O efeito da TBA é superior no manejo do blefarospasmo em relação à distonia oromandibular.

Anterior and posterior sagittal shift in cervical dystonia: a clinical and electromyographic study, including a new EMG approach of the longus colli muscle.

Anterior and posterior sagittal shift of the head are less common postures in cervical dystonia and, as such, have not been comprehensively studied. In this article, we have detailed both our clinical and electromyography (EMG) findings in 11 patients with prominent dystonic sagittal shift of the head. A new technique of injection of the longus colli, based on a laterocervical approach under EMG guidance, is described. We have detailed the clinical phenotypes of dystonic posterior sagittal shift or "double chin" posture (4 patients) and anterior sagittal shift or "goose neck" posture (7 patients) and proposed specific botulinum toxin (BoNT) treatment protocols for these postures. Seven patients with the goose-neck posture responded well (70%-90% benefit) to BoNT injections. Six patients responded to splenius capitii injections alone, and 1 patient needed, in addition, the injection into both sterno-cleido-mastoid muscles. Four patients with the double-chin posture responded well to BoNT injection (50%-80% benefit). Two patients responded to suprahyoid injection alone, and 2 patients needed, in addition, the injection into the sterno-cleido-mastoid and longus colli muscles. Dysphagia was avoided in all of the double-chin patient group by adjusting our injection technique into the suprahyoid and longus colli muscles. The individualised toxin BoNT protocols have resulted in an improved benefit. The new Longus colli injection technique has allowed for a therapeutic effect of botulinum toxin without causing dysphagia.

Aripiprazole in Meige Syndrome: Clinical Response and Implications for Treatment Development.

INTRODUCTION: Meige syndrome is a segmental form of dystonia where botulinum toxins are the preferred treatment option. However, its invasive nature, treatment costs, partial responsiveness, and benefit duration are some of their limitations. METHODS: Six consecutive subjects with Meige syndrome were treated only with aripiprazole. RESULTS: A dramatic response was obtained in all subjects during the first weeks of treatment. Aripiprazole mean ± SD daily dose was 7.9 ± 3.6 mg. Three subjects developed parkinsonism related to aripiprazole treatment; the former improved after reducing the dosage, without significant worsening of cranial dystonia. After a mean ± SD follow-up of 2.0 ± 0.7 years, clinical benefit persists over time, with a mean percentage reduction of Unified Dystonia Rating Score of 75.6% ± 8.4%. CONCLUSIONS: Aripiprazole should be considered as an alternative treatment option among subjects with Meige syndrome, especially in those refractory to botulinum toxin injections. The clinical response shown in our patients may lead to treatment development.

Botulinum toxin treatment of dystonic anterocollis: What to inject.

BACKGROUND: Anterocollis (AC) is a rare form of cervical dystonia, which responds poorly to botulinum toxin treatment. OBJECTIVES: To recognise the different clinical phenotypes of AC and to detail the selection of muscles from the results of treating a cohort of 15 AC patients with Botulinum Toxin. METHODS: The study was performed using prospectively collected data. We included 15 patients with cervical dystonia and AC posture, treated between 2016 and 2019 in our joint Neuro-ENT clinic. We excluded patients with posterior cervical muscle weakness and patients with Parkinsonism. We characterised the primary dystonic posture of every AC patient as posterior sagittal shift, head flexion or neck flexion, or a combination of the three. RESULTS: All AC patients had a more widespread dystonic picture with a majority having Meige syndrome, but AC was the most problematic feature. Treatment with botulinum toxin required the injection not only of the deep cervical flexor (DCF), but also the sterno-cleido-mastoid (SCM) and moreover the supra-hyoid (SH) muscles. The choice between the longus capiti and the longus colli depended on the AC posture. Half of the patients had a dramatic improvement with 90% satisfaction or above. CONCLUSION: AC posture is a complex but treatable type of CD. A joint Neuro-ENT clinic is an ideal setting in which to target all the dystonic muscles. This allows the injection of the longus capiti (under nasal endoscopic approach) as well as the supra-hyoid and SCM muscles in the same session.

Oromandibular dystonia questionnaire (OMDQ-25): a valid and reliable instrument for measuring health-related quality of life.

OBJECTIVE: To develop and validate a disease-specific instrument to measure health-related quality of life in patients with oromandibular dystonia. DESIGN: Prospective, cross-sectional observational study. SETTING: Tertiary referral clinic, North East England, UK. METHODS: The Oromandibular Dystonia Questionnaire (OMDQ-25) was evaluated in a group of 30 patients for psychometric properties, including its sensitivity to change after Botulinum Toxin A treatment. Comparison to a generic instrument was enabled using the Glasgow Health Scale Inventory and the Glasgow Benefit Inventory (GBI). RESULTS: Item generation passed tests for scaling assumptions. Reliability was proven (Cronbach's α > 0.78; test-retest > 0.9) and validity supported with both convergent validity and discriminant validity. Sensitivity to change after Botulinum Toxin A treatment was established for all subscales (P ≤ 0.005), and in comparison with the GBI, all subscales showed large effect sizes. CONCLUSION: The Oromandibular Dystonia Questionnaire (OMDQ-25) is the first reliable and valid instrument to measure health-related quality of life in patients with oromandibular dystonia.

The role of the injection botulinum toxin A in cases of blepharospasm syndrome, hemifacial spasm and Meige's syndrome.

BACKGROUND: Benign Essential Blepharospasm Symdrome is a neuropathologic disorder. The cause of blepharospasm is multifactorial. It is unlikely that a single defect in this elusive control centre is the primary cause of this disease. OBJECTIVES: To evaluate the role of botulinum toxin A in treating cases of Essential Blepharospasm Symdrome, Hemifacial Spasm and Meige's Syndrome, and to assess orbicularis oculi muscle post treatment, in the patients who attended neuro-ophthalmology clinic and the general outpatient department of BP Koirala Lions Centre for Ophthalmic studies. METHODS: A prospective, interventional study was carried out on all the patients of Essential Blepharospasm Symdrome, Hemifacial Spasm and Meige's syndrome who underwent treatment with botulinum toxin A in BP Koirala Lions Centre for Ophthalmic studies during a study period of one and half years. Pre- treatment grading of the spasm was done with Jankovic spasm grading and post treatment response was seen with assessment of orbicularis oculi muscle and improvement in functional impairment scale. Relevant findings were noted. RESULTS: A total of 40 cases were enrolled in the study. The mean Jankovic spasm grading in cases of essential blepharospasm, hemifacial spasm and Meige's syndrome was 3.61 (+/- Standard deviation 0.50, range 3-4), 3.21 (+/- Standard deviation 0.63, range 2-4) and 3.67 (+/- Standard deviation 0.57, range 3-4) respectively. The mean value for reappearance of significant spasms (in months) in cases of essential blepharospasm, hemifacial spasm and Meige's syndrome was 4.3 (+/- Standard deviation 1.6, range 2.0-6.5), 5.8 (+/- Standard deviation 1.4, range 3-8) and 4.5 (+/- Std.deviation 2.8, range 2.5-6.5) respectively. Blepharoptosis was the commonest complication accounting for 66.6% of the complications. CONCLUSIONS: The movement disorders like Essential Blepharospasm, Hemifacial Hpasm and Meige's syndrome are treated by different modalities. An acceptable and effective treatment modality has been a long felt need in these cases. Our study has shown that injection of botulinum toxin A has been a safe and effective method of treating these cases in Nepal.

Treatment of blepharospasm and Meige's syndrome with abo- and onabotulinumtoxinA: long-term safety and efficacy in daily clinical practice.

INTRODUCTION: Thirty years after their approval, botulinum toxin injections still are the first-line therapy for blepharospasm. The aim of our study was to analyze long-term data concerning safety and efficacy in a large cohort over decades. METHODS: Treatment data of all patients with blepharospasm and Meige´s syndrome in our outpatient clinic having undergone at least three subsequent treatment sessions with current onabotulinumtoxinA or abobotulinumtoxin A were analyzed with respect to the course of dose, effect duration, side effects, patients´ satisfaction and occurrence/reasons for treatment discontinuation. RESULTS: The observation period was up to 18 years for onabotulinumtoxinA and 29 years for abobotulinumtoxinA with a total of 1778 and 9319 treatment sessions in 69 patients with onabotulinumtoxinA, 281 with abobotulinumtoxin A and 2 of these having used both products. The dose increased in the first years followed by a stable dose in the following years. The mean dose was 39.1/198.7 mouse units (onabotulinumtoxinA/abobotulinumtoxinA). In over 25% of all sessions, inhibition of the eyelid opening was effectively treated with pretarsal injections. The most common adverse events included ptosis (4%/5%), epiphora/sicca (4%/5%), double vision (1%/1%) and facial asymmetry (1%/1%). Reasons for therapy discontinuation were change to a nearby doctor, age, other diseases, spontaneous improvement, side effects or possible treatment failure. Only one patient was tested positive for neutralizing antibodies against botulinum toxin A. CONCLUSION: The treatment of blepharospasm and Meige's syndrome with onabotulinumtoxinA and abobotulinumtoxinA is safe and effective, also over a long observation period of up to 29 years.

A comparison of jaw-closing and jaw-opening idiopathic oromandibular dystonia.

Oromandibular dystonia (OMD) is a form of focal dystonia that affects masticatory, lower facial, and lingual muscles. We compared the clinical variables and response to treatment between patients with idiopathic jaw-closure C-OMD (n = 11) and jaw-opening dystonia O-OMD (n = 12) seen in our Movement Disorders clinic over the last 10 years. The co-existence of dystonia in other regions and sensory tricks were significantly more prevalent in O-OMD (P = 0.049 and 0.03, respectively). Male gender, orobuccolingual dyskinesias (facial grimacing, lip biting, tongue dyskinesias, platysma contractions and bruxism) and better response to botulinum toxin injections were more frequent in C-OMD but remained a trend.

[Case of oromandibular dystonia presenting with severe impairment of mouth-opening, and a marked effect by administration of baclofen].

We report a 67-year-old woman with idiopathic oromandibular dystonia (OMD). She could neither open the mouth nor take meals due to involuntarily strong mouth-closing. The movement of face, pharynx and tongue were normal, and she could open the mouth slightly when jaw and cheek were touched (sensory trick). Chvostek sign and Trousseau sign were negative, and opisthotonus was not recognized. The laboratory data including calcium, phosphorous and cerebrospinal fluid were within normal limits, head and cervical MRI, temporomandibular joints-Xp and needle electromyography were normal. The surface electromyography revealed that masseter and chin muscles contracted synchronously. This result meant dystonia around the mouth. The clinical course and physical examination did not support the diagnosis of tetanus, tetany or bulldog response. She was diagnosed as OMD. She had peroral administration of baclofen, because this drug is a GABA-derivative and acts as a muscular relaxant. Her clinical symptoms and dystonic pattern on the surface electromyography improved markedly after the administration. Baclofen is an effective drug for treatment of oromandibular dystonia.

[Spasmodic dysphonia in course of Meige's syndrome--case report]. / Przypadek dysfonii spastycznej, wystepujacej w przebiegu zespolu Meige'a.

INTRODUCTION: The Meige's syndrom is characterized by the presence of bilateral, symmetrical, dystonic cramp of face muscles or muscles of middle line of body, the respiratory muscles and muscles of throat. The etiology of Meige's syndrome is uncertain. The disorders of basal ganglia function and neurotransmitters' imbalance (dopamine and acetylocholine) can be with reason of pronouncement of symptoms presumably. MATERIAL AND METHOD: Our aim was to introduce a case of 71 years old patient in whom we diagnosed spasmodic dysphonia in course of Meige's Syndrom. Patient has been treated by 3 years with Botulin toxin. The spasmodic Dysphonia occurred after over 2 years from appearing of first syndrom's symptoms. RESULTS: Sonorous voice during rehabilitation was got during expressing syllables and short bisyllabic words. Patient stays still under phoniatric care. CONCLUSIONS: Patients with spasmodic dysphonia ought to be examined by a interdisciplinary medical team.

Clinical application of botulinum toxin type B in movement disorders and autonomic symptoms.

OBJECTIVE: [corrected] To evaluate efficacy and safety of botulinum toxin type B (BTX-B) in treatment of movement disorders including blepharospasm, oromandibular dystonia, hemifacial spasm, tremor, tics, and hypersecretory disorders such as sialorrhea and hyperhidrosis. METHODS: A retrospective study of BTX-B injections in treatment of 58 patients with various neurological disorders was performed. The mean follow-up time was 0.9 +/- 0.8 years. Results of the first and last treatment of patients with at least 3 injection sessions were compared. RESULTS: The response of 58 patients to a total of 157 BTX-B treatment sessions was analyzed. Of the 157 treatment sessions, 120 sessions (76.4%) resulted in moderate or marked improvement while 17 sessions (10.8%) had no response. The clinical benefits after BTX-B treatment lasted an average of 14 weeks. Of the 41 patients with at least 3 injection sessions (mean 10 +/- 8.6), most patients needed increased dosage upon the last session compared to the first session. Nineteen patients (32.8%) with 27 sessions (17.2%) reported adverse effects with BTX-B treatment. CONCLUSIONS: Though most patients require increased dosage to maintain effective response after repeated injections, BTX-B is an effective and safe treatment drug for a variety of movement disorders, as well as drooling and hyperhidrosis.

[Meige's syndrome or segmental craniocervical dystonia: terminology, history and contemporary view].

The term "Meige's syndrome" or "Breughel's syndrome" is used by specialists for the description of blepharospasm with the involuntary movements in the lower part of the face and/or masseter muscle. These eponyms "Meige's syndrome" and "Breughel's syndrome" make some mess in the terminology. The term "segmental craniocervical dystonia" joins different blepharospam-plus phenotypes and reflects contemporary perceptions about its genetic and pathophysiological community. Botulinotherapy is in fact the only way of symptomathic treatment of craniocervical dystonias.