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1.
Sci Rep ; 11(1): 8742, 2021 04 22.
Artigo em Inglês | MEDLINE | ID: mdl-33888857

RESUMO

Deep-brain stimulation (DBS) is an effective treatment for patients with Meige syndrome. The globus pallidus interna (GPi) and the subthalamic nucleus (STN) are accepted targets for this treatment. We compared 12-month outcomes for patients who had undergone bilateral stimulation of the GPi or STN. Forty-two Asian patients with primary Meige syndrome who underwent GPi or STN neurostimulation were recruited between September 2017 and September 2019 at the Department of Neurosurgery, Peking University People's Hospital. The primary outcome was the change in motor function, including the Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) and disability subscale (BFMDRS-D) at 3 days before DBS (baseline) surgery and 1, 3, 6, and 12 months after surgery. Secondary outcomes included health-related quality of life, sleep quality status, depression severity, and anxiety severity at 3 days before and 12 months after DBS surgery. Adverse events during the 12 months were also recorded. Changes in BFMDRS-M and BFMDRS-D scores at 1, 3, 6, and 12 months with DBS and without medication did not significantly differ based on the stimulation target. There were also no significant differences in the changes in health-related quality of life (36-Item Short-Form General Health Survey) and sleep quality status (Pittsburgh Sleep Quality Index) at 12 months. However, there were larger improvements in the STN than the GPi group in mean score changes on the 17-item Hamilton depression rating scale (- 3.38 vs. - 0.33 points; P = 0.014) and 14-item Hamilton anxiety rating scale (- 3.43 vs. - 0.19 points; P < 0.001). There were no significant between-group differences in the frequency or type of serious adverse events. Patients with Meige syndrome had similar improvements in motor function, quality of life and sleep after either pallidal or subthalamic stimulation. Depression and anxiety factors may reasonably be included during the selection of DBS targets for Meige syndrome.


Assuntos
Estimulação Encefálica Profunda , Globo Pálido/fisiopatologia , Núcleo Subtalâmico/fisiopatologia , Idoso , Feminino , Humanos , Masculino , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Estudos Retrospectivos
2.
J Neurol Neurosurg Psychiatry ; 91(12): 1343-1348, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33028603

RESUMO

OBJECTIVES: To study the efficacy and safety of bilateral globus pallidus internus deep brain stimulation (GPi-DBS) in refractory Meige syndrome (MS) and evaluate the psychiatric disorders before and after surgery. METHODS: Twenty-two patients with MS treated with bilateral GPi-DBS were retrospectively analysed before surgery and after continuous neurostimulation. Before surgery, patients were assessed by the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), Self-Rating Depression Scale, Medical Outcomes Study 36-Item Short-Form General Health Survey (SF-36) and Pittsburgh Sleep Quality Index (PQSI), which corresponded to motor symptoms, depressive state, quality of life and sleep quality, respectively. The implantable pulse generator of each patient was activated at 1 month after surgery. At 1 month, 3 months, 6 months and 12 months after continuous neurostimulation, all patients were evaluated by the same scales above. RESULTS: The BFMDRS movement scores decreased from 15.0±5.3 before surgery to 3.5±4.5 at 12 months after neurostimulation, with a mean improvement of 78% (p<0.001). The BFMDRS disability scores improved from 7.4±4.9 before surgery to 4.0±4.6 at 12 months after neurostimulation, with a mean improvement of 56% (p<0.001). The postoperative SF-36 scores had the remarkable improvement compared with baseline scores. Impaired sleep quality was found in 82% of patients and depression in 64% before surgery, which didn't neither obtained amelioration after continuous neurostimulation. CONCLUSIONS: Bilateral pallidal neurostimulation is a beneficial therapeutic option for refractory MS, which could improve the motor symptoms except for depression and sleep quality.


Assuntos
Estimulação Encefálica Profunda/métodos , Depressão/psicologia , Globo Pálido , Síndrome de Meige/terapia , Qualidade de Vida , Sono , Idoso , Transtornos da Articulação/epidemiologia , Transtornos de Deglutição/epidemiologia , Tontura/epidemiologia , Feminino , Transtornos Neurológicos da Marcha/epidemiologia , Humanos , Hipestesia/epidemiologia , Neuroestimuladores Implantáveis , Masculino , Síndrome de Meige/fisiopatologia , Síndrome de Meige/psicologia , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Resultado do Tratamento
3.
Neurol India ; 66(1): 71-76, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29322963

RESUMO

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.


Assuntos
Toxinas Botulínicas/farmacologia , Síndrome de Meige/tratamento farmacológico , Síndrome de Meige/fisiopatologia , Fármacos Neuromusculares/farmacologia , Língua/fisiopatologia , Adulto , Idade de Início , Idoso , Toxinas Botulínicas/administração & dosagem , Eletromiografia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Fármacos Neuromusculares/administração & dosagem , Resultado do Tratamento , Gravação em Vídeo , Adulto Jovem
4.
J Neurosurg ; 128(3): 897-902, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-28548593

RESUMO

OBJECTIVE Subthalamic nucleus deep brain stimulation has been shown to be effective in reducing symptoms of primary Meige syndrome. However, assessments of its efficacy and safety have been limited to several case reports and small studies. METHODS The authors performed a retrospective study to assess the efficacy and safety of bilateral subthalamic nucleus stimulation in 15 patients with primary Meige syndrome who responded poorly to medical treatments or botulinum toxin injections. Using the movement and disability subscores of the Burke-Fahn-Marsden Dystonia Rating Scale, the authors evaluated the severity of patients' dystonia and related before surgery and at final follow-up during neurostimulation. The movement scale was assessed based on preoperative and postoperative video documentation by an independent rater who was unaware of each patient's neurostimulation status. Quality of life was assessed with the Medical Outcomes Study 36-Item Short-Form General Health Survey. RESULTS The dystonia movement subscores in 14 consecutive patients improved from 19.3 ± 7.6 (mean ± standard deviation) before surgery to 5.5 ± 4.5 at final follow-up (28.5 ± 16.5 months), with a mean improvement of 74% (p < 0.05). The disability subscore improved from 15.6 ± 4.9 before surgery to 6.1 ± 3.5 at final follow-up (p < 0.05). In addition, the postoperative SF-36 scores increased markedly over those at baseline. The authors also found that bilateral stimulation of the subthalamic nucleus immediately improved patient symptoms after stimulation and required lower stimulation parameters than those needed for pallidal deep brain stimulation for primary Meige syndrome. Four adverse events occurred in 3 patients; all of these events resolved without permanent sequelae. CONCLUSIONS These findings provide further evidence to support the long-term efficacy and safety of subthalamic nucleus stimulation as an alternative treatment for patients with medically intractable Meige syndrome.


Assuntos
Estimulação Encefálica Profunda/métodos , Síndrome de Meige/terapia , Núcleo Subtalâmico/fisiopatologia , Adulto , Idoso , Feminino , Humanos , Masculino , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento
5.
J Neurol Sci ; 383: 153-157, 2017 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-29246604

RESUMO

BACKGROUND: The aim of the present study was to report the short-term as well long-term results of bilateral pallidal stimulation in 6 consecutive patients for severe debilitating craniocervical dystonia (Meige syndrome) using Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). METHODS: We evaluated 6 consecutive patients with the diagnosis of intractable long-standing craniocervical dystonia. The formal objective assessment included the motor and disability BFMDRS scores. The BFMDRS assessment was performed before and after it roughly annually up to 60months when bilateral pallidal stimulation was switched on and compared to baseline BFMDRS scores. We present short-term (3months postoperatively) follow-up as well long-term (from 6 to 60months) results. Baseline BFMDRS scores and subsequent follow-up BFMDRS scores were compared with the use of a Wilcoxon signed-rank test for matched pairs. A two-tailed probability level of 5% (p<0.05) was considered significant. RESULTS: Bilateral GPi DBS improved the BFMDRS total movement score by 65% at short-term follow-up and by 53% at long-term follow-up when compared to baseline scores. Subscores for eyes at short-term follow-up were reduced by 78%, for mouth by 58%, and for speech/swallowing by 49%. This improvements for individual subscores were maintained at long-term follow-up and were as follows for eyes by 67%, mouth by 50% and speech/swallowing by 39%. The BFMDRS disability score was reduced by 48% at short-term follow-up and by 55% at long-term follow-up when compared to baseline scores. CONCLUSION: Our results showed that bilateral GPi DBS in craniocervical dystonia is effective and safe. Phasic dystonic movements like blepharospasm or oromandibular dystonia responded very fast and favorable to pallidal stimulation reducing disabilities.


Assuntos
Estimulação Encefálica Profunda , Globo Pálido , Síndrome de Meige/terapia , Idoso , Estimulação Encefálica Profunda/métodos , Feminino , Seguimentos , Globo Pálido/fisiopatologia , Humanos , Masculino , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Estudos Prospectivos , Resultado do Tratamento
6.
J Neurol Sci ; 372: 162-170, 2017 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-28017205

RESUMO

'Meige's syndrome' is a type of cranial dystonia characterized by blepharospasm and oromandibular dystonia and can be associated with complex movement of lower facial muscles, mouth, jaw, tongue, pharyngeal and cervical muscles. Frequently, blepharospasm is the earliest clinical manifestation, which spreads over a period of time to involve other cranial and extra-cranial muscles. Common characteristics of this syndrome are well known, but their variety is wide. Different eponyms such as "Breughel syndrome", "Wood syndrome", "Blepharospasm plus", "Segmental cranial dystonia" and "Segmental cranio-cervical dystonia" have been used to describe this entity with numerous anatomical variations. In the majority of the patients Meige's syndrome is primary or idiopathic, where the cause of spasm is not known, however secondary cases can occur following prolonged use of neuroleptics or secondary to underlying brain disorders. This syndrome has also been described in patients with essential tremor, Parkinson's disease and atypical Parkinsonism. Neurophysiological features are similar to other focal dystonia characterized by abnormal plasticity and impaired inhibition. Most of the patients are successfully treated with injection of botulinum toxin, however deep brain stimulation has emerged as a good therapeutic option in intractable patients. The objective of this review is to understand whether patients who develop Meige's syndrome are different from patients who manifest blepharospasm or oromandibular dystonia alone.


Assuntos
Síndrome de Meige/história , Síndrome de Meige/terapia , História do Século XIX , História do Século XX , História do Século XXI , Humanos , Síndrome de Meige/epidemiologia , Síndrome de Meige/fisiopatologia
7.
Neurol Sci ; 37(5): 769-75, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26838523

RESUMO

Bilateral hemifacial spasm and Meige syndrome can be easily confused due to their similar clinical manifestation. Here, we aimed to investigate the application of electrophysiological methods and magnetic resonance tomographic angiography (MRTA) in the differentiation between hemifacial spasm and Meige syndrome. 10 patients with bilateral hemifacial spasm and 9 patients with Meige syndrome received electrophysiological monitoring of nerves. There were two males and eight females with bilateral hemifacial spasm, aged 16-58 years with a course of 5-54 months. For the patients with Meige syndrome, there were three males and six females, aged 51-68 years with a course of 12-36 months. All patients received conventional MRTA of the brain blood vessels before decompression. We found that all patients with Meige syndrome showed synchronous contraction of bilateral orbicularis oculi muscles and (or) burst discharge from orbicularis oris muscles in surface electromyography (sEMG). However, those with hemifacial spasm presented with bilaterally asynchronous burst discharge. Electromyography for patients with Meige syndrome did not record abnormal muscle response (AMR), but recorded AMR for those with bilateral hemifacial spasm. The offending vessels were compressed in patients with hemifacial spasm in MRTA, while MRTA results were generally negative for those with Meige syndrome. Combining sEMG and AMR detection in EMG and MRTA, bilateral hemifacial spasm can be differentiated from Meige syndrome with a reduction of misdiagnosis rate.


Assuntos
Potencial Evocado Motor/fisiologia , Espasmo Hemifacial/diagnóstico por imagem , Espasmo Hemifacial/fisiopatologia , Angiografia por Ressonância Magnética , Síndrome de Meige/diagnóstico por imagem , Síndrome de Meige/fisiopatologia , Adolescente , Adulto , Idoso , Eletromiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
8.
Ophthalmic Plast Reconstr Surg ; 31(3): e65-6, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25951177

RESUMO

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.


Assuntos
Apraxias/tratamento farmacológico , Blefarospasmo/tratamento farmacológico , Inibidores da Captação de Dopamina/uso terapêutico , Síndrome de Meige/tratamento farmacológico , Metilfenidato/uso terapêutico , Administração Oral , Adulto , Apraxias/fisiopatologia , Blefarospasmo/fisiopatologia , Feminino , Humanos , Masculino , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade
9.
Am J Ophthalmol ; 156(1): 173-177.e2, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23541393

RESUMO

PURPOSE: To report the clinical success and incidence of adverse events of repetitive botulinum toxin treatment of 15 years or greater. DESIGN: Retrospective cohort study. METHODS: The study sample consisted of 37 patients from a clinical practice, 11 male and 26 female. Inclusion criteria consisted of patients treated a minimum of 15 consecutive years for facial dystonia. Seven patients had hemifacial spasm, 4 Meige syndrome, and 26 benign essential blepharospasm. Main outcome measures consisted of treatment efficacy and adverse events. RESULTS: Mean treatment duration was 19.4 years (SD 2.2) with an average of 62 (SD 22) treatments of 70.2 (SD 20.8) neurotoxin units. Mean duration of treatment efficacy was 127 days (SD 37) with a 5% physician-reported minor adverse event rate and no major adverse events over each patient's clinical course. Patients reported no major and 20% incidence of minor adverse events over the treatment course. CONCLUSION: Results suggest that long-term botulinum toxin treatment produces clinical success in the alleviation of facial dystonia symptoms. Treatment produced a low incidence of major adverse events and minor adverse events. Previous studies may under-report clinical success and over-report adverse events because of study design.


Assuntos
Blefarospasmo/tratamento farmacológico , Toxinas Botulínicas Tipo A/uso terapêutico , Toxinas Botulínicas/uso terapêutico , Espasmo Hemifacial/tratamento farmacológico , Síndrome de Meige/tratamento farmacológico , Fármacos Neuromusculares/uso terapêutico , Idoso , Blefarospasmo/fisiopatologia , Toxinas Botulínicas/efeitos adversos , Toxinas Botulínicas Tipo A/efeitos adversos , Feminino , Seguimentos , Espasmo Hemifacial/fisiopatologia , Humanos , Incidência , Injeções Subcutâneas , Masculino , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Fármacos Neuromusculares/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento
10.
Mov Disord ; 26(13): 2409-14, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21913223

RESUMO

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.


Assuntos
Toxinas Botulínicas/administração & dosagem , Eletromiografia/métodos , Injeções Intramusculares/métodos , Síndrome de Meige/tratamento farmacológico , Músculos do Pescoço/efeitos dos fármacos , Torcicolo/tratamento farmacológico , Adulto , Idoso , Feminino , Humanos , Masculino , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Músculos do Pescoço/fisiopatologia , Torcicolo/classificação , Torcicolo/fisiopatologia , Resultado do Tratamento
12.
Mov Disord ; 26(4): 691-8, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-21312284

RESUMO

Deep brain stimulation of the globus pallidus internus (GPi DBS) is effective in the treatment of primary segmental and generalized dystonia. Although limb, neck, or truncal dystonia are markedly improved, orofacial dystonia is ameliorated to a lesser extent. Nevertheless, several case reports and small cohort studies have described favorable short-term results of GPi DBS in patients with severe Meige syndrome. Here, we extend this preliminary experience by reporting long-term outcome in a multicenter case series, following 12 patients (6 women, 6 men) with Meige syndrome for up to 78 months after bilateral GPi DBS. We retrospectively assessed dystonia severity based on preoperative and postoperative video documentation. Mean age of patients at surgery was 64.5 ± 4.4 years, and mean disease duration 8.3 ± 4.4 years. Dystonia severity as assessed by the Burke-Fahn-Marsden Dystonia Rating Scale showed a mean improvement of 45% at short-term follow-up (4.4 ± 1.5 months; P < 0.001) and of 53% at long-term follow-up (38.8 ± 21.7 months; P < 0.001). Subscores for eyes were improved by 38% (P = 0.004) and 47% (P < 0.001), for mouth by 50% (P < 0.001) and 56% (P < 0.001), and for speech/swallowing by 44% (P = 0.058) and 64% (P = 0.004). Mean improvements were 25% (P = 0.006) and 38% (P < 0.001) on the Blepharospasm Movement Scale and 44% (P < 0.001) and 49% (P < 0.001) on the Abnormal Involuntary Movement Scale. This series, which is the first to demonstrate a long-term follow-up in a large number of patients, shows that GPi DBS is a safe and highly effective therapy for Meige syndrome. The benefit is preserved for up to 6 years.


Assuntos
Estimulação Encefálica Profunda/métodos , Globo Pálido/fisiologia , Síndrome de Meige/terapia , Idoso , Análise de Variância , Eletrodos , Feminino , Humanos , Estudos Longitudinais , Masculino , Síndrome de Meige/fisiopatologia , Pessoa de Meia-Idade , Atividade Motora/fisiologia , Índice de Gravidade de Doença , Resultado do Tratamento
13.
Parkinsonism Relat Disord ; 17(2): 123-5, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21147544

RESUMO

Primary Meige syndrome is an idiopathic movement disorder that manifests as craniofacial and often cervical dystonias. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has emerged as a powerful surgical option in the treatment of primary generalized or segmental dystonia. However, the experience with GPi-DBS in Meige syndrome is limited. We followed 5 patients with disabling Meige syndrome treated by bilateral GPi-DBS for 49 ± 43.7 (mean ± SD) months. All patients were assessed before surgery and at the last follow-up after surgery using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) which includes both the movement and disability scales. Bilateral GPi-DBS produced a sustained and long-lasting improvement in dystonia symptoms associated with Meige syndrome. At the last follow-up, the mean scores of BFMDRS movement and disability scales improved significantly by 84 ± 6.8% (range, 75-94%) and 89 ± 8.1% (range, 80-100%), respectively. Bilateral pallidal stimulation is a beneficial therapeutic option for long-term relief of the disabling dystonia symptoms in Meige syndrome.


Assuntos
Estimulação Encefálica Profunda/métodos , Globo Pálido/fisiologia , Síndrome de Meige/fisiopatologia , Síndrome de Meige/terapia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
14.
Neurol Neurochir Pol ; 45(6): 590-9, 2011.
Artigo em Polonês | MEDLINE | ID: mdl-22212990

RESUMO

Deep brain stimulation (DBS) is an established and accepted treatment modality of generalized dystonia. The stereotactic target to be approached with DBS leads is the internal segment of the globus pallidus (GPi). Bilateral GPi stimulation in patients suffering from primary generalized dystonia reduced dystonic movement not only in the trunk and limbs but also in the neck and face. These observations have led to the use of GPi stimulation in patients with severe torticollis and Meige syndrome refractory to pharmacological agents as well to botulinum toxin injections. An increasing number of reports indicate the effectiveness of GPi stimulation in the treatment of intractable focal and segmental dystonia. Moreover, DBS can be performed simultaneously on both sides during one operative session. This treatment modality is reversible and safer when compared to stereotactic ablative techniques. In future, DBS can become an alternative treatment for intractable focal and segmental dystonia.


Assuntos
Estimulação Encefálica Profunda/métodos , Síndrome de Meige/terapia , Torcicolo/terapia , Terapia por Estimulação Elétrica , Globo Pálido , Humanos , Síndrome de Meige/fisiopatologia , Procedimentos Neurocirúrgicos/métodos , Torcicolo/fisiopatologia
15.
Clin Otolaryngol ; 35(5): 390-6, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-21108749

RESUMO

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.


Assuntos
Síndrome de Meige/fisiopatologia , Síndrome de Meige/psicologia , Qualidade de Vida , Inquéritos e Questionários , Adulto , Idoso , Toxinas Botulínicas Tipo A/uso terapêutico , Estudos Transversais , Feminino , Humanos , Masculino , Síndrome de Meige/tratamento farmacológico , Pessoa de Meia-Idade , Observação , Estudos Prospectivos , Psicometria , Reprodutibilidade dos Testes , Sensibilidade e Especificidade
16.
Mov Disord ; 25(4): 405-6, 2010 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-20213832

RESUMO

The Moches were a pre-Columbian culture from Peru, who had a fine ceramic technique and used to represent diseases. One example is the potter presented here which represents a man with a probable Meige's syndrome and may be the first artistic representation of this disease.


Assuntos
Cultura , Etnicidade , Medicina nas Artes , Síndrome de Meige/história , Síndrome de Meige/fisiopatologia , Blefarospasmo/história , Blefarospasmo/fisiopatologia , Cerâmica/história , História Antiga , Humanos , Peru
17.
J Plast Reconstr Aesthet Surg ; 63(1): 59-64, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19010100

RESUMO

We have proposed a hypothetical mechanism to involuntarily sustain the effective eyelid retraction, which consists of not only voluntary but also reflexive contractions of the levator palpebrae superior muscle (LPSM). Voluntary contraction of fast-twitch fibres of the LPSM stretches the mechanoreceptors in Mueller's muscle to evoke trigeminal proprioception, which induces continuous reflexive contraction of slow-twitch fibres of the LPSM through the trigeminal proprioceptive nerve fibres innervating the mechanoreceptors in Mueller's muscle via the oculomotor neurons, as a tonic trigemino-oculomotor reflex. In the common skeletal mixed muscles, electrical stimulation of the proprioceptive nerve, which apparently connects the mechanoreceptors in muscle spindles to the motoneurons, induces the electromyographic response as the Hoffmann reflex. To verify the presence of the trigemino-oculomotor reflex, we confirmed whether intra-operative electrical simulation of the transverse trigeminal proprioceptive nerve on the proximal Mueller's muscle evokes an electromyographic response in the LPSM under general anaesthesia in 12 patients. An ipsilateral, phasic, short-latency response (latency: 2.8+/-0.3 ms) was induced in the ipsilateral LPSM in 10 of 12 subjects. As successful induction of the short-latency response in the ipsilateral LPSM corresponds to the Hoffmann reflex in the common skeletal mixed muscles, the present study is the first electromyographic verification of the presence of the monosynaptic trigemino-oculomotor reflex to induce reflexive contraction of the LPSM. The presence of the trigemino-oculomotor reflex may elucidate the unexplainable blepharoptosis due to surgery, trauma and tumour, all of which may damage the trigeminal proprioceptive nerve fibres to impair the trigemino-oculomotor reflex.


Assuntos
Eletromiografia , Pálpebras/fisiologia , Síndrome de Meige/fisiopatologia , Contração Muscular/fisiologia , Músculo Esquelético/fisiologia , Fibras Nervosas Mielinizadas/fisiologia , Nervo Trigêmeo/fisiologia , Piscadela/fisiologia , Estimulação Elétrica , Músculos Faciais/inervação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculos Oculomotores/inervação , Propriocepção/fisiologia , Reflexo/fisiologia
18.
Parkinsonism Relat Disord ; 15(7): 483-9, 2009 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-19457699

RESUMO

Frequently, blepharospasm is associated with involuntary movements of the platysma, lower face and masticatory muscles. Similarly, masticatory dystonia may occur in isolation or in combination with dystonia of other cranial and cervical muscles. The non-possessive and possessive forms of Meige and Brueghel syndromes have been variably and imprecisely ascribed to various anatomical variations of craniocervical dystonia. Herein, the origin of eponymic terms as applied to craniocervical dystonia is reviewed as support for proposed elimination of these eponyms from clinical usage. Although the term "segmental craniocervical dystonia" more accurately captures the combination of blepharospasm and dystonia of other head and neck muscles, delineation of craniocervical subphenotypes is essential for etiological/genetic and treatment studies. To conclude, the clinical features, epidemiology, pathophysiology and therapeutic management of segmental craniocervical dystonia are examined with a particular focus on "blepharospasm-plus" subphenotypes.


Assuntos
Síndrome de Meige , Antidiscinéticos/uso terapêutico , Toxinas Botulínicas/uso terapêutico , Distonia/complicações , Distonia/terapia , Músculos Faciais/efeitos dos fármacos , Músculos Faciais/fisiopatologia , História do Século XX , História do Século XXI , Humanos , Síndrome de Meige/história , Síndrome de Meige/patologia , Síndrome de Meige/fisiopatologia , Síndrome de Meige/terapia
19.
J Neurol ; 255(6): 881-4, 2008 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18338193

RESUMO

OBJECTIVE: Deep brain stimulation (DBS) has emerged as a useful therapeutic option for patients with insufficient benefit from conservative treatment. METHODS: Nine patients with chronic DBS who suffered from cervical dystonia (4), generalized dystonia (2), hemidystonia (1), paroxysmal dystonia (1) and Meige syndrome (1) were available for formal follow-up at three years postoperatively, and beyond up to 10 years. All patients had undergone pallidal stimulation except one patient with paroxysmal dystonia who underwent thalamic stimulation. RESULTS: Maintained improvement was seen in all patients with pallidal stimulation up to 10 years after surgery except in one patient who had a relative loss of benefit in dystonia ratings but continued to have improved disability scores. After nine years of chronic thalamic stimulation there was a mild loss of efficacy which was regained when the target was changed to the pallidum in the patient with paroxysmal dystonia. There were no major complications related to surgery or to chronic stimulation. Pacemakers had to be replaced within 1.5 to 2 years, in general. CONCLUSION: DBS maintains marked long-term symptomatic and functional improvement in the majority of patients with dystonia.


Assuntos
Encéfalo/fisiopatologia , Estimulação Encefálica Profunda/estatística & dados numéricos , Distúrbios Distônicos/terapia , Adulto , Encéfalo/cirurgia , Estimulação Encefálica Profunda/métodos , Estimulação Encefálica Profunda/normas , Distonia/fisiopatologia , Distonia/terapia , Distúrbios Distônicos/fisiopatologia , Feminino , Seguimentos , Globo Pálido/fisiologia , Humanos , Masculino , Síndrome de Meige/fisiopatologia , Síndrome de Meige/terapia , Pessoa de Meia-Idade , Vias Neurais/fisiologia , Marca-Passo Artificial , Tálamo/fisiologia , Tempo , Torcicolo/fisiopatologia , Torcicolo/terapia , Resultado do Tratamento
20.
Acta Neurol Scand ; 118(3): 198-202, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18336624

RESUMO

BACKGROUND: Pallidal deep brain stimulation (DBS) of globus pallidus internus (Gpi) has emerged as an effective treatment for dystonia. The experience is however limited concerning focal dystonias and to date only a few cases of pallidal DBS in the treatment of Meige syndrome have been published. METHODS/RESULTS: We here present a patient with Meige syndrome in whom unilateral pallidal DBS failed to improve the axial symptoms, but bilateral stimulation resulted in a major improvement. The Burke-Fahn-Marsden score (BFM) improved by 71.5% and the patient's blepharospasm was abolished. CONCLUSIONS: The results suggest bilateral pallidal DBS may be an effective treatment for Meige syndrome.


Assuntos
Estimulação Encefálica Profunda , Síndrome de Meige/terapia , Adulto , Idade de Início , Blefarospasmo/etiologia , Distonia/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Síndrome de Meige/complicações , Síndrome de Meige/fisiopatologia
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