title: Irritability in Krabbe's disease: dramatic response to low-dose morphine.
abstract: Krabbe's disease is characterized by normal neonatal development with subsequent regression and profound, medically intractable irritability. Two female infants presented at 5 months of age with increasing irritability, abnormal motor control, and developmental regression. Investigations confirmed the diagnosis of Krabbe's disease. Maximal treatment of gastroesophageal reflux and nitrazepam 0.1 mg/kg by mouth three times daily were unsuccessful in controlling irritability. Morphine was initiated and titrated to 0.06 mg/kg by mouth every 6 hours in Patient 1 and 0.1 mg/kg by mouth every 8 hours in Patient 2, resulting in remarkably successful control of irritability. The diagnosis of Krabbe's disease is devastating for families and is compounded by the marked irritability. Management is difficult, but in these two infants, irritability was successfully controlled with low-dose morphine.
authors.affiliation: Department of Pediatrics, Division of Neurology, Dalhousie University, IWK Health Centre, Halifax, Nova Scotia, Canada.
authors.forename: W A
authors.initials: WA
authors.lastname: Stewart
authors.forename: K E
authors.initials: KE
authors.lastname: Gordon
authors.forename: P R
authors.initials: PR
authors.lastname: Camfield
authors.forename: E P
authors.initials: EP
authors.lastname: Wood
authors.forename: J M
authors.initials: JM
authors.lastname: Dooley
mesh_terms: D000701:Analgesics, Opioid / Q000008:administration & dosage / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D017809:Fatal Outcome; D005260:Female; D005764:Gastroesophageal Reflux / Q000150:complications*; D006801:Humans; D007223:Infant; D007508:Irritable Mood / Q000187:drug effects*; D007965:Leukodystrophy, Globoid Cell / Q000150:complications / Q000188:drug therapy*; D009020:Morphine / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D000701:Analgesics, Opioid; D009020:Morphine