Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UNIPROT:P30536 (
PBS
)
9,886
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Aim:
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. In the present study, we aimed to validate the construct validity and reliability of the pediatric (SARA
GAIT/POSTURE
) sub-scale.
Methods:
We included 28 EOA patients [15.5 (6-34) years; median (range)]. For inter-observer reliability, we determined the ICC on EOA SARA
GAIT/POSTURE
sub-scores by three independent pediatric neurologists. For convergent validity, we associated SARA
GAIT/POSTURE
sub-scores with: (1)
Ataxic gait
Severity Measurement by Klockgether (ASMK; dynamic balance), (2) Pediatric Balance Scale (
PBS
; static balance), (3) Gross Motor Function Classification Scale -extended and revised version (GMFCS-E&R), (4) SARA-kinetic scores (SARA
KINETIC
; kinetic function of the upper
and
lower limbs), (5) Archimedes Spiral (AS; kinetic function of the upper limbs), and (6) total SARA scores (SARA
TOTAL
; i.e., summed SARA
GAIT/POSTURE
, SARA
KINETIC
, and SARA
SPEECH
sub-scores). For discriminant validity, we investigated whether EOA co-morbidity factors (myopathy and myoclonus) could influence SARA
GAIT/POSTURE
sub-scores.
Results:
The inter-observer agreement (ICC) on EOA SARA
GAIT/POSTURE
sub-scores was high (0.97). SARA
GAIT/POSTURE
was strongly correlated with the other ataxia and functional scales [ASMK (
r
s
= -0.819;
p
< 0.001);
PBS
(
r
s
= -0.943;
p
< 0.001); GMFCS-E&R (
r
s
= -0.862;
p
< 0.001); SARA
KINETIC
(
r
s
= 0.726;
p
< 0.001); AS (
r
s
= 0.609;
p
= 0.002); and SARA
TOTAL
(
r
s
= 0.935;
p
< 0.001)]. Comorbid myopathy influenced SARA
GAIT/POSTURE
scores by concurrent muscle weakness, whereas comorbid myoclonus predominantly influenced SARA
KINETIC
scores.
Conclusion:
In young EOA patients, separate SARA
GAIT/POSTURE
parameters reveal a good inter-observer agreement and convergent validity, implicating the reliability of the scale. In perspective of incomplete discriminant validity, it is advisable to interpret SARA
GAIT/POSTURE
scores for comorbid muscle weakness.
...
PMID:Construct Validity and Reliability of the SARA Gait and Posture Sub-scale in Early Onset Ataxia. 2932 69
