Cerebral Palsy after Neonatal Encephalopathy: How Much Is Preventable?

OBJECTIVES: To determine the expected proportion of term cerebral palsy (CP) after neonatal encephalopathy (NE) that could theoretically be prevented by hypothermia and elucidate the perinatal factors associated with CP after NE in those who do not meet currently used clinical criteria required to qualify for hypothermia ("cooling criteria"). STUDY DESIGN: Using the Canadian CP Registry, we categorized children born at ≥ 36 weeks with birth weight ≥ 1800 g with CP after moderate or severe NE according to the presence or absence of cooling criteria. Maternal, perinatal, postnatal, and placental factors were compared between the 2 groups. A number needed to treat of 8 (95% CI 6-17) to prevent one case of CP was used for calculations. RESULTS: Among the 543 term-born children with CP, 155 (29%) had moderate or severe NE. Sixty-four of 155 (41%) met cooling criteria and 91 of 155 (59%) did not. Shoulder dystocia was more common in those who did not meet cooling criteria (OR 8.8; 95% CI 1.1-71.4). Low birth weights (20% of all singletons), small placentas (42%), and chorioamnionitis (13%) were common in both groups. CONCLUSIONS: The majority of children with CP after NE did not meet cooling criteria. An estimated 5.1% (95% CI 2.4%-6.9%) of term CP after NE may be theoretically prevented with hypothermia. Considering shoulder dystocia as an additional criterion may help recognize more neonates who could potentially benefit from cooling. In all cases, a better understanding of the antenatal processes underlying NE is essential in reducing the burden of CP.

Normal imaging in patients with cerebral palsy: what does it tell us?

OBJECTIVE: To identify distinctive clinical features characterizing children with cerebral palsy (CP) and normal-appearing magnetic resonance imaging (MRI) findings. STUDY DESIGN: Using a population-based CP registry, the Registre de la Paralysie Cérébrale au Québec (Quebec Cerebral Palsy Registry), various antenatal, perinatal, and postnatal predictor variables, as well as current phenotype, were compared in patients with normal-appearing MRI findings and those with abnormal MRI findings. RESULTS: Of the 213 patients evaluated, 126 (60%) had MRI imaging results available and were included in our analysis. Of these 126 patients, 90 (71%; 51 males, 39 females) had abnormal findings and 36 (29%; 17 males and 19 females) had normal-appearing findings. Compared with other CP variants, normal-appearing MRI was more prevalent (P = .001) in dyskinetic CP (72.7%; 8 of 11) and less prevalent (P = .002) in spastic hemiplegic CP (10%; 4 of 40). There were no significant differences between the 2 groups (P > .05) in terms of the prevalence of perinatal or postnatal clinical features or clinical outcomes. Furthermore, 42% (15 of 36) of the children with normal-appearing MRI exhibited a high degree of functional disability (Gross Motor Functional Classification System IV-V), compared with 33% (30 of 90) with abnormal MRI. CONCLUSION: No clinical features, except a higher prevalence of dyskinetic CP, was identified in the children with normal-appearing MRI. More refined imaging techniques may be needed to evaluate patients with normal-appearing MRI findings. Furthermore, genetic or functional, rather than gross structural lesions, may underlie the pathophysiology of CP in this cohort. Finally, the high proportion of substantial functional disability underscores the importance of continuous follow-up even in the absence of early structural abnormalities on imaging.

Clinical clues to differentiating inherited and noninherited etiologies of childhood ataxias.

OBJECTIVE: To identify clinical features at presentation that differentiate inherited and noninherited etiologies of childhood ataxias. STUDY DESIGN: A retrospective chart review analysis was conducted on 167 patients evaluated in neurology outpatient clinics for ataxia or ataxia-related symptoms. The frequency of clinical features, determined a priori, in the 2 groups was compared. RESULTS: A larger proportion of patients were diagnosed with a nongenetic cause than with a genetic cause (89% [148 patients] vs 11% [19 patients]). The majority of patients in the nongenetic group (56% [83/148]) presented early for medical evaluation, compared with 31% (6/19) in the genetic group. Consanguinity (16% vs 4%) and positive family history (16% vs 2%) were more frequent in the genetic group. Presenting symptoms of abnormal gait (95% vs 57%) and muscle weakness (47% vs 8%), including physical findings of abnormal muscle tone (63% vs 32%), abnormal reflexes (63% vs 16%), clonus (26% vs 9%), dysmetria (32% vs 5%), pes cavus (21% vs 1%), sensory deficits (16% vs 0%), and nonneurologic musculoskeletal abnormalities (58% vs 19%), were more prevalent in the genetic group. CONCLUSION: Certain clinical features can help delineate between inherited and noninherited causes of childhood ataxia and thus guide physicians in the targeted evaluation of patients.

Motor function at school age in children with a preschool diagnosis of developmental language impairment.

OBJECTIVES: To evaluate fine motor (FM) and gross motor (GM) function shortly after school entry in children with a preschool diagnosis of developmental language impairment (DLI). STUDY DESIGN: A cohort of children (n = 70) diagnosed at pre-school age with DLI was reevaluated in elementary school. Language, cognitive, and motor outcomes were assessed through the use of the Battelle Developmental Inventory (BDI). Language was further assessed through the use of the Vineland Adaptive Behavior Scale, Peabody Picture Vocabulary, and Expressive One Word Picture Vocabulary Tests. Performance below -1.5 SD of the normative mean on any test was considered to represent impairment. RESULTS: Forty-three children (mean age, 7.4 +/- 0.7 years) underwent reassessment at a mean of 3.8 +/- 0.7 years after initial preschool assessment. Mean scores for BDI motor domains (FM, 78.3 +/- 11.4; GM, 84.9 +/- 13.3) fell below normative values. Twenty-two children (52%) had motor impairment (FM, 17 of 42; GM, 15 of 42); 35 of 43 (81%) continued to have language impairment. BDI communication raw scores correlated most strongly with FM (rho = 0.73, P < .001) and GM (rho = 0.58, P = .003) raw scores but showed only moderate correlations with cognitive raw scores (rho = 0.41, P = .05). CONCLUSIONS: Impaired motor function is an important comorbidity in DLI. Factors critical to motor performance may also contribute to language deficits in DLI.

Predictors of developmental disabilities after open heart surgery in young children with congenital heart defects.

OBJECTIVES: To determine the prevalence of persistent developmental impairments in children with congenital heart defects and to identify factors that enhance risk for an adverse outcome. STUDY DESIGN: Eligible infants (n = 131) <2 years of age requiring open heart surgery were recruited prospectively. Subjects were assessed during surgery and again 12 to 18 months later with standardized developmental assessments and formal neurologic examinations. RESULTS: Mean age at follow-up testing was 19.1 +/- 6.6 months. Assessments indicated that 41% had abnormal neurologic examinations. Gross and/or fine motor delays were documented in 42%, and 23% demonstrated global developmental delay. Univariate and multiple regression models identified the following factors increasing the risk for persistent developmental deficits: preoperative and acute postoperative neurodevelopmental status and microcephaly, type of heart lesion, length of deep hypothermic circulatory arrest, age at surgery, and days in the intensive care unit (P <.05). CONCLUSIONS: Children with congenital heart defects commonly have ongoing neurologic, motor, and developmental deficits well after surgical correction. The cause is multifactorial and includes brain injury before, during, and after heart surgery.