Dystonic Respiratory Dysregulation and Other Breathing Disorders Associated with Dystonia (P01.223)

Abstract

Objective: To characterize respiratory complaints in dystonia. Background Respiratory difficulties are sometimes reported by patients with cranial-cervical dystonia (Mehanna R, Jankovic J. Respiratory problems in neurologic movement disorders. Parkinsonism Relat Disord. 2010;16: 628-38). This dystonia-related respiratory disorder, however, has not been well characterized. Design/Methods: We review 13 dystonic patients with various respiratory complaints and describe their symptoms and response to botulinum toxin injections. Results: The male to female ratio was 4/9 and the mean age 56.84. Cranial-cervical dystonia was the most frequent diagnosis affecting 9 (69%) patients. The cause of dyspnea was an upper airway dystonic obstruction in 9 (69%) patients, diaphragmatic dysfunction in one (8%) and a combination of both in the remaining 3 (23%) patients. Emergent tracheostomy was required in 2 (15%) patients. Among the 5 patients who underwent laryngoscopy, one had an adductor spasmodic dysphonia and 4 had normal vocal cord motion. Pulmonary function tests were performed in 5 patients and were normal. Videofluoroscopy was performed in 4 patients, showed decreased diaphragmatic movement in 2 and was normal in the 2 others. Among the 8 patient who received botulinum toxin injections and were not lost to follow up, 6 (75%) reported improvement in their respiratory distress and 2 (25%) reported no improvement. Conclusions: In some patients with dystonia, gasping, stridor, interrupted flow of speech, paradoxical breathing, dyspnea on exertion, and other respiratory symptoms suggest involvement of the upper airways, diaphragm, or both as a result of “dystonic respiratory dysregulation”. Botulinum toxin injections may be at least partially beneficial in some patients. This series of patients draws attention to respiratory distress as a potentially serious, even life-threatening, complication of dystonia. Disclosure: Dr. Mehanna has nothing to disclose. Dr. Jankovic has received personal compensation for activities with Allergan, Inc., Chelsea Therapeutics, Serono Inc., Merz Pharma, Lundbeck Research USA, Inc, Teva Neuroscience as a consultant. Dr. Jankovic has received personal compensation in an editorial capacity for Medlink: Neurology in Clinical Practice. Dr. Jankovic has received research support from Allergan, Inc, Allon Therapeutics, Ceregene, Inc., Chelsea Therapeutics; Diana Helis Henry Medical Research Foundation, Serono Inc., Huntington9s Disease Society of America, Huntington Study Group, Impax Pharmaceuticals, Ipsen Limited, Lundbeck Research USA, Inc.