bluuebunny/crossref_metadata_2025_split huggingface dataset:
Health Status and Health Care Utilization of Multiple Sclerosis in Canada
Authors: Pohar Sheri L. , Jones C. Allyson , Warren Sharon , Turpin Karen V.L. , Warren Kenneth 
Published: December 2014

Background:Persons with multiple sclerosis (MS) represent a small segment of the population, but given the progression of the disease, they experience substantial physical, psychosocial and economic burdens.Objective:The primary aim was to compare demographic characteristics, health status, health behaviours, health care resource utilization and access to health care of the community dwelling populations with and without MS.Methods:Cross-sectional survey using data from the Canadian Community Health Survey (CCHS 1.1). Adjusted analyses were performed to assess differences between persons with MS and the general population, after controlling for age and sex. Normalized sampling weights and bootstrap variance estimates were used.Results:Respondents with MS were 7.6 times (95% CI: 5.4, 10.7) more likely to have health-related quality of life scores that reflected severe impairment than respondents without MS. Respondents with MS were 12.2 times (95% CI: 8.6, 17.2) to rate their health as ‘poor’ or ‘fair’ than the general population. Urinary incontinence and chronic fatigue syndrome were 18.7 times (95% CI: 12.5, 28.2) and 21.9 times (95% CI: 11.9, 40.3), more likely to be reported by respondents with MS than those without. Differences between the two populations also existed in terms of health care resource utilization and access and health behaviours.Conclusion:Large discrepancies in health status and health care utilization existed between persons with MS who reside in the community and the general population according to all indicators used. Health care needs of persons with MS were also not met.
Motor Neuron Degeneration in a 20-Week Male Fetus: Spinal Muscular Atrophy Type 0
Authors: Sarnat Harvey B. , Trevenen Cynthia L. 
Published: December 2014

Background:Neuropathological changes in degenerating motor neurons are well documented in the term neonate with spinal muscular atrophy, but not at midgestation.Methods:Postmortem neuropathological examination was performed in a 20-week male fetus with a hypoplastic left cardiac anomaly.Results:Selective degeneration of spinal and hypoglossal motor neurons was an incidental finding. Degenerating motor neurons were not immunoreactive with neuronal nuclear antigen (NeuN) or neuron-specific enolase (NSE), as were the normal motor neurons. Synaptophysin reactivity was reduced around the soma of degenerating normal motor neurons. Ubiquitin and tau were expressed in degenerating motor neurons. Gliosis, inflammation and microglial activation were lacking in the ventral horns of the spinal cord. Laryngeal striated muscle was unaltered for age. No cerebral malformations or hypoxic-ischaemic changes were found.Conclusion:This case represents an early motor neuronal degeneration and corresponds to the recently described “type 0” spinal muscular atrophy. Lack of contractures is attributed to the early fetal age, since most muscular growth occurs in the second half of gestation.
Neurocardiogenic Syncope: Frequency and Consequences of its Misdiagnosis as Epilepsy
Authors: Josephson Colin B. , Rahey Susan , Sadler R. Mark 
Published: December 2014

Background:Neurocardiogenic syncope (NCS) can be mistaken as a seizure. We reviewed the frequency and diagnostic consequences of this misdiagnosis.Methods:A retrospective review of outpatient adult epilepsy clinic charts (QEII health Sciences Centre, Halifax, NS) was conducted to identify NCS patients referred with a provisional diagnosis of seizures. Charts were reviewed in detail with an emphasis on the consequences of misdiagnosis.Results:Of 1506 consecutive referrals to the epilepsy clinic, 194 (12.9%) ultimately had a clinical diagnosis of NCS. Mean age was 38 +/- 16 years (mean age of syncopal onset was 28 +/- 16 years). Two-thirds of referrals were from primary care physicians (including emergency departments) and 18% from neurologists. Thirty-five percent were prescribed antiepileptic drugs (AEDs) prior to referral with eight patients (4.1%) experiencing hypersensitivity reactions. Three of five women had adverse pregnancy outcomes while taking AEDs. One-third of patients had restrictions placed on their driving privileges while 11 patients (5.7%) had their employment interrupted. Diagnostic modalities used in the work-up included EEG (90%), CT head (51%), and MRI head (15%).Conclusions:NCS is commonly misdiagnosed as epilepsy. Some patients had an incorrect diagnosis for > 10 years. Patients with this misdiagnosis are often excessively investigated, inappropriately treated, and have unnecessary restrictions placed on driving and employment.
Treating Newly Diagnosed Epilepsy: The Canadian Choice
Authors: Burneo J. G. , McLachlan R. S. 
Published: December 2014

Background:Choosing an antiepileptic medication to treat a patient with epilepsy can be a complicated process during which the treating physician must base her or his decision on efficacy and safety of each of many available drugs. The lack of comparative studies between medications is one of the reasons.Methods:We conducted a survey on the management of newly diagnosed epilepsy in adult patients. The surveyed were adult and pediatric neurologists with a subspecialty interest in epilepsy who were working in academic institutions or private practice across Canada. Scenarios presented were grouped in categories according to the epilepsy syndrome (absence epilepsy, juvenile myoclonic epilepsy, undetermined idiopathic generalized epilepsy, symptomatic or cryptogenic partial epilepsy, and unclassified epilepsy), the patient's gender and age. First and second step in medical treatment for status epilepticus were surveyed as well.Results:Forty one of 64 experts responded the survey (responder rate of 66%). The results revealed a consensus among Canadian epileptologists that the first choice of antiepileptic medication in generalized epilepsies was between valproate in men (chosen by 88% of respondents) and lamotrigine in women. In localization-related epilepsies, carbamazepine was the preferred drug of choice (chosen by 90% of respondents). In the treatment of status epilepticus, an initial intravenous dose of lorazepam (95% of respondents), followed by a second dose of lorazepam or intravenous phenytoin in case the initial dose of lorazepam failed, were the treatments preferred.
Neuromonitoring During Surgery for Paediatric Spinal Deformity in Canada (2007)
Authors: Norton Jonathan , Hedden Douglas 
Published: December 2014

Background:Neuromonitoring during paediatric (and adult) spinal deformity surgery helps to reduce the risk of both permanent and short term neurological damage. A shortage of neurophysiologists and technicians limits the availability of this service. Not all surgeons believe neuromonitoring offers neuroprotection during spinal surgery. This study aimed to document the degree to which paediatric patients undergoing spinal deformity correction surgery have their spinal cord function monitored.Methods:A questionnaire was sent electronically to all of the surgical members of the Canadian Paediatric Spinal Deformity Study Group.Results:Results were received from 9/9 centres indicating that monitoring was performed in 7/9 centres, with one further centre awaiting staffing. Whilst half of those centres that do monitor only monitor sensory and motor evoked potentials, the remaining centres also use EMG and EEG to assess the state of the patient intraoperatively.Conclusions:Despite a shortage of staff, most paediatric spinal deformity surgeons in Canada who wish to, are able to neurophysiologically monitor their surgical cases. Neuromonitoring appears to be becoming a standard of care, at least for paediatric spinal deformity surgery. There is an urgent need for the establishment of national standards for both technologists and interpreters, as well as training programmes for both these groups.
Trigeminal Neuralgia: Outcomes after Gamma Knife Radiosurgery
Authors: Knafo Henri , Kenny Brendan , Mathieu David 
Published: December 2014

Background:Trigeminal neuralgia (TN) often remains difficult to treat despite multiple available medications, and can severely impact on the quality of life of affected patients. Gamma knife radiosurgery has recently emerged as a minimally-invasive alternative to surgery for patients suffering from drug-resistant TN. The goal of this study was to report the short-term efficacy of gamma knife radiosurgery for TN and assess its impact on the quality of life of patients treated in the first 18 months of our experience.Methods:Patients with medically-refractory TN or with unacceptable drug side effects were considered for radiosurgery. A maximum dose of 80 Gy was administered to the affected nerve using a single 4-mm isocenter. Follow-up assessments were made at 2, 4 and 6 months, with evaluation of pain relief, drug reduction and quality of life. Factors impacting treatment response were assessed using Cox regression analysis.Results:A total of 67 patients were treated. Significant pain relief was seen in 77.6% of patients, including 32.6% who became pain-free. Patients were able to discontinue all medications in 34.3% or reduce drug intake by more than 50% in an additional 28.4% of cases. No variable was found to predict pain relief although older age (>66 years) approached statistical significance. Sensory side effects were seen in 14.9% of patients. Quality of life improved in the majority of patients after radiosurgery.Conclusions:Gamma knife radiosurgery is a safe and effective management alternative for trigeminal neuralgia, providing good or excellent pain relief and improvement in quality of life in the majority of patients with few side effects.
Precentral Knob Corresponds to the Primary Motor and Premotor Area
Authors: Shinoura N. , Suzuki Y. , Yamada R. , Tabei Y. , Saito K. , Yagi K. 
Published: December 2014

Purpose:Cortical mapping during awake surgery assesses intraoperative neurological change in response to electrical stimulation to provide direct information regarding the anatomical localization of the primary motor area (M1). The goal of the present study was to analyze the reliability of the identification of the precentral knob in the axial image of magnetic resonance imaging or functional MRI (fMRI) for the detection of M1.Materials and Methods:Among patients with brain tumors within or near M1 in whom awake surgery was employed from April 2004 through March 2007, 14 cases were analyzed in which either the M1 or premotor area (PMA) was successfully detected by mapping during awake surgery.Results:The precentral knob was localized to the PMA in 4 cases and to M1 in 10 cases. By contrast, the gyrus activated by hand clenching in fMRI on the affected side at least partially corresponded to M1 in all cases, while those on the unaffected side corresponded to M1 in 12 of 12 cases.Conclusion:These results indicate that the precentral knob corresponds to PMA as well as to M1, whereas the gyrus activated in fMRI corresponds to M1 on the affected and unaffected side.
Standardization and Detailed Characterization of the Syngeneic Fischer/F98 Glioma Model
Authors: Mathieu David , Lecomte Roger , Tsanaclis Ana Maria , Larouche Annie , Fortin David 
Published: December 2014

Introduction:Adequate animal glioma models are mandatory for the pursuit of preclinical research in neuro-oncology. Many implantation models have been described, but none perfectly emulate human malignant gliomas. This work reports our experience in standardizing, optimizing and characterizing the Fischer/F98 glioma model on the clinical, pathological, radiological and metabolic aspects.Materials and methods:F98 cells were implanted in 70 Fischer rats, varying the quantity of cells and volume of implantation solution, and using a micro-infusion pump to minimize implantation trauma, after adequate coordinates were established. Pathological analysis consisted in hematoxylin and eosin (H&E) staining and immunohistochemistry for GFAP, vimentin, albumin, TGF-b1, TGF-b2, CD3 and CD45. Twelve animals were used for MR imaging at 5, 10, 15 and 20 days. Corresponding MR images were compared with pathological slides. Two animals underwent 18F-FDG and 11C-acetate PET studies for metabolic characterization of the tumors.Results:Implantation with 1x104 cells produced a median survival of 26 days and a tumor take of 100%. Large infiltrative neoplasms with a necrotic core were seen on H&E. Numerous mitosis, peritumoral infiltrative behavior, and neovascular proliferation were also obvious. GFAP and vimentin staining was positive inside the tumor cells. Albumin staining was observed in the extracellular space around the tumors. CD3 staining was negligible. The MR images correlated the pathologic findings. 18F-FDG uptake was strong in the tumors.Conclusion:The standardized model described in this study behaves in a predictable and reproducible fashion, and could be considered for future pre-clinical studies. It adequately mimics the behavior of human malignant astrocytomas.
Survey of Management of Severe Head Injury in Canada
Authors: Jacka Michael J. , Zygun David 
Published: December 2014

Objective:To determine: 1. the degrees of consensus and disagreement among Canadian critical care clinicians regarding the appropriateness (benefit exceeding risk) of common therapeutic manoeuvres in patients with severe closed head injury (CHI), and 2. the frequency with which clinicians employed these manoeuvres.Methods:The study design was a systematic scenario-based survey of all neurosurgeons and critical care physicians treating patients with severe CHI in Canada.Results:In the scenario of acute epidural hematoma with mass effect, respondents agreed very strongly that surgery was appropriate. Clinicians reported mannitol and hypertonic saline as appropriate. Beyond these two interventions, agreement was less strong, and the use of the extraventricular drain (EVD), phenytoin, cooling, hyperventilation, nimodipine, and jugular venous oximetry (JVO) were of uncertain appropriateness. Steroids were considered inappropriate. In a scenario of diffuse axonal injury (DAI), clinicians agreed strongly that fever reduction, early enteral feeding, intensive glucose control, and cerebral perfusion pressure (CPP)-directed management were appropriate. The use of mannitol, hypertonic saline, EVD, JVO, narcotics and propofol were also appropriate. Neuromuscular blockade, surgery, and hyperventilation were of uncertain appropriateness. The appropriateness ratings of the interventions considered in the scenario of an intracranial contusion mirrored the DAI scenario. In general, correlations between the reported appropriateness and frequency of use of each intervention were very high. An exception noted was the use of the JVO. The correlation between CPP-guided therapy and the use of the EVD was weak.Conclusions:This survey has described current practice with regard to treatment of patients with severe CHI. Areas of variation in perceived appropriateness were identified that may benefit from further evaluation. Suggested priorities for evaluation include the use of osmotic diuretics, anticonvulsants, and intracranial manometry.
Metastatic Melanoma to the Pituitary Gland
Authors: McCutcheon Ian E. , Waguespack Steven G. , Fuller Gregory N. , Couldwell William T. 
Published: December 2014

Background:Metastasis to the pituitary gland is unusual, and occurs most often in patients with carcinomas of the breast or lung. Despite its propensity for spread to the brain, metastatic melanoma has rarely been described within the sella.Methods:We report two cases of malignant melanoma pathologically confirmed within the pituitary, both metastatic from a primary site on the chest wall. In each patient, transsphenoidal resection of the tumor was incomplete and each received local radiotherapy after surgery.Results:One patient recurred quickly and developed brain metastasis as well. He died four months after resection of the pituitary metastasis, but the second patient survived six months without recurrence. As intrasellar metastasis portends widespread systemic disease and may be synchronous with parenchymal brain metastasis, survival in such patients is limited regardless of adjunctive therapy.Conclusions:Such cases are likely to arise more commonly in future due to the increasing incidence of melanoma. Identifying them by imaging alone is difficult due to inconsistent signal characteristics on MRI (as shown by these cases) and the confusion introduced by any associated intratumoral hemorrhage.
Ophthalmoplegic Migraine: Inflammatory Neuropathy with Secondary Migraine?
Authors: McMillan H J , Keene D L , Jacob P , Humphreys P 
Published: December 2014

Background:This critical review provides a summary of the clinical presentation, neuroimaging, treatment and prognosis in pediatric ophthalmoplegic migraine (OM). The features of OM are not in keeping with its classification as a migraine-variant.Method:We review 3 new and 37 reported pediatric OM cases.Results:Headache was an inconsistent feature, with 25% patients showing no evidence of pain at the initial OM episode.Patients demonstrated:1) prolonged time for symptom resolution to occur (median time 3 weeks); 2) tendency for recurrent episodes to have more severe and persistent nerve involvement; 3) evidence of permanent neurological sequelae with recurrent episodes (30% of patients); 4) rapid improvement and shortened duration with corticosteroid therapy and; 5) transient, reversible MRI contrast enhancement of the affected cranial nerve (86% of patients). These features would not be expected in primary migraine headache.Conclusion:A detailed understanding of the natural history of OM is essential for the clinical. This review provides support that OM may result from cranial nerve inflammation with headache a secondary and later feature of this condition.
The Script Concordance Test: A New Tool Assessing Clinical Judgement in Neurology
Authors: Lubarsky Stuart , Chalk Colin , Kazitani Driss , Gagnon Robert , Charlin Bernard 
Published: December 2014

Background:Clinical judgment, the ability to make appropriate decisions in uncertain situations, is central to neurological practice, but objective measures of clinical judgment in neurology trainees are lacking. The Script Concordance Test (SCT), based on script theory from cognitive psychology, uses authentic clinical scenarios to compare a trainee’s judgment skills with those of experts. The SCT has been validated in several medical disciplines, but has not been investigated in neurology.Methods:We developed an Internet-based neurology SCT (NSCT) comprising 24 clinical scenarios with three to four questions each. The scenarios were designed to reflect the uncertainty of real-life clinical encounters in adult neurology. The questions explored aspects of the scenario in which several responses might be acceptable; trainees were asked to judge which response they considered to be best. Forty-one PGY1-PGY5 neurology residents and eight medical students from three North American neurology programs (McGill, Calgary, and Mayo Clinic) completed the NSCT. The responses of trainees to each question were compared with the aggregate responses of an expert panel of 16 attending neurologists.Results:The NSCT demonstrated good reliability (Cronbach alpha = 0.79). Neurology residents scored higher than medical students and lower than attending neurologists, supporting the test’s construct validity. Furthermore, NSCT scores discriminated between senior (PGY3-5) and junior residents (PGY1-2).Conclusions:Our NSCT is a practical and reliable instrument, and our findings support its construct validity for assessing judgment in neurology trainees. The NSCT has potentially widespread applications as an evaluation tool, both in neurology training and for licensing examinations.
Myocardial Infarction Following t-PA for Acute Stroke
Authors: Mehdiratta M. , Murphy C. , Al-Harthi A. , Teal P. A. 
Published: December 2014

Background and Purpose:Complications of intravenous (IV) thrombolysis with tissue plasminogen activator (t-PA) for acute stroke are commonly related to hemorrhage, anaphylaxis, or arterial re-occlusion. Embolic complications of t-PA are beginning to be recognized with increased use of t-PA for acute ischemic stroke. We hypothesize that disruption of intra-cardiac thrombus may result in myocardial infarction (MI) after use of t-PA for acute ischemic stroke.Summary of Cases:We describe three cases of acute MI immediately following IV t-PA infusion for acute stroke. In patient #1 apical thrombus was visualized on cardiac echocardiogram accounting for the MI after t-PA for acute stroke. Patient #2 had fresh thrombus seen on cardiac catherization after use of t-PA for acute stroke. Patient #3 developed a significant troponin rise 15 hours after the t-PA for stroke infusion with an echocardiogram revealing new wall motion abnormalities. Patient # 1 and #2 died secondary to multi-organ failure.Discussion:Acute MI immediately following t-PA treatment for stroke is a rare but serious complication. The disruption of intra-cardiac thrombus and subsequent embolization to the coronary arteries may be an important mechanism in the development of MI after t-PA treatment for acute ischemic stroke.
A Novel GDAP1 Mutation P78L Responsible for CMT4A Disease in Three Moroccan Families
Authors: Bouhouche Ahmed , Birouk Nazha , Benomar Ali , Ouazzani Reda , Chkili Taïeb , Yahyaoui Mohamed 
Published: December 2014

Background:The gene encoding the ganglioside-induced-differentiation-associated protein 1 (GDAP1) has been associated with both axonal and demyelinating neuropathy. Up to date, 25 mutations in the GDAP1 gene have been reported in patients from different origins.Methods:Three Moroccan families with early onset ARCMT1 and autosomal recessive inheritance were genotyped to test linkage to 8q21.3 and their GDAP1 gene coding exons screened for mutations.Results:A novel C233T transversion at codon 78 (P78L) was detected in 6 patients from 3 unrelated families. The mutation was found to be homozygous in two families and compound heterozygous in association with the already reported S194X mutation in one family. The P78L mutation was associated with a common haplotype suggesting a Moroccan founder mutation. The patients had symptoms within the two first years of life and developed common phenotype of CMT4A with evident hoarse-voice in two cases with the longer disease duration.Conclusion:P78L mutation was associated with a common haplotype suggesting a common ancestor.