REVIEW
Introdução: A Síndrome de Meige é uma distonia craniana que acomete adultos e associa distonia orofacial com blefaroespasmo. Toda a fisiopatologia ainda não foi totalmente elucidada, mas já se sabe que cursa com excitabilidade anormal do córtex sensório-motor e vias interneuronais do tronco encefálico associadas a fatores ambientais e predisposição genética. Existem diferentes tipos de tratamentos possíveis que incluem tanto vias farmacológicas quanto invasivas como propostas terapêuticas. A Estimulação Cerebral Profunda do Globus Pallidus Internus (GPi-DBS) surgiu como uma forma estabelecida de neuromodulação invasiva para a Síndrome de Meige, demonstrando ter um efeito significativo na melhora dos sintomas. Relato de Caso: um caso de Síndrome de Meige previamente tratado com procedimentos convencionais para a condição, como tratamento farmacológico, blefaropexia, toxina botulínica, todos sem sucesso. Após múltiplas falhas, o paciente foi submetido a um GPi-DBS, que evoluiu com melhora de 80% dos sintomas, melhorando também sua qualidade de vida. Além disso, também foi realizada uma revisão da literatura de outros casos de GPi-DBS, que, em quase 100% dos casos, foi demonstrada uma melhora significativa do quadro. Conclusão: o GPi-DBS tem sido utilizado como uma importante ferramenta no tratamento da Síndrome de Meige, apresentando alta eficácia na melhora dos sintomas e na qualidade de vida dos pacientes.
Background: Meige’s Syndrome is cranial dystonia in adults that associates orofacial dystonia with blepharospasm. The whole pathophysiology has not been fully elucidated yet but it is already known that it courses with abnormal excitability of the sensorimotor cortex and brainstem interneuronal pathways associated with environmental factors and genetic predisposition. There are different types of possible therapies that include both pharmacological and invasive procedures as therapeutic proposals. The Deep Brain Stimulation of Globus Pallidus Internus (GPi-DBS) emerged as an established form of invasive neuromodulation for Meige’s Syndrome which has been demonstrated to have a remarkable effect on symptoms improvement. Case presentation: a case of Meige’s Syndrome previously treated with conventional procedures for the condition, such as pharmacological treatment, blepharopexy, botulinum toxin, all without success, was reported. After multiple failures, the patient was submitted to a GPi-DBS, which evolved with an improvement of 80% of the symptoms, improving also the patient’s quality of life. Also, a literature review ofother cases of GPi-DBS, which almost 100% demonstrated a significant improvement of the condition was performed. Conclusion: GPi-DBS has been used as an important tool to treat Meige’s Syndrome, showing high efficacy in the symptoms improvement and quality of the patients’ life.
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1 Medical student, Medical School of ABC, São Paulo, SP, Brazil.
2 Medical student, Department of Neurosurgery, Santa Paula Hospital, São Paulo, SP, Brazil.
3 Medical student, Faculty of Medical Sciences of Santa Casa de São Paulo, São Paulo, SP, Brazil.
4 Medical student, Pontifical Catholic University of Sorocaba, São Paulo, SP, Brazil.
5 MD, Neurosurgeon, Professor, Department of Medicine, Faculty of Medical Sciences of Santa Casa de São Paulo, São Paulo, SP, Brazil.
6 MD, PhD, Neurosurgeon, Department of Functional Neurosurgery, Santa Casa de São Paulo Hospital, São Paulo, SP, Brazil.
7 MD, PhD, Neurosurgeon, Santa Paula Hospital, São Paulo, SP, Brazil.
8 MD, PhD, Neurosurgeon, Department of Neurosurgery, Santa Paula Hospital, São Paulo; Professor, Faculty of Medical Sciences of Santa Casa de São Paulo; Professor, Medical School of ABC,
São Paulo, SP, Brazil
Received Jan 12, 2022
Accepted Mar 23, 2022
