The neurobehavioral phenotype in mucopolysaccharidosis Type IIIB: An exploratory study

  title={The neurobehavioral phenotype in mucopolysaccharidosis Type IIIB: An exploratory study},
  author={Elsa G. Shapiro and Kelly King and Alia Ahmed and Kyle R Rudser and Robin K Rumsey and Brianna Yund and Kathleen A. Delaney and Igor Nestrasil and Chester B. Whitley and Michael Potegal},
  booktitle={Molecular genetics and metabolism reports},
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… CONTINUE READING
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  • E. Shapiro, I. Nestrasil
  • Ahmed, et al., Quantifying behaviors of children…
  • 2015
Highly Influential
12 Excerpts

Manual for the Kaufman Assessment Battery for Children, second ed

  • A. S. Kaufman
  • American Guidance Service, Circle Pines MN,
  • 2004
Highly Influential
4 Excerpts


  • E. Shapiro, I. Nestrasil
  • Delaney, et al., A prospective natural [2…
  • 2016

Wraith , Management of mucopolysaccharidosis type III

  • M. A. Cleary, E. J.
  • Arch . Dis . Child .
  • 2016

Acquired autistic behaviors in children with MPSIIIA ,

  • K. Rudser R. Rumsey, K. Delaney
  • J . Pediatr .
  • 2014


  • K. Delaney, K. Rudser
  • Yund, et al., Methods in neuropsychological…
  • 2013

Methods in neuropsychological assessment in children with neurodegenerative disease : San fi lippo syndrome

  • K. Rudser K. Delaney, B. Yund
  • J . Inherit . Metab . Dis . Rep .
  • 2013

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