Dysarthria in children and adults with ataxia telangiectasia.


Journal

Developmental medicine and child neurology
ISSN: 1469-8749
Titre abrégé: Dev Med Child Neurol
Pays: England
ID NLM: 0006761

Informations de publication

Date de publication:
04 2021
Historique:
accepted: 08 12 2020
pubmed: 2 2 2021
medline: 20 8 2021
entrez: 1 2 2021
Statut: ppublish

Résumé

To investigate the characteristics and severity of dysarthria in children and adults with ataxia telangiectasia. All children and adults with ataxia telangiectasia who visited our multidisciplinary outpatient clinic for ataxia telangiectasia were asked to participate in this study, which took place in March 2019. To evaluate dysarthria, we used the Radboud Dysarthria Assessment in adults (older than 18y) and the paediatric Radboud Dysarthria Assessment in children (5-18y), including the observational tasks 'conversation' and 'reading', and the speech-related maximum performance tasks 'repetition rate', 'phonation time', 'fundamental frequency range', and 'phonation volume'. Speech intelligibility was measured using the Intelligibility in Context Scale. Twenty-two individuals (15 children [5-17y], seven adults [19-47y]; 14 males and eight females; mean age 19y, SD 15y 2mo) participated. Dysarthria was present in all participants and characterized by ataxic components in adults and similar uncontrolled movements in children. In most participants, speech was mildly to mildly/severely affected. Almost all participants had an abnormal score for at least one maximum performance task. Dysarthria in ataxia telangiectasia is characterized by uncontrolled, ataxic, and involuntary movements, resulting in monotonous, unstable, slow, hypernasal, and chanted speech. Dysarthria in ataxia telangiectasia is characterized by uncontrolled, ataxic, and involuntary movements. Dysarthria in ataxia telangiectasia results in monotonous, unstable, slow, hypernasal, and chanted speech. Dysarthria in ataxia telangiectasia can be assessed using the Radboud Dysarthria Assessment and the paediatric Radboud Dysarthria Assessment.

Identifiants

pubmed: 33521952
doi: 10.1111/dmcn.14811
pmc: PMC7986845
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

450-456

Subventions

Organisme : Twan foundation
Organisme : A-T Children's Project

Commentaires et corrections

Type : CommentIn

Informations de copyright

© 2021 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.

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Auteurs

Stefanie J G Veenhuis (SJG)

Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, the Netherlands.

Nienke J H van Os (NJH)

Department of Pediatric Neurology, Radboud University Medical Center, Nijmegen, the Netherlands.

Marjo H J C van Gerven (MHJC)

Donders Institute for Brain, Cognition and Behaviour, Department of Rehabilitation, Radboud University, Nijmegen, the Netherlands.

Leenke van Haaften (L)

Donders Institute for Brain, Cognition and Behaviour, Department of Rehabilitation, Radboud University, Nijmegen, the Netherlands.

Elisabeth H Mulder (EH)

Donders Institute for Brain, Cognition and Behaviour, Department of Rehabilitation, Radboud University, Nijmegen, the Netherlands.
Faculty of Arts at Radboud University, Nijmegen, the Netherlands.

Corry M R Weemaes (CMR)

Department of Pediatric Infectious Diseases and Immunology, Radboud University Medical Center, Nijmegen, the Netherlands.

Michèl A A P Willemsen (MAAP)

Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, the Netherlands.
Department of Pediatric Neurology, Radboud University Medical Center, Nijmegen, the Netherlands.

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