Publications by authors named "Tjitske F Lawerman"

Article Synopsis
  • - The study aimed to assess how accurately early-onset ataxia (EOA) can be recognized compared to conditions like developmental coordination disorder (DCD) and central hypotonia, using scientifically validated features.
  • - Researchers analyzed 32 children with diagnoses of EOA, DCD, and hypotonia through videotaped assessments by three neurologists, focusing on inter-observer agreement and phenotypic profiles using a specific scoring system.
  • - Results indicated that while EOA could be reliably distinguished from central hypotonia, it was not easily differentiated from DCD, highlighting that incorporating scientifically validated EOA features improved consensus on diagnoses.
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In children, gait and posture assessment provides a crucial marker for the early characterization, surveillance and treatment evaluation of early onset ataxia (EOA). For reliable data entry of studies targeting at gait and posture improvement, uniform quantitative biomarkers are necessary. Until now, the pediatric test construct of gait and posture scores of the Scale for Assessment and Rating of Ataxia sub-scale (SARA) is still unclear.

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Aim: For reliable assessment of ataxia severity in children, the Childhood Ataxia and Cerebellar Group of the European Pediatric Neurology Society aimed to validate the Scale for Assessment and Rating of Ataxia (SARA) according to age.

Method: Twenty-two pediatric ataxia experts from 15 international institutions scored videotaped SARA performances in 156 typically developing children (4-16y: m/f=1; 12 children per year of age; including nine different nationalities). We determined age-dependency and reliability of pediatric SARA scores by a mixed model.

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Early-Onset Ataxia (EOA) and Developmental Coordination Disorder (DCD) are two conditions that affect coordination in children. Phenotypic identification of impaired coordination plays an important role in their diagnosis. Gait is one of the tests included in rating scales that can be used to assess motor coordination.

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Aim: To determine whether ataxia rating scales are reliable disease biomarkers for early onset ataxia (EOA).

Method: In 40 patients clinically identified with EOA (28 males, 12 females; mean age 15y 3mo [range 5-34y]), we determined interobserver and intraobserver agreement (interclass correlation coefficient [ICC]) and discriminant validity of ataxia rating scales (International Cooperative Ataxia Rating Scale [ICARS], Scale for Assessment and Rating of Ataxia [SARA], and Brief Ataxia Rating Scale [BARS]). Three paediatric neurologists independently scored ICARS, SARA and BARS performances recorded on video, and also phenotyped the primary and secondary movement disorder features.

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Article Synopsis
  • - The study aimed to evaluate how well different specialists agree on assessing early-onset ataxia (EOA) and to see if the Scale for Assessment and Rating of Ataxia (SARA) could serve as a useful indicator.
  • - Seven specialists assessed 40 patients, and agreement levels were statistically significant but only moderately strong, suggesting some inconsistencies in their evaluations.
  • - SARA gait subscores effectively distinguished between 'indisputable' and 'mixed' EOA phenotypes, indicating that better scores relate to a higher likelihood of primary ataxia, which could help refine EOA classification in future research.
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Aim: The aim of the study was to determine whether paediatric ataxia speech subscores are reliably applicable for international early-onset ataxia (EOA) databases. If so, we reasoned that ataxia speech subscores should be associated with ataxia scores and involve high interobserver agreement, including those for internationally applicable Scale for Assessment and Rating of Ataxia (SARA) syllable repetition tasks (SARASRT).

Method: Three independent paediatric neurologists and a speech therapist scored speech in 52 healthy children (mean age 10y, range 4-16y) and 40 individuals with EOA (mean age 15y, range 5-34y).

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