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Longitudinal neurocognitive outcome in an adolescent with Hurler-Scheie syndrome

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

Published 15 September 2006 Volume 2006:2(3) Pages 381—386

T David Elkin1, Gail Megason2, Anita Robinson2, Hans-Georg O Bock3, Gregory Schrimsher1, Joseph Muenzer2


The University of Mississippi Medical Center, Departments of 1Psychiatry, 2Pediatrics, 3Preventive Medicine, The University of North Carolina at Chapel Hill, Jackson, MS, USA


Abstract: 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.


Keywords: Hurler-Scheie syndrome, adolescent, neurocognitive function

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