bluuebunny/crossref_metadata_2025_split huggingface dataset:
Access to Spine Care: A Tale of Two Cities
Authors: Hurlbert R. John , Mobbs Ralph , Teo Charles 
Published: December 2014

ABSTRACT:Introduction:As governments struggle with increasing demand for accountability within the Canadian Health Care System and set wait-time standards, it is important to objectify data to allow a true understanding of present limitations and to facilitate comparisons to other systems. The purpose of this study was to compare wait list times for a cohort of patients requiring spinal surgery in Calgary, Alberta to a similar cohort in Sydney, Australia.Methods:From January 1 until June 30, 2006 all outpatients admitted for spinal surgery to the Foothills Hospital were identified by the surgeons’ office. Two time periods were quantified from their charts: (1) time from referral to surgical consultation; and (2) time from surgical consultation to operative intervention. From July 1 until December 31, 2006 patients were similarly identified through Neurosurgical offices at the Prince of Wales Public and Private Hospitals in Sydney, Australia.Results:Four hundred ninety-one surgical patients were captured during the six month period in Calgary and 155 patients during the subsequent six months in Sydney. The majority of patients in Sydney were treated in the Private Health Care system. Public patients in Sydney have access to a surgical consultant twice as fast as public patients in Calgary while private patients have access ten times faster. Access to operating room time within the public system is a rate limiting step in both countries. However, Sydney private patients receive their surgery four times faster than Calgary patients.Conclusions:Compared to Calgary, access to specialized spine care in Sydney appears more efficient not only in the Private but also the Public Health Care System. Part of this efficiency may arise from offloading from the public into the private system. Solutions proposed to reduce wait list times should consider benefits of a Private Health Care System.
A Novel Mutation in a Large French-Canadian Family with LGMD1B
Authors: Chrestian Nicolas , Valdmanis Paul N. , Echahidi Najmeddine , Brunet Denis , Bouchard Jean-Pierre , Gould Peter , Rouleau Guy A. , Champagne Jean , Dupré Nicolas 
Published: December 2014

ABSTRACTBackground:Limb girdle muscular dystrophy type 1B is an autosomal dominant disease characterized by late onset proximal muscle involvement associated with cardiac complications such as atrioventricular conduction blocks, dilated cardiomyopathy, and sudden death.Objective:Define the full phenotypic spectrum of a new mutation in the LMNA gene causing limb girdle muscular dystrophy type 1B.Methods:We identified a large French Canadian family with the LGMD 1B phenotype and a cardiac conduction disease phenotype that carried a new mutation in the LMNA gene and sought to define its full phenotypic spectrum by performing complete neurological and cardiac evaluations, muscle biopsy, RNA and DNA studies.Results:The proband and 12 living at risk relatives were tested. In total, we identified seven carriers of a new (IVS9-3C>G) LMNA gene mutation. Of the three symptomatic patients, all had cardiac involvement, but only two presented proximal limb weakness. The one available muscle biopsy demonstrated a normally expressed lamin A/C protein, localized at the nuclear envelope. RNA study revealed a loss of exon 10 transcription caused by the IVS9-3C to G splicing mutation.Conclusions:We have identified a new mutations in the LMNA gene in a French-Canadian family. This diagnosis has important implications for affected patients and their siblings since they may eventually require pacemaker implantation.
Restless Legs Syndrome in Patients with Chronic Obstructive Pulmonary Disease
Authors: Kaplan Yuksel , Inonu Handan , Yilmaz Ayse , Ocal Serpil 
Published: December 2014

Objective:To evaluate the prevalence of restless legs syndrome (RLS) in patients with chronic obstructive pulmonary disease (COPD) and the relationship between RLS and clinical/laboratory findings of COPD.Methods:One hundred and thirty-four COPD patients without secondary causes of RLS were included. Thirty-nine (29.1%) patients were diagnosed with RLS and classified as Group 1. The control group consisted of 65 age-matched COPD patients without RLS. Group 1 was divided into subgroups according to the Johns Hopkins Severity (JHS) scale. Patients with a score of 0, 1, or 2 were classified as JHS 0-2 and those with a score of 3 as JHS 3. Group 1 and the control group and subgroups were compared for clinical and laboratory characteristics.Results:We found that the duration of COPD was longer and that airway obstruction, hypercapnia, and hypoxia were more evident in patients with RLS than those without. Similar differences were also detected between JHS subgroups 3 (more severe) and 0-2. Polyneuropathy frequency was significantly higher in Group 1 compared to controls. However, Group 1 subgroups showed a similar frequency of polyneuropathy. In a multivariate analysis, hypercapnia made a significant independent contribution to both JHS 0-2 and JHS 3 patients when RLS severity was set as the dependent variable. Polyneuropathy and the duration of COPD were significant independent variables for patients in the JHS 3 subgroup. Polyneuropathy was the strongest predictor for the JHS 3 patients.Conclusions:We conclude that RLS is frequent in COPD, particularly in patients with severe hypoxemia/hypercapnia and in late stages of the disease.
Intravascular Lymphoma with Conus Medullaris Syndrome Followed by Encephalopathy
Authors: Savard Martin , Verreault Steve , Gould Peter V. , Bernier Vincent , Bouchard Jean-Pierre 
Published: December 2014

Background:Intravascular large cell lymphoma (ILCL) is a diagnostic challenge, with neurological, cutaneous and constitutional symptoms. The natural history is usually an evolution to a comatose state. As invasive procedures are usually required for diagnosis, recognizing the typical clinical pattern is critical since an effective treatment is available.Method:After an extensive literature review of the subject, we report a case of ILCL, analyzing clinical, laboratory, radiological and pathological data. We will also give a special attention to the clinical picture of a conus medullaris (CM) lesion with subsequent encephalopathy in the same patient.Results:We report here a 61-year-old woman with a paraplegia caused by a CM lesion, evolving about one year latter to encephalopathy and eventual coma, with the diagnosis of ILCL confirmed by autopsy. The present case is similar to eight other cases in literature who had CM lesion associated with ILCL, knowing that 80-90% of these patients will eventually evolve to encephalopathy without treatment. Conclusions: ILCL is a recognized but rare cause of coma. Diagnosing it is tremendously important since it is fatal if left untreated. We propose that this specific picture (conus medullaris lesion, eventually evolving to encephalopathy) is quite characteristic and will directly result in better outcome if recognized.
Sphenoid Sinus Mucocele - Rupture Causing Brainstem Inflammation and Stroke
Authors: Busche Kevin , Morrish Wiliam F. 
Published: December 2014

Background:Posterior rupture of a sphenoid sinus mucocele is a rare cause of brainstem injury.Methods:Case report.Results:A healthy young woman with a history of prior surgical excision of nasal polyps presented with a headache and evolving neurologic symptoms. The clinical presentation and imaging studies were consistent with a posterior rupture of a large sphenoid mucocele with subsequent inflammatory changes in the brainstem and evidence of brainstem stroke. Relatively rapid recovery coincided temporally with the resolution of local inflammation. Longer-term recovery continued over months, as expected following stroke.Conclusions:Posterior rupture of a sphenoid sinus mucocele may cause brainstem injury by multiple mechanisms.
Severe Head Injury: Clinicians’ Awareness of the Literature
Authors: Jacka Michael J , Des Ordons Amanda Roze , Zygun David 
Published: December 2014

Objectives:1. To determine the awareness of the literature concerning therapeutic manoeuvres in severe closed head injury (CHI) among Canadian critical care clinicians and neurosurgeons, 2. To identify factors that affect utilization of these manoeuvres, and 3. To compare reported appropriateness and frequency of use with #1 and #2.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:Fifty-nine of 99 neurosurgeons and 82 of 148 critical care physicians responded (57%). The majority of respondents were not able to identify the highest level of published evidence for most manoeuvres, except for the avoidance of corticosteroids (51%). The factor identified by most respondents as being most important in motivating use of any given manoeuvres was the level of published evidence (25%). Although reported appropriateness and frequency of use of most manoeuvres correlated well with each other, they did not correlate with awareness of evidence. In the case of corticosteroids, there was a strong correlation between non-use of steroids and awareness of evidence (R = - 0.30, p = 0.0003).Conclusions:Respondents to this survey of Canadian physicians treating patients with severe head injury reported published evidence as being the most significant factor affecting use of a therapy. However, most respondents did not correctly identify the highest published level of evidence for most therapies. This study has identified difficulty with research translation that may have clinical implications.
NRAMP1Polymorphism and Viral Factors in Sardinian Multiple Sclerosis Patients
Authors: Gazouli Maria , Sechi Leonardo , Paccagnini Daniela , Sotgiu Stefano , Arru Giannina , Nasioulas George , Vassilopoulos Dimitrios 
Published: December 2014

Background:Multiple sclerosis (MS) is believed to be an autoimmune disease occurring in genetically predisposed individuals after an appropriate environmental exposure such as viral infections. Recent studies suggest a significant association between MS and the functional 5’-(GT)n polymorphism in the promoter region of theNRAMP1gene. In the present study we aimed to evaluate the contribution of the allelic variation in theNRAMP1promoter to MS susceptibility and to study the role of viral infection in relation to specificNRAMP1genotypes, in a Sardinian cohort.Methods:Sixty MS patients and 66 healthy individuals were genotyped, and screened for the presence of Epstein-bar virus (EBV) and JC virus (JCV) sequences.Results:Consistent with previous autoimmune disease studies, allele 3 at the functional 5’(GT)n promoter region repeat polymorphism, was significantly overrepresented among MS patients when compared to controls (p=0.02). The EBV and JCV sequences were detected in 8/60 (13.33%) and in 4/60 (6.66%) of MS patients respectively and in 5/66 (7.57%) and in 0/66 of controls.Conclusion:The allelic variation in the NRAMP1 promoter may contribute to MS susceptibility in the Sardinian population. The viral sequences were not confined to a specific NRAMP1 genotype.
Acute Stroke with Hyperdense Middle Cerebral Artery Sign Benefits from IV rtPA
Authors: Tartaglia M C , Di Legge S , Saposnik G , Jain V , Chan R , Bussière M , Hachinski V , Frank C , Hesser K , Pelz D 
Published: December 2014

Objectives:The current management of acute ischemic stroke is intravenous (IV) recombinant tissue plasminogen activator (rtPA). The presence of a hyperdense middle cerebral artery sign (HMCAS) on pre-treatment head computed tomogram (CT) is considered a poor prognostic sign. We compared the clinical outcome in IV rtPA-treated patients with and without a HMCAS.Design:Retrospective analysis of prospectively collected cases treated with IV rtPA within three hours. Inclusion criteria were the presence of: i) an anterior circulation stroke; ii) a pre-treatment CT available; iii) a pre-treatment National Institutes of Health (NIH) stroke scale (NIHSS) score; and iv) a modified Rankin Score (mRS) at three months.Results:One hundred and thirty patients were eligible for the analysis, 64 (49%) had a HMCAS. The HMCAS group had a trend toward a higher mean (±SD) pre-treatment NIHSS score compared to the non-HMCAS group (13.9±6 vs. 12.2±6; p=0.12). Accordingly, there were more patients with severe strokes (NIHSS>10) in the HMCAS group compared to the non-HMCAS one (48/64=75% vs. 35/66=53%; p=0.009). The mean (±SD) NIHSS score 24 hours after treatment was 10.6 (±8) in the HMCAS group and 8.3 (±7) in the non-HMCAS group (p=0.08). In a multiple logistic regression analysis, the only independent predictor of poor outcome (mRS 3-6) was pre-treatment NIHSS score (p<0.001).Conclusion:Patients with a HMCAS receiving IV rtPA did not fare worse at three months despite a greater proportion of patients with more severe strokes. Based on the current knowledge, IV rtPA remains a good treatment for patients with a HMCAS within three hours of symptom onset.
Prevalence of Multiple Sclerosis in Canada: A Systematic Review
Authors: Poppe Alexandre Y. , Wolfson Christina , Zhu Bin 
Published: December 2014

Background:Studies of the prevalence of multiple sclerosis (MS) in Canada have generally been isolated to specific regions. Given the importance of multiple sclerosis as a cause of disability in adults, a comprehensive review of Canadian MS prevalence examining current data, interregional variation, deficiencies in knowledge and frontiers for research is timely.Methods:A systematic review of all studies addressing the prevalence of MS in Canada or regions within Canada, published in English or French since 1985, was conducted. Studies were identified using MEDLINE, EMBASE and bibliographic review. Ten studies were evaluated for methodological rigour and a test of heterogeneity across studies was performed and a measure of consistency (I2) estimated.Results:Studies were generally of high quality. Nine were restricted to regions within Canada and one provided an estimated national prevalence based on self-reported cases. All reported a high prevalence (>50 per 100 000). Latitude and longitude gradients were not striking while assessment of heterogeneity confirmed that regional differences were unlikely to be the result of sampling variability.Conclusions:This review confirms Canada as a country of very high MS prevalence and it is the first study to demonstrate that variation in regional estimates represents true differences in prevalence within Canada. Avenues for future MS prevalence research, including adoption of a national MS registry, are proposed.
Progressive Supranuclear Palsy: A Review of Co-existing Neurodegeneration
Authors: Keith-Rokosh J , Ang L C 
Published: December 2014

Objectives:The neuropathological findings of 32 progressive supranuclear palsy (PSP) cases over a period of 17 years were reviewed.Results:Of the 26 cases with adequate clinical data, 20 patients either presented with cognitive dysfunction or developed a cognitive impairment subsequently during the course of the disease. Co-existing changes of argyrophilic grains and corticobasal degeneration (CBD) were found in 28% and 32% of the cases respectively. Alzheimer-related pathology was found in 69% of cases but only 18.75% of cases fulfilled the consortium to establish a registry for Alzheimer's disease (CERAD) criteria for either definite or probable Alzheimer's disease. Lewy bodies were noted in four cases (12.5%), all in the subcortical regions. Only seven cases of PSP showed no pathological evidence of other co-existing neurodegenerative diseases. The severity of the cerebrovascular pathology in this cohort was insufficient to explain any clinical symptomatology.Conclusions:As in previous studies, this study has demonstrated the frequent co-existence of pathological changes usually noted in other neurodegenerative diseases in PSP. Whether these coexisting pathological changes contribute to the cognitive impairment in PSP remains uncertain.
Reliability of the MMSE Administered In-Person and by Telehealth
Authors: McEachern Wendaline , Kirk Andrew , Morgan Debra G. , Crossley Margaret , Henry Carol 
Published: December 2014

Background:Recent advances in telehealth have improved access to health care for those in rural areas. It is important that examinations conducted via telehealth are comparable to in-person testing. A rural and remote memory clinic in Saskatoon provided an opportunity to compare scores on the Mini-Mental State Examination (MMSE) administered in-person and via telehealth.Methods:After an initial one day assessment in Saskatoon, patients were seen in follow-up at 6 and 12 weeks. Individual patients were randomly assigned to either in-person follow-up assessment in Saskatoon or telehealth assessment in their home community. Patients who initially received in-person assessments were seen by telehealth for their next follow-up visit and vice-versa. The same neurologist administered MMSEs at all visits. The first 71 patients with both 6 and 12 week follow-up assessments were included in this study. The scores of in-person and telehealth MMSE administrations were compared using the methods of Bland and Altman as well as a paired t-test.Results:MMSE scores did not differ significantly between telehealth (22.34 +/- 6.35) and in-person (22.70 +/- 6.51) assessments.Conclusion:Telehealth provides an acceptable means of assessing mental status of patients in remote areas.
In Situ Cranioplasty for Hyperostosing Meningiomas of the Cranial Vault
Authors: Bloch Orin , McDermott Michael W. 
Published: December 2014

Abstract:Objective:Hyperostosis of the bone overlying meningiomas has been reported in up to 50% of cases. The skull becomes infiltrated by meningothelial tumor cells, necessitating removal of the hypertrophied bone to achieve a complete tumor resection. Unfortunately, aesthetic reconstruction of large bony defects can pose a significant challenge intra-operatively. Custom cranioplasty implants are very expensive and can only be fabricated after the bony defect exists, requiring a second surgery for implantation. Although numerous composite materials exist to repair the defects at the time of tumor resection, the challenge is to create an implant that fits appropriately without shifting and approximates the natural curvature of the skull. We have developed a technique for an “in situ cranioplasty” using a composite construct with strength in compression and tension.Technique:After the skull is reshaped by shaving down part of the hyperostotic bone, titanium mesh is molded to the surface of the skull and screwed into the surrounding normal bone. The bone flap is then removed by drilling a trough at the outer margin of the tumor-involved skull and removing a ring of normal surrounding bone. The central portion of tumor involved skull is then craniectomized. The mesh can be reapplied and the full thickness of the central bone can be reconstructed with polymethylmethacrylate, yielding a solid construct perfectly matched to the patient's natural head shape.Conclusion:This novel technique yields a sturdy, aesthetic, and cost-effective result which can be used to address any cranial vault defect at the time of tumor resection.
Gender, Patient Comfort and the Neurosurgical Operating Room
Authors: Zener Rebecca , Bernstein Mark 
Published: December 2014

Abstract:Background:Neurosurgical patients may be unaware of components of their intra-operative care. The relationship between patient gender and comfort level in the neurosurgical operating room (OR) has not been previously studied. Our objective was to gain insight into patients' perspective of the OR environment, including staffing and observers, the role of medical students, catheterization, exposure, and verbiage, using a qualitative needs assessment.Methods:Face-to-face semi-structured interviews were conducted with 20 patients (14 female, six male) who had a neurosurgical operation under general anesthetic within the previous two years. The majority underwent craniotomy for benign tumours. Interviews were transcribed and subjected to modified thematic analysis.Results:Nine themes emerged: 1) perception of the intra-operative environment varies between men and women; 2) lacking awareness about observers is anxiety-provoking for women; 3) being unaware of the hands-on involvement of students is a concern for all patients; 4) disclosure of implantation of foreign and permanent materials into patients is important; 5) catheterization is anxiety provoking for women; 6) pre-operative menstruation screening may minimize embarrassment for women; 7) patients perceive extraneous conversation as a distraction for surgeons; 8) patients trust their surgeon; 9) a relationship exists between interviewer gender and patient comfort in the interview.Conclusion:Although most male and female patients are unaware of OR activities, they are generally not fearful since they trust their surgeon. Women appear to have greater information needs. Patients' information needs must be met without provoking anxiety and yet preserving their personal sense of modesty in the intra-operative environment.
Head Pre-Cooling Improves Symptoms of Heat-Sensitive Multiple Sclerosis Patients
Authors: Reynolds Luke F. , Short Christine A. , Westwood David A. , Cheung Stephen S. 
Published: December 2014

Abstract:Background:Damage to the central nervous system by Multiple Sclerosis (MS) leads to multiple symptoms, including weakness, ambulatory dysfunction, visual disturbances and fatigue. Heat can exacerbate the symptoms of MS whereas cooling can provide symptomatic relief. Since the head and neck areas are particularly sensitive to cold and cooling interventions, we investigated the effects of cooling the head and neck for 60 minutes on the symptoms of MS.Methods:We used a double blinded, placebo controlled, cross-over study design to evaluate the effects of head and neck cooling on six heat-sensitive, stable, ambulatory females with MS (Extended Disability Status Scale 2.5-6.5). To isolate the effects of perceived versus physiological cooling, a sham cooling condition was incorporated, where subjects perceived the sensation of being cooled without any actual physiological cooling. Participants visited the clinic three times for 60 minutes of true, sham, or no cooling using a custom head and neck cooling hood, followed by evaluation of ambulation, visual acuity, and muscle strength. Rectal and skin temperature, heart rate, and thermal sensation were measured throughout cooling and testing.Results:Both the true and sham cooling elicited significant sensations of thermal cooling, but only the true cooling condition decreased core temperature by 0.37°C (36.97±0.21 to 36.60±0.23°C). True cooling improved performance in the six minute walk test and the timed up-and-go test but not visual acuity or hand grip strength.Conclusions:Head and neck cooling may be an effective tool in increasing ambulatory capacity in individuals with MS and heat sensitivity.