Meige syndrome secondary to basal ganglia injury: A potential cause of acute respiratory distress

Background: Meige syndrome is a movement disorder that includes blepharospa sm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress a s a feature of such dystonias occurs infrequently. A review of the literatu re 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 b lepharospasm combined with oromandibular and cervical dystonias. Neuroimagi ng demonstrated bilateral damage localized to the globus pallidus. Years la ter, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms. Results: Increa sing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheot omy. A partial therapeutic benefit of tetrabenazine was demonstrated. Concl usion: This case highlights two interesting aspects of Meige's syndrome: (1 ) Focal bilateral basal ganglia lesions appear to be responsible for this p atient's movement disorder which is consistent with relative overactivity o f the direct pathway from striatum to globus pallidus internal and substant ia nigra pars reticularis; (2) Respiratory involvement in a primarily crani ofacial dystonia to the point of acute airway compromise.