Literature DB >> 22724898

Early behavioural manifestation of Smith-Magenis syndrome (del 17p11.2) in a 4-month-old boy.

Christa Einspieler1, Hiroyo Hirota, Mariko Yuge, Sunao Dejima, Peter B Marschik.   

Abstract

OBJECTIVE: There is little systematic data on early neurodevelopmental functioning of infants with Smith-Magenis syndrome, since early diagnosis is rare.
METHODS: A boy with cytogenetically confirmed Smith-Magenis syndrome was videotaped at 4 months and 1 week of age. His posture and spontaneous movements were analysed without knowing the diagnosis.
RESULTS: The motor repertoire appeared significantly reduced; fidgety general movements, which are typical of that age, were missing. Posture was abnormal and overall movements were jerky and monotonous. The findings indicate a severe motor impairment by no more than 4 months of age.
CONCLUSION: It was concluded that an absence of fidgety movements that goes along with subtle dysmorphic features indicates an increased risk of maldevelopment and justifies the need to refer for genetic evaluation with the potential of facilitating earlier diagnosis.

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Year:  2012        PMID: 22724898     DOI: 10.3109/17518423.2011.654281

Source DB:  PubMed          Journal:  Dev Neurorehabil        ISSN: 1751-8423            Impact factor:   2.308


  11 in total

1.  Smith-Magneis syndrome: behavioural phenotype mimics ADHD.

Authors:  Sundar Gnanavel
Journal:  BMJ Case Rep       Date:  2014-01-06

2.  Developmental profile of speech-language and communicative functions in an individual with the preserved speech variant of Rett syndrome.

Authors:  Peter B Marschik; Ralf Vollmann; Katrin D Bartl-Pokorny; Vanessa A Green; Larah van der Meer; Thomas Wolin; Christa Einspieler
Journal:  Dev Neurorehabil       Date:  2013-07-19       Impact factor: 2.308

3.  The motor repertoire in 3- to 5-month old infants with Down syndrome.

Authors:  Dafne Herrero; Christa Einspieler; Carolina Y Panvequio Aizawa; Akmer Mutlu; Hong Yang; Alice Nogolová; Jasmin Pansy; Karin Nielsen-Saines; Peter B Marschik
Journal:  Res Dev Disabil       Date:  2017-06-03

Review 4.  A Novel Way to Measure and Predict Development: A Heuristic Approach to Facilitate the Early Detection of Neurodevelopmental Disorders.

Authors:  Peter B Marschik; Florian B Pokorny; Robert Peharz; Dajie Zhang; Jonathan O'Muircheartaigh; Herbert Roeyers; Sven Bölte; Alicia J Spittle; Berndt Urlesberger; Björn Schuller; Luise Poustka; Sally Ozonoff; Franz Pernkopf; Thomas Pock; Kristiina Tammimies; Christian Enzinger; Magdalena Krieber; Iris Tomantschger; Katrin D Bartl-Pokorny; Jeff Sigafoos; Laura Roche; Gianluca Esposito; Markus Gugatschka; Karin Nielsen-Saines; Christa Einspieler; Walter E Kaufmann
Journal:  Curr Neurol Neurosci Rep       Date:  2017-05       Impact factor: 5.081

5.  Cerebral Palsy: Early Markers of Clinical Phenotype and Functional Outcome.

Authors:  Christa Einspieler; Arend F Bos; Magdalena Krieber-Tomantschger; Elsa Alvarado; Vanessa M Barbosa; Natascia Bertoncelli; Marlette Burger; Olena Chorna; Sabrina Del Secco; Raye-Ann DeRegnier; Britta Hüning; Jooyeon Ko; Laura Lucaccioni; Tomoki Maeda; Viviana Marchi; Erika Martín; Catherine Morgan; Akmer Mutlu; Alice Nogolová; Jasmin Pansy; Colleen Peyton; Florian B Pokorny; Lucia R Prinsloo; Eileen Ricci; Lokesh Saini; Anna Scheuchenegger; Cinthia R D Silva; Marina Soloveichick; Alicia J Spittle; Moreno Toldo; Fabiana Utsch; Jeanetta van Zyl; Carlos Viñals; Jun Wang; Hong Yang; Bilge N Yardımcı-Lokmanoğlu; Giovanni Cioni; Fabrizio Ferrari; Andrea Guzzetta; Peter B Marschik
Journal:  J Clin Med       Date:  2019-10-04       Impact factor: 4.241

6.  Monozygotic twins with Rett syndrome: Phenotyping the first two years of life.

Authors:  Christa Einspieler; Peter B Marschik; Wanderley Domingues; Victor B Talisa; Katrin D Bartl-Pokorny; Thomas Wolin; Jeff Sigafoos
Journal:  J Dev Phys Disabil       Date:  2014-04

7.  General movements in genetic disorders: A first look into Cornelia de Lange syndrome.

Authors:  Peter B Marschik; Marina Soloveichick; Christian Windpassinger; Christa Einspieler
Journal:  Dev Neurorehabil       Date:  2013-12-04       Impact factor: 2.308

8.  Behavioral disturbance and treatment strategies in Smith-Magenis syndrome.

Authors:  Alice Poisson; Alain Nicolas; Pierre Cochat; Damien Sanlaville; Caroline Rigard; Hélène de Leersnyder; Patricia Franco; Vincent Des Portes; Patrick Edery; Caroline Demily
Journal:  Orphanet J Rare Dis       Date:  2015-09-04       Impact factor: 4.123

Review 9.  The General Movement Assessment Helps Us to Identify Preterm Infants at Risk for Cognitive Dysfunction.

Authors:  Christa Einspieler; Arend F Bos; Melissa E Libertus; Peter B Marschik
Journal:  Front Psychol       Date:  2016-03-22

10.  Highlighting the first 5 months of life: General movements in infants later diagnosed with autism spectrum disorder or Rett Syndrome.

Authors:  Christa Einspieler; Jeff Sigafoos; Sven Bölte; Katrin D Bratl-Pokorny; Rebecca Landa; Peter B Marschik
Journal:  Res Autism Spectr Disord       Date:  2014-01-09
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