CASE REPORT |
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Year : 2013 | Volume
: 16
| Issue : 3 | Page : 409-410 |
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Adductor laryngeal breathing dystonia in NBIA treated with botulinum toxin-A
Vinod Rai1, Vinay Goyal1, Garima Shukla1, Girija Rath2, Madhuri Behari1
1 Department of Neurology, All India Institute of Medical Sciences, New Delhi, India 2 Department of Neuroanaesthesia, All India Institute of Medical Sciences, New Delhi, India
Correspondence Address:
Vinay Goyal Department of Neurology, All India Institute of Medical Sciences, New Delhi India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0972-2327.116919
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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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