title: [Efficacy of botulinum toxin type A against cervical dystonia and muscular hypertonia in persons with severe motor and intellectual disabilities with respiratory problems and/or dysphagia].
abstract: We administered intramuscular injections of botulinum toxin type A (BTX-A) in 11 persons with cervical dystonia (CD) and muscular hypertonia (MH). All patients had severe motor and intellectual disabilities (SMID). Furthermore, in 10 patients, SMID was accompanied by respiratory problems and/or dysphagia. Three patients received night nasal intermittent positive pressure ventilation and 3 had undergone tracheotomy; 5 patients had upper respiratory problems. Because of these complications, BTX-A dose was gradually increased in those patients until the desired effect was obtained (mean last dose, 6.8 u/kg/dose). All patients were clinically assessed with the Tsui scale before treatment with BTX-A. At 1, 2, 4, and 8 weeks after BTX-A injections, responses to the injections were assessed with the Tsui scale repetitively in all patients. Significant or mild improvements in the Tsui scale scores were observed in 8 patients without any severe adverse effects. In addition, some improvements in respiration and body weight gain were observed. We observed a reduction in the number of oral medications in 10 cases. Administration of BTX-A for the treatment of SMID has numerous benefits, not all of which can be explained by Tsui scale scores. BTX-A is safe and has potential for use in the treatment of CD and MH with respiratory problems and/or dysphagia.
authors.affiliation: Department of Pediatrics, Tokyo Metropolitan Tobu Medical Center for Persons with Developmental/Multiple Disabilities, Tokyo. yokoyama@nicoco.jp
authors.forename: Mina
authors.initials: M
authors.lastname: Yokoyama
authors.forename: Manami
authors.initials: M
authors.lastname: Honda
authors.forename: Yumi
authors.initials: Y
authors.lastname: Okoshi
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Masuyama
authors.forename: Yuji
authors.initials: Y
authors.lastname: Iwasaki
authors.forename: Masataka
authors.initials: M
authors.lastname: Arima
mesh_terms: D000293:Adolescent; D000328:Adult; D019274:Botulinum Toxins, Type A / Q000008:administration & dosage / Q000627:therapeutic use*; D002675:Child, Preschool; D003680:Deglutition Disorders / Q000150:complications; D005260:Female; D006801:Humans; D007273:Injections, Intramuscular; D008607:Intellectual Disability / Q000150:complications; D008297:Male; D008875:Middle Aged; D009122:Muscle Hypertonia / Q000188:drug therapy; D009465:Neuromuscular Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D014103:Torticollis / Q000150:complications / Q000188:drug therapy*
chemical_list: D009465:Neuromuscular Agents; D019274:Botulinum Toxins, Type A