Epidermoid cysts are tumors familiar to neurosurgeons, but intramedullary epidermoid cysts are rare. The authors report the case of a 6-year-old girl presenting with progressive paraparesis. A midthoracic intramedullary mass was revealed on myelography and magnetic resonance (MR) imaging and confirmed as an intramedullary epidermoid cyst at surgery, at which time the cyst was removed. This is the fourth report documenting a purely intramedullary epidermoid cyst occurring in a child. The pathology and etiology, epidemiology, clinical features, radiology (including MR image characteristics), and surgical treatment of such rare intramedullary benign tumors are discussed. Magnetic resonance imaging reduces the delay in diagnosis of spinal cord tumors but should be guided by clinical judgment.

BACKGROUND.Surgical removal and radiation therapy are associated with significant risk for morbidity in the pediatric population with craniopharyngioma. Intracystic therapies have been utilized in some centers to potentially decrease morbidity associated with cystic craniopharyngioma. The aim of the study was to review the Canadian experience with intracystic bleomycin therapy (ICB).METHODS.All centers in the Canadian Pediatric Brain Tumor Consortium (CPBTC) were invited to participate in a retrospective review of this treatment. A questionnaire was sent to each center. The data were analyzed at British Columbia's Children's Hospital.RESULTS.In all, 6 of 17 centers utilized ICB and submitted data. A total of 17 of 19 patients with the intention to treat received bleomycin. Twelve were treated at the time of diagnosis, and 5 at the time of recurrence. Five patients achieved a complete response, 6 achieved a partial response, and 5 achieved a minor response to bleomycin. One patient was stable for 2.8 years. At the time of last follow‐up, 8 patients have not required further intervention. Complications included transient symptomatic peritumoral edema (2 patients), precocious puberty (1 patient) and panhypopituitarism (2 patients). The median follow‐up was 4 years (range, 0.5–10.2 years). The median progression‐free survival was 1.8 years (range, 0.3–6.1 years). One patient died of a massive infarct secondary to radiation‐induced moyamoya syndrome.CONCLUSIONS.ICB was found to be well tolerated in this group of children. ICB may be a feasible and effective therapy for certain children with craniopharyngioma. Bleomycin may delay the need for aggressive surgery or radiation therapy for several years. Prospective multiinstitutional clinical trials are required to further evaluate the feasibility, effectiveness, and dose schedules of this treatment. Cancer 2007. © 2007 American Cancer Society.

Vagal nerve stimulation (VNS) has shown promising results in various cohorts of non-surgical refractory epilepsy in adults and children. However studies report a significant delay between implantation and clinical response. We describe a cohort of 28 children and adolescents prospectively followed, classified by epileptic syndromes and treated with VNS using a 6-week rapid ramping protocol between January 2000 and March 2005. Our cohort showed favorable outcome within 6 months which was sustained at 24 months: 68% (19/28) showing >or=50% reduction in seizure frequency, including 14% (4/28) who became seizure-free. VNS was particularly efficacious in children with cryptogenic generalized and partial epilepsies. Although adverse events occurred in 68% (19/28) of patients, most were transient. In conclusion, rapid ramping is associated with an early and lasting response in most children but with a slightly higher side-effect rate.

Clone pTB16 has been isolated by differential screening of a human glioma cDNA library. Northern blot analysis has shown that pTB16 expression is several times (>11-fold) higher in gliomas than in a primitive neuroectodermal tumor. This observation was supported by in situ hybridization and extended to nine other gliomas. Expression was virtually absent in adenocarcinoma cells metastasized to brain. Malignant gliomas showed stronger hybridization than benign gliomas, while blood capillaries did not show hybridization. pTB16 mRNA was also shown to be expressed in established glioma cell lines and at high levels in epileptic foci, indicating that expression of the gene may be limited to certain cell types and that its upregulation is not merely a consequence ofcellular proliferation. Nucleotide sequence analysis identified pTB16 as the human counterpart for rat testicular sulfated glycoprotein 2 (SGP-2), whose function in the reproductive system remains unknown. Although SGP-2 transcripts, and hence pTB16, were recently shown to be increased in neurodegenerative diseases such as scrapie in hamsters and Alzheimer disease in humans, our observations with brain tumors and epilepsy are suggestive of a role for pTB16 in neuropathologies in general and support the hypothesis of its involvement in tissue remodeling and cell death.

Object Ischemic cerebral vascular accidents (CVAs) in children result in epilepsy in 25% of patients, which is refractory in 7% of cases. Repeated seizures worsen the global and cognitive prognosis of these patients. To evaluate the prognosis of epilepsy and cognitive development in children with refractory seizures following a CVA, the authors retrospectively studied the effectiveness of periinsular hemispherotomy in the treatment of these patients. Methods Between March 1995 and November 2007, 8 children who suffered from stroke-induced refractory epilepsy underwent a periinsular hemispherotomy. All patients' charts were reviewed in a retrospective manner. Age at the time of the CVA, imaging studies, cause of the ischemic event, onset of the first seizure, patient's handedness, the extent of the parenchymal damage, electroencephalography findings, type of epileptic seizures, number of seizures per day, number of antiepileptic medications, preoperative neuropsychological evaluation, and surgical outcome with regard to the patient's seizure activity were analyzed. Results There were 7 boys and 1 girl in this study. The mean age at stroke was 23 months (range birth–5 years). The mean age at onset of epilepsy was 22 months (range 0–60 months). The mean age at the time of the hemispherotomy was 7 years (range 54–130 months). The average delay prior to the hemispherotomy was 5 years and 3 months (range 23–115 months). Prior to surgery, the average number of seizures per day was 35 (range 5–100). The average number of antiepileptic medications introduced before the hemispherotomy was 8 (range 6–12). Six patients required only 1 surgical intervention and 2 necessitated 2 separate operations:1 underwent a 2-staged hemispherotomy and the other underwent a prior callosotomy. There were no reported surgical complications in this series. Seven children are seizure free. However, the remaining child, after a 3.5-year disease-free interval, has recently started having seizures. No child demonstrated an improvement in neuropsychological evaluation. Conclusions The periinsular hemispherotomy must be considered an alternative in the therapeutic approach to stroke-induced pediatric refractory epilepsy. It is effective in controlling seizure activity. The authors believe the delay before hemispherotomy must be shortened in children with post-CVA refractory epilepsy.