Keith E. Aronyk

Pediatric Temporal Lobectomy for Epilepsy

Background: Temporal lobectomy in adults is an accepted form of treatment for patients with intractable complex partial seizures. There have been few long-term studies of children undergoing temporal lobectomy for epilepsy. Methods: We reviewed the pediatric cases of temporal lobectomy for intractable epilepsy performed by the Comprehensive Epilepsy Program at the University of Alberta Hospitals between 1988 and 2000. All patients had preoperative and postoperative clinical evaluations, seizure charts, drug levels, EEG, CT/MRI, long-term video EEG monitoring and neuropsychological testing. The patients were reassessed at 6 weeks, 6 months and 1 year postoperatively, then yearly. The duration of follow up was 1–10 years (mean 5 years). Results: Forty-two patients were studied (25 males and 17 females). Age at surgery ranged from 18 months to 16 years. The interictal EEG was abnormal in 38 of the 42 patients. Twenty-two patients had focal epileptic discharge and 1 had generalized epileptic discharge. Focal slowing was seen in 9 patients and diffuse slowing in 5 patients. CT scan was abnormal in 17 of 39 patients and normal in 22 of 39. MRI was abnormal in 34 of 42 patients and normal in 8 of 42. Pathology included brain tumors in 14 patients, mesial temporal sclerosis in 8, focal cortical dysplasia in 4, tuberous sclerosis in 4, dual pathology in 4, porencephalic cyst in 1 and normal pathology or gliosis in 6. Thirty-three of 42 patients (78%) were seizure-free following surgery and an additional 5 (12%) had a decrease in seizure frequency. Three patients had complications, but there were no deaths. Conclusion: Temporal lobectomy is a safe and effective treatment for children with intractable complex partial seizures. Seventy-eight percent of patients are seizure-free following the surgery and there are few complications. MRI is superior to CT scan for detection of temporal lobe pathology yet failed to detect abnormalities in some patients. The most common pathologies found were brain tumors, mesial temporal sclerosis and developmental lesions. In addition to seizure control, many patients experienced improvement in cognitive and psychosocial function following surgery.

Anatomic details of intradural channels in the parasagittal dura: a possible pathway for flow of cerebrospinal fluid.

OBJECTIVE The absorption of cerebrospinal fluid occurs primarily by means of arachnoid granulations (AG) in the superior sagittal sinus (SSS) and the lacunae laterales (LL) in the parasagittal dura. Previous descriptions of this region suggest a network of intradural channels, but finer details of extent and relationship between channels and AG were not addressed. Therefore, we undertook an anatomic study of cadaveric parasagittal dura. METHODS The SSS and parasagittal dura of 20 formalin-fixed adult cadavers and 15 autopsy specimens from patients ranging in age from 18 weeks of gestation to 80 years were studied by use of a light microscope, a scanning electron microscope, and corrosion casting. Intradural injections into the parasagittal region were performed in two formalin-fixed and four autopsy specimens from adults by use of normal saline and corrosion casting. RESULTS Extensive networks of intradural channels from 0.02 to 2.0 mm in diameter were noted in all of the specimens. Channels either were connected to the SSS at intervals along the side wall or drained directly into the LL, which extended up to 3 cm from midline. Channels lined with endothelium stained positive for Factor VIII, as did the endothelium of the LL and SSS. In some places, the network of channels seemed to coalesce to form LL. The underside of the dura was coarse and trabeculated where the channels were abundant, and AG were interdigitated between these trabeculae. In regions of the dura where channels were sparse or absent, the dural underside was smooth and lacked AG. Underlying cortical veins opened directly into the SSS and were unrelated to intradural channels. Intradural parasagittal injections from the epidural side accessed the SSS by way of channels using pressures between 0 and 20 cm H2O at 1.5 ml/min. CONCLUSION These channels may represent a pathway for the flow of cerebrospinal fluid from AG to the SSS.

Pediatric epilepsy surgery at the University of Alberta: 1988-2000

Epilepsy surgery is considered a treatment option for patients with intractable seizures. Relatively few studies of efficacy, safety, and long-term outcome are available for the pediatric age group. This study describes a 12-year experience with pediatric epilepsy surgery at the University of Alberta. Records of pediatric epilepsy surgery patients admitted to the Comprehensive Epilepsy Program at the University of Alberta between 1988 and 2000 were reviewed. All patients received preoperative and postoperative clinical evaluation, seizure charts, testing of drug levels, electroencephalogram, computed tomography/magnetic resonance imaging, neuropsychologic testing, and long-term video electroencephalogram monitoring. The patients were reassessed after surgery at 6 weeks, 6 months, and 1 year and then yearly. The duration of follow-up was 1 year to 12 years. Forty-two patients underwent temporal lobectomies; 35, extratemporal resection. The age at surgery ranged from 6 months to 16 years. Thirty-two (76%) of temporal lobe patients became seizure-free (Engel Class I) vs 24 (68%) for the extratemporal group (Engel Class I). One patient (2%) in the temporal group had an Engel Class II outcome and one patient (3%) in the extratemporal group had the same Engel Class II outcome. Three patients (4%) manifested postoperative complications, and there were no deaths. Patients reported improvement in cognitive abilities, behavior, and quality of life after the surgery. Epilepsy surgery in children is effective and safe. Many children are seizure-free after the operation and remain so, although the results of temporal lobectomy are better than for extratemporal resections. There are few complications, and children often have an improved quality of life.

The effects of labor and delivery on spinal cord function and ambulation in patients with meningomyelocele

Two hundred and eight patients with meningomyeloceles were reviewed to assess the role of birth trauma in the pathogenesis of their neurological deficit. Vaginal breech-delivered patients appeared to have poorer neurological function in the newborn period. The factors responsible include: (1) a mid or upper lumbar level, that by sparing hip flexors and knee extensors, predisposes to breech presentation; (2) limb posturing due to residual neurological function and intrauterine positioning that limits the accurate neurological assessment of the newborn; (3) nervous system or soft tissue injury due to labor and delivery. Regardless of the mechanism, vaginal breech-delivered patients showed improvement in function, and eventually exhibited ambulatory function comparable to those infants born by cesarian section or vaginally after vertex presentation and labor. In our patient population, elective cesarian section did not offer a spinal cord or ambulatory advantage over vaginal delivery for those pregnancies presenting in a vertex fashion. Thus, it should be reserved for fetuses who are presenting breech or who have other obstetrical indications for operative delivery.

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.

Pathology and Neuroimaging in Pediatric Temporal Lobectomy for Intractable Epilepsy

Objectives: Firstly, to study the pathology at surgery in children undergoing temporal lobectomy for intractable partial epilepsy. Secondly, to compare neuroimaging techniques (CT, MRI) in the preoperative detection of pathology. Lastly, to examine the surgical outcome in children. Methods: Forty-two pediatric patients undergoing temporal lobectomy for intractable epilepsy at the Comprehensive Epilepsy Program at the University of Alberta Hospital between the years 1988–1998 were studied. Patients had extensive preoperative investigations including CT and MRI. The pathology at surgery was reviewed and compared to preoperative neuroimaging. Charts were reviewed to determine surgical outcome. Results: Brain tumors were the most common pathology, found in 13/42 patients. Mesial temporal sclerosis (MTS) was found in 8 patients and dual pathology in an additional 5. Focal cortical dysplasia (FCD) was seen in 4 patients, 1 patient had a porencephalic cyst and 4 patients had tubers of tuberous sclerosis. Seven patients had no specific pathology detected. MRI was clearly more sensitive than CT in the detection of pathology. MRI was abnormal in 27/42 cases (64%), while CT scan was found to be abnormal in only 12/39 (31%). Surgical outcome was excellent, with 34/42 patients (80%) having an Engel class I outcome. One patient had significant improvement with an Engel class II outcome, 3 (7%) had little improvement (Engel class III) and 4 (10%) were unchanged (Engel class IV). Three patients (7%) had surgical complications. Conclusions: A wide variety of developmental pathology is seen following temporal lobectomy for intractable epilepsy of childhood. Brain tumors, FCD and MTS are common. MRI is superior to CT in the detection of pathology, which may be subtle in children. Surgical outcome is excellent, with most children being seizure free and few complications being seen.

Glutathione levels and chemosensitizing effects of buthionine sulfoximine in human malignant glioma cells

SummaryBiopsy samples and cultured cells derived from them were obtained from 39 patients with malignant glioma and were analyzed for 1) glutathione (GSH) content; 2) sensitivity to 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) and/or nitrogen mustard (HN2) treatment and 3) the effect of buthionine sulfoximine (BSO) treatment on BCNU and/or HN2 cytotoxicity. The average GSH concentration of biopsy specimens was lower than those of cultured cells (2.36±0.44 vs. 11.42±2.32 nmol/106 cells). While some of the tumor specimens were sensitive to either BCNU or HN2, the majority were resistant to both. However, 8 of 23 tumors tested showed enhanced sensitivity to BCNU following treatment with BSO. Five of 17 tumors were similarly sensitized to HN2 by BSO. These results suggest that BSO chemosensitization may be of value for certain patients and that screening assays may help identify treatment-sensitive individuals.

Neural activity generated in the neural placode and nerve roots in the neonate with spina bifida

OBJECT The authors conducted a study to determine the neurophysiological capacity of the neural placode in spina bifida neonates and to determine if the spinal nerve roots in these neonates had normal stimulation. METHODS The authors present a case series of 2 neonates born with open neural tube defects who underwent neural tube closure within 24 hours of birth. Neurophysiological monitoring and electrical stimulation of the placode and nerve roots was performed before and after closure of the neural tube. RESULTS Stimulation of nerve roots resulted in evoked electromyographic responses in distinct muscle groups, indicative of the myotome innervation pattern. Stimulation threshold did not change significantly after closure of the placode. Stimulation within the placode generated an alternating pattern of activity in the left and right legs. CONCLUSIONS Closure of the neural tube did not affect the stimulation threshold of the nerve roots, which remained easily excitable. The viability of the nerve roots suggests that they may be candidates for neural prostheses in the future. The neural placode contains basic neural elements for generating a locomotor-like pattern in response to tonic neural inputs.

Rosette-forming Glioneuronal Tumors in the Posterior Third Ventricle

Rosette-forming glioneuronal tumor (RGNT) is a rare brain tumor found almost exclusively within the fourth ventricle. These grade I tumors were first included in the World Health Organization (WHO) Classification for the central nervous system in 20071. Since then, approximately 49 cases have been published. There are no reported cases of RGNT within the third ventricle, although this tumor has also been described in the pineal region, the tectum and within the aqueduct. Supratentorial localization is quite rare, with only three published: one was found in the septum pellucidum, another within the optic nerve in neurofibromatosis type 1 (NF1) patient and one in a patient with multiple RGNT in the lateral ventricle. The authors report the first case series in the literature of RGNT occurring within the posterior third ventricle.

Iatrogenic complications following shunting the subarachnoid space in infancy

Background Of those patients who require extracranial shunting to manage CSF circulation disorders, some of the most challenging are those whose shunts have been placed in the subarachnoid space in infancy or childhood. While clinically well in childhood, with few if any revisions, a symptom complex may develop in mid to late childhood that is characterized by chronic, often non-specific headaches and intermittent intracranial hypertension associated with abducens palsy and/or papilloedema. Imaging shows some combination of decompressed CSF spaces, acquired hindbrain herniation or posterior fossa crowding, diploic hypertrophy, narrow spinal canal and meningeal thickening. ICP monitoring does not always provide clarification of the clinical ICP correlation.