bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Canadian Epilepsy Database and Registry
Authors: McLachlan R.S. 
Published: February 2016

ABSTRACT:Epilepsy encompasses medical, psychological, social and demographic factors which are best studied in large populations. The Canadian Epilepsy Database and Registry (CEDAR) is a comprehensive English language, multicentre epilepsy database which has been developed to study the impact of these and other factors on epilepsy in Canada. In addition, it can be used locally in the clinic for office record keeping, automated printout of a referral letter or graphing seizures and other data over time. The data in CEDAR are similar to what is found in the patient's chart. There are 20 participating centres across Canada which have entered data on over 8000 adult and pediatric epilepsy patients. The information in CEDAR will be available for research purposes to centres entering data as well as to academic researchers, the pharmaceutical industry and government agencies.
Postirradiated and Nonirradiated Gliosarcoma: Immunophenotypical Profile
Authors: Ang Lee C. , Perry James R. , Bilbao Juan M. , Ozane Wayne , Peschke Eva , Young Beverley , Nelson Nahid 
Published: February 2016

AbstractBackground: Thirty-one gliosarcomas (25 nonirradiated and 6 postirradiated tumors) were selected based on the presence of two distinctive areas: a malignant gliomatous and a sarcomatous component. In all cases, the sarcomatous component appears like fibrosarcoma or malignant fibrous histiocytoma. Two tumors showed additional areas consisting of osteochondroid differentiation. Methods: All tumors were examined using antibodies against Ulex europaeus agglutinin I (UEA), glial fibrillary acidic protein (GFAP), vimentin (VM), epithelial membrane antigen (EMA), desmin, collagen IV, alpha-1-antitrypsin (a-l-AT) and smooth muscle actin (SMA). Results: While the VM highlighted the sarcomatous areas of all tumors there were only scattered spindle cells that were GFAP-positive in the same areas. The a-l-AT was diffusely reactive in the sarcomatous areas in 20 cases. Focal immunoreactivity was seen with SMA (20 tumors), UEA (8 tumors), EMA (5 tumors), collagen IV (5 tumors) and desmin (4 tumors) in the nonvascular sarcomatous cells. Conclusions: The range of immunophenotypical expression is likely to be a reflection of the capacity of a multipotential progenitor to undergo divergent differentiation. There is very little morphological difference between the postirradiated and nonirradiated tumors except that a higher proportion of postirradiated tumors are immunoreactive to SMA and desmin.
The Occurrence of Multiple Sclerosis in the Hutterites of North America
Authors: Hader Walter J. , Peter Seland T. , Hader Mary B. , Harris Colleen J. , Dietrich Dennis W. 
Published: February 2016

AbstractObjective: 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.
Abscess Within a Brain Metastasis
Authors: Pui Ng Wai , Lozano Andres 
Published: February 2016

AbstractBackground: 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.
Excitation and Inhibition in Epilepsy
Authors: Engel Jerome 
Published: February 2016

AbstractThe term epileptic seizures is used to describe a variety of paroxysmal signs and symptoms resulting from a large number of underlying pathological processes. Generalized tonic-clonic convulsions, therefore, reflect entirely different basic neuronal mechanisms than those of typical absences. Animal research suggests that the former result from disturbances that involve disinhibilion, whereas the abnormality giving rise to the latter requires intact, if not enhanced, inhibition in order to sustain hypersynchronous neuronal discharges. Investigations in human mesial temporal lobe epilepsy and chronic experimental animal models indicate that partial seizures can also reflect multiple underlying mechanisms, with some disinhibitory in type, while others appear to be hypersynchronous and associated with enhanced inhibition. Just as more than one epileptogenic disturbance can result in transition to ictus, it is likely that more than one mechanism can be engaged to terminate epileptic seizures, that these diverse processes could result in different postictal manifestations, and that they could conceivably contribute differently to the development of interictal behavioral disturbances. An appreciation for the fact that epilepsy is not merely an increase in excitation and/or a decrease in inhibition, but a variety of complicated neuronal interactions in different patients, or even at different times in the same patient or same seizure, is essential to the development of effective treatments to control epileptic seizures and prevent their consequences.
Epidemiological Study of Ruptured Intracranial Aneurysms in the Saguenay-Lac-Saint-Jean region (Quebec, Canada)
Authors: Mathieu Jean , Pérusse Louis , Allard Pierre , Prévost Claude , Cantin Léo , Bouchard Jean-Marie , DeBraekeleer Marc 
Published: February 2016

AbstractBackground: Using a population-based register of the Saguenay-Lac-Saint-Jean region (Quebec, Canada), the genealogical reconstruction of 533 individuals with intracranial aneurysm (IA) showed a familial aggregation (the presence of aneurysm in two or more first- to third-degree relatives) for 159 (29.8%) of them; this proportion is much higher than reported elsewhere. Objective: As part of an ongoing project to assess a genetic predisposition to intracranial aneurysms in the Saguenay-Lac-Saint-Jean population, the objective of the present study was to determine whether age-specific rates of ruptured cerebral aneurysms were higher than in other populations. Design: A retrospective study of cases of proven ruptured I As which were hospitalized during the 1973 to 1992 period was conducted. Age-adjusted rates were computed and compared to those reported in the Helsinki population. Results: We identified 412 cases of ruptured aneurysms. The age-adjusted incidence rate was 7.2/100,000/year (6.2 for men, 8.1 for women), which is similar to the incidence rates reported in other studies. Although the mean age at time of rupture was younger (46.6 years ± 13.8) than usually reported, no increase in age-specific incidence rates was detected. Conclusions: The results of this epidemiological study neither support nor reject the hypothesis of a genetic predisposition to intracranial aneurysms in the Saguenay-Lac-Saint-Jean population.
A Novel Approach to the Determination and Characterization of HIV Dementia
Authors: Brouwers P. , Mohr E. , Hildebrand K. , Hendricks M. , Claus J.J. , Baron I.S. , Young M. , Pierce P. 
Published: February 2016

ABSTRACT:Background: Neuropsychological studies of the pattern and extent of cognitive impairment in HIV-infected patients have mostly used deviations from control values and/or cut-off scores as criteria for classification of dementia. There is, however, no agreement as to how to define impairment, and classification is imprecise. Method: The current study used a dementia classification matrix, developed with a step-wise linear discriminant analysis of neuropsychological data from patients with primary neurodegenerative dementias, to classify symptomatic HIV patients as demented or non-demented, and further to differentiate cortical and subcortical dementia patterns. Thirty-two male and 2 female patients (mean age 39 ± 2) with symptomatic HIV disease (mean absolute CD4 count 195 ±41) participated in the study. Results: Thirty-five per cent of patients were classified as demented. Of these, 83% showed a subcortical pattern and 17% a cortical profile of deficits. Significant differences between patients classified as subcortically demented and those categorized as normal on neuropsychological measures associated with subcortical integrity further validated the classification. Measures of psychiatric status between subgroups were similar. Conclusion: Since certain treatments may delay or reverse cognitive deficits, the use of an objective classification method based on discriminant analysis may help to identify patients who may benefit from therapy.
A Normal Bias Toward a Pictorially Defined Top in Line Bisection
Authors: Toth Cory , Kirk Andrew 
Published: February 2016

ABSTRACT:Background: We set out to determine whether separable visual and representational components underlie normal subjects’ upward and distal biases in bisecting vertical and radial lines under visual guidance. Methods: Thirty-four normal subjects were asked to bisect lines oriented horizontally, vertically, and radially. Human silhouette figures were placed at either end of each line. These figures were presented upright or upside down in order to pictorially define a “top” to each line independent of the actual top of the visual field. Results: Although subjects erred toward the top of the visual field, they also demonstrated a significant bias toward the heads of the figures for lines in all spatial orientations. Conclusions: This result supports the existence of two biases: one toward the upper visual field, and another toward an internally represented “top” as suggested pictorially. These findings provide further support for the hypothesis that normal subjects’ upward and distal biases on bisection of vertical and radial lines under visual guidance have both representational and visual-based components.
Post-traumatic Frontal Lobe Epilepsy with Structural Changes: Excellent Results After Cortical Resection
Authors: Cukiert Arthur , Olivier André , Andermann Frederick 
Published: February 2016

ABSTRACT:Background: The observation that resection of structural lesions of the frontal lobe provides an effective treatment of intractable epilepsy has been made by surgeons specializing in this field for over sixty years! However, the excellent results achieved when such resections are coupled with the use of modern diagnostic and operative technologies are not fully appreciated by the modern clinician. Methods: We review the results of resection of large post traumatic frontal lesions in six patients with intractable frontal seizures. No invasive EEG recording was required pre-operatively. Results: The imaging characteristics of the underlying lesions, usually due to depressed skull fractures are illustrated. Surgical treatment rendered five patients seizure free and the sixth had over 85% improvement in seizure frequency. Conclusions: Modern imaging permits ready visualization of the post traumatic lesions with cause intractable seizures. It is important to recognize that resection of these post traumatic scars usually leads to excellent seizure control.
Pre-radiation Chemotherapy for Malignant Glioma in Adults
Authors: Kirby Sarah , Macdonald David , Fisher Barbara , Gaspar Laurie , Cairncross Gregory 
Published: February 2016

ABSTRACT: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.
Add-on Gabapentin for Refractory Seizures in Patients With Brain Tumours
Authors: Perry James R. , Sawka Carol 
Published: February 2016

ABSTRACT: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.
Intracranial Chondroma. Report of Two Cases and Review of the Literature
Authors: hacerte Daniel , Gagné Francois , Copty Michel 
Published: February 2016

ABSTRACT: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.
Reciprocal Inhibition in Hemiplegia: Correlation with Clinical Features and Recovery
Authors: Okuma Yasuyuki , Lee Robert G. 
Published: February 2016

ABSTRACT:Background:Previous reports have described changes in reciprocal la inhibition in hémiplégie patients, but correlations between the amount of la inhibition and the clinical deficits have not been well established.Methods:We studied reciprocal inhibition between ankle flexors (tibialis anterior) and extensors (soleus) in 16 hemiplegic patients at various stages following a stroke and in 26 control subjects. The amount of disynaptic la inhibition was determined from the short latency suppression of the soleus or tibialis anterior H-reflexes by conditioning stimulation of the antagonistic muscle nerves. Results:Disynaptic la inhibition from peroneal nerve afférents to soleus motoneurones was increased in patients who showed good recovery of function with mild spasticity. However, it was not changed, or even sometimes diminished, in patients who made a poor recovery and had more marked extensor spasticity. In patients where serial recordings were obtained there was an increase in la inhibition during the recovery period following stroke. la inhibition to the tibialis anterior motoneurones tended to be greater in the poor recovery patients with marked spasticity than in the good recovery patients. The late (Dl) inhibition, presumably due to presynaptic inhibition, was decreased in the patients, although consistent correlations between the amount of this inhibition and the clinical features were not clearly demonstrated.Conclusions:Changes in excitability of la inhibitory pathways can be correlated with some of the clinical features seen in hemiplegia. Increased la inhibition of soleus motoneurones during recovery may be a mechanism to compensate for loss of descending motor commands.
Symptomatic Dystonias Associated with Structural Brain Lesions: Report of 16 Cases
Authors: Kostià Vladimir S. , Stojanovié-Svetel Marina , Kacar Aleksandra 
Published: February 2016

ABSTRACT:Background:Symptomatic (secondary) dystonias associated isolated lesions in the brain provide insight into etiopathogenesis of the idiopathic form of dystonia and are a basis for establishing the possible correlation between the anatomy of a lesion and the type of dystonia according to muscles affected.Methods:In 358 patients with differently distributed dystonias, a group of 16 patients (4.5%) was encountered in whom dystonia was associated with focal brain lesions.Results:Of the 16 patients, 3 patients had generalized, 3 segmental and 4 hemidystonia, while the remaining 6 patients had focal dystonia. The most frequent etiologies were infarction in 7, and tumor in 4 patients. These lesions were usually found in the lenticular and caudate nucleus, thalamus, and in the case of blepharospasm in the upper brainstem.Conclusions:Our results support the suggestion that dystonia is caused by a dysfunction of the basal ganglia.
Historical Neurology and Neurosurgery On the Names of Babiński
Authors: Gasecki Andrew P. , Hachinski Vladimir 
Published: February 2016

ABSTRACT:The 100th anniversary of the discovery of the extensor plantar response will be celebrated in 1996. It was Joseph François Félix Babinski who became known worldwide for the sign that bears his name. In order to help Joseph in establishing his career, brother Henri gave up his aspirations and abandoned engineering. Clovis Vincent, ‘father’ of French neurosurgery and pupil of Joseph, stated: “Joseph Babinski lived for science, and Henri lived for his brother; without Henri Babinski, Joseph would not have accomplished that much”. However, Henri’s name became famous in all Paris for a cookbook Gastronomie Pratique written under the pseudonym of ‘Ali-Bab.’ Throughout Joseph’s career his surname remained distorted despite his own efforts to spell and pronounce it correctly. Several people can claim the name Babinski, but in neurology and neurosurgery there is only one, Joseph.
Tibial Mononeuropathy from a Lower Limb Synovial Cyst
Authors: Sun J.C.L. , Wallace C. , Zochodne D.W. 
Published: February 2016

AbstractBackgroundTibial mononeuropathy due to compression by a synovial cyst is uncommon with fewer than 10 cases reported in the literature.MethodCase Study.ResultsTibial motor mononeuropathy with focal conduction block between the popliteal fossa and ankle was identified by electrophysiological testing. Magnetic resonance imaging and surgical exploration identified a synovial cyst compressing the nerve just distal to the popliteal fossa.ConclusionsThe combination of electrophysiological localization and imaging in cases of unexplained tibial mononeuropathy may identify an unexpected structural cause.
Supratentorial Ectopic Ependymoma
Authors: Vernet Olivier , Farmer Jean-Pierre , Meagher-Villemure Kathleen , Montes José L. 
Published: February 2016

AbstractBackgroundEpendymomas usually arise from the ventricular surface.MethodsWe report an 11-year-old female who presented with a supratentorial ectopic ependymoma.ResultsThe patient presented with a two-month-history of progressive headache, nausea and vomiting. Examination revealed papilledema, horizontal nystagmus, diplopia on upward gaze, and right pronator drift. CT scan showed an enhancing left precentral subcortical lesion measuring 3 cm in diameter with associated edema and mass effect. Its medial border was located 3 cm from the ependymal surface of the ventricle. A firm tumour was dissected from the centrum semiovale white matter, and removed in toto as confirmed on MRI. Pathological examination revealed histological, immuno-histochemical and electron microscopic features consistent with an ependymoma. Spine MRI and bone marrow aspirate, as well as lumbar puncture of cytology failed to show any dissemination.ConclusionFrom the literature review, this represents an exceptional ependymoma located at the distance from the ventricular system or cisterns. Different pathogenic alternatives are discussed.