bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Incidence Trends of Primary Brain Tumors in Saskatchewan from 1970 to 2001
Authors: Pirouzmand F. , Sadanand V. 
Published: December 2014

Objective:There has been a paucity of information on the epidemiology of primary brain tumors (BTs) in Canada. This study documents epidemiology of primary BTs in Saskatchewan over three decades to define their current state, changing pattern over years and relative distribution in two geographically defined areas of the province.Methods:Data on all primary BTs from 1970 to 2001 from the Brain Tumor Registry in Saskatchewan was collected. The aggregate data on primary BTs including the time-series for incidence, age, geographic location and sex were statistically analyzed using SPSS 13. Poisson regression was used to model the incidence as a function of decade of diagnosis and age at the time of diagnosis.Results:The average annual incidence of primary BTs was 11.1 per 100,000 person-years (male 12.5 per 100.000 and female 9.8 per 100.000). Males constituted 54.5% of all these tumors. The age distribution of tumors was bimodal with peaks at 5 years and 65 years. During this time, the incidence of primary BTs has increase predominantly in non-malignant types. No difference was found in the rate of all the diagnosed primary BTs combined, meningioma and lymphoma between the northern part (Regina) and southern part (Saskatoon) of the province.Conclusions:The incidence of BTs in Saskatchewan is more than previously reported in Canada. There is a temporal trend in increasing incidence of some of the BTs predominantly in the non-malignant types. No spatial difference in the incidence of primary BTs was shown in this province. These data will provide useful information to guide the future studies on BTs changing patterns, possible etiologies and efficient resource allocation for management of these diseases.
Possible Anticipation in Hereditary Spastic Paraplegia Type 4 (SPG4)
Authors: Reddy P. Leema , Seltzer William K. , Grewal Raji P. 
Published: December 2014

Objective:We report a multigenerational family with uncomplicated hereditary spastic paraplegia type 4 and apparent anticipation. Genetic analysis of the proband revealed a frame shift mutation (5 base pair deletion) in exon 9 of the SPG4 gene encoding the spastin protein. We hypothesized that this deletion mutation may be dynamic and variability in the size of the deletion could account for the anticipation.Methods:Clinical and genetic analysis of this family and the deletion mutation.Results:In this family, the age of onset, which ranges from 3 to 50 years shows an average decrease in the age of onset of 21.8 years per transmission over three generations. Genetic analysis of multiple family members indicates that all affected members carry the same c.1340_1344deITATAA mutation and that it is not dynamic.Conclusion:In this family, other molecular mechanisms may contribute to development of anticipation.
SPG4 Founder Effect in French Canadians with Hereditary Spastic Paraplegia
Authors: Meijer Inge A. , Dupré Nicolas , Brais Bernard , Cossette Patrick , St-Onge Judith , Rioux Marie-France , Benard Melanie , Rouleau Guy A. 
Published: December 2014

Background:The most common cause of autosomal dominant Hereditary Spastic Paraplegia (HSP) is mutations in the SPG4 gene. We have previously identified novel SPG4 mutations in a collection of North American families including the c.G1801A mutation present in two families from Quebec. The aim of this study is to estimate the frequency of the c.G1801A mutation in the French Canadian (FC) population and to determine whether this mutation originates from a common ancestor.Methods:We collected and sequenced exon 15 in probands of 37 families. Genotypes of markers flanking the SPG4 gene were used to construct haplotypes in five families. Clinical information was reviewed by a neurologist with expertise in HSP.Results:We have identified three additional unrelated families with the c.G1801A mutation and haplotype analysis revealed that all five families share a common ancestor. The mutation is present in 7% of all our FC families and explains half of our spastin linked FC families. The phenotype associated with the c.G1801A genotype is pure HSP with bladder involvement.Conclusion:In this study we have determined that the relative frequency of the c.G1801A mutation in our FC collection is 7%, and approximately 50% in the spastin positive FC group. This mutation is the most common HSP mutation identified in this population to date and is suggestive of a founder effect in Quebec.
Period Prevalence of Epilepsy in Children in BC: A Population-Based Study
Authors: Schiariti Veronica , Farrell Kevin , Houbé Jill S. , Lisonkova Sarka 
Published: December 2014

Background:Most estimates of the prevalence of seizure disorders in Canada derive from national surveys which differ in sampling and case-finding methods. This study used health care utilization data to make a population-based estimate of the prevalence of epileptic seizures and of epilepsy in children in British Columbia (BC).Methods:All BC residents between 0-19 years-of-age in 2002-3 enrolled in the Medical Services Plan were included. Epileptic seizures were defined using ICD-9 codes; health care utilization data was obtained from BC Linked Health Database. The period prevalence of epileptic seizures and of epilepsy was determined by age, urban/rural region and socioeconomic status.Results:8,125 of 1,013,816 children were identified as having an epileptic seizure of which 5,621 were classified as epilepsy - 5.5 per 1,000 children (95% CI: 5.4-5.7). The prevalence of epilepsy in infants and preschoolers was higher than that reported in the literature. A higher prevalence of epilepsy was observed also among those with low socioeconomic status. A higher prevalence of epilepsy was observed in those health regions with a higher proportion of First Nations and a lower prevalence was observed in health regions with a higher proportion of visible minorities.Conclusions:Age-specific prevalence rates in BC children for epilepsy, determined from population-based administrative records, were similar to published data except in children under five years. We found a gradient of increased prevalence with decreased level of income. Prevalence rates based on utilization data have the potential to guide program planning for children with epileptic seizures.
Arterial Onyx Embolisation of Intracranial DAVFs with Cortical Venous Drainage
Authors: Chew Jason , Weill Alain , Guilbert François , Raymond Jean , Audet Marie-Eve , Roy Daniel 
Published: December 2014

Purpose:To present our experience with the endovascular management of intracranial dural arteriovenous fistulas with direct cortical venous drainage by trans-arterial embolisation using Onyx.Materials & Methods:Between January 2004 and April 2008, 12 consecutive high grade intracranial dural arteriovenous fistulas (Cognard type III (eight patients) or IV (three patients)) were treated by trans-arterial embolisation with Onyx. The majority of cases were treated by Onyx embolisation alone. One case had additional embolisation with n-butyl-2-cyanoacrylate at the same session. Imaging follow-up was obtained in all but one patient (mean 3.6 months).Results:Nine patients had a technical success at the end of the embolisation procedure with complete angiographic exclusion of the fistula. Two patients had a small residual fistula at the end of embolisation, one of which had residual mild cortical venous drainage. Both were stable at follow-up angiography. One patient had a residual fistula supplied by the ophthalmic artery, which was thought to be unsafe to embolise and was sent for surgery, which was curative. In one patient the microcatheter ruptured, with a fragment of the distal microcatheter left in the occipital artery. No clinical complications were observed in this series at clinical follow-up (mean 3.3 months). Two patients were noted to have significant radiation dose.Conclusion:Endovascular management of intracranial dural arteriovenous fistulas with direct venous cortical drainage by trans-arterial Onyx embolisation is a safe and effective treatment according to our experience. Fluoroscopy times and radiation dose may be a concern.
Percutaneous Muscle Biopsies: Review of 900 Consecutive Cases at London Health Sciences Centre
Authors: Derry Kendra L. , Nicolle Michael N. , Keith-Rokosh Julia A. , Hammond Robert R. 
Published: December 2014

Objective:In the present study we review our experience with 900 consecutive percutaneous muscle biopsies over the period 1993 to 2007. We examined the advantages and limitations of the procedure, biopsy site preferences, diagnostic range, frequency of diagnoses and quality of histopathology. Demographics, referral patterns and patients' perceptions of the procedure were also assessed.Methods:Cases were identified through the London Health Sciences Centre Department of Pathology database. Standard biopsy procedures were followed using a manual trocar style instrument. With a neuropathology technologist in attendance at all biopsies, biopsies were oriented in the fresh state and snap frozen.Results:Most referrals for muscle biopsy were from neuromuscular neurologists. The procedure was found to be efficient, well-tolerated and produced high quality specimens in all diagnostic categories. No major complications occurred. Failure to obtain an adequate tissue sample, although uncommon (<2%), was usually due to marked obesity, edema or muscle wasting. Bleeding at the site was rarely problematic and no wound infections were reported.Conclusions:Needle muscle biopsies represent an efficient alternative to open biopsies when peripheral nerve sampling is not required and when large tissue samples are not needed for extensive biochemical analyses.
Resiniferatoxin Mediated Ablation of TRPV1+ Neurons Removes TRPA1 as Well
Authors: Pecze László , Pelsőczi Péter , Kecskés Miklós , Winter Zoltán , Papp András , Kaszás Krisztián , Letoha Tamás , Vizler Csaba , Oláh Zoltán 
Published: December 2014

Objectives:Resiniferatoxin, the most potent agonist of inflammatory pain/vanilloid receptor/cation channel (TRPV1) can be used for neuron subtype specific ablation of pain generating cells at the level of the peripheral nervous system by Ca2+-excytotoxicity. Molecular neurosurgery is an emerging technology either to alleviate severe pain in cancer or treat/prevent different local neuropathies. Our aim was determining sensory modalities that may be lost after resiniferatoxin treatment.Methods:Newborn or adult mice were treated with resiniferatoxin, then changes in chemical and heat sensitivity were correlated with alterations of the cell composition of sensory ganglions.Results:Only mice treated at adult age became less sensitive to heat stimuli, while both treatment groups lost sensitivity to specific vanilloid agonists of TRPV1 and, interestingly, to allyl-isothiocyanate, a selective agonist of TRPA1. Our in vivo and post mortem analytical results confirmed that TRPV1 and TRPA1 function together and resiniferatoxin-mediated neurosurgery removes both sensor moleculesDiscussion:In adult mice resiniferatoxin causes: i) desensitization to heat and ii) sensitization to cold. Cold hyperalgesia, an imbalance in thermosensation, might be conferred by a prominent cold receptor that is expressed in surviving resiniferatoxin-resistant sensory neurons and compensates for pain signals lost with TRPA1 and TRPV1 double positive cells in the peripheral nervous system.
Cerebral Proliferative Angiopathy
Authors: Eesa Muneer , Sharma Pranshu , Goyal Mayank 
Published: December 2014

A 31-year-old male presented with transient numbness of the right hand, progressing to involve the forearm. The symptoms lasted for around 30 minutes without any associated weakness or positive motor phenomena. An evaluation in the emergency room revealed no focal deficits.Acomputer tomogram (CT) scan performed in the emergency room suggested a few abnormal vascular structures in relation to the left hemisphere, with no evidence of hemorrhage. The patient was referred for magnetic resonance imaging (MRI) and conventional angiography.The MRI showed a diffuse network of densely enhancing vascular spaces involving the left middle cerebral artery (MCA) and anterior cerebral artery (ACA) territories without a clear nidus or abnormal parenchymal signal change (Figure 1). There was no involvement of the basal ganglia or thalami. Given the size and extent of the abnormality, there was a relative paucity of draining vessels, which were only moderately enlarged.
Acute Glomerulonephritis Presenting With PRES: A Report of 4 Cases
Authors: Wirrell E C , Hamiwka L D , Hamiwka L A , Grisaru S , Wei X 
Published: December 2014

Objective:Posterior reversible encephalopathy syndrome (PRES) occurs most commonly in the setting of known hypertension or use of immunosuppressive agents.Design and Methods:We report four previously-well children who presented acutely with altered mentation, seizures and visual disturbances and were diagnosed with PRES.Results:Only one child had a history of gross hematuria prior to the seizure. All four were discovered to be hypertensive only after onset of their neurological symptoms, and were subsequently diagnosed with glomerulonephritis. All four had rapid resolution of neurological symptoms with adequate treatment of hypertension.Conclusions:Blood pressure must be measured promptly in all children presenting with these symptoms. If elevated, the diagnosis of PRES should be strongly considered and a workup for renal disease pursued.
Multimodal Evoked Potentials of Kennedy's Disease
Authors: Lai Tsu-Hsien , Soong Bing-Wen , Chen Jen-Tse , Chen Yen-Yu , Lai Kuan-Lin , Wu Zin-An , Liao Kwong-Kum 
Published: December 2014

Background:Kennedy's disease (KD) is an X-linked recessive polyglutamine disease. Traditionally, it is a lower motor neuron syndrome with additional features such as gynecomastia and tremor. Sensory symptoms are minimal if ever present. We used multimodal evoked potential (EPs) tests to study the distribution of the involvement of the disease.Methods:Visual, brainstem auditory, somatosensory and motor EPs were studied in six KD patients. All of them had typical presentations and had been proved genetically.Results:Abnormal findings were noted as follows: prolonged peak latencies of visual EPs, increased hearing threshold level, inconsistent brainstem auditory EPs, decreased amplitudes of cortical potentials of somatosensory EPs, and increased motor threshold to transcranial magnetic stimulation.Conclusions:Our multimodal EP studies showed that KD involved multiple levels of the nervous system. It implies the widespread effects of the mutant androgen receptors.
LRRK2 Screening in a Canadian Parkinson's Disease Cohort
Authors: Grimes D. A. , Racacho L. , Han F. , Panisset M. , Bulman D. E. 
Published: December 2014

Background:Mutations in the leucine-rich repeat kinase 2 gene (LRRK2) have become the most common known cause for developing Parkinson's disease. The frequency of mutations described in the literature varies widely depending on the population studied with most reports focusing only on screening for the most common G2019S mutation in exon 41.Methods:In this study seven exons (19, 24, 25, 31, 35, 38, and 41) in LRRK2 where mutations have been reported were screened in 230 unselected Parkinson's disease patients using denaturing high-performance liquid chromatography.Results:The sequencing of samples with heteroduplex profiles revealed five novel and two known intronic sequence variants. In our cohort, we were unable to detect any of the known mutations in these exons or identify novel mutations within the LRRK2 gene.Conclusions:Therefore, despite the availability of diagnostic LRRK2 genetic testing it is unlikely to yield a positive result in this population.
Coincidence vs Cause: Cure in Three Glioblastoma Patients Treated with Brachytherapy
Authors: Dehdashti Amir R. , Sharma Sunjay , Laperriere Normand , Bernstein Mark 
Published: December 2014

Background:Very long term survival after diagnosis of malignant glioma has been described in individual case reports. Survival of more than 10 years is extremely rare, especially when identified in 3 out of 71 patients assigned to one arm of a randomized controlled trial.Patients:Three patients survived 11, 16, and 18 years following the diagnosis of glioblastoma and treatment with surgery, conventional fractionated radiation, and high-activity iodine-125 boost brachytherapy as part of a randomized controlled trial.Conclusion:Despite this apparent cause and effect relationship, statistical analysis shows no relationship between these cures and treatment with brachytherapy. Cure of glioblastoma remains rare.
Oxidative Stress after Subarachnoid Hemorrhage in gp91phox Knockout Mice
Authors: Liu Shimin , Tang Jiping , Ostrowski Robert P , Titova Elena , Monroe Cara , Chen Wanqiu , Lo Wendy , Martin Robert , Zhang John H 
Published: December 2014

Background:Oxidative stress largely contributes to early brain injury after subarachnoid hemorrhage (SAH). One of the major sources of reactive oxygen species is NADPH oxidase, upregulated after SAH. We hypothesized that NADPH oxidase-induced oxidative stress plays a major causative role in early brain injury after SAH.Methods:Using gp91phox knockout (ko) and wild-type (wt) mice, we studied early brain injury in the endovascular perforation model of SAH. Mortality rate, cerebral edema, oxidative stress, and superoxide production were measured at 24 h after SAH. Neurological evaluation was done at 23 h after SAH surgery.Results:Genotyping confirmed the existence of a nonfunctional gp91phox gene in the ko mice. CBF measurements did not show differences in SAH-induced acute ischemia between ko and wt mice. SAH caused a significant increase of water content in the ipsilateral hemisphere as well as an increase of Malondialdehyde (MDA) levels and superoxide production. There were no significant differences in post-SAH mortality rate, brain water content and the intensity of the oxidative stress between knockout and wild type groups of mice.Conclusions:Our results suggest that gp91phox is not critically important to the early brain injury after SAH. An adaptive compensatory mechanism for free radical production in knockout mice is discussed.
Canadian Minor Hockey Participants’ Knowledge about Concussion
Authors: Cusimano Michael D. , Chipman Mary L. , Volpe Richard , Donnelly Peter 
Published: December 2014

Abstract:Background and Objectives:In Canada and the USA, ice hockey is a cause of traumatic brain injury. Post-concussive symptoms are the most important feature of the diagnosis of concussion in sports and it is recommended that athletes not return to play while still symptomatic. Lack of knowledge of concussions could therefore be one of the main detriments to concussion prevention in hockey. The purpose of this research is to describe what minor league hockey players, coaches, parents and trainers know about concussion and its management.Methods:A questionnaire to assess concussion knowledge and return to play guidelines was developed and administered to players at different competitive levels (n = 267), coaches, trainers and parents (total adults n = 142) from the Greater Toronto Area.Results:Although a majority of adults and players could identify mechanisms responsible for concussion, about one-quarter of adults and about a quarter to a half of children could not recall any symptoms or recalled only one symptom of a concussion. A significant number of players and some adults did not know what a concussion was or how it occurred. Almost half of the players and a fifth of the adults incorrectly stated that concussion was treated with medication or physical therapy. Nearly one quarter of all players did not know if an athlete experiencing symptoms of concussion should continue playing.Conclusions:This study demonstrated that a significant number of people held misconceptions about concussion in hockey which could lead to serious health consequences and creates a need for better preventive and educational strategies.
Primary Stenting for Recurrent Stenosis Following Carotid Endarterectomy
Authors: Bussière Miguel , Pelz David M. , Lownie Stephen P. 
Published: December 2014

Abstract:Background:Carotid angioplasty and stenting is an accepted alternative treatment for severe restenosis following carotid endarterectomy. Balloons may not be required to effectively treat these lesions, given their altered histopathology compared to primary atherosclerotic plaque and tendency to be less calcified. Primary stenting using self-expanding stents alone may, therefore, be a safe and effective treatment for restenosis post-carotid endarterectomy.Methods:We review our experience in the treatment of 12 patients with symptomatic severe restenosis following carotid endarterectomy with primary stent placement alone.Results:Self-expanding stent placement alone reduced the mean internal carotid artery stenosis from 85% to 29%. Average peak systolic velocity determined at the time of ultrasonography decreased from 480 cm/s at initial presentation to 154 cm/s post-stent deployment and further decreased to 104 cm/s at one year follow-up. The stented arteries remained widely patent with no evidence of restenosis. A single peri-procedural ipsilateral transient ischemic event occurred. There were no cerebral or cardiac ischemic events recorded at one year of follow-up.Conclusions:In this series, primary stent placement without use of angioplasty balloons was a safe and effective treatment for symptomatic restenosis following carotid endarterectomy.
Neuromuscular Function in Survivors of the Acute Respiratory Distress Syndrome
Authors: Angel Michael J. , Bril Vera , Shannon Patrick , Herridge Margaret S. 
Published: December 2014

Background:Survivors of acute respiratory distress syndrome (ARDS) report generalized weakness and reduced exercise tolerance up to two years following discharge from the intensive care unit (ICU). Persistent neuromuscular complications of ARDS may contribute to the functional disability observed in these patients.Methods:Sixteen ARDS survivors underwent comprehensive neurological evaluation and standardized electrodiagnostic testing 6 to 24 months after ICU discharge. Four of these patients agreed to open muscle biopsy.Results:Seven of sixteen patients had clinically significant focal compressive mononeuropathies. Electrodiagnostic testing failed to reveal any changes attributable to critical illness polyneuropathy or myopathy. All four muscle biopsies were abnormal, and although the pathological features were structurally non-specific, the presence of an acquired myopathy remains possible. Four patients had persistent mixed sensory complaints but had normal electrodiagnostic evaluation.Conclusions:The high frequency of mononeuropathies highlights the need for vigilance in daily ICU care. The findings also suggest that complaints of weakness and reduced exercise capacity in ARDS survivors may be related to combined effects of compressive neuropathies and generalized longstanding structural changes in muscle and may support an organic basis for longterm functional disability.