Enrique C. G. Ventureyra

Traumatic intracranial aneurysms in childhood and adolescence

We report four pediatric traumatic intracranial aneurysms occurring before the age of 10 years. Two of these aneurysms were the results of closed head injury. The remaining two were iatrogenic aneurysms which occurred in unusual circumstances. These four children represent 33% of the pediatric intracranial aneurysms seen at the Childrens Hospital of Eastern Ontario from 1974 to 1992. Diagnosis of traumatic intracranial aneurysms requires a high index of suspicion: any head-injured or postoperative child who experiences delayed neurologic deterioration, or who fails to improve as expected following treatment, should promptly undergo diagnostic intracranial imaging. Documented subarachnoid hemorhage, intracerebral or intraventricular hemorrhage, or subdural haematoma in this clinical setting should be further investigated by cerebral angiography to exclude a traumatic aneurysm or other vascular lesion. Traumatic aneurysms typically arise at the skull base or from distal anterior or middle cerebral arteries or branches consequent to direct mural injury or to acceleration-induced shear. Reported traumatic aneurysms account for 14%–39% of all pediatric aneurysms. Iatrogenic aneurysms also occur with unecpected frequency during. childhood and adolescence. Pediatric traumatic cerebral aneurysms may present early or late. Most present early with intracranial hemorrhage. Late presentation occurs infrequently, typically as an aneurysmal mass. Once diagnosed, these aneurysms should be promptly treated by craniotomy employing routine microsurgical techniques, or in some cases, by endovascular detachable balloon techniques. Delay in operative treatment entails significant risks of repeated hemorrhage and death. Outcome in these children is primarily determined by the extent of traumatic cerebral injury and the preoperative clinical status. The latter directly depends upon diagnosis of the aneurysm prior to either initial or repeated hemorrhage.

The effects of head injury in children on neuropsychological and behavioural functioning

In a prospective study 76 children were divided into three groups on the basis of severity of head injury as defined by the Glasgow Coma Scale and duration of increased intracranial pressure. The children were administered a neuropsychological test battery and behavioural ratings were made by parents and teachers at three intervals: time of hospital discharge and 3 and 9 months post-initial testing. There were cognitive deficits related to severity of injury with the greatest difference in abilities observed between the severe and the other two groups. The greatest differences in skills were on the Performance IQ and timed tests of visual-motor speed and co-ordination. The greatest improvement in skills occurred in the first 3 months post-injury. Several children in coma for up to 4 weeks were able to obtain normal IQ scores. In the mild and moderate injury groups very few had behavioural change while in the severe group approximately 90% had one learning or adjustment difficulty and 40% had three or more problems.

Transcutaneous vagus nerve stimulation for partial onset seizure therapy

Abstract A new concept for transcutaneous vagus nerve stimulation for control of partial onset seizures is described. The rationale for the application of this innovative noninvasive method is discussed.

Spontaneous intracranial hemorrhage of structural origin during the first year of life

Abstract Symptomatic intracranial hemorrhage (ICH) in term infants is not common, but when it occurs it is usually secondary to trauma, coagulation disorders and/or hypoxia. The possibility of a structural cause for an infantile ICH is unfortunately not seriously considered until very late. In this paper we report the cases of five full-term infants, each of whom developed ICH secondary to a structural lesion during the 1st year of life. Three presented during the newborn period. A congenital saccular aneurysm of the middle cerebral artery in an 8-month old male infant; a posterior fossa arteriovenous malformation in a 2-week old female neonate; a deep parietal cavernous angioma in a 6.5-month-old male infant; a temporoparietal low-grade astrocytoma in a 12-day old male neonate and a temporoparietal desmoplastic ganglioglioma in a 9-day-old male neonate were the structural lesions that were causative for hemorrhage. In all cases but one, the diagnosis was reached by computerized tomography and/or magnetic resonance imaging. All infants underwent surgery for the removal of the hematoma and of the lesion causative for the bleed. All are alive at 19, 3, 11.5, 10, and 5 years, respectively. We discuss the diagnosis of ICH with special emphasis on contemporary imaging modalities and stress the benefits of aggressive and timely surgical treatment. We then consider a concise analysis of the world literature on the occurrence of structural causes of ICH during infancy.

Cortical spreading depression releases ATP into the extracellular space and purinergic receptor activation contributes to the induction of ischemic tolerance

Cortical Spreading Depression (CSD) is a well-studied model of preconditioning that provides a high degree of tolerance to a subsequent ischemic event in the brain. The present study was undertaken in order to determine whether the release of ATP during CSD could contribute to the induction of ischemic tolerance. Direct measurement of ATP levels during CSD indicates that with each CSD wave ATP is released into the extracellular space at levels exceeding 100 microM. Cultures of rat primary cortical neurons exposed to low levels of extracellular ATP developed tolerance to subsequent oxygen-glucose deprivation (OGD) or metabolic hypoxia. The preconditioning effect requires new protein synthesis and develops with time, suggesting that a complex genomic response is required for the induction of tolerance. Multiple purinergic receptors are involved in mediating tolerance, with P2Y receptor activation having the greatest effect. Although extracellular adenosine or glutamate may make a small contribution, most of the tolerance was found to be induced independently of adenosine or glutamate receptor activation. Multiple signal transduction pathways mediate the response to extracellular ATP with the protein kinase A pathway and activation of phospholipase C contributing the most. The results are consistent with the proposal that CSD releases ATP into the extracellular space and the subsequent activation of P2Y receptors makes a major contribution to the induction of ischemic tolerance in the brain.

Medical and cognitive outcome in children with traumatic brain injury.

BACKGROUND Head injury is an important cause of morbidity and mortality in pediatrics. Comprehensive studies on outcome are scarce despite significant clinical concern that multiple areas of functioning may be impaired following moderate to severe head injury. The literature suggests that sequelae include not only medical problems but also impairments in cognitive functioning. METHODS A retrospective medical and psychology chart review of patients, age 1-18 years, admitted to the Childrens Hospital of Eastern Ontario with moderate (Glasgow Coma Scale [GCS] 9-12) or severe head injury (GCS < or = 8) from November 1, 1993 until December 31, 1998 was conducted. Correlations were performed between medical variables (i.e., GCS, Pediatric Risk of Mortality [PRISM] III score, duration of ICU and hospital stay) and measures of intelligence and memory functioning. RESULTS Eighty-three children age 1 to 18 were included. Seventy percent of the children were classified as having a severe head injury. There was a mortality rate of thirteen percent. Younger age at injury, lower GCS, and higher PRISM III scores predicted higher mortality. Medical complications were documented systematically. Forty-four patients underwent at least one cognitive assessment and 17 of these children had intelligence testing at three points in time: baseline (< four months), early recovery (five to 15 months) and follow-up (16 to 38 months). The mean intelligence and memory scores fell within the average range at the latest point in follow-up. For those children who underwent three serial assessments, the mean verbal and performance IQ fell within the low average range at baseline improving significantly to fall within the average range by early recovery. Continued improvements were apparent in verbal memory beyond early recovery, with the mean obtained at follow-up falling within 1 SD of the normative mean. Despite the return to normal ranges for the group means the proportion of scores falling below 1.5 standard deviations from the mean was greater than population norms for verbal IQ, performance IQ and verbal memory. Lower GCS scores and longer duration of stay in ICU or hospital were predictive of lower nonverbal intelligence. Lower GCS was also predictive of lower visual memory scores. CONCLUSIONS This study describes a population of Canadian children who suffered moderate or severe traumatic brain injury. Initial GCS was the best predictor of mortality and cognitive outcome. These children demonstrated a temporal improvement in intelligence and memory functioning, with their mean performance on these cognitive measures falling within the average range at 16 to 38 months postinjury, although there was considerable variability in the outcomes between individuals.

Brain tumors in childhood and adolescence

Brain tumors are the second most common neoplasm in childhood and adolescence. With the recent advances in technology, changes in tumor incidence have been reported. This study examines this statement. A 19-year retrospective case review of primary brain tumors in persons younger than 18 years of age at time of diagnosis, who had permanent residence in our catchment area, was performed. Data were examined for changes in presenting symptoms and signs and incidence rates for tumors on the basis of anatomic location and histologic tumor type. An incidence rate of 2.76 per 100,000 people younger than 18 years of age was found. During the period of this study a small, but significant, trend toward increasing incidence was evident. No changes in patterns of presentation or duration of symptoms before diagnosis was observed. The incidence rate based on histologic tumor diagnosis remained fairly constant during the study period.

Bone marrow metastasis in astrocytic gliomata

With the increasing survival time of many pediatric patients with malignancies, unexpected symptoms or signs require diligent search for rare complications or second cancers related to the disease or treatment. We recently encountered a patient with extensive glioblastoma multiforme who developed pancytopenia six months after completion of treatment with craniospinal radiation and chemotherapy with etoposide and cyclophosphamide. Bone marrow aspirate and biopsy confirmed bone marrow metastasis from the brain tumor. He showed good partial remission with chemotherapy with carmustine and cis-platinum as demonstrated by serial bone marrow aspirate for cytology and cytogenetics and enjoyed good quality of life for eight months. 14 other patients with astrocytic glioma, two of whom are children, are reported in the literature to have diffuse bone marrow metastasis. Therefore, in patients with malignant astrocytic tumor, bone marrow metastasis, though not common, should be considered when bone pain or cytopenias occur, especially when prolonged.

Epilepsy surgery in the first 3 years of life: A Canadian survey

Objective:  To determine the clinical characteristics, surgical challenges, and outcome in children younger than 3 years of age undergoing epilepsy surgery in Canada.

Temporal lobe gangliomas in children

The ganglioglioma, a mixed tumor with neuronal and glial elements, occurs occasionally in children and young adults. Its incidence, clinical presentation, radiological features, and natural history have not been clearly defined. A review of 111 primary brain tumors treated at our institution shows that 9.0% were gangliogliomas. The temporal lobe was the most common site involved. All patients presented with seizures but were neurologically intact. The CT scan showed a partially calcified mass, frequently isodense or nearly so, with minimal enhancement. Complete removal produced dramatic improvement in seizure control. Radiotherapy and/or chemotherapy are reserved for tumors showing malignant changes in the astrocytic component. Patients with chronic epilepsy, or whose seizures escape control despite anticonvulsants, should be examined with this tumor in mind. Following removal of these low-grade temporal lobe tumors, fewer seizures are seen, and the long-term prognosis is very good.