Background: Focal gliomas involving the midbrain tectum and tegmentum have been identified as having a better prognosis than diffuse tumors affecting the brain stem. However, only limited information is available concerning treatment effectiveness and long term outcome for these patients. Methods: A retrospective, population-based cancer registry survey was performed to assess the clinical features and treatment courses of patients with focal midbrain tumors. Results: Sixteen patients with midbrain gliomas were identified; eight had tectal gliomas and eight tegmental gliomas. Thirteen patients presented with symptoms related to hydrocephalus, and 12 required a ventriculoperitoneal shunt. Seven patients underwent surgery directed at the tumor. Eight patients underwent initial radiation therapy and none had initial chemotherapy. One patient diagnosed at age 18 months had a rapidly growing tumor after 14 months of follow up which has responded to chemotherapy. The mean survival of this patient population was 84 months (range 3-280 months) after diagnosis, with only one tumor related death occurring (280 months after diagnosis). Survival was not affected by tumor location within the midbrain (tegmental or tectal) or by whether radiation therapy was or was not administered. Conclusions: Patients with focal midbrain gliomas require symptom control aimed at treatment of hydrocephalus, or mass effect from the tumor. However the extended survival of this population suggests that routine aggressive surgical debulking is often not required. Furthermore, the routine use of radiation therapy or chemotherapy for all such patients is questioned.

Purpose: To review our experience with pre-radiation chemotherapy for malignant glioma. Methods: Consecutive adults with newly diagnosed glioblastoma, anaplastic astrocytoma, anaplastic oligodendroglioma and anaplastic mixed glioma with a Karnofsky Performance Score of 60 or greater were treated with one cycle of procarbazine, lomustine and vincristine or lomustine alone, prior to radiation. Computed tomographic scans were obtained soon after surgery, eight weeks later, after radiation, and at regular intervals thereafter. The effects of chemotherapy and subsequent radiation and durations of tumor control and survival were assessed in this single arm, single center, prospective trial. Results: Thirty-seven patients started chemotherapy, 36 were rescanned eight weeks after diagnosis. Five patients (16%) responded to the first cycle of chemotherapy, three had glioblastoma and two anaplastic oligodendroglioma. Seven (19%) progressed during the first cycle, 6 had glioblastoma; with the addition of radiation one progressive case responded, three stabilized, and three continued to progress. Median times to progression and median durations of survival were 26 weeks and 60 weeks for the entire group, 24 weeks and 44 weeks for glioblastoma, and greater than 104 weeks for anaplastic astrocytoma. Conclusions: Most patients with glioblastoma do not respond to one cycle of nitrosourea-based chemotherapy given prior to radiation, but patients with anaplastic oligodendroglioma sometimes do. Patients with anaplastic astrocytoma may not respond to one cycle of chemotherapy, but often respond to subsequent radiation. Judging by survival results, radiation can be delayed eight weeks without appearing to compromise patient outcome. Implications: Pre-radiation chemotherapy with newer agents can be evaluated more fully in the future knowing that brief delays in radiation are unlikely to yield substantially inferior results.

Objective: To report the occurrence, clinical characteristics and genealogical analysis of multiple sclerosis in the Hutterites of North-Western United States and Western Canada. Background: The incidence of multiple sclerosis is reported to be lower or rare in certain ethnic groups and genetic isolates and was previously observed to be absent in the Hutterite population. Methods: After long-term surveillance, six patients were identified and clinical examinations and laboratory investigations including VER and MRI were completed. Results: The six cases included two brothers, two first cousins, male and female, another male and female, all representing two of the three endogamous groups of Hutterites, are linked to two common ancestors through lines of descent dating to 1723. The individual pedigrees were analyzed from extensive genealogical records covering eight generations. Conclusion: The incidence of multiple sclerosis in Hutterites is low in a high risk area of North America. A specific mode of inheritance pattern has not been established and a common founder effect may play a role in the development of multiple sclerosis. The genetic contribution of the Hutterites seems greater than previously recognized.

Background: The term, “triphasic wave” originally described an EEG pattern believed to be a marker for a specific stage of hepatic coma. For 4 decades, the diagnostic and prognostic specificity of the pattern remains controversial. Its pathophysiology also continues to be elusive. Methods: EEG recordings were obtained in three patients known or suspected to have primary generalized epilepsy. In 2 patients, the EEGs were part of long-term monitoring using simultaneous video-EEG telemetry. For the third patient, the EEG was secured only during the post-ictal unconsciousness. These 3 patients were specifically selected because of the presence of triphasic waves in their EEGs. Results: Triphasic waves were observed in the EEG of the 3 patients only during post-ictal unconsciousness. The pattern was transient, being preceded by generalized suppression and delta slow waves and followed by theta activities. Alpha rhythms supervened when the patients became fully alert. Conclusion: A post-ictal state should be considered in unconscious patients with triphasic EEG waves.

Background: Seizures associated with intracranial neoplasms are occasionally refractory to conventional anti-epileptic drugs. Gabapentin (GBP) is one of several novel anti-epileptic drugs effective as an add-on therapy for intractable seizures but has not been studied in patients with cerebral tumours. Patients and Methods: We used GBP in a open-label add-on fashion to treat 14 patients with intractable seizures associated with intracranial tumours including four glioblastomas, four metastases, three recurrent glioblastomas, and one each of anaplastic and low grade astrocytoma and meningioma. GBP was added if optimization of pre-existing therapy failed and was titrated until seizures were controlled. Results: One patient experienced adverse drowsiness. Follow-up ranged from 3–24 weeks during which time 7 patients died from disease progression. Concurrent therapy included dexamethasone in eight, cranial irradiation in four, and radiosurgery in one. Responder rate (number with at least 50% fewer seizures) was. 100% and persisted throughout follow-up. Complete resolution of seizures occurred in 8/14 patients. Conclusions: GBP was well tolerated in patients with brain tumours. Seizure frequency was reduced in all patients and efficacy persisted over time; however, the mechanism of this improvement is unclear. Concurrent therapy, regression of frequency to the mean, and the lack of controls may account for apparent benefit. In addition, because GBP may interact with a leucine-related neuronal binding site we also speculate that this novel mechanism of action may have been enhanced in our patients due to the abnormal blood-brain barrier associated with cerebral tumours. Further investigation and a controlled trial are warranted.

Background: We report experience with patients presenting with a specific combination of symptoms: unilateral sciatica, unilateral sensibility loss in the dermatomes SI to S5 (hemi-saddle) and subjective micturation problems secondary to ruptured lumbar disc. Because of its similarities with a cauda equina syndrome, this combination of symptoms was thought to be a unilateral cauda equina syndrome and it was called hemi-cauda equina syndrome. Consequently, it was treated as an emergency. Methods: Ten patients were evaluated. They compromised 2.3% of all patients undergoing lumbar discectomy. Results: Outcome is good with only 10% persisting minor neurologic deficit (sensibility loss in dermatomes S3 to S5). With the exception of urinary retention or incontinence, duration of symptoms and signs does not seem to influence outcome. Comparing signs, symptoms and radiographic findings with those of a cauda equina syndrome which were recently and thoroughly studied, they were found to be more severe in cases of cauda equina syndrome. Especially, the good outcome, (apparently unrelated to the duration of symptoms in cases of hemi-cauda equina syndrome) contrasted with the treatment results of cauda equina syndrome. Conclusions: We defined the hemi-cauda equina syndrome from ruptured disc as a combination of unilateral leg pain, unilateral sensibility loss in dermatomes SI to S5 and sphincter paralysis (proven urinary retention or incontinence). Motor deficit is not necessarily present. Emergency surgery is warranted. Patients presenting with micturation complaints other than urinary retention or incontinence do not suffer from a hemi-cauda equina syndrome.

Background: Localizing critical brain functions such as language in children is difficult and generally requires invasive techniques. Recently sensory, motor and language functions in adults have been mapped to specific brain locations using functional imaging techniques. Of these techniques, functional MRI (fMRI) is the least invasive and has the highest spatial and temporal resolution. Its use in adults is well documented but application to children has not been as well described. In the present study lateralization and localization of language was evaluated with fMRI prior to epilepsy surgery in a nine-year-old male with complex partial seizures, attentional difficulty and decreased verbal proficiency. Methods: Two language paradigms well studied in adults (read, verb generation) and two additional language paradigms (antonym generation, letter fluency) were studied using whole brain fMRI after stimulus items and timing were adjusted to achieve the desired performance level during imaging. The patient was also conditioned to the magnet environment prior to imaging. Results: Word reading and letter fluency tasks produced lateralized and localized activation similar to that seen in adults. The patient had no language deficits following an anterior 2/3 dominant temporal lobe resection. Conclusions: With modifications of protocols such as those detailed in this report, this non-invasive method for localizing language function is feasible for the presurgical evaluation of children as well being applicable for a variety of developmental language issues.

Background: Neoplastic metastases to the cerebellum are a frequent complication in patients with carcinoma. However, the co-existence of an abscess within a CNS metastasis is a rare event. Methods: We report the case of a 79-year-old woman with two cerebellar abscesses within metastatic lesions in the cerebellum. She presented with a rapidly progressing syndrome characterized by elevated intracranial pressure and cerebellar findings. Results: At surgery, a purulent exudate within discrete metastatic tumours was identified. Pathological and microbiological examinations confirmed the coexistence of an abscess within a metastatic carcinoma. Significance and Conclusion: The radiological diagnosis of intracranial abscesses and metastases can be non-specific and brain imaging may not reliably identify both processes when they coexist. Furthermore, brain metastases often contain liquefied material which can be mistaken for tissue necrosis rather than an infectious process. It is important to be aware that a brain metastasis can also be infected. Therefore, acquisition of lesionai tissue for both pathological and microbiological examinations is essential for accurate diagnosis and to direct optimal therapy in situations where the intracranial lesion could be either an abscess or a metastatic deposit.

Introduction: Chondromas are rare intracranial tumors. The authors present two cases of intracranial intradural chondroma, one originating from the falx cerebri and the other from the dura mater of the convexity. Method and Results: Diagnostic procedures, including magnetic resonance imaging, and surgical findings are described. In both cases, pre-operative diagnosis could have been at least suspected, and the tumor was completely removed, without recurrence after a follow-up of many years. The pathogenesis and pathological findings are discussed, and cases from the literature are reviewed. Conclusion: Benign intradural chondroma has a good prognosis, with no recurrence after surgical excision in most cases.

Background: Intramedullary spinal cord abscess due to Listeria Monocytogenes is an uncommon condition usually affecting immunocompromised patients. Method: Case study. Results: A 69-year-old man presented with 3 weeks history of subacute paralysis of both lower limbs and the left upper limb. Myelogram and CT scan showed a widened upper cervical cord. CSF revealed lymphocytosis, moderately elevated protein and depressed glucose. A gadolinium-enhanced MRI showed diffuse cervical cord edema with two ring-enhancing lesions at C2-C3. Blood and CSF cultures grew Listeria Monocytogenes. He received IV ampicillin and gentamycin; the latter was discontinued after 1 month due to nephrotoxicity. Serial MRI over the next 3 months showed significant reduction in the size of these abscesses. The patient made a modest improvement in the power of his lower limbs, however he remained bed-ridden. Aside from being a mild, diet-controlled diabetic, there was no evidence of immunosuppression. Conclusion: Listeria spinal cord abscess is a treatable disorder and should be considered in the differential diagnosis in patients with a subacute onset of spinal cord dysfunction.

In 1909, in an isolated community hospital, on the northern tip of the Province of Newfoundland and Labrador, Canada, Dr. John Mason Little, Jr. performed electrical stimulation of the cerebral cortex, prior to cortical excision, as treatment of recurrent cerebral seizures in three patients. Extracts from Dr. Little’s written records of the clinical features, the neurosurgical procedures and cerebral cortical stimulation are summarised. A brief review of the contemporaneous history of neurosurgical procedures for epilepsy provides a prospective of Dr. Little’s remarkable surgical virtuosity.