title: Management of the behavioural manifestations of Hunter syndrome.
abstract: This article reviews the behavioural manifestations of, and the strategies for managing, Hunter syndrome (mucopolysaccharidosis (MPS) type II), a rare X-linked lysosomal storage disorder caused by a deficiency of the enzyme iduronate-2-sulphatase. Hunter syndrome is generally considered to have two manifestations: an attenuated form and a severe form; in the latter, the person has pronounced cognitive decline. Infants with either phenotype usually appear normal at birth, but may show some somatic signs. Children with the severe phenotype show developmental delay and changes in behaviour patterns at about 18 months to 4 years of age. To varying degrees, patients with the severe form manifest behavioural disorders such as hyperactivity, aggression, impulsivity, anxiety and sleep disturbances. Medications, such as antipsychotics, benzodiazepines and anticonvulsants, have been tried with varying degrees of success. Behavioural management strategies may be a worthwhile approach, although published data are lacking. For sleep disturbances, behavioural modification plus melatonin or benzodiazepine may be effective treatments.
authors.affiliation: Clinical Nurse Specialist, Manchester Children's Hospital.
authors.forename: Jane
authors.initials: J
authors.lastname: Roberts
authors.affiliation: Clinical Nurse Specialist, Birmingham Children's Hospital.
authors.forename: Catherine
authors.initials: C
authors.lastname: Stewart
authors.affiliation: Lead Paediatric Neuropsychologist, Birmingham Children's hospital.
authors.forename: Shauna
authors.initials: S
authors.lastname: Kearney
mesh_terms: D014150:Antipsychotic Agents / Q000627:therapeutic use; D032763:Behavior Control / Q000379:methods*; D001569:Benzodiazepines / Q000627:therapeutic use; D002648:Child; D002653:Child Behavior Disorders / Q000209:etiology / Q000628:therapy*; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D016532:Mucopolysaccharidosis II / Q000150:complications*
chemical_list: D014150:Antipsychotic Agents; D001569:Benzodiazepines
keywords: Behavioural problems; Hunter syndrome; Lysosomal storage disorder; Mucopolysaccharidosis (MPS) type II