1/2. meige syndrome secondary to basal ganglia injury: a potential cause of acute respiratory distress.BACKGROUND: meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on meige syndrome and the relationship between dystonias and respiratory compromise is presented. methods: A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms. RESULTS: Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated. CONCLUSION: This case highlights two interesting aspects of Meige's syndrome: (1) Focal bilateral basal ganglia lesions appear to be responsible for this patient's movement disorder which is consistent with relative overactivity of the direct pathway from striatum to globus pallidus internal and substantia nigra pars reticularis; (2) Respiratory involvement in a primarily craniofacial dystonia to the point of acute airway compromise.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
2/2. Staged deep brain stimulation for refractory craniofacial dystonia with blepharospasm: case report and physiology.OBJECTIVE AND IMPORTANCE: We report the intraoperative results, subsequent course, and 1-year follow-up evaluation of a patient with medication-refractory craniofacial dystonia for whom we planned bilateral globus pallidus internus (GPi) deep brain stimulation (DBS) implantation but delayed the left GPi DBS implantation because of robust intraoperative effects of right GPi DBS. CLINICAL PRESENTATION: A 47-year-old patient had a 5-year history of progressively severe, bilateral craniofacial dystonia with blepharospasm (Meige's syndrome) that was refractory to medications and to botulinum toxin (A and B) injections. blepharospasm interfered with his ability to perform his duties as a Special Forces soldier and ended his military career. INTERVENTION: Under stereotactic guidance (magnetic resonance imaging and computed tomographic image fusion, Cosman-Roberts-Wells frame, and University of florida surgical navigation software) and with detailed microelectrode mapping (four microelectrode passes), a DBS electrode was implanted in the right posteroventral GPi. Microelectrode recordings were taken to document electrophysiological activity of neurons in the region, and intraoperative macrostimulation was performed. The patient was followed up for 6 months with right unilateral GPi DBS, and later a left GPi DBS electrode was placed. CONCLUSION: Although DBS for primary generalized dystonia is commonly performed by simultaneously implanting bilateral GPi electrodes, it may be reasonable in cases of refractory blepharospasm and/or craniofacial dystonia to use a staged procedure for implantation in selected patients. Additionally, the physiology, especially that encountered in the striatum, may help to elucidate the pathophysiological basis for refractory blepharospasm and Meige's syndrome. More cases will be needed to determine the significance of the results reported in this article.- - - - - - - - - - ranking = 0.5keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |