Age‐related reference values for the pediatric Scale for Assessment and Rating of Ataxia: a multicentre study

Lawerman, T.F.; Brandsma, R.; Burger, Huibert; Burgerhof, Johannes G. M.; Sival, Deborah A

doi:10.1111/dmcn.13507

Cited by 44 publications

(43 citation statements)

References 21 publications

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“…In children, the reliability of this method is very high (intraclass correlation coefficient=0.997) . We accounted for age‐related influences on the SARA and PBS scores by using age‐corrected SARA and PBS values. For rating scale information, an explanation of the age corrections, and age‐corrected SARA score values see Appendix S2 and Table S1 (online supporting information).…”

Section: Methodsmentioning

confidence: 99%

“…When the latter is due to DCD, it can be associated with other disorders such as attention‐deficit/hyperactivity disorder, and also with mildly ataxic, dystonic, or choreatic features that are not excluded from the diagnosis . Physiologically delayed motor coordination in very young children is excluded from the DCD diagnosis, since it reflects typical brain development . The underlying aetiology of DCD is still unclear, but dysfunctional activation within the cerebellar, basal ganglia, and corticospinal networks has been suggested .…”

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confidence: 99%

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Paediatric motor phenotypes in early‐onset ataxia, developmental coordination disorder, and central hypotonia

Lawerman

Brandsma

Maurits

et al. 2019

Develop Med Child Neuro

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AIMS To investigate the accuracy of phenotypic early-onset ataxia (EOA) recognition among developmental conditions, including developmental coordination disorder (DCD) and hypotonia of central nervous system origin, and the effect of scientifically validated EOA features on changing phenotypic consensus.METHOD We included 32 children (4-17y) diagnosed with EOA (n=11), DCD (n=10), and central hypotonia (n=11). Three paediatric neurologists independently assessed videotaped motor behaviour phenotypically and quantitatively (using the Scale for Assessment and Rating of Ataxia [SARA]). We determined: (1) phenotypic interobserver agreement and phenotypic homogeneity (percentage of phenotypes with full consensus by all three observers according to the underlying diagnosis); (2) SARA (sub)score profiles; and (3) the effect of three scientifically validated EOA features on phenotypic consensus. RESULTS Phenotypic homogeneity occurred in 8 out of 11, 2 out of 10, and 1 out of 11 patients with EOA, DCD, and central hypotonia respectively. Homogeneous phenotypic discrimination of EOA from DCD and central hypotonia occurred in 16 out of 21 and 22 out of 22 patients respectively. Inhomogeneously discriminated EOA and DCD phenotypes (5 out of 21) revealed overlapping SARA scores with different SARA subscore profiles. After phenotypic reassessment with scientifically validated EOA features, phenotypic homogeneity changed from 16 to 18 patients.

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Section: Methodsmentioning

confidence: 99%

mentioning

confidence: 99%

Paediatric motor phenotypes in early‐onset ataxia, developmental coordination disorder, and central hypotonia

Lawerman

Brandsma

Maurits

et al. 2019

Develop Med Child Neuro

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“…Ongoing assessment of children's physical ability and function with pCMS is also important to track progress and assist with development of their therapy program. Outcome measures that may be used include the pediatric SARA (Scale for the Assessment and Rating of Ataxia) [30,58] or BARS (Brief Ataxia Rating Scale) [57], which enable a quantification of severity of ataxia. These may be used alongside more functional and participation/quality of life measures, for example the Bruininks-Oseretsky test of Motor Proficiency 2 [6] or the Pediatric Evaluation of Disability Inventory [24].…”

Section: Rehabilitation Of Cerebellar Motor Problems During and Aftermentioning

confidence: 99%

Post-operative cerebellar mutism syndrome: rehabilitation issues

et al. 2019

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Introduction Tumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing numbers of survivors are at high risk for developing adverse and long-term neurological deficits including deficits of cognition, behavior, speech, and language. Post-operative cerebellar mutism syndrome (pCMS) is a well-known and frequently occurring complication of cerebellar tumor surgery in children. In the acute stage, children with pCMS may show deterioration of cerebellar motor function as well as pyramidal and cranial neuropathies. Most debilitating is the mutism or the severe reduction of speech and a range of neurobehavioral symptoms that may occur. In the long term, children that recover from pCMS continue to have more motor, behavioral, and cognitive problems than children who did not develop pCMS after cerebellar tumor surgery. The severity of these long-term sequelae seems to be related to the length of the mute phase. Aim of this narrative review The impact of pCMS on patients and families cannot be overstated. This contribution aims to discuss the present knowledge on the natural course, recovery, and rehabilitation of children with pCMS. We suggest future priorities in developing rehabilitation programs in order to improve the long-term quality of life and participation of children after cerebellar tumor surgery and after pCMS in particular.

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“…Some studies have confirmed the validity of this scale for Friedreich's ataxia, spinocerebellar ataxia, and posterior fossa tumor . Although additional reports indicate that the SARA is age‐dependent and reliable in normal children, another author objected to this conclusion . In short, the SARA requires further assessments of validity and reliability.…”

Section: Behavioral Assessments Of Pmdsmentioning

confidence: 99%

Current behavioral assessments of movement disorders in children

et al. 2018

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Pediatric movement disorders (PMDs) are common and have recently received increasing attention. As these disorders have special clinical features, the selection of appropriate behavioral assessment tools that can clearly distinguish movement disorders from other diseases (eg, epilepsy and neuromuscular disorders) is crucial for achieving an accurate diagnosis and treatment. However, few studies have focused on behavioral assessments in children. The present report attempts to provide a critical review of the available subjective and objective assessment tests for common PMDs. We believe that the principles of objectification, multi-purpose use, and simplification are also applicable to the selection and development of satisfactory pediatric behavioral assessment tools. We expect that the development of wearable sensors, virtual reality, and augmented reality will lead to the establishment of more reliable and simple tests. In addition, more rigorous randomized controlled trials that have been specifically designed to evaluate behavioral testing in children are also expected in the future.

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Age‐related reference values for the pediatric Scale for Assessment and Rating of Ataxia: a multicentre study

Cited by 44 publications

References 21 publications

Paediatric motor phenotypes in early‐onset ataxia, developmental coordination disorder, and central hypotonia

Paediatric motor phenotypes in early‐onset ataxia, developmental coordination disorder, and central hypotonia

Post-operative cerebellar mutism syndrome: rehabilitation issues

Current behavioral assessments of movement disorders in children

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