Skip to main content
Article thumbnail
Location of Repository

Longitudinal neurocognitive outcome in an adolescent with Hurler-Scheie syndrome

By T David Elkin, Gail Megason, Anita Robinson, Hans-Georg O Bock, Gregory Schrimsher and Joseph Muenzer


An adolescent with Hurler-Scheie syndrome is reported. This now 15 year-old-young woman was initially diagnosed at age 4. She was assessed for neurocognitive functioning at ages 5, 13, and 15 years. Results show a significant decline in intellectual functioning from the superior range to the average range from age 5 to age 13, and then no change from age 13 to age 15. The relationship between Hurler-Scheie syndrome, premorbid intellectual functioning, and cognitive–behavioral interventions are discussed in light of the longitudinal neurocognitive effects of this disease

Topics: Case Reports
Publisher: Dove Medical Press
OAI identifier:
Provided by: PubMed Central

Suggested articles


  1. (1992). Assessment of children.
  2. (1998). Follow-up of nine patients with Hurler syndrome after bone marrow transplantation.
  3. (1995). Neuropsychological outcomes of several storage diseases with and without bone marrow transplantation.
  4. (2002). Outcome of second hematopoietic cell transplantation in Hurler syndrome. Bone Marrow Transplant,
  5. (2004). Research on outcomes of pediatric traumatic brain injury: current advances and future directions. Dev Neuropsychol,
  6. (1999). The mucopolysaccharidoses.
  7. (1995). The mucopolysaccharidoses. In

To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.