Dysarthria in children and adults with ataxia telangiectasia

Autor: Nienke J H van Os, Michèl A.A.P. Willemsen, Stefanie J.G. Veenhuis, Leenke van Haaften, Marjo H.J.C. van Gerven, Elisabeth H Mulder, Corry M.R. Weemaes
Rok vydání: 2021
Předmět:
030506 rehabilitation
medicine.medical_specialty
congenital
hereditary
and neonatal diseases and abnormalities
lnfectious Diseases and Global Health Radboud Institute for Molecular Life Sciences [Radboudumc 4]
Context (language use)
Phonation volume
Audiology
Intelligibility (communication)
03 medical and health sciences
Dysarthria
0302 clinical medicine
Developmental Neuroscience
Medicine
Outpatient clinic
Humans
Speech
Phonation
Child
Involuntary movement
business.industry
Cerebral Palsy
Original Articles
medicine.disease
Disorders of movement Donders Center for Medical Neuroscience [Radboudumc 3]
nervous system diseases
Pediatrics
Perinatology and Child Health
Ataxia-telangiectasia
Original Article
Neurology (clinical)
medicine.symptom
0305 other medical science
business
030217 neurology & neurosurgery
Zdroj: Developmental Medicine & Child Neurology, 63, 4, pp. 450-456
Developmental Medicine & Child Neurology, 63, 450-456
Developmental Medicine and Child Neurology
ISSN: 0012-1622
Popis: Aim To investigate the characteristics and severity of dysarthria in children and adults with ataxia telangiectasia. Method 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. Results 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. Interpretation Dysarthria in ataxia telangiectasia is characterized by uncontrolled, ataxic, and involuntary movements, resulting in monotonous, unstable, slow, hypernasal, and chanted speech. What this paper adds 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.
What this paper adds 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.
Databáze: OpenAIRE
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