Adductor laryngeal breathing dystonia in NBIA treated with botulinum toxin-A

作者:Rai Vinod; Goyal Vinay*; Shukla Garima; Rath Girija; Behari Madhuri
来源:Annals of Indian Academy of Neurology, 2013, 16(3): 409-410.
DOI:10.4103/0972-2327.116919

摘要

We report a rare case of neurodegeneration with brain iron accumulation (NBIA) presented with episodic inspiratory stridor. A 10-year-old boy presented with 3-year history of gradually progressive spastic gait and generalized dystonia (involving all four limbs, neck, jaw, and speech). MRI brain showed "Eye of Tiger" sign. He recently developed severe inspiratory stridor associated with almost gasping respiration. Direct video laryngoscopy showed paradoxical vocal cord closure during inspiration. He was treated with EMG-guided botulinum toxin-A injection given into bilateral thyroarytenoid muscles, resulting in dramatic response with complete disappearance of the stridor within a week. The effect lasted 18 months.

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