The Neurobehavioral Phenotype in Mucopolysaccharidosis Type IIIB: an Exploratory Study.

ABSTRACT:

Objectives

Our goal was to describe the neurobehavioral phenotype in mucopolysaccharidosis Type IIIB (MPS IIIB). Parents report that behavioral abnormalities are a major problem in MPS III posing serious challenges to parenting and quality-of-life for both patient and parent. Our previous research on MPS IIIA identified autistic symptoms, and a Klüver-Bucy-type syndrome as indicated by reduced startle and loss of fear associated with amygdala atrophy. We hypothesized that MPS IIIB would manifest similar attributes when assessed with the same neurobehavioral protocol.

Methods

Ten patients with MPS IIIB were compared with 9 MPS IIIA patients, all older than 6. 8 younger children with Hurler syndrome (1H) were chosen as a comparison group for the Risk Room procedure; MPS IH doe

SUBMITTER: Shapiro E 

PROVIDER: S-EPMC4762067 | biostudies-literature | 2016 Mar

REPOSITORIES: biostudies-literature