Our objective was to determine the incidence rate of stroke and stroke subtypes in children. We reviewed the medical records, autopsy records, and brain imaging studies of all children with a possible stroke within the Greater Cincinnati metropolitan area population of nearly 1.3 million during 1988 and 1989. Traumatic brain hemorrhages and germinal matrix hemorrhages were excluded. Of the 295,577 children in Greater Cincinnati, medical records of 178 children were screened. Sixteen cases (13 whites and three blacks) less than age 15 years fit strictly defined criteria for first-ever stroke. The incidence rate for cerebral infarction was 1.2 cases per 100,000 (95% confidence interval, 0.3 to 2.0). The combined incidence rate for intracerebral hemorrhage and subarachnoid hemorrhage was 1.5 cases per 100,000 children (95% confidence interval, 0.4 to 2.3). The incidence rate of all stroke in white children was 2.6 cases per 100,000 (95% confidence interval, 1.2 to 4.1), compared to 3.1 per 100,000 in black children (95% confidence interval, 0 to 6.6). The combined 30-day mortality for intracerebral hemorrhage and subarachnoid hemorrhage was 22% (two of nine) compared to 14% (one of seven) for cerebral infarction. We conclude that in contrast to the picture in adults, hemorrhagic stroke among infants and children is at least as common as ischemic infarction.

Summary:Purpose: To present the results of transcallosal surgical resection of hypothalamic hamartoma (HH) in 26 patients with refractory epilepsy in a prospective outcome study.Methods: Patients with refractory epilepsy symptomatic to HH were referred for surgical resection of their HH (mean age, 10.0 years; range, 2.1-24.2 years). A transcallosal, interforniceal approach was used to remove and/or disconnect the hamartoma. Volumetry was obtained on pre-and postoperative brain MRI scans to determine percentage of resection. Outcome assessment included determination of postoperative seizure frequencies in comparison to baseline and the incidence of postoperative complications. Postoperative changes in cognitive and behavioral functioning, in comparison to baseline, were elicited by parental report.Results: The average postoperative follow-up interval was 20.3 months (range, 13-28 months). Fourteen (54%) patients were completely seizure free, and nine (35%) had at least a 90% improvement in total seizure frequency. Parents reported postoperative improvement in behavior in 23 (88%) patients and in cognition in 17 (65%) patients. Transient postoperative memory disturbance was seen in 15 (58%) patients, but persisted in only two (8%). Two (8%) patients had persisting endocrine disturbance requiring hormone replacement therapy (diabetes insipidus and hypothyroidism in one each). With univariate analysis, the likelihood of a seizure-free outcome correlated with younger age, shorter lifetime duration of epilepsy, smaller preoperative HH volume, and 100% HH resection.Conclusions: Refractory epilepsy associated with HH can be safely and effectively treated with surgical resection by a transcallosal, interforniceal approach. Short-term memory deficits appear to be transient for most patients, and family perception of the impact of surgery on cognitive and behavioral domains is favorable. Complete resection yields the best result.

No abstract

Hypothalamic hamartomas (HHs) are rare developmental tumors that cause seizures or pituitary axis dysfunction, usually beginning in childhood. We analyzed HH tissue from 57 patients whose tumors were resected through recently developed transcallosal interforniceal and transventricular endoscopic surgical approaches. All cases were composed of abnormally distributed but cytologically normal neurons and glia, including fibrillary astrocytes and oligodendrocytes. Neuronal elements predominated in most cases, but a relative increase in astrocytic elements was seen with increasing age. All had various sized nodular foci of neurons as well as areas of diffusely distributed neurons with interspersed glial cells. Smaller neurons predominated, and most cases had only a few interspersed large ganglion cells. Immunohistochemistry demonstrated extensive production of synapse-associated proteins. Immunohistochemistry for phosphorylated and nonphosphorylated neurofilament and alpha-internexin demonstrated staining patterns consistent with mature neurons. In contrast to cortical dysplasia, atypical large ganglion-like balloon cells were almost never seen. In summary, although their number and distribution vary, mature smaller neurons were the most prominent and most consistent histologic feature of HH. Nodules of these small neurons were a universal feature of the microarchitecture of HH lesions associated with epilepsy. Characterization of these neurons may aid in understanding the mechanism of seizure development in HH.