Abstract

Background: Meige syndrome is a rare neurological condition characterized by oromandibular dystonia and blepharospasm. Treatment modalities includes medications like anticholinergics, dopamine antagonist, GABA agonist, physical or occupational therapy, baclofen injections, and surgical interventions like ablation or deep brain stimulation of Globus pallidus internus nuclei [GPI]. Case Details: We present a case of 31 year old male patient with abnormal involuntary movement of head, neck, left shoulder and arm since three years, diagnosed as “Meige syndrome”. Patient was planned for right GPI radiofrequency ablation [RFA]. Thorough preoperative assessment was done. Significant findings were severe head and neck dystonia, and anticipated difficult airway. Surgery was planned under monitored anesthesia care with titrated sedation to allow for intraoperative neurologic testing. On the day of surgery, patient had severe cervical dystonia, not subsiding even after sedation with dexmedetomidine, requiring change of anaesthesia plan to general anaesthesia with secured airway as an airway intervention would have been difficult after application of the head frame. Standard balanced anaesthesia with nasal endotracheal tube was administered.BIS was used to monitor depth of anaesthesia. Surgeon required intraoperative testing during RFA of right GPI. About 20 minutes prior to testing, anaesthetic agents were stopped and BIS around 90 was documented. Patient cooperated well for neurological testing during localization and RFA of right GPI. After RFA, general anesthesia was continued. Procedure was uneventful, patient was extubated and shifted to ward after observation in recovery room and discharged on postoperative day three. Discussion and Clinical relevance: RFA of GPI is a modality of treatment for patient with Meige syndrome. Anaesthesia management may be challenging due to exacerbation of the abnormal involuntary movements during surgery. Anesthesia may be in form of monitored anesthesia care with sedation or general anesthesia with intra-operative testing during lesioning. Anesthesia drugs need to be titrated to facilitate awake testing during radio-frequency ablation procedure. Conclusion: Exacerbation of dystonia may necessitate change of anesthesia plan to general anesthesia with awake testing during lesioning. Patient must be counselled in preoperative period to cooperate for testing. Team work, planning and patient counselling for cooperation during procedure, aids in safe conduct of procedure.

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