Open AccessAdductor laryngeal breathing dystonia in NBIA treated with botulinum toxin-A
Author(s) -
Vinod Rai,
Vinay Goyal,
Gaurav Shukla,
Girija Prasad Rath,
Madhuri Behari
Publication year - 2013
Publication title -
annals of indian academy of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.427
H-Index - 31
eISSN - 1998-3549
pISSN - 0972-2327
DOI - 10.4103/0972-2327.116919
Subject(s) - medicine , stridor , dystonia , botulinum toxin , breathing , anesthesia , larynx , laryngoscopy , surgery , airway , intubation , psychiatry
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.