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1.

Background

Long-term follow-up data after different vascular types of ischemic perinatal stroke is sparse. Our aim was to study neurodevelopmental outcomes following neonatal and presumed perinatal ischemic middle cerebral artery territory stroke (arterial ischemic stroke, AIS) and periventricular venous infarction (PVI).

Methods

A prospective consecutive cohort of 40 term-born children with perinatal stroke (21 AIS, 19 PVI) was identified through the Estonian Paediatric Stroke Database. While 48% of the children with AIS were diagnosed during the neonatal period, all the children with PVI had presumed perinatal stroke. Outcomes based on the Paediatric Stroke Outcome Measure (PSOM) and Kaufman Assessment Battery for Children – Second Edition (K-ABC-II), in relation to extent and laterality of stroke, were defined.

Results

At a median age of 7 years 6 months (range 3.6–13y), there was a trend towards worse neurodevelopmental outcome in participants with AIS when compared to PVI (mean total PSOM scores 3.1 and 2.2, respectively; p = 0.06). Combined deficits of motor, language and cognitive/behavioural functions were significantly more common among children with AIS (90%) when compared to children with PVI (53%, p = 0.007). General cognitive ability (by K-ABC-II) was significantly lower in the AIS subgroup (mean 79.6; 95% CI 72.3–87.0), but children with PVI (91.6; 95% CI 85.5–97.8) also had poorer performance than the age-equivalent normative mean. Large extent of stroke was associated with poorer neurodevelopmental outcome and lower cognitive performance in children following AIS but not in PVI.

Conclusion

In this national cohort, poor long-term neurodevelopmental outcome after perinatal ischemic stroke was seen irrespective of the vascular type or time of diagnosis of stroke. However, the spectrum of neurological deficits is different after perinatal AIS and PVI, with combined deficits more common among children following AIS.  相似文献   

2.

Introduction

The purposes of this study were to evaluate parental attitudes toward general vaccination protocols and increase parental knowledge of the human papilloma virus (HPV) vaccine.

Methods

A nonprobability convenience sample (N = 75) using a pre-/postintervention study design was conducted in a pediatric office in southern New Jersey. The Parental Attitudes Module measured the general disposition toward having children receive any type of vaccine. The HPV Knowledge Survey was a second tool used to specifically measures knowledge of the HPV vaccine. A self-directed computer-based learning was part of the educational intervention.

Results

A paired t test showed that HPV Knowledge Survey postintervention scores were significantly higher than HPV Knowledge Survey preintervention scores (t = ?10.585, p < .001). The Parental Attitudes Module and the HPV Knowledge Survey pretest showed a positive moderate relationship (rs = .552, p < .001).

Discussion

In the 10 years since the HPV vaccine has been on the market, there is a continued need to increase parental knowledge about the HPV vaccine to close the gap on vaccine nonadherence. A self-directed, computer-based learning tablet appears to be an effective tool to educate parents or legal guardians about the purpose, efficacy, and safety of the HPV vaccine.  相似文献   

3.

Objective

To evaluate relationships between whole blood (B-Al) and hair aluminum (H-Al) levels in healthy infants and their immunization history and development.

Methods

We conducted a cross-sectional study of 9- to 13-month-old children recruited from an urban primary care center, excluding those with a history of renal disease or receipt of either aluminum-containing pharmaceuticals or parenteral nutrition. Aluminum levels were measured using inductively coupled plasma-mass spectrometry. Correlation with Bayley Scales of Infant and Toddler Development, Third Edition (BSID) and vaccine-related aluminum load was assessed via linear regression models.

Results

The median age of 85 participants was 287 days. B-Al (median, 15.4 ng/mL; range, 0.9–952 ng/mL) and H-Al (median 42,542 ng/g; range, 2758–211,690 ng/g) were weakly correlated (Spearman ρ = 0.26; P = .03). There was no significant correlation between B-Al or H-Al and estimated aluminum load from vaccines. B-Al was not correlated with BSID composite or subscale scores. Although H-Al was not correlated with BSID scores in models including all data (n = 85), it was inversely correlated with motor composite (P < .02; Wald = 5.88) and the gross motor subscale (P = .04; Wald = 4.38) in models that excluded an extreme outlying H-Al value.

Conclusions

Infant B-Al and H-Al varied considerably but did not correlate with their immunization history. Likewise, there was no correlation between B-Al and infant development or between H-Al and language or cognitive development. An inverse correlation between H-Al and BSID motor scores deserves further investigation.  相似文献   

4.

Objective

We examined the self-reported preparedness of hospitalized adolescents and young adults (AYA) for transition from pediatric to adult-oriented health care with regard to: 1) previous health care transition (HCT) preparation, 2) Self-Determination Theory (SDT) constructs of health self-management autonomy and competence, and 3) their perception of medical knowledge, attitudes, and concerns.

Methods

From 2013 to 2015, 139 hospitalized patients aged 15 to 21 years completed a 40-item survey on HCT preparation, attitudes, concerns, and perception of knowledge adapted in part from validated questionnaires of the Department of Health and Human Services, Maternal and Child Health Bureau, and SDT Treatment Self-Regulation Study.

Results

Fewer than 40% of all respondents endorsed previous HCT preparation such as providers discussing taking responsibility for their health, transitioning to adult providers, and only 20% had discussed future health insurance needs. Of our AYA population, 84% had 1 or more special health care needs. Older patients, female patients, and those with increased HCT preparation scores had increased autonomous motivation, positive attitudes toward transition, yet also increased transition concerns. Higher autonomous motivation and perceived competence correlated with increased perception of knowledge (P = .002, < .001 respectively) and more positive attitudes toward transition planning (P < .001, .054 respectively). Multivariate regression analysis revealed those with increased HCT preparation and those with increased perceived competence had increased perception of knowledge (β = .25, P = .005 and β = .35, P < .001).

Conclusions

Our findings suggest that hospitalized AYA received limited education and preparation regarding key elements of HCT to adult-oriented health care. Moreover, those previously exposed to transition preparation efforts were more likely to have motivation and a sense of competence in HCT skills.  相似文献   

5.

Introduction

Shapiro syndrome (SS) is characterized by spontaneous recurrent episodes of hypothermia, hyperhidrosis and corpus callosum (CC) agenesis. Less than 60 cases have been reported to date and the pathogenic mechanism as well as the prognosis of this syndrome are still debated. We describe the clinical features and long-term follow-up of a pediatric cohort of SS patients.

Methods

We collected 13 (10 novel) pediatric cases of SS and report their long-term follow-up and neurological outcome.

Results

All patients experienced recurring hypothermia, with body temperature below 35 °C during the episodes, often accompanied by hyperidrosis. CC agenesis was an inconstant structural feature in the present series (2/13 patients). Seven patients received antiepileptic drugs (AEDs) or other drug therapy for a mean period of 12 months. At long-term follow-up (mean = 61 months, range: 60–96), all individuals were free from episodes of paroxysmal hypothermia independently from previous AED use or other drug therapy.

Conclusion

Paroxysmal hypothermia, the core symptom of SS, behaved as a age-dependent feature in our cohort, supporting a good long-term prognosis for SS. A prompt diagnosis of SS is crucial to avoid unnecessary diagnostic investigations.  相似文献   

6.

Introduction

Many patients with cerebral palsy (CP) suffer chronic pain as one of the most limiting factors in their quality of life. In CP patients, pain mechanisms are not well understood, and pain therapy remains a challenge. Quantitative sensory testing (QST) might provide unique information about the functional status of the somatosensory system and therefore better guide pain treatment.

Objectives

To understand better the underlying pain mechanisms in pediatric CP patients, we aimed to assess clinical and pain parameters, as well as QST profiles, which were matched to the patients' cerebral imaging pathology.

Patients and methods

Thirty CP patients aged 6–20 years old (mean age 12 years) without intellectual impairment underwent standardized assessments of QST. Cerebral imaging was reassessed. QST results were compared to age- and sex-matched controls (multiple linear regression; Fisher's exact test; linear correlation analysis).

Results

CP patients were less sensitive to all mechanical and thermal stimuli than healthy controls but more sensitive to all mechanical pain stimuli (each p < 0.001). Fifty percent of CP patients showed a combination of mechanical hypoesthesia, thermal hypoesthesia and mechanical hyperalgesia; 67% of CP patients had periventricular leukomalacia (PVL), which was correlated with mechanic (r = 0.661; p < 0.001) and thermal (r = 0.624; p = 0.001) hypoesthesia.

Conclusion

The combination of mechanical hypoesthesia, thermal hypoesthesia and mechanical hyperalgesia in our CP patients implicates lemniscal and extralemniscal neuron dysfunction in the thalamus region, likely due to PVL. We suspect that extralemniscal tracts are involved in the original of pain in our CP patients, as in adults.  相似文献   

7.

Objective

To evaluate prospectively the effectiveness of cognitive behavioral therapy (CBT) in children with autism spectrum disorder (ASD).

Methods

Drug-naïve children who met the DSM-V criteria for a diagnosis of ASD were recruited from a day care center, specialized in long-term treatment of children and adolescents with ASD. Symptom assessment was performed using the Aberrant Behavior Checklist (ABC) before (base-line) and after 12 months (follow-up) of CBT.

Results

Nine boys with a mean age of 6 (±2.0) years were included. Compared to baseline, significant improvements of symptoms of irritability (p = 0.012), hyperactivity (p = 0.008) and lethargy (p = 0.008) were observed at follow-up.

Conclusion

Results indicate that CBT is an effective therapy for children with ASD. Larger studies are needed to give more details about which symptoms respond best in these patients.  相似文献   

8.

Introduction

This study examined the association between early weight gain (0–6 months) and risk for overweight, as defined by the Institute of Medicine, at 1 year among infants of low-income Hispanic immigrant mothers.

Method

Weight-for-age data were extracted from electronic medical records of 335 infants with gestations of 37 weeks or longer and birthweights appropriate for gestational age and without medical problems likely to interfere with growth or feeding. Logistic regression models were constructed to examine the impact of early weight changes on weight status at 1 year.

Results

By 12 months, 36.7% of infants had crossed weight-for-age of 84.1% or greater on World Health Organization growth charts. In adjusted models, infants had 20.8 (95% confidence interval = [19.8, 44.0]) times the odds of reaching this benchmark at 1 year for each z score increase at 0 to 6 months.

Discussion

The study highlights a time-sensitive opportunity for interventions to reduce risk for overweight for this vulnerable population.  相似文献   

9.

Objective

Diagnostic errors (DEs), which encompass failures of accuracy, timeliness, or patient communication, cause appreciable morbidity but are understudied in pediatrics. Pediatricians have expressed interest in reducing high-frequency/subacute DEs, but their epidemiology remains unknown. The objective of this study was to investigate the frequency of two high-frequency/subacute DEs and one missed opportunity for diagnosis (MOD) in primary care pediatrics.

Methods

As part of a national quality improvement collaborative, 25 primary care pediatric practices were randomized to collect 5 months of retrospective data on one DE or MOD: elevated blood pressure (BP) and abnormal laboratory values (DEs), or adolescent depression evaluation (MOD). Relationships between DE or MOD proportions and patient age, gender, and insurance status were explored with mixed-effects logistic regression models.

Results

DE or MOD rates in pediatric primary care were found to be 54% for patients with elevated BP (n = 389), 11% for patients with abnormal laboratory values (n = 381), and 62% for adolescents with an opportunity to evaluate for depression (n = 400). When examining the number of times a pediatrician may have recognized an abnormal condition but either knowingly or unknowingly did not act according to recommended guidelines, providers did not document recognition of an elevated BP in 51% of patients with elevated BP, and they did not document recognition of an abnormal laboratory value without a delay in 9% of patients with abnormal laboratory values.

Conclusions

DEs and MODs occur at an appreciable frequency in pediatric primary care. These errors may contribute to care delays and patient harm.  相似文献   

10.

Objective

To determine whether educational media as actually used by low-income families promote parent–child cognitive stimulation activities.

Methods

We performed secondary analysis of the control group of a longitudinal cohort of mother–infant dyads enrolled postpartum in an urban public hospital. Educational media exposure (via a 24-hour recall diary) and parent–child activities that may promote cognitive stimulation in the home (using StimQ) were assessed at 6, 14, 24, and 36 months.

Results

Data from 149 mother–child dyads, 93.3% Latino, were analyzed. Mean (standard deviation) educational media exposure at 6, 14, 24, and 36 months was, respectively, 25 (40), 42 (58), 39 (49), and 39 (50) minutes per day. In multilevel model analyses, prior educational media exposure had small positive relationship with subsequent total StimQ scores (β = 0.11, P = .03) but was nonsignificant (β = 0.08, P = .09) after adjusting for confounders (child: age, gender, birth order, noneducational media exposure, language; mother: age, ethnicity, marital status, country of origin, language, depressive symptoms). Educational media did predict small increases in verbal interactions and toy provision (adjusted models, respectively: β = 0.13, P = .02; β = 0.11; P = .03). In contrast, more consistent relationships were seen for models of the relationship between prior StimQ (total, verbal interactions and teaching; adjusted models, respectively: β = 0.20, P = .002; β = 0.15, P = .006; β = 0.20, P = .001) and predicted subsequent educational media.

Conclusions

Educational media as used by this sample of low-income families does not promote cognitive stimulation activities important for early child development or activities such as reading and teaching.  相似文献   

11.

Background

Data on paediatric deep brain stimulation (DBS) is limited, especially for long-term outcomes, because of small numbers in single center series and lack of systematic multi-center trials.

Objectives

We seek to systematically evaluate the clinical outcome of paediatric patients undergoing DBS.

Methods

A German registry on paediatric DBS (GEPESTIM) was created to collect data of patients with dystonia undergoing DBS up to the age of 18 years. Patients were divided into three groups according to etiology (group 1 inherited, group 2 acquired, and group 3 idiopathic dystonia).

Results

Data of 44 patients with a mean age of 12.8 years at time of operation provided by 6 German centers could be documented in the registry so far (group 1 n = 18, group 2 n = 16, group 3 n = 10). Average absolute improvement after implantation was 15.5 ± 18.0 for 27 patients with pre- and postoperative Burke-Fahn-Marsden Dystonia Rating scale movement scores available (p < 0.001) (group 1: 19.6 ± 19.7, n = 12; group 2: 7.0 ± 8.9, n = 8; group 3: 19.2 ± 20.7, n = 7). Infection was the main reason for hardware removal (n = 6). 20 IPG replacements due to battery expiry were necessary in 15 patients at 3.7 ± 1.8 years after last implantation.

Discussion

Pre- and postoperative data on paediatric DBS are very heterogeneous and incomplete but corroborate the positive effects of DBS on inherited and acquired dystonia. Adverse events including relatively frequent IPG replacements due to battery expiry seem to be a prominent feature of children with dystonia undergoing DBS. The registry enables collaborative research on DBS treatment in the paediatric population and to create standardized management algorithms in the future.  相似文献   

12.

Objectives

To elaborate the imaging phenotype associated with a homozygous c.743C > del frameshift mutation in DAG1 leading to complete absence of both α- and β-dystroglycan previously reported in a consanguineous Israeli-Arab family.

Methods

We analyzed prenatal and postnatal imaging data of patients from a consanguineous Israeli-Arab kindred harboring the DAG1 mutation.

Results

The imaging studies (fetal ultrasound, CT scan and postnatal MRI) demonstrated: flat cortex (abnormally thick with irregular pebbled cortical-white matter border on MRI), hydrocephalus, scattered small periventricular heterotopia and subependymal hemorrhages and calcifications, z-shaped brainstem, and in addition an occipital encephalocele, vermian agenesis, and an elongated and thick tectum (tectocerebellar dysraphia).

Conclusions

The novel association of cobblestone malformation with tectocerebellar dysraphia as part of WWS is characteristic of the homozygous c.743C > del frameshift mutation in the DAG1 gene.  相似文献   

13.

Background

Ataxia telangiectasia (AT) is a neurodegenerative cerebellar disorder, caused by mutations in the ATM gene, involved in DNA repair. Radiosensitivity, progressive ataxia, immune deficiency and malignancies, are well known symptoms, but urological manifestations are scarcely described.

Objective

To characterize urologic manifestations in a large cohort of AT patients.

Methods

Retrospective cross-sectional chart study comprising 52 AT patients followed at a National AT Center.

Results

25% of the cohort (13 patients/8 males) had urologic symptoms, which presented at 11 ± 4.3 years. The most common symptom was secondary enuresis affecting 15% of the patients (8 children/4 males). Incontinence appeared at 8 ± 6.2 years of age, and resolved spontaneously within 15 ± 8.3 months in 6 patients. It preceded loss of ambulatory capacity by 1–2 years in 7 patients. Lumbosacral MRI were normal (4 children) and urine cultures (all) were negative. Urodynamic evaluation that was performed in only one patient revealed overactive bladder. Additional manifestations were macroscopic hematuria due to bladder telangiectasia in a 12-year-old, and renal cell carcinoma in a 22-year-old. Other manifestations unrelated to AT were neprolithiasis, vesico-ureteral reflux and scrotal pain, each in 1 patient.

Discussion

Transient secondary enuresis is a frequent finding in AT patients, heralding loss of ambulatory capacity, tough it's pathophysiological mechanism is largely no understood.  相似文献   

14.

Objective

To understand the effect of a health messaging intervention focused on provider communication about vaccination on mothers' willingness to vaccinate children against human papillomavirus (HPV) and seasonal influenza.

Methods

A total of 2476 mothers of 9- to 13-year-olds in the United States completed a Web-based survey in August 2014. Mothers were randomized to 1 of 2 groups targeting HPV or influenza vaccine. Mothers whose child had not received the target vaccine (ie, zero doses of HPV vaccine/no prior-year administration of influenza vaccine) were randomized to the intervention. The study used a 3 × 2 between-subjects design; illustrated vignettes depicted 1 of 3 levels of provider recommendation strength (brief mention of vaccination, strong recommendation of vaccination, or personal disclosure of vaccination of own children), and presence or absence of information comparing safety of vaccination to the safety of a common daily activity. Outcome was mothers' willingness to have their child receive the target vaccine. Perceived benefits of vaccination were assessed before viewing the intervention and were included as a covariate in analyses, along with child gender.

Results

For HPV vaccine, there was a main effect of safety information (F(1,684) = 7.99, P = .005) and perceived benefits of vaccination (F(1,684) = 221.64, P < .001) on mothers' willingness to vaccinate. For influenza, perceived benefits of vaccination significantly related to willingness to vaccinate (F(1,462) = 105.78, P < .001). Child gender was not associated with willingness.

Conclusions

Provider communication about vaccination may need to be tailored to the vaccine in question. A next step to increasing coverage for both HPV and influenza vaccines may be an intervention aimed at increasing mothers' perceived benefits of vaccination.  相似文献   

15.

Background

Herbal medicines are particularly regarded as an alternative or complement to conventional pharmaceuticals in the treatment and prevention of respiratory tract infections (RTIs). Therefore, the purpose of this review was to identify evidence for herbal therapy in the treatment of RTIs concerning effectiveness and safety.

Methods

Medline/PubMed, Scopus, and the Cochrane Library were searched through February 12, 2015. Randomized controlled trials that compared herbal therapy with no treatment, placebo, or any pharmaceutical medication in children and adolescents (age 0 to 18 years) with RTI were eligible.

Results

Eleven trials with 2181 participants were included. No clear evidence for Echinacea (4 trials) or an herbal compound preparation (1 trial) in preventing RTI symptoms was found. Meta-analysis revealed evidence for efficacy (responder rates: risk ratio [RR], 2.56; 95% confidence interval [CI], 1.54–4.26; P < .01; heterogeneity: I2 = 38%; chi-square = 9.63; P = .14) and safety (patients with adverse events: RR, 1.06; 95% CI, 0.42–2.66; P = .9; heterogeneity: I2 = 72%; chi-square = 10.64; P = .01) of Pelargonium sidoides in treating RTI symptoms compared with placebo (6 trials).

Conclusions

Because of conflicting evidence in the included studies no concrete conclusion on effects of Echinacea could be drawn so far. In the case of Pelargonium sidoides, meta-analysis revealed moderate evidence for efficacy and safety in the treatment of RTIs in children.  相似文献   

16.
17.

Background

In non-walking children with severe spasticity, daily care can be difficult and many patients suffer from pain. Selective dorsal rhizotomy (SDR) reduces spasticity in the legs, and therefore has the potential to improve daily care and comfort.

Aim

To examine effects of SDR on daily care and comfort in non-walking children with severe spasticity due to different underlying neurological conditions.

Methods

Medical history, changes in daily care and comfort and satisfaction with outcome were assessed retrospectively in non-walking children who underwent SDR in our center, with a mean follow-up of 1y 7m (range 11m–4y 3m). All eligible patients (n = 24, years 2009–2014) were included.

Results

Mean age at SDR was 12y 4m (SD 4y 3m, range 2y 8m–19y 3m). Associated orthopaedic problems were frequent. Seven patients underwent scoliosis correction in the same session. Most improvements were reported in dressing (n = 16), washing (n = 12) and comfort (n = 10). Median score for satisfaction was 7 on a scale of 10 (range 1–9). SDR resulted in reduction of spasticity in leg muscles. In nine patients dystonia was recorded post-operatively, mainly in children with congenital malformations and syndromes.

Interpretation

SDR is a single event intervention that can improve daily care and comfort in non-walking children with severe spasticity, and can safely be combined with scoliosis correction. Despite the improvements, satisfaction is variable. Careful attention is necessary for risk factors for dystonia, which may be unmasked after SDR.  相似文献   

18.

Objective

No standardized curricula exist for training residents in the special needs of children with medical complexity. We assessed resident satisfaction, knowledge, and behavior after implementing a novel online curriculum composed of multimedia modules on care of children with medical complexity utilizing virtual simulation.

Methods

We conducted a randomized controlled trial of residents across North America. A Web-based curriculum of 6 self-paced, interactive, multimedia modules was developed. Readings for each topic served as the control curriculum. Residents were randomized to 1 of 2 groups, each completing 3 modules and 3 sets of readings that were mutually exclusive. Outcomes included resident scores on satisfaction, knowledge-based assessments, and virtual simulation activities.

Results

Four hundred forty-two residents from 56 training programs enrolled in the curriculum, 229 of whom completed it and were included in the analysis. Subjects were more likely to report comfort with all topics if they reviewed modules compared to readings (P ≤ .01 for all 6 topics). Posttest knowledge scores were significantly higher than pretest scores overall (mean increase in score 17.7%; 95% confidence interval 16.0, 19.4), and the mean pre–post score increase for modules was significantly higher than readings (20.9% vs 15.4%, P < .001). Mean scores on the verbal handoff virtual simulation increased by 1.1 points (95% confidence interval 0.2, 2.0, P = .02). There were no significant differences found in pre–post performance for the device-related emergency virtual simulation.

Conclusions

There was high satisfaction, significant knowledge acquisition, and specific behavior change after participating in this innovative online curriculum. This is the first multisite, randomized trial assessing satisfaction, knowledge impact, and behavior change in a virtually simulated environment with pediatric trainees.  相似文献   

19.

Background

Annually 14.000 children with traumatic brain injury (TBI) are admitted to the Emergency Department (ED) in the Netherlands. Presentation varies and a specific entity comprises the juvenile head trauma syndrome (JHTS) with secondary deterioration after a mild trauma. As outcome of JHTS can be fatal, early recognition is essential.

Aim

To outline the epidemiology and clinical features of JHTS, in comparison to paediatric mild TBI patients without JHTS.

Methods

Retrospective study of 570 patients with mild TBI admitted to the ED of a level-one trauma centre from 2008 to 2014. Diagnosis of JHTS by experienced neurologists was compared with diagnosis by physicians at the ED.

Results

Physicians at the ED diagnosed JHTS more frequently (14%) compared to experienced neurologists (8%). JHTS occurred after a lucid interval varying from 5 to 225 min (mean 44 (SD 64)) with changes in consciousness. JHTS patients were younger compared to mild TBI patients (4.1 (SD 2.4) vs. 7.3 (SD 5.7), p < 0.01), (range: 1–10 years). Falls occurred more often in JHTS (84% vs. 69%, p = 0.03) and at presentation, vomiting (42% vs. 22%, p < 0.01) and changed behaviour (29% vs. 1%, p = 0.03) were more present compared to the mild TBI group.

Conclusion and discussion

JHTS occurs more often in children up to 10 years with falls as major cause of injury. Clues for recognition of this syndrome comprise changes in consciousness and vomiting or changed behaviour on presentation at the ED. For clinical practice, these factors should guide the decision for hospital admission or discharge.  相似文献   

20.

Aim

To compare physiological age-relatedness between dyskinesia (dystonia/choreoathetosis), dystonia and ataxia rating scale scores in healthy children.

Method

Three movement disorders specialists quantified dyskinetic-like features in healthy children (n = 52; 4–16 years) using the Dyskinesia Impairment Scale (DIS = DIS-choreoathetosis (DIS-C) + DIS-dystonia (DIS-D)). We compared the age-related regression coefficients of the DIS with data processed from previous studies on dystonia and ataxia rating scales (Burke-Fahn-Marsden Movement and Disability Scales (BFMMS and BFMDS) and Scale for Assessment and Rating of Ataxia (SARA), International Cooperative Ataxia Rating Scale (ICARS) and Brief Ataxia Rating Scale (BARS)).

Results

Dyskinetic scores were obtained in 79% (DIS); 65% (DIS-D) and 17% (DIS-C) versus dystonic and ataxic scores in 98% (BFMMS) and 89% (SARA/ICARS/BARS) of the children. Age-related DIS and DIS-D scores (B = ?0.90 and 0.77; p < 0.001) were correlated with age-related BFMMS scores (B = ?0.49; p < 0.001; r = 0.87; p < 0.001), whereas DIS-C scores were age-independent. Ataxic scores revealed stronger age-related regression coefficients than dyskinetic and dystonic scores (4–8 years; p < 0.05).

Interpretation

In healthy children, comparison between physiological dyskinesia, dystonia and ataxia rating scale scores revealed: 1. inverse age-relatedness for dystonic and ataxic scores, but not for choreoathetotic scores, 2. interrelated dystonic DIS-D and BFMMS scores, 3. the strongest age-related expression by ataxic scores. In healthy children, these physiological movement disorder-like features are interpreted as an expression of the developing underlying motor centres.  相似文献   

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