Early development and regression in Rett Syndrome

This study utilized developmental profiling to examine symptoms in 14 girls with genetically confirmed Rett syndrome and whose families were participating in the Australian Rett syndrome or InterRett database. Regression was mostly characterized by loss of hand and/or communication skills (13/14) ex...

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Main Authors: Lee, J.Y.L., Leonard, H., Piek, Jan Patricia, Downs, Jennepher
Format: Journal Article
Published: Wiley-Blackwell Publishing, Inc. 2013
Subjects:
Online Access:http://hdl.handle.net/20.500.11937/47088
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author Lee, J.Y.L.
Leonard, H.
Piek, Jan Patricia
Downs, Jennepher
author_facet Lee, J.Y.L.
Leonard, H.
Piek, Jan Patricia
Downs, Jennepher
author_sort Lee, J.Y.L.
building Curtin Institutional Repository
collection Online Access
description This study utilized developmental profiling to examine symptoms in 14 girls with genetically confirmed Rett syndrome and whose families were participating in the Australian Rett syndrome or InterRett database. Regression was mostly characterized by loss of hand and/or communication skills (13/14) except one girl demonstrated slowing of skill development. Social withdrawal and inconsolable crying often developed simultaneously (9/14), with social withdrawal for shorter duration than inconsolable crying. Previously acquired gross motor skills declined in just over half of the sample (8/14), mostly observed as a loss of balance. Early abnormalities such as vomiting and strabismus were also seen. Our findings provide additional insight into the early clinical profile of Rett syndrome.
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publishDate 2013
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spelling curtin-20.500.11937-470882019-02-19T05:35:12Z Early development and regression in Rett Syndrome Lee, J.Y.L. Leonard, H. Piek, Jan Patricia Downs, Jennepher Rett syndrome development autistic symptoms MECP2 regression This study utilized developmental profiling to examine symptoms in 14 girls with genetically confirmed Rett syndrome and whose families were participating in the Australian Rett syndrome or InterRett database. Regression was mostly characterized by loss of hand and/or communication skills (13/14) except one girl demonstrated slowing of skill development. Social withdrawal and inconsolable crying often developed simultaneously (9/14), with social withdrawal for shorter duration than inconsolable crying. Previously acquired gross motor skills declined in just over half of the sample (8/14), mostly observed as a loss of balance. Early abnormalities such as vomiting and strabismus were also seen. Our findings provide additional insight into the early clinical profile of Rett syndrome. 2013 Journal Article http://hdl.handle.net/20.500.11937/47088 10.1111/cge.12110 Wiley-Blackwell Publishing, Inc. fulltext
spellingShingle Rett syndrome
development
autistic symptoms
MECP2
regression
Lee, J.Y.L.
Leonard, H.
Piek, Jan Patricia
Downs, Jennepher
Early development and regression in Rett Syndrome
title Early development and regression in Rett Syndrome
title_full Early development and regression in Rett Syndrome
title_fullStr Early development and regression in Rett Syndrome
title_full_unstemmed Early development and regression in Rett Syndrome
title_short Early development and regression in Rett Syndrome
title_sort early development and regression in rett syndrome
topic Rett syndrome
development
autistic symptoms
MECP2
regression
url http://hdl.handle.net/20.500.11937/47088