Use of the Dyskinesia Impairment Scale in non-ambulatory dyskinetic cerebral palsy.

AIM: To assess the responsiveness, concurrent validity, and feasibility of the Dyskinesia Impairment Scale (DIS) in non-ambulatory patients with dyskinetic cerebral palsy (CP). METHOD: The study is a secondary analysis of data collected in the IDYS trial, a randomized controlled trial on the effects of intrathecal baclofen (ITB). The DIS and Barry-Albright Dystonia Scale (BADS) were conducted at baseline and after 3 months of ITB or placebo treatment. Responsiveness was assessed by comparing the effect sizes and correlation of change after treatment between the DIS and BADS. Concurrent validity was evaluated by assessing the correlations between scales. Feasibility was evaluated for each DIS item by the number of participants who successfully accomplished the item. RESULTS: Thirty-three non-ambulatory patients (9 females, 24 males) with dyskinetic CP (ITB-treated: n=17, mean [SD] age: 14y 1mo [4y 1mo]; placebo-treated: n=16, mean [SD] age: 14y 7mo [4y]) were included in the study. The effect sizes for BADS and DIS were similar in The ITB-treated group (-0.29 and -0.22 respectively). Changes after treatment on the DIS dystonia subscale correlated with changes on the BADS (r=0.64; p<0.001). The DIS dystonia subscale and BADS correlated at baseline and follow-up (r=0.78; p<0.001 and r=0.79; p<0.001). Not all DIS activity items could be performed in this sample of patients. INTERPRETATION: For non-ambulatory patients with dyskinetic CP, the responsiveness of the DIS equalled the responsiveness of BADS. Concurrent validity was adequate. Feasibility for activity items was restricted in patients with severe dyskinetic CP. WHAT THIS PAPER ADDS: The Dyskinesia Impairment Scale (DIS) and Barry-Albright Dystonia Scale showed similar responsiveness in non-ambulatory patients with dyskinetic cerebral palsy (CP). No floor or ceiling effect was observed for DIS in non-ambulatory participants. The concurrent validity of DIS was adequate in non-ambulatory participants. Patients with dyskinetic CP in Gross Motor Function Classification System levels IV and V could not perform all DIS activity items.

Reliability of the Assisting Hand Assessment in adolescents.

AIM: To investigate the interrater and test-retest reliability of the Assisting Hand Assessment in adolescents (Ad-AHA) with cerebral palsy (CP) and to evaluate the alternate-form reliability of different test activities. METHOD: Participants were 112 adolescents with unilateral CP (60 males, 52 females; mean age 14y 5mo [standard deviation {SD} 2y 8mo], Manual Ability Classification System levels I-III). Reliability was evaluated using intraclass correlation coefficients (ICC), smallest detectable change (SDC), and Bland-Altman plots. RESULTS: ICCs for interrater (n=38) and test-retest reliability (n=31) were excellent: 0.97 (95% CI 0.94-0.98) and 0.99 (95% CI 0.98-0.99) respectively. The alternate-form reliability of different test activities was excellent for children (age 10-12y, n=30) performing the School-Kids AHA and Ad-AHA Board Game 0.99 (95% CI 0.98-0.99) and for adolescents (age 13-18y) performing the Ad-AHA Board Game compared to the Ad-AHA Present (n=28) 0.99 (95% CI 0.95-0.98), or the Ad-AHA Sandwich (n=29) 0.99 (95% CI 0.98-0.99) tasks. SDC for test-retest was 4.5 AHA-units. INTERPRETATION: Ad-AHA scores are consistent across different raters and occasions. The good alternate-form reliability indicates that the different test activities can be used interchangeably in adolescents with unilateral CP. Differences greater than or equal to 5 AHA-units can be considered a change beyond measurement error. The use of logit based AHA-units makes change comparable for persons at different ability levels.

Intrathecal baclofen treatment in dystonic cerebral palsy: a randomized clinical trial: the IDYS trial.

BACKGROUND: Dystonic cerebral palsy is primarily caused by damage to the basal ganglia and central cortex. The daily care of these patients can be difficult due to dystonic movements. Intrathecal baclofen treatment is a potential treatment option for dystonia and has become common practice. Despite this widespread adoption, high quality evidence on the effects of intrathecal baclofen treatment on daily activities is lacking and prospective data are needed to judge the usefulness and indications for dystonic cerebral palsy. The primary aim of this study is to provide level one clinical evidence for the effects of intrathecal baclofen treatment on the level of activities and participation in dystonic cerebral palsy patients. Furthermore, we hope to identify clinical characteristics that will predict a beneficial effect of intrathecal baclofen in an individual patient. METHODS/DESIGN: A double blind placebo-controlled multi-center randomized clinical trial will be performed in 30 children with dystonic cerebral palsy. Patients aged between 4 and 25 years old with a confirmed diagnosis of dystonic cerebral palsy, Gross Motor Functioning Classification System level IV or V, with lesions in the cerebral white matter, basal ganglia or central cortex and who are eligible for intrathecal baclofen treatment will be included. Group A will receive three months of continuous intrathecal baclofen treatment and group B will receive three months of placebo treatment, both via an implanted pump. After this three month period, all patients will receive intrathecal baclofen treatment, with a follow-up after nine months. The primary outcome measurement will be the effect on activities of and participation in daily life measured by Goal Attainment Scaling. Secondary outcome measurements on the level of body functions include dystonia, spasticity, pain, comfort and sleep-related breathing disorders. Side effects will be monitored and we will study whether patient characteristics influence outcome. DISCUSSION: The results of this study will provide data for evidence-based use of intrathecal baclofen in dystonic cerebral palsy.