Outcome of Pallidal Deep Brain Stimulation in Meige Syndrome

OBJECTIVE: Meige syndrome is the combination of blepharospasm and oromandibular dystonia. We assessed the surgical results of bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) in patients with medically refractory Meige syndrome. METHODS: Eleven patients were retrospectively analyzed with follow-ups of more than 12 months. The mean follow-up period was 23.1 +/- 6.4 months. The mean age at time of surgery was 58.0 +/- 7.8 years. The mean duration of symptoms was 8.7 +/- 7.6 years. DBS electrodes were placed under local anesthesia using microelectrode recording and stimulation. After 2.4 +/- 1.3 days of trial tests, the stimulation device was implanted under general anesthesia. Patients were evaluated using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). RESULTS: BFMDRS total movement scores improved by 59.8%, 63.5%, 74.1%, 74.5%, and 85.5% during the immediate postoperative period of test stimulation, 3, 6, 12, and 24 months (n = 5) after surgery, respectively. The BFMDRS total movement scores were reduced gradually and the results reached statistical significance in the postoperative period (test period, p < 0.001; 3 months, p < 0.001; 6 months, p = 0.003; 12 months, p < 0.001; 24 months, p = 0.042). There was no statistical difference between 12 months and 24 months. BFM subscores improved by 63.3% for the eyes, 80.9% for the mouth, 68.4% for speech/swallowing, and 87.9% for the neck at 12 months after surgery. The adverse effects were insignificant. CONCLUSION: The bilateral GPi-DBS can be effective for the treatment of intractable Meige syndrome without significant side effects.

Meige's syndrome

The Meige's syndrome 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. A disorder of basal ganglia function along with perhaps neurotransmitter imbalance [dopamine and acetylcholine] is likely to be the mechanism involved in the causation of this disorder. It is a rare condition and only a limited number of cases have been reported in literature. However, many patients may remain undiscovered or misdiagnosed. Our aim is to introduce a case of 68 years old man who was referred to neurology department with chief complaint of oromandibular dystonia and dysphasia. He was treated successfully with Botulinum toxin

Oromandibular dystonia and persistent psychiatric symptoms in extra-pontine myelinolysis.

A 32 years female presented with gradually progressive dysarthria, dysphagia, oromandibular dystonia and mild generalized weakness. She had several episodes of acute psychotic behavior. She had abnormal saccadic eye movements, generalized hypertonia and exaggerated jerks in upper limbs. She was previously treated in a peripheral hospital for severe vomiting and diarrhea. MRI of brain revealed symmetrical T-2 weighted hyperintensities in bilateral putaminal and caudate region along with pons and midbrain suggesting demyelination due to a metabolic insult. Her power improved gradually over days and the dysarthria, dysphagia and oromandibular dystonia improved gradually over several weeks with supportive measures but the psychiatric manifestations are still persisting.

Ovarian Granulosa Cell Tumor presenting as Meigs' Syndrome with elevated CA125

Herein, a rare case of ovarian granulosa cell tumor, presenting as Meigs' syndrome, with elevated carbohydrate antigen 125 (CA125), is reported. A 69-year-old woman was admitted for the investigation of abdominal fullness and dyspnea. A preoperative examination revealed a huge pelvic tumor and an abdominopelvic magnetic resonance image (MRI) assumed ovarian cancer. A chest computed tomography (CT) scan revealed pleural effusion. A laparotomy confirmed the huge mass to be an ovarian tumor. A total abdominal hysterectomy (TAH), with a bilateral salpingo-oophorectomy (BSO) and partial omentectomy, was performed. Although short-term intrathoracic drainage was required, the hydrothorax and ascites rapidly resolved in the postoperative period.

Clinical application of botulinum toxin type B in movement disorders and autonomic symptoms / 中国医学科学杂志(英文版)

<p><b>OBJECTIVE</b>[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.</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Swallowing disorders in patients with blepharospasm

El blefaroespasmo es una distonía focal caracterizada por el cierre involuntario de los ojos debido a la contracción anormal de los músculos orbiculares de los párpados. Cuando el blefaroespasmo se asocia a la presencia de movimientos involuntarios oromandibulares se denomina síndrome de Meige. El objetivo de este estudio fue investigar la presencia de alteraciones deglutorias en pacientes con blefaroespasmo y síndrome de Meige. Se incluyeron 20 pacientes consecutivos que fueron estudiados mediante vídeo fluoroscopia con técnica de bario. Se investigaron las 4 etapas de la deglución. El 90% de los pacientes (18 casos) presentó trastornos en la deglución. Las alteraciones más comúnmente halladas fueron caída prematura del alimento, 15 casos (83%) y resíduos valeculares, 14 casos (78%). El 67% de anormalidades se observó en la tercera etapa de la deglución. El 89% de los pacientes (16) presentó más de un trastorno deglutorio. Se observó una correlación positiva y estadísticamente significativa entre el número de hallazgos patológicos y la edad de los pacientes y la duración de la enfermedad. De acuerdo a lo publicado, la prevalencia de desórdenes de la deglución en pacientes sanos de edad avanzada es del 44%. En nuestra serie alcanzó el 90%, lo cual sugiere que nuestros hallazgos podrían estar relacionados no sólo con la edad, sino también con la posibilidad de que el compromiso distónico en pacientes con blefaroespasmo sea mayor de lo que se aprecia clínicamente extendiéndose más allá de los músculos orofaciales.

Toxina botulínica: aplicaçöes clínicas / Botulinum toxin: clinic aplication

A toxina botulínica (TOXBA) produz paralisia muscularpela inibiçäo pré-sináptica da liberaçäo da acetilcolina. A eliminaçäo de sua toxicidade natural através de sofisticadas técnicas laboratoriais tornou possível clínica no tratamento de uma diversidade de desordens de movimentos até entäo de difícil controle. A filosofia de seu uso repousa na identificaçäo dos principais músculos envolvidos no movimento anormal e na paralisaçäo dos mesmos pela injeçäo tópica de TOXBA, de modo a bloquear o movimento anormal em questäo. O procedimento, em mäos experimentadas, é até simples e tem a vantagem de ser um método poco invasivo e de poder ser realizado ambulatorialmente. Além de seguro, tem elevada eficácia. A experiência pessoal do autor e a literatura mostram que melhora significativa é obtidaem estrabismo, 85 por cento; blefaroespasmo, 70-90 por cento; distonia oromadibular, 70 por cento; distonia cervical (torcicolo espasmódico), 50-90 por cento; distonia laríngea, 80-100 por cento; distonia de mäo, 40-80 por cento; e espasmo hemifacial, 90 por cento...

Síndrome de distiquiasis linfedema / Distichiasis-lymphedema syndrome

Introducción. El síndrome de distiquiasis-linfedema es una anomalía congénita poco frecuente que transmite con carácter autosómico dominante. Se caracteriza por presentar una fila accesoria de pestañas a lo largo de la lámina posterio del borde libre del párpado a nivel de los orificios de las glándulas de Meibomio y linfedema que generalmente se presenta en la adolescencia. Caso Clínico. Se reporta el caso de una familia con síndrome de distiquiasis-linfedema en la cual el caso índice presentaba distiquiasis y la madre distiquiasis, linfedema crónico e hipoestesia corneal. Se describen los aspectos genéticos, clínicos y de tratamiento más relevantes de esta enfermedad. Conclusión. Es importante que el médico pediatra y oftalmólogo conozcan el síndrome de distiquiasis-linfedema para poder reconocer a tiempo las alteraciones oftalmológicas y sistémicas asociadas y así evitar sus complicaciones

Botulinum A toxin treatment for blepharospasm and Meige syndrome: report of 100 patients.

One hundred patients (9.09 per cent) with blepharospasm from a grand total of 1,100 patients (at the Movement Disorders Clinic at Siriraj Hospital) who had been treated with botulinum A toxin (BTX-A) injection between 1989 and 1996 were analysed. The 100 patients comprised 65 females and 35 males with a female to male ratio of 1.86:1. Their mean age was 53.3 years (S.D. 12.03). Sixty patients had idiopathic blepharospasm, 31 patients were diagnosed with Meige syndrome (blepharospasm plus oromandibular dystonia) and nine patients with segmental dystonia (Meige syndrome plus cervical dystonia). The mean duration of suffering was 39.22 months (S.D. 44.83). Each patient received 30-50 IU of BTX-A injections according to the standard Siriraj injection pattern. Nine patients were lost to follow-up. The results of BTX-A injection were classified as: excellent result (an improvement of more than 75 per cent) in 83.51 per cent; a good response (an improvement of 50-75 per cent) in 13.19 per cent; a moderate response (an improvement of 25-50 per cent) in 2.20 per cent; and minimal or no response in 1.10 per cent. The complications of BTX-A injection were transient minimal ptosis (9.89 per cent), transient double vision (1.10 per cent) and excessive lacrimation (1.10 per cent). The efficacy of BTX-A injection lasted one to two months in 1.10 per cent, two to three months in 23.08 per cent, three to four months in 45.05 per cent, four to five months in 16.48 per cent, five to six months in 4.40 per cent and more than six months in 9.89 per cent. Botulinum A toxin injection is a simple and effective out-patient treatment for patients with blepharospasm, causing no systemic side-effects and minor transient local complications. The only drawback of this treatment is its high cost (100 IU cost 300 US dollars).

Clinical and demographic features of Meige's syndrome.

Fifty five patients with Meige's syndrome were examined for clinical and demographic features. The mean age of onset was 52.3 years. The peak age of onset was in the sixth decade with a male to female ratio of 1.11:1. The mean duration of illness was 3.7 years. Commonest initial symptom was increased blinking, seen in 30 cases (54.5%). Twenty five patients (45.4%) had complete syndrome of blepharospasm with oromandibular dystonia, whereas 24 patients (43.6%) had blepharospasm alone and the rest (6 patients, 10.9%) had oromandibular dystonia. The extension of spasm beyond cranial muscles was observed in 10 patients (18.1%). Eleven patients had family history of dystonia or other extrapyramidal disorders. Incidence of depression was high in these cases.

Distonías craneales: perfil clínico y neurofarmacológico en 123 casos / Cranial dystonias: clinical and neuropharmacological profile in 123 cases

El blefaroespasmo aislado (BE) y/o asociado a distonía oromadibular (DOM) constituye una de las formas más frecuentes de distonía focal/segmentaria de inicio en la edad adulta. En los últimos 10 años evaluamos 123 pacientes con BE y BE + DOM. Observamos una predominancia de 3 a 1 en mujeres sobre varones, con una edad promedio de inicio de los síntomas de 52 años. Si bien algunos pacientes presentaron un comienzo unilateral, todos evolucionaron en forma bilateral. La remisión espontánea de los síntomas ocurrió en sólo 3 pacientes. El error diagnóstico más frecuente consistió en interpretar este cuadro como psicógeno u ocular. Los estudios por imágenes (TC y MRI) demostraron anormalidades en 12 casos que correspondieron a lesiones en ganglios basales en 6 de los mismos. En nuestra serie, 4 pacientes presentaron historia familiar de distonía craneal. El tratamiento de elección fue la administración local por vía subcutánea de toxina botulínica. Estos hallazgos son homologables a otras series internacionales. Creemos que el reconocimiento clínico-epidemiológico de esta afección, a menudo incapacitante, facilitará un mejor y más rápido diagnóstico y ofrecerá una posibilidad terapéutica sencilla que demuestra un alto grado de seguridad y eficacia

Blefaroespasmo. Distonía oromandibular / Blepharospasm. Distonia oromandibular

El blefaroespasmo es una distonía focal que compromete el orbicularis oculi, que en casos severos produce una ceguera funcional. Hemos evaluado las características clínicas y perfil neurofarmacológico en 100 casos consecutivos estudiados en los últimos 9 años en el Hospital Universitario de Buenos Aires, Argentina. La población estuvo compuesta por 69 mujeres y 39 hombres cuyas edades oscilaron entre 29 y 78 años. El 61 por ciento presentó blefaroespasmo mientras que el resto tenía una distonía oromandibular asociada. La gran mayoría comenzó en la 5ta. década y el comienzo fue unilateral en el 12 por ciento pero en todos los casos evolucionó a una forma bilateral. Sólo el 60 por ciento había sido previamente diagnosticado en forma correcta, mientras que en el 40 por ciento restante el diagnóstico se difirió hasta varios años el tratamiento más efectivo fue la infiltración del orbicular de los párpados con toxina botulínica (88 por ciento) seguido por el lisuride en dosis bajas que benefició al 56 por ciento y el trihexifenidilo en el 54.5 por ciento de los tratados. Nuestros resultados sugieren que el cuadro puede ser manejado con éxito luego de hacer un correcto diagnóstico. Las infiltraciones con toxina botulínica debieran ser efectuadas por neurólogos con experiencia en movimientos anormales.

Meige's syndrome.

A case of Meige's syndrome is reported who presented with blepharospasm and oromandibular dystonia along with dislocation of jaw, due to severe dystonia. Such severe form of dystonia are very rare.

Sulpiride in Meige's Syndrome: Possible Role of Glutamate

Sulpiride, a selective antagonist for adenylate cyclase-independent dopamine receptors, was administrated to 25 patients with blepharospasm and oromandibular dystonia(Meige's syndrome). Of the 25, 7 patients (28%) exhibited marked and lasting improvement with sulpiride and 12 patients (48%) showed mild or transient improvement. This favorable therapeutic response to sulpiride suggests that striatal glutamate underactivity may play a role in the pathophysiology of Meige's syndrome as a primary or secondary defect.

Tratamento da doença de Meige com droga agonista de receptores GABA / Treatment of Meige disease with GABA receptors agonist

A doença de Meige é distúrbio de movimento que consiste no aparecimento espontâneo de blefarospamo associado a movimentos dispônicos de musculatura orofacial. Associadamente podem ser encontrados torcicolo espasmódico disfonia espática e distonia de extremidades. Várias hipóteses foram formuladas para explicar esse distúrbio, tendo em vista a resposta a drogas com açäo conhecida nos sitemas de neurotransmissores do cérebro. Algumas evidências apontam para um estado de preponderância dopaminérgica e, nesse sentido, justifica-se a estimulaçäo da atividade GABA, sabendo-se que esse neurotransmissor age sobre uma da alças de controle da produçäo de dopamina na substância negra. Por essa razäo investigamos a açäo de um agonista GABA, o baclofen, sobre a doença de Meige. Foram incluídos no protocolo 5 pacientes, 4 mulheres e um homem, com idade variando entre 50 e 63 anos e duraçäo da doença variando entre 4 meses e 18 anos. Todos apresentavam blefarospasmo-distonia orofacial e, além disso três apresentavam disfonia espástica e um distonia de extremidades. A droga era iniciada em dose de 20mg/dia, aumentada em 10mg a cada três ser obtida resposta ou sugirem efeitos colaterais. Um dos pacientes apresentou melhora marcada do blefarospasmo-distonia orofacial e outro melhora moderada dos mesmos sintomas em avaliaçäo 30 dias após establilizaçäo da dose. Näo houve melhora da distonia espástica e ocorreu melhora a moderada da distonia de extremidades. Näo podemos afirmar que a melhora observada ao fim de um mês se mantenha, ou mesmo que melhora mais significativa fosse observada em avaliaçäo feita mais tardiamente. Concluimos que o baclofen pode ser útil, pelo menos por algum tempo, na doença de Meige