title: Nocturnal hypoventilation in children with nonprogressive neuromuscular disease.
abstract: Eight patients between 4 and 24 years of age with nonprogressive neuromuscular disease sought medical attention because of severe nocturnal hypoventilation. There were two types of findings: subacute with progressive early morning headaches and daytime drowsiness and acute with ventilatory failure and cor pulmonale. Seven patients were ambulant. Seven were successfully treated with either a cuirass negative pressure ventilator or a positive pressure ventilator via a tracheostomy. The ventilatory assistance was only used at night and resulted in rapid resolution of early morning symptoms and a return to full daytime activity. One patient died as a result of an intercurrent respiratory infection before respiratory support could be given. It is important to be aware of this potentially life-threatening complication in patients with an otherwise good prognosis and of the benefit to be derived from active treatment.
authors.affiliation: Department of Paediatrics, Royal Postgraduate Medical School, Hammersmith Hospital, London, England.
authors.forename: J Z
authors.initials: JZ
authors.lastname: Heckmatt
authors.forename: L
authors.initials: L
authors.lastname: Loh
authors.forename: V
authors.initials: V
authors.lastname: Dubowitz
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D000860:Hypoxia / Q000209:etiology; D008297:Male; D009468:Neuromuscular Diseases / Q000150:complications*; D010092:Oximetry; D012121:Respiration, Artificial; D012891:Sleep Apnea Syndromes / Q000209:etiology* / Q000601:surgery / Q000628:therapy; D014139:Tracheostomy; D014797:Vital Capacity