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Aim: To create a shortened, more user-friendly Second Edition of the Dyskinesia Impairment Scale (DIS-II) to assess dystonia and choreoathetosis, and evaluate its construct validity and reliability., Method: Scale development included an online expert meeting (n = 21) and iterative discussions within the research group (n = 6). A Rasch measurement model analysis on DIS scores from individuals with dyskinetic cerebral palsy or inherited/idiopathic dystonia (n = 123, 74 males, mean age 14 years, SD 5 years) was performed to evaluate the construct validity and reliability of the DIS-II., Results: The DIS-II evaluates dystonia and choreoathetosis in action and rest in 11 body regions, with action items scored from 0 to 3 and rest items 0 to 2. The number of videos to record are reduced from 26 to 14 and the items to score are reduced from 144 to 88. Rating scale functioning, goodness-of-fit evaluation, principal component analysis, and targeting met the predefined quality criteria of the study and construct validity was therefore considered good. Furthermore, person reliability indicated that the DIS-II can separate individuals into eight distinct ability levels., Interpretation: The DIS-II provides valid and reliable measures for dystonia and choreoathetosis, and reduces the administration and scoring time compared with the DIS. The DIS-II logit scores (interval level data) enhance comparison over time and between individuals in clinical practice and research., What This Paper Adds: Compared with the Dyskinesia Impairment Scale (DIS), the shortened edition (DIS-II) requires half of the number of videos to be scored. The DIS-II has a simplified rating scale, requiring scoring of 88 instead of 144 items. The DIS-II has shown excellent reliability and good construct validity. The interval properties of the DIS-II are superior to the ordinal level outcome measures of the DIS., (© 2022 Mac Keith Press.)

Aim: To provide insight into outcome and long-term safety and efficacy of deep brain stimulation (DBS), from the perspective of individuals with Lesch-Nyhan disease (LND) and their families., Method: We used patient-centered outcome measures to assess long-term outcomes of DBS for 14 individuals (mean [SD] age 10y 10mo [5y 6mo], range 5-23y, all males) with LND, after an average duration of 5y 6mo (range 11mo-10y 5mo) after surgery. We compared these results with a comprehensive review of previously published cases., Results: Patients and their families reported that DBS of the globus pallidus can be effective both for motor and behavioral disturbances in LND. However, outcome measures were often not significantly changed owing to substantial variability among individuals, and were overall less positive than in previous reports based on clinician assessments. In addition, there was an unexpectedly high rate of adverse events, tempering overall enthusiasm for the procedure., Interpretation: Although DBS might be an effective treatment for LND, more research is needed to understand the reasons for response variability and the unusually high rates of adverse events before DBS can be recommended for these patients. What this paper adds Individuals with Lesch-Nyhan disease and their families report variable efficacy of deep brain stimulation. Long-term outcomes are associated with a high adverse event rate., (© 2021 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.)

Aim: To evaluate the long-term effects of selective dorsal rhizotomy (SDR) 10 years or more after the procedure and complications observed any time after SDR in children with cerebral palsy (CP)., Method: Embase, PubMed, and the Cochrane Library were searched from their individual dates of inception through 1st June 2018 for full-text original articles in English that described long-term follow-up after SDR in children with CP. The authors independently screened publications to determine whether they met inclusion criteria; thereafter all authors extracted data on patient characteristics, the proportion of the original cohort being followed-up, and the reported outcomes., Results: Of the 199 studies identified, 16 were included in this evaluation: 14 were case series and two studies reported a retrospectively assigned comparison group. Evidence concerning function was limited by study design differences, clinical variability, loss to follow-up, and heterogeneity across trials., Interpretation: At 10 years or more follow-up, available studies generate low-level evidence with considerable bias. No functional improvement of SDR over routine therapy is documented. Furthermore, the long-term effects of SDR with respect to spasticity reduction is unclear, with many studies reporting a high amount of add-on spasticity treatment. More long-term follow-up using robust scientific protocols is required before it can be decided whether the use of SDR as routine therapy for children with CP is to be recommended or not., What This Paper Adds: Ten years after selective dorsal rhizotomy, available studies supply inconclusive evidence on functional outcomes. The long-term effect on spasticity is uncertain, studies reported a substantial need for add-on treatment. Short- and long-term complications seem frequent but are not reported in a consistent manner., (© 2019 The Authors Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.)

Aim: To describe health-related quality of life (HRQoL), pain, fatigue, and other health variables in young adults with cerebral palsy (CP), and to explore associations with the Gross Motor Function Classification System - Expanded and Revised (GMFCS-ER) and physical activity., Method: This was a cross-sectional study of 61 young adults at a mean age of 21 years 2 months (standard deviation 8mo, range 20-22y) with CP, from a geographically defined area. Data collection included: Short Form 36 version 2 for HRQoL, Brief Pain Inventory - Short Form, Fatigue Severity Scale, level of physical activity, medical history, and physical examination., Results: Overall HRQoL equalled that of population norms; however self-reported physical health was lower in GMFCS-ER levels III to V compared to GMFCS-ER levels I to II. Self-reported mental health was, inversely, lower in GMFCS-ER levels I to II compared to GMFCS-ER levels III to V. Pain prevalence was 49%, and pain was present across all GMFCS-ER levels. Fatigue, as well as sleep problems, had 41% prevalence, with fatigue severity decreasing with increasing level of physical activity., Interpretation: General HRQoL in young adults with CP was comparable to population norms. Pain and fatigue are important to address in high motor-functioning individuals also. Physical activity could be a possible protective factor against fatigue., What This Paper Adds: Health-related quality of life in young adults with cerebral palsy (CP) was comparable to population norms. Pain, fatigue, and sleep problems occurred at all Gross Motor Function Classification System levels. There is a possible protective effect of physical activity on fatigue., (© 2019 Mac Keith Press.)

Aim: To elucidate the natural course of benign paroxysmal torticollis, the relationship of this disorder to migraine and other paroxysmal diseases, and to analyse candidate genes., Method: This was a case series of children with benign paroxysmal torticollis of infancy (BPTI) diagnosed from 1998 to 2005, at Astrid Lindgren Children's Hospital, Stockholm, Sweden. A neurological examination and a formalized motor assessment were performed from 2005 to 2007. At a second follow-up, in 2014 to 2015, the children and their parents were interviewed and candidate genes analysed., Results: The mean age of the eight females and three males included in the second follow-up was 13 years 9 months (SD 2y 2mo). All motor assessments were normal. Five had developed migraine, abdominal migraine, and/or cyclic vomiting. Prophylactic treatment or migraine-specific medication during attacks were not needed. No paroxysmal tonic upgaze, benign paroxysmal vertigo, epilepsy, episodic ataxia, or paroxysmal dyskinesia was reported. Rare genetic variants in CACNA1A and ATP1A2 were found in two children. Five had a family history of migraine., Interpretation: BPTI is transient and does not lead to neurological sequelae. Most children afflicted experience either a mild migraine or no paroxysmal disorder at all in their adolescence. Genetic variants in candidate genes were few, indicating potential genetic heterogeneity., What This Paper Adds: After resolution of their benign paroxysmal torticollis of infancy (BPTI), children display no gross motor delay. Most adolescents who previously had BPTI have not developed migraine. No mutations in candidate genes, known to cause hemiplegic migraine, were found. Associated symptoms are often lacking during episodes of torticollis., (© 2018 Mac Keith Press.)

Aim: The aim of this study is to evaluate the long-term effects of selective dorsal rhizotomy (SDR), 15 to 20 years after surgery in patients with cerebral palsy., Method: Eighteen children (four females, 14 males; mean age at SDR 4y 7mo, SD 1y 7mo) with bilateral spastic cerebral palsy (CP), were prospectively assessed after SDR. This study focuses on the outcome 15 to 20 years after the procedure. The assessments include the Modified Ashworth Scale for spasticity, the Gross Motor Function Measure (GMFM-88), the Wilson Mobility Scale, The Health-Related Quality of Life Health Survey, SF-36v2, and the Brief Pain Inventory., Results: The effect of normalized muscle tone in lower extremities after SDR was sustained after a median of 17 years. The best gross motor function capacity, according to the GMFM score, was seen at the 3-year follow-up, thereafter a gradual decline followed. Half of the individuals reported low intensity pain and interference. Compared to a norm sample the physical health component of SF-36v2 was slightly lower and the mental health component slightly higher., Interpretation: The spasticity-reducing effect of SDR does not improve long-term functioning, nor prevent contractures, but it can possibly reduce the pain often experienced by individuals with CP., (© 2014 Mac Keith Press.)

Aim: Accommodation is the ability of the eye to change focus in order to maintain a sharp image of objects at various distances. The accommodative process is largely unknown in children and requires new assessment techniques. The aim of the study was to investigate this process in children with and without cerebral palsy (CP)., Method: In a descriptive case-control study, children with CP (n=15; nine females, six males; median age 14y) and 21 typically developing children (11 females, 10 males; median age 12y) underwent standard ophthalmological examination and examination by the PowerRefractor. Six of the children had spastic bilateral CP, five had spastic unilateral CP, and four had dyskinetic CP. The children's Gross Motor Function Classification System (GMFCS) levels were as follows: level I, seven children; level II, two children; level III, three children; and level IV, three children. The PowerRefractor measures accommodation in response to minus lens stimuli. Continuous measurements of refraction/accommodation, eye position, and pupil size are obtained. The Kruskal-Wallis analysis of variance (ANOVA) and Mann-Whitney U test were used for between-group analysis (α=0.05), and Friedman ANOVA was used for within-group analysis., Results: The stimuli-response gain (input/output) was approximately 80% in typical children inducing a focusing error (0.2-0.5D) increasing with minus lens power. Children with CP accommodated significantly less (gain: ~30%; p<0.001), inducing a larger focusing error (1.1-1.7D) increasing with minus lens power. The accommodative response was slower and more variable in children with CP. The pupil response did not mirror the accommodative response., Interpretation: Children with CP exhibit problems in generating an appropriate accommodative response. This can affect everyday living and reading skills., (© 2013 Mac Keith Press.)

Aim: The aim of this study was to evaluate the long-term effects of selective dorsal rhizotomy (SDR) in children with cerebral palsy (CP)., Method: Nineteen children (four females, 15 males; mean age 4y 7mo, SD 1y 7mo) with bilateral spastic CP, were prospectively assessed at baseline and 18 months, 3 years, and 10 years after SDR. Assessments included the Modified Ashworth Scale for spasticity, the Gross Motor Function Measure 88 (GMFM-88) and the Wilson gait scale for ambulation, neurological investigations, and passive joint range of motion assessment. A 10-year retrospective chart review was added for orthopaedic surgery after SDR., Results: Baseline muscle tone at the hip, knee, and ankle level displayed a high degree of spasticity that normalized after SDR. After 10 years there was a slight recurrence of spasticity at the knee and ankle. Joint range of motion declined from a maximum at 3 years after SDR to the 10-year follow-up. Median ambulatory status was best 3 years after SDR and then declined. The GMFM-88 score increased from the median baseline value of 51 to 66 (p=0.002) and 76 (p<0.001) at the initial follow-ups. After 10 years there was a decline in gross motor function with a reduction in the GMFM-88 score to 62 (p=0.022). Within 10 years, 16 out of 19 patients had a mean of three orthopaedic surgeries (SD 2.8), soft tissue surgery being the most common., Interpretation: The spasticity-reducing effect of SDR, although pronounced, did not seem to improve long-term functioning or prevent contractures. This suggests that contracture development in CP is not mediated by spasticity alone., (© 2011 The Authors. Developmental Medicine & Child Neurology © 2011 Mac Keith Press.)

Aim: To investigate relationships between hand function, brain lesions, and corticomotor projections in children with unilateral cerebral palsy (CP)., Method: The study included 17 children (nine males, eight females; mean age 11.4 [SD 2.4] range 7-16 y), with unilateral CP at Gross Motor Function Classification System level I and Manual Ability Classification System level I or II. Hand function was assessed with the Box and Blocks test and Assisting Hand Assessment (AHA). Conventional structural magnetic resonance images were assessed visually for type, location, and extent of brain lesions. Single-pulse transcranial magnetic stimulation (TMS) provided information on organization of corticomotor projections., Results: The most favourable hand function was seen in children who had white-matter damage of immaturity with mild white-matter loss and contralateral motor projections. Children with ipsilateral projections had the most impaired function. Nevertheless, in this subgroup a range of ability was seen (AHA 29-59%). Motor-projection patterns appeared to be influenced by lesion extent and location, but not by lesion type., Interpretation: Combining information from structural magnetic resonance images and TMS can improve prediction of hand function. A wide variation in hand function was seen within all motor-projection patterns. Although the most impaired hand function was seen in the ipsilateral motor-projection group, some children in this group had fairly good ability. Such information is important for treatment planning.

The long-term effects of botulinum toxin A (BoNT-A) treatment in children with cerebral palsy (CP) are still elusive. We studied a prospective clinical cohort of 94 children with different subtypes (50% spastic diplegic CP, 22% hemiplegic CP, 25% tetraplegic CP, 3% dyskinetic CP), sex (55% male, 45% female), severity according to Gross Motor Function Classification System (29% Level I, 15% Level II, 16% Level III, 17% Level IV, 23% Level V), and age (median 5y 4mo, range 11mo-17y 8mo). The longest follow-up time was 3 years 7 months (median 1y 6mo) and included a maximum of eight injections per muscle (median two injections to a specific muscle). Outcome measurements were muscle tone (Modified Ashworth Scale) and joint range of motion (ROM). Assessments were made at a minimum before and 3 months after each injection. Ninety-five per cent confidence intervals for differences from baseline were used to identify significant changes. BoNT-A injections induced reduction of long-term spasticity in all muscle-groups examined: the gastrocnemius, hamstring, and adductor muscles. The reduction in tone was most distinct in the gastrocnemius muscle, and each repeated injection produced an immediate reduction in muscle tone. However, improvement in ROM was brief and measured only after the first injections, whereupon the ROM declined. Thus, the results suggest that BoNT-A can be effective in reducing muscle tone over a longer period, but not in preventing development of contractures in spastic muscles. The dissociation between the effects on muscle tone and ROM indicates that development of contractures is not coupled to increased muscle tone only, but might be caused by other mechanisms.

This study was designed to determine whether children with cerebral palsy (CP) showed more co-activity than comparison children in non-prime mover muscles with regard to the prime mover during maximum voluntary isometric contraction (MVIC) of four lower-extremity muscles. Fourteen children with spastic diplegic CP (10 males, four females; age range 4-10y), seven children with spastic hemiplegic CP (five males, two females; age range 5-10y), and 14 comparison children (eight males, six females; age range 4-11y) participated in the study. Gross Motor Function Classification System levels of the children with CP were as follows: eight children at Level I, seven children at Level II, five children at Level III, and one child at Level I V. Surface electromyographic recordings were made simultaneously from the vastus lateralis (VL), medial hamstrings (MH), tibialis anterior, and lateral gastrocnemius (LG) muscles during maximal voluntary contraction. Children with CP showed higher co-activity than the comparison children in both antagonist and adjacent muscles. This was particularly true when VL, MH, or LG muscles were engaged in MVIC. These findings may contribute to the weakness and abnormal movement patterns seen in CP, and they have implications for treatment.

We examined muscle recruitment patterns in children with cerebral palsy (CP) and comparison children without CP under conditions of maximum voluntary contractions. Three groups of children participated in the study: (1) 12 children with diplegic CP (eight males, four females; age range 4-10 y, mean age 7 y [SD 2 y 4 mo]); (2) six children with hemiplegic CP (four males, two females; age range 5-10 y, mean age 7 y 4 mo [SD 2 y]); and (3) 13 comparison children with normal motor function (seven males, six females; age range 4-11 y, mean age 7 y 2 mo, [SD 2 y]). The children with CP were classified according to the Gross Motor Function Classification System: eight were Level I, five were Level II, four were Level III, and one was Level IV. Surface electromyography was recorded from four proximal and distal lower extremity (LE) muscles. Children with CP more frequently activated a muscle other than the intended prime mover first, compared with the comparison children, especially when the prime mover was a distal muscle. For example, during ankle plantar flexion, when the lateral gastrocnemius muscle was the prime mover, children with hemiplegia showed preactivation of the tibialis anterior muscle and children with diplegia showed medial hamstring coactivation. In conclusion, children with CP showed considerable differences to the comparison children in how LE muscles were voluntarily activated. Greater understanding of muscle recruitment patterns under a variety of tasks may provide new directions for motor control retraining or other forms of intervention.