International Paediatric Stroke Study: stroke associated with cardiac disorders.

BACKGROUND AND HYPOTHESIS: The aetiologies of arterial ischaemic stroke in children are diverse and often multifactorial. A large proportion occurs in children with cardiac disorders. We hypothesized that the clinical and radiographic features of children with arterial ischaemic stroke attributed to cardiac disorders would differ from those with other causes. METHODS: Using the large population collected in the prospective International Paediatric Stroke Study, we analysed the characteristics, clinical presentations, imaging findings, and early outcomes of children with and without cardiac disorders. RESULTS: Aetiological data were available for 667 children with arterial ischaemic stroke (ages 29 days to 19 years). Cardiac disorders were indentified in 204/667 (30.6%), congenital defects in 121/204 (59.3%), acquired in 40/204 (19.6%), and isolated patent foramen ovale in 31/204 (15.2%). Compared to other children with stroke, those with cardiac disorders were younger (median age 3.1 vs. 6.5 years; P < 0.001) and less likely to present with headache (25.6% vs. 44.6%; P < 0.001), but were similar in terms of gender and presentation with focal deficits, seizures, or recent infection. Analysis of imaging data identified significant differences (P = 0.005) in the vascular distribution (anterior vs. posterior circulation or both) between groups. Bilateral strokes and haemorrhagic conversion were more prevalent in the cardiac disorders group. CONCLUSIONS: Cardiac disorders were identified in almost one-third of children with arterial ischaemic stroke. They had similar clinical presentations to those without cardiac disorders but differed in age and headache prevalence. Children with cardiac disorders more frequently had a 'cardioembolic stroke pattern' with a higher prevalence of bilateral strokes in both the anterior and posterior circulations, and a greater tendency to haemorrhagic transformation.

Postoperative encephalopathy with choreoathetosis.

Since the 1980s, survival of children with CHD has increased significantly with the introduction of new surgical techniques that incorporate cardiorespiratory arrest (CRA), extracorporeal circulation (ECC), and deep hypothermia. However, an increase in survival has been associated with an increase in recognized postoperative neurological complications. Postoperative encephalopathy with choreoathetosis, also known as "postpump chorea", is one of these well-defined neurological complications and was first reported in 1961. Postpump chorea is considered one of the most devastating neurological complications following cardiac surgery. However, the exact etiology and pathophysiology of this complication is unknown. Several factors may contribute to the postoperative development of choreoathetoid movements, including deep hypothermia (core body temperature < 20ºC) with total circulatory arrest, use of cardiopulmonary bypass, and variability in blood pH and PaCO(2) resulting in fluctuations in cerebral blood flow. The length of time children are affected by choreoathetoid movements and long-term neurological outcome in these children varies and largely depends upon the form of postoperative encephalopathy that they develop, described as either mild or severe. Several groups suggest that age at time of surgery plays a role in the risk of developing postpump chorea, with a tendency for older children to develop the severe persistent form.

Interrater reliability of the Pediatric National Institutes of Health Stroke Scale (PedNIHSS) in a multicenter study.

BACKGROUND AND PURPOSE: Stroke is an important cause of death and disability among children. Clinical trials for childhood stroke require a valid and reliable acute clinical stroke scale. We evaluated interrater reliability (IRR) of a pediatric adaptation of the National Institutes of Health Stroke Scale. METHODS: The pediatric adaptation of the National Institutes of Health Stroke Scale was developed by pediatric and adult stroke experts by modifying each item of the adult National Institutes of Health Stroke Scale for children, retaining all examination items and scoring ranges of the National Institutes of Health Stroke Scale. Children 2 to 18 years of age with acute arterial ischemic stroke were enrolled in a prospective cohort study from 15 North American sites from January 2007 to October 2009. Examiners were child neurologists certified in the adult National Institutes of Health Stroke Scale. Each subject was examined daily for 7 days or until discharge. A subset of patients at 3 sites was scored simultaneously and independently by 2 study neurologists. RESULTS: IRR testing was performed in 25 of 113 a median of 3 days (interquartile range, 2 to 4 days) after symptom onset. Patient demographics, total initial pediatric adaptation of the National Institutes of Health Stroke Scale scores, risk factors, and infarct characteristics in the IRR subset were similar to the non-IRR subset. The 2 raters' total scores were identical in 60% and within 1 point in 84%. IRR was excellent as measured by concordance correlation coefficient of 0.97 (95% CI, 0.94 to 0.99); intraclass correlation coefficient of 0.99 (95% CI, 0.97 to 0.99); precision measured by Pearson ρ of 0.97; and accuracy measured by the bias correction factor of 1.0. CONCLUSIONS: There was excellent IRR of the pediatric adaptation of the National Institutes of Health Stroke Scale in a multicenter prospective cohort performed by trained child neurologists.

Ribosomal DNA assay of culture-negative Streptococcus pneumoniae meningitis.

Culture-negative bacterial meningitis with secondary complications remains a significant challenge. Optimal treatment requires identification of the infecting organism. While the gold standard for diagnosis remains cerebrospinal fluid culturing, a significant number of cultures remain negative despite clinical evidence of meningitis. This patient illustrates the usefulness of polymerase chain reaction technology in identifying a specific organism, in an otherwise culture-negative bacterial meningitis with spinal cord abscess.

Postmortem intracranial neuropathology in children following cardiac transplantation.

At Loma Linda University Children's Hospital, the medical information of 405 pediatric patients who received orthotopic cardiac transplantation were reviewed. Of those who died (n=136), 86% (n=117) underwent postmortem examinations, and the brain was examined in 61% (n=82, male=39). The number and type of intracranial lesions present were compiled, and these were matched to underlying functional cardiac disease categories. Intracranial abnormalities were present in 87%. Infarct was the most common primary central nervous system pathology in hypoplastic left heart syndrome (41%) but was also observed frequently in children with obstructive lesions (37%), cyanotic disease (31%), or cardiac shunting (29%). Secondary findings included extraparenchymal hemorrhage in obstructive lesions (31%); hypoxic changes occurred in 15% of patients with cyanotic disease and in 14% of those with cardiac shunting. Thirty-three percent of children with restrictive lesions had no neuropathology reported. Postmortem examination brain weights were matched against age and sex norms, with 29% of females and 36% of males below two standard deviations. These findings revealed that intracranial pathology was present in the majority of transplanted children who underwent postmortem examination, and that infarctive changes constituted the most common neuropathologic abnormality. Additionally, a number of children had significantly reduced brain weight.