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Background and Purpose— Prior annualized estimates of pediatric ischemic stroke incidence have ranged from 0.54 to 1.2 per 100 000 US children but relied purely on diagnostic code searches to identify cases. We sought to obtain a new estimate using both diagnostic code searches and searches of radiology reports and to assess the relative value of these 2 strategies. Methods— Using the population of 2.3 million children (International Classification of Diseases, 9th Revision codes suggestive of stroke and cerebral palsy; and (2) radiology reports for key words suggestive of infarction. Cases were confirmed through chart review. We calculated sensitivities and positive predictive values for the 2 search strategies. Results— We identified 1307 potential cases from the International Classification of Diseases, 9th Revision code search and 510 from the radiology search. A total of 205 ischemic stroke cases were confirmed, yielding an ischemic stroke incidence of 2.4 per 100 000 person-years. The radiology search had a higher sensitivity (83%) than the International Classification of Diseases, 9th Revision code search (39%), although both had low positive predictive values. For perinatal stroke, the sensitivity of the stroke International Classification of Diseases, 9th Revision codes alone was 12% versus 57% for stroke and cerebral palsy codes combined; the radiology search was again the most sensitive (87%). Conclusions— Our incidence estimate doubles that of prior US reports, a difference at least partially explained by our use of radiology searches for case identification. Studies relying purely on International Classification of Diseases, 9th Revision code searches may underestimate childhood ischemic stroke rates, particularly for neonates.

Objective: To determine if diffusion tensor imaging (DTI) metrics of the pyramidal tracts correlate with motor outcome in infants presenting with motor dysfunction. Methods: DTI tractography of the pyramidal tracts was performed in 21 patients with clinical motor dysfunction who were less than 30 months of age and in 22 age-matched controls. We plotted tract-specific DTI metrics (fractional anisotropy, parallel diffusivity, transverse diffusivity, and mean diffusivity) against age for the controls and generated normative curves. For each patient, we calculated the deviation from the normative curves. Patients returned for a neurodevelopmental evaluation when they were over 36 months of age, and motor outcome measures were performed. We analyzed the association between normative deviation in DTI metrics and motor outcome measures using linear and logistic regression models. Results: Normative deviation in fractional anisotropy and transverse diffusivity were significantly correlated with all measures of motor outcome. Lower fractional anisotropy and higher transverse diffusivity compared to controls were associated with worse motor outcome. Furthermore, children who were eventually diagnosed with permanent motor dysfunction had lower fractional anisotropy and higher transverse diffusivity compared with those whose motor dysfunction normalized. Conclusions: Diffusion tensor imaging metrics correlate with motor outcome in infants presenting with motor dysfunction. The identification of a quantitative imaging marker that can be applied to infants at the time of clinical presentation has implications for the evaluation of early motor dysfunction.

Background and Purpose— Although hemorrhagic strokes (HS) account for half of all strokes in children, rates and predictors of recurrent HS have not been studied. Methods— We collected data on all documented cases of HS (intracerebral hemorrhage, subarachnoid hemorrhage, and intraventricular hemorrhage, except neonatal intraventricular hemorrhage), among 2.3 million children ( Results— Among 116 children with atraumatic incident HS followed for a mean of 4.2 years, 11 had a recurrent HS at a median of 3.1 months (range 7 days to 5.7 years), yielding an overall 5-year cumulative recurrence rate (CRR) of 10% (95% CI, 58% to 18%). Sixty-four percent of recurrences were within the first 6 months. Whereas children with idiopathic HS (n=29) had no recurrences, children with structural lesions (vascular malformations or tumors) had a 5-year cumulative recurrence rate of 13% (95% CI, 7% to 25%; 9 recurrences among 71 children; P Conclusions— Overall, 1 in 10 children with HS experienced a recurrence within 5 years, despite available therapies. Whereas idiopathic HS rarely recurred, and HS due to medical etiologies tended to recur acutely, children with structural lesions had a high and prolonged risk for recurrence.

BACKGROUND: Risk factors for ventriculoperitoneal (VP) shunt complications have not been assessed with population cohort data since the advent of modern surgical techniques. We examined demographic factors and VP shunt complications in a population-based retrospective cohort study of all nonfederal California hospital admissions between 1990 and 2000. METHODS: We identified all admissions in a statewide administrative hospital discharge database that indicated aVP shunt insertion. Repeat hospital admissions within California generating a discharge diagnosis of surgical shunt complication, defined as a shunt replacement, revision, removal, or exploration, represented the primary outcome of interest. We performed Kaplan-Meier survival analyses and Cox proportional hazards modeling to analyze sex, age, ethnicity, socioeconomic status based on payer status, and type of hydrocephalus in relation to the risk of shunt complications. RESULTS: The annual population incidence ofVP shunt placement was 5.5 per 100,000. Among 14,455 individuals with 65,040 person-years of follow-up, the cumulative complication rate at 5 years was 32%. Children demonstrated a higher rate of shunt complications than did adults at 5 years (48 versus 27%, P < 0.0001). The following demographic factors were independently associated with increased risk of shunt complications: male sex (hazard ratio [HR], 1.1; 95% confidence interval [Cl], 1.03-1.2), low socioeconomic status (HR, 1.2; 95% CI, 1.1-1.3), and age younger than 19 years (HR, 1.6; 95% CI, 1.4-1.7). Compared with communicating hydrocephalus, obstructive hydrocephalus was also associated with increased risk of shunt complications (HR, 1.7; 95% CI, 1.5-1.9). CONCLUSION: VP shunt complications are common, especially in children. Further study is needed to explain the associations between demographic factors and elevated shunt complication rates.

Using a California-wide hospital discharge database, the authors analyzed all first admissions for stroke in children 1 month through 19 years of age from 1991 through 2000. Incidence rates were estimated as the number of first hospitalizations divided by the person-years at risk; case fatality rates were based on in-hospital deaths.The authors identified 2,278 first admissions for childhood stroke, yielding an annual incidence rate of 2.3 per 100,000 children (1.2 for ischemic stroke, 1.1 for hemorrhagic stroke). Compared with whites, black children were at higher risk of stroke (for ischemic stroke, relative risk [RR] 2.59, 95% CI 2.17 to 3.09, p0.0001; subarachnoid hemorrhage [SAH], RR 1.59, CI 1.06 to 2.33, p = 0.02; intracerebral hemorrhage [ICH], RR 1.66, CI 1.23 to 2.13, p = 0.0001). Hispanics, however, had a lower risk of ischemic stroke (RR 0.70, CI 0.60 to 0.82, p0.0001) and ICH (RR 0.77, CI 0.64 to 0.93, p = 0.0004), whereas Asians had similar risks as whites. Boys were at higher risk for all stroke types than girls (ischemic stroke, RR 1.25, CI 1.11 to 1.40, p = 0.0002; SAH, RR 1.24, CI 1.00 to 1.53, p = 0.047; ICH, RR 1.34, CI 1.16 to 1.56, p = 0.0001). After eliminating cases with coexisting sickle cell disease, excess stroke risk persisted in blacks; after elimination of trauma, excess stroke risk persisted in boys. Case fatality rates were similar among different ethnic groups. Compared with girls, boys had a higher case fatality rate for ischemic stroke (17 vs 12%; p = 0.002) but not for ICH or SAH.Rates of hospitalization for stroke are higher among black children and boys; sickle cell disease and trauma do not fully account for these findings.

Objective: To investigate the excess mortality due to remote symptomatic epilepsy, taking account of frequency and type of seizures.Methods: The authors compared mortality in persons with (n = 8,156) and without (n = 72,526) history of epilepsy in a 1988 to 1999 California population of persons with mild developmental disabilities. Subjects had traumatic brain injury, cerebral palsy, Down syndrome, autism, or no identifiable condition. There were 506,204 person-years of data, with 1,523 deaths. Excess death rates and standardized mortality ratios were computed for the persons in the study with epilepsy, relative to those in the study without epilepsy. Controlled comparisons were made using logistic regression on person-years.Results: Compared to subjects with no epilepsy, the excess mortality was six (deaths per 1,000 persons per year) for persons with a recent (Conclusions: Persistent seizures are associated with increased mortality in remote symptomatic epilepsy. Mortality is highest among individuals with status epilepticus or generalized convulsions.

Studies have documented declining mortality from stroke in adults over the past two decades, with black adults at greater risk of death from stroke than whites in all years. As these findings have been attributed to control of stroke risk factors that are less important in children, trends and demographics of childhood stroke mortality are of interest, but have not been explored.The authors analyzed death certificate data for ischemic and hemorrhagic stroke (subarachnoid hemorrhage [SAH] and intracerebral hemorrhage [ICH]) in children under 20 years of age in the United States for the years 1979 through 1998, covering approximately 1.5 billion person-years.Childhood mortality from stroke declined by 58% overall, with reductions in all major subtypes (ischemic stroke decreased by 19%; SAH, by 79%; ICH, by 54%). Black ethnicity was a risk factor for mortality from all stroke types (relative risk 1.74 for ischemic stroke; 1.76 for SAH; 2.06 for ICH; p0.0001 for all types). Male sex was a risk factor for mortality from SAH (relative risk 1.30, p0.0001) and ICH (relative risk 1.21, p0.0001), but not from ischemic stroke (relative risk 1.02, p = 0.76).Mortality from stroke in US children has decreased dramatically over the last 20 years. Black children are at greater risk of death from all stroke types than are white children. As control of known stroke risk factors is unlikely to account for declining stroke mortality and ethnic differences in children, unrecognized stroke risk factors may be important.

Objective The objective of the study was to determine the relationship between nighttime delivery and neonatal encephalopathy (NE). Study Design The design of the study was a retrospective population-based cohort of 1,864,766 newborns at a gestation of 36 weeks or longer in California, 1999-2002. We determined the risk of NE associated with nighttime delivery (7:00 pm to 6:59 am ). Results Two thousand one hundred thirty-one patients had NE (incidence 1.1 per 1000 births). Nighttime delivery was associated with increased NE (odds ratio [OR], 1.13; 95% confidence interval [CI], 1.03–1.20), birth asphyxia (OR, 1.18; 95% CI, 1.08–1.29), and neonatal seizures (OR, 1.17; 95% CI, 1.07–1.28). In adjusted analyses, nighttime delivery was an independent risk factor for NE (OR, 1.10; 95% CI, 1.01–1.21), as were severe intrauterine growth retardation (OR, 3.8; 95% CI, 3.1–4.8); no prenatal care (OR, 2.0; 95% CI, 1.4–2.9); primiparity (OR, 1.5; 95% CI, 1.4–1.7); advanced maternal age (OR, 1.3; 95% CI, 1.16–1.45); and infant male sex (OR, 1.3; 95% CI, 1.2–1.4). Conclusion Future studies of time of delivery may generate new strategies to reduce the burden of NE.

OBJECTIVE Racial and ethnic disparities in cerebral palsy have been documented, but the underlying mechanism is poorly understood. We determined whether low birth weight accounts for ethnic disparities in the prevalence of cerebral palsy and whether socioeconomic factors impact cerebral palsy within racial and ethnic groups. METHODS In a retrospective cohort of 6.2 million births in California between 1991 and 2001, we compared maternal and infant characteristics among 8397 infants with cerebral palsy who qualified for services from the California Department of Health Services and unaffected infants. RESULTS Overall, black infants were 29% more likely to have cerebral palsy than white infants (relative risk: 1.29 [95% confidence interval: 1.19-1.39]). However, black infants who were very low or moderately low birth weight were 21% to 29% less likely to have cerebral palsy than white infants of comparable birth weight. After we adjusted for birth weight, there was no difference in the risk of cerebral palsy between black and white infants. In multivariate analyses, women of all ethnicities who did not receive any prenatal care were twice as likely to have infants with cerebral palsy relative to women with an early onset of prenatal care. Maternal education was associated with cerebral palsy in a dose-response fashion among white and Hispanic women. Hispanic adolescent mothers (aged

Neonatal encephalopathy remains a major cause of neurologic and developmental disability in term infants. Approximately one in 5 affected infants die during the neonatal period, and another one fourth have permanent motoror cognitive dysfunction. Previous studies suggest that brain injury occurs at-or near-the time of birth and so might be helped by postnatal interventions. The investigators used magnetic resonance imaging (MRI) to define patterns of injury in 173 term newborn infants having neonatal encephalopathy. MRI was done 1 to 24 days after birth, the median interval being 6 days. MRI distinguished between a watershed-predominant pattern (involving white matter, especially in the vascular watershed and possibly extending to cortical gray matter); a basal ganglia/thalamus-predominant pattern (involving the deep gray nuclei and perirolandic cortex, extending to the total cortex in severe cases); and a normal state. Neuroradiologists without knowledge of the clinical findings interpreted the MRI scans. Cognitive development was assessed by the Mental Development Index of the Bayley Scales of Infant Development II. The watershed pattern of injury was found in 45% of infants and the basal ganglia/thalamus pattern in 25%; 30% had normal MRI findings. Injury patterns could not be related to maternal substance use, gestational diabetes, premature rupture of membranes, preeclampsia, or intrauterine growth restriction. The basal ganglia/thalamus pattern of injury was associated with more severe clinical signs, more intensive resuscitation at birth, more advanced encephalopathy, and more severe seizures. This pattern also correlated with the most marked impairment of motor and cognitive outcomes at age 30 months. No newborn infant with normal MRI findings had a functional motor deficit. More than half (56%) of surviving infants with the basal ganglia/thalamus pattern had spastic quadriparesis. Additional watershed injury did not worsen neuromotor scores when the basal ganglia/thalamus-predominant pattern was observed. Neuromotor scores did not change from ages 12 to 30 months. These findings show that different patterns of brain injury in term infants with neonatal encephalopathy are associated with different neurodevelopmental outcomes. Knowledge of the pattern of injury can help to identify infants who might benefit most from measures that protect the brain after injury as well as those who may benefit from rehabilitative services.

Objective: Fahr9s disease is a well-described neurodegenerative disorder in adults, but few reports describe it in pediatric patients. We present two children with Fahr9s disease. Background Fahr9s disease is a rare progressive disorder characterized by idiopathic bilateral calcification of the basal ganglia. The underlying etiology is unknown. Patients typically exhibit a movement disorder and psychiatric symptoms. Diagnosis is based on characteristic imaging findings and the exclusion of systemic causes of secondary basal ganglia calcification. Design/Methods: We describe two teenagers who presented to an academic center with neurologic complaints, and who were diagnosed with Fahr9s disease. Results: Case one. DJ is a 14 year old girl with a two year history of slowly progressive movement disorder characterized by dystonia, poor balance and bradykinesia, and progressive psychiatric symptoms of aggression and impulse control disorder. Brain MRI showed symmetrical T2 shortening in bilateral globus pallidi. Head CT showed heavy mineralization within bilateral globus pallidi consistent with calcium deposits. Systemic causes of secondary basal ganglia calcification were ruled out, and family history was non-contributory. Case two. TU is a 14 year old boy with a two year history of severe anxiety and difficulty with social interactions. He recently developed simple motor tics, but has no other movement disorder. Head CT showed bilateral calcifications in the deep grey nuclei, mostly within globus pallidi, but also involving the thalamus and white matter. Brain MRI confirmed the presence of bilateral mineralization within bilateral globus pallidi. Systemic causes of secondary basal ganglia calcification were ruled out, and family history was non-contributory. Conclusions: Fahr9s disease, a neurodegenerative disorder typically diagnosed in late adulthood, has rarely been described in children. We present two teenagers with classic clinical and radiographic features of Fahr9s disease. Children with a movement disorder and psychiatric symptoms should be evaluated for Fahr9s disease. Disclosure: Dr. Singhal has nothing to disclose. Dr. Van Haren has nothing to disclose. Dr. Wu has nothing to disclose.

ContextChorioamnionitis has been implicated in the pathogenesis of cerebral palsy, but most studies have not reported a significant association. Cystic periventricular leukomalacia (cPVL) is believed to be a precursor of cerebral palsy in preterm infants.ObjectivesTo determine whether chorioamnionitis is associated with cerebral palsy or cPVL and to examine factors that may explain differences in study results.Data SourcesSearches of MEDLINE (1966-1999), Index Medicus (1960-1965), Doctoral Dissertation Abstracts On-Line (1861-1999), bibliographies, and online conference proceedings (1999) were performed for English-language studies with titles or abstracts that discussed prenatal risk factors for cerebral palsy or cPVL.Study SelectionOf 229 initially identified publications, meta-analyses were performed on studies that addressed the association between clinical (n = 19) or histologic (n = 7) chorioamnionitis and cerebral palsy or cPVL in both preterm and full-term infants. Inclusion criteria were: presence of appropriate exposure and outcome measures, case-control or cohort study design, and provision of sufficient data to calculate relative risks (RRs) or odds ratios with 95% confidence intervals (CIs). Studies evaluating risk of cerebral palsy following maternal fever, urinary tract infection, or other maternal infection were collected, but not included in the meta-analysis.Data ExtractionInformation from individual studies was abstracted using standardized forms by 2 independent observers blinded to authors' names, journal titles, and funding sources.Data SynthesisUsing a random effects model, clinical chorioamnionitis was significantly associated with both cerebral palsy (RR, 1.9; 95% CI, 1.4-2.5) and cPVL (RR, 3.0; 95% CI, 2.2-4.0) in preterm infants. The RR of histologic chorioamnionitis and cerebral palsy was 1.6 (95% CI, 0.9-2.7) in preterm infants, and histologic chorioamnionitis was significantly associated with cPVL (RR, 2.1; 95% CI, 1.5-2.9). Among full-term infants, a positive association was found between clinical chorioamnionitis and cerebral palsy (RR, 4.7; 95% CI, 1.3-16.2). Factors explaining differences in study results included varying definitions of clinical chorioamnionitis, extent of blinding in determining exposure status, and whether individual studies adjusted for potential confounders.ConclusionOur meta-analysis indicates that chorioamnionitis is a risk factor for both cerebral palsy and cPVL.

Background: Although Chiari I malformation is increasingly recognized in children, little is known about its clinical presentation in this age group. Objective: To evaluate the relationship between clinical and MRI features of pediatric Chiari I malformations. Methods: We performed a chart review and MRI analysis of 49 children with Chiari I malformation. The degree of tonsillar ectopia was compared with age at onset, presence of syringomyelia, and a neurologic severity score as follows: asymptomatic = 0, symptomatic with normal neurologic examination = 1, and symptomatic with abnormal examination = 2. Results: Age at onset of symptoms ranged from 10 months to 14 years. Fifty-seven percent of patients were asymptomatic. Headache and neck pain were the most frequent complaints. Syringomyelia was detected in 14% of patients and skull base abnormalities in 50%. The magnitude of tonsillar ectopia (5 to 23 mm) correlated with severity score ( p = 0.04) but not with other clinical measures. Conclusions: The clinical symptoms of Chiari I malformations in children are nearly identical to those seen in adults. Children with greater amounts of tonsillar ectopia on MRI are more likely to be symptomatic.