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van CCT (UK)

van CCT (UK)

Cognitie en het ontwikkelende brein in galactosemie.

- candidate number9364
- NTR NumberNTR2869
- ISRCTNISRCTN wordt niet meer aangevraagd.
- Date ISRCTN created
- date ISRCTN requested
- Date Registered NTR26-apr-2011
- Secondary IDsNL27398.068.09 METC azM/UM
- Public TitleCognitie en het ontwikkelende brein in galactosemie.
- Scientific TitleCognition in Classic Galactosemia: What happens to the developing brain?
- hypothesisSpecific areas of language production are affected in children and adolescents with classic galactosemia, in particular syntactic production. Further, it is hypothesized that the galactosmic patients will show a profile of general cognitive functions deviating from typically developing children and adolescents.
- Healt Condition(s) or Problem(s) studiedClassic galactosemia
- Inclusion criteriaFor patients, a diagnosis of classic galactosemia as diagnosed by GALT enzyme activity assay or GALT-gene mutation analysis (information obtained from treating physician); Age between 10 and 18 years old.
- Exclusion criteriaAny other disorder or disease that could affect cognitive functioning independently of classic galactosemia (an exception is made for the diagnosis of ADHD, because this disorder is common in this group).
- mec approval receivedyes
- multicenter trialno
- randomisedno
- groupFactorial
- Type2 or more arms, non-randomized
- Studytypeobservational
- planned startdate 1-aug-2009
- planned closingdate1-apr-2012
- Target number of participants52
- InterventionsN/A
- Primary outcome1. The performance on the language-related behavioural paradigms (four paradigms, each focusing on one aspect of speech processing or production);
2. The event-related potentials (ERPs) extracted from the EEG recorded during the behavioural tasks and.
- Secondary outcomeThe performance on the neuropsychological test battery (i.e. a composition of tests focusing on the major cognitive domains: memory, attention, speech and language, visuo-motor skills).
- TimepointsTwo sessions will be scheduled: One for the neuropsychological test and one for the EEG study.
- Trial web siteN/A
- statusinclusion stopped: follow-up
- Sponsor/Initiator Academic Hospital Maastricht (azM)
- Funding
(Source(s) of Monetary or Material Support)
Galactosemie Onderzoek Fonds (GOF)
- PublicationsN/A
- Brief summaryRationale:
Classic galactosemia is a hereditary disorder caused by the body’s inability to breakdown galactose, a sugar mainly found in milk. A galactose-restricted diet (soy diet) is the basis of therapy. This, however, does not prevent the emergence of long-term complications. One of these long-term complications is related to cognition. Reduced intelligence quotient (IQ) scores as well as impairments in speech and language have been reported. In fact, ninety percent of affected children have speech problems, usually diagnosed as Childhood Apraxia of Speech (CAS) (i.e. an impairment of motor programming of speech musculature). However, at present, CAS is over-diagnosed. This means that there are patients that receive a treatment for CAS, while other speech disabilities are being overlooked and not addressed properly resulting in suboptimal treatment. Our preliminary research concerning speech shows that syntax is a major problem in these children. The innovative approach in this study, examining the different levels of speech (e.g. semantic and syntactical speech) in combination with behavioral data and simultaneous brain activity recordings, aims to elucidate which levels of speech are specifically impaired in these children. Pinpointing at which level speech and other cognitive functions are affected is imperative to design successful treatment approaches with a better outcome.

To elucidate which areas of speech are specifically affected in children and adolescents with classic galactosemia; and to sketch a profile of the galactosemic patients’ general cognitive functions and of the development of these cognitive functions.

Study design:
Observational case control design consisting of a neuropsychological assessment and a behavioural paradigm with simultaneous electroencephalographic brain activity recording.
- Main changes (audit trail)
- RECORD26-apr-2011 - 6-mei-2011

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