bluuebunny/crossref_metadata_2025_split huggingface dataset:
Severe Proximal Myopathy with Remarkable Recovery after Vitamin D Treatment
Authors: Al-Said Yousef A. , Al-Rached Hiyam S. , Al-Qahtani Hussien A. , Jan Mohammed M.S. 
Published: December 2014

Background:Osteomalacia is an uncommon cause of muscle weakness. Our objectives were to describe features of myopathy associated with Vitamin D deficiency and examine the contributing factors leading to osteomalacic myopathy in our region.Methods:Patients identified retrospectively for the six year period ending in December 2006 with the diagnosis of osteomalacia and/or Vitamin D deficiency associated proximal muscle weakness were included. They were followed in three major centers in western Saudi Arabia. Clinical, biochemical, radiological, and electrophysiological findings were collected before and after Vitamin D treatment by chart review.Results:Forty seven female patients aged 13-46 years (mean 23.5, SD 4.5) were included. All were veiled and covered heavily when outside the house for social and cultural reasons. Only eight (17%) had adequate varied diet with daily milk ingestion. All patients presented with progressive proximal muscle weakness lasting 6-24 months (mean 14) prior to our evaluation. The weakness was severe in six (13%) patients leading to wheel chair bound states. Associated musculoskeletal pain involving the back, hips, or lower limbs was common (66%). Osteomalcia was the referral diagnosis in only 11 patients and the remaining 36 (77%) patients were misdiagnosed. All patients had metabolic and radiological profiles suggestive of osteomalacia. Remarkable recovery was documented in all patients following oral cholecalciferol and calcium supplementation.Conclusions:Vitamin D deficiency is an important treatable cause of osteomalacic myopathy in Saudi Arabia. The diagnosis is frequently delayed or missed. Screening for Vitamin D deficiency in patients with acquired myopathy is needed to identify this treatable disorder.
Development of a Concise QOL Questionnaire for Brain Tumor Patients
Authors: Goffaux Philippe , Boudrias Marie , Mathieu David , Charpentier Catherine , Veilleux Nadia , Fortin David 
Published: December 2014

Background:The purpose of this study was to develop and validate a self-administered questionnaire to measure the health-related quality of life (QOL) of patients with brain cancer. We wanted to assess both core and disease-specific concerns in a single, easy-to-use instrument, thus promoting concision and clinical utility. The questionnaire departs from its predecessors in that it was designed for- and validated among French speaking Canadians.Methods:A focus group of health professionals was used to develop items for the questionnaire, which was later validated with 105 patients suffering from brain cancer. The underlying structure of the questionnaire was investigated using principal component analysis and confirmed using a principal factor analysis.Results:The final version of the questionnaire contains 30 items. Seven multi-item scales, tapping into distinct dimensions of QOL, were uncovered (i.e., functional well-being, symptom severity/fear of death, social support/acceptance of disease, autonomy in personal care, digestive symptomatology, neurocognitive function, and pain). Assessment of reliability revealed elevated internal consistency for each of the seven scales (Cronbach coefficient alpha ≥≥.65), whereas known-groups validity (anchor-based approach) revealed that the different dimensions uniquely discriminated between patients with different functional levels (Karnofsky Performance Scores) and clinical status (exposure to neurosurgery, radiotherapy, and use of chemotherapy and anticonvulsants).Conclusion:Our QOL questionnaire, the Sherbrooke Neuro-Oncology Assessment Scale, or SNAS, taps into both core and disease-specific issues relevant to neuro-oncology patients. It has good validity and reliability, and clearly reflects the multidimensional nature of QOL. Depending on the research focus, it may be used in clinical trials to track the impact of disease and/or treatment on satisfaction, functional status, and general well-being.
Adjuvant Chemotherapy for Adults with Malignant Glioma: A Systematic Review
Authors: Perry James , Laperriere Normand , Zuraw Lisa , Chambers Alexandra , Spithoff Karen , Cairncross J. Gregory 
Published: December 2014

Objective:This systematic review examines the role of chemotherapy following surgery and external beam radiotherapy for adults with newly diagnosed malignant glioma.Methods:MEDLINE, EMBASE, and the Cochrane Library databases were searched to August 2006 to identify relevant randomized controlled trials (RCTs) and meta-analyses. Proceedings from the 1997 to 2006 annual meetings of the American Society of Clinical Oncology were also searched.Results:Two RCTs reported a survival advantage in favour of radiotherapy with concomitant and adjuvant temozolomide compared with radiotherapy alone in patients with anaplastic astrocytoma or glioblastoma. Twenty-six RCTs and two meta-analyses detected either no advantage or a small survival advantage in favour of adjuvant chemotherapy.Conclusion:Concomitant temozolomide during radiotherapy and post-radiation adjuvant temozolomide is recommended for all patients ages 18-70 with newly diagnosed glioblastoma multiforme who are fit for radical therapy (ECOG 0-1). Temozolomide may be considered in other situations (i.e., ECOG 2, biopsy only, age >70, intermediate grade glioma), but there is no high-level evidence to support this decision. Moreover, there are few data on long-term toxicities or quality of life with temozolomide. Adjuvant chemotherapy may be an option for younger patients with anaplastic (grade 3) astrocytoma and patients with pure or mixed oligodendroglioma. However, there is no evidence of a survival advantage from adjuvant chemotherapy in these patients, and treatment-related adverse effects and their impact upon quality of life are poorly studied. The combination of procarbazine, lomustine, and vincristine (PCV) is not recommended for patients with anaplastic oligodendroglioma and oligoastrocytoma.
A Case-Control Study of Tissue Plasminogen Activator for Acute Ischemic Stroke
Authors: Reid J M , Phillips S J , Gubitz G J , Jarrett J , Christian C , Dai D 
Published: December 2014

Background:Randomized trials demonstrate that intravenous tissue plasminogen activator (tPA) improves outcome in acute ischemic stroke (AIS). To assess translation of this efficacy into effectiveness in routine clinical practice we performed a case-control study of tPA treatment for AIS in a single hospital.Methods:151 tPA-treated AIS patients (1996-2005) were matched 1:1 with blinding to outcome to controls from a prospective registry based on age, gender, pre-stroke Oxford handicap scale (OHS), stroke severity, and subtype. The outcomes were in-hospital death, symptomatic intracranial hemorrhage (SICH), length-of-stay (LOS), discharge OHS and long-term survival.Results:In-hospital mortality (23% vs. 24%) or long-term survival (median follow-up of 2 years) was not different between cases and controls (p=0.83). SICH occurred in 7.8% (95% CI 4.2-13.5%) of tPA-treated patients. Median LOS was non-significantly shorter for cases (13 [7-29] vs. 16 [8-32] days, p=0.14) but significantly shorter in tPA-treated vs. non-treated women (14 [7-28] vs. 20 [11-34] days, p=0.04). At discharge 6.6% (95% CI 1.1-12.0%) more tPA-treated patients than controls had no disability (OHS ≤1, p=0.02). However, there was no difference in discharge independence rates or proportion discharged home.Conclusion:We demonstrate minor improvements in early recovery after stroke with tPA but the impact is less dramatic than that reported in randomized trials. This may relate to timing of treatment and the type of patients treated.
PLEDS : corrélations cliniques
Authors: Fitzpatrick W. , Lowry N. 
Published: December 2014

Objective:We reviewed our experience in 96 consecutive patients exhibiting periodic lateralized epileptiform discharges (PLEDs) on EEG.Methods:EEG reports from January 1, 1999 to September 30, 2006 were screened for the term ‘PLEDs’ and its variants. A retrospective chart review, including examination of neuroimaging and other investigations, was conducted on each patient identified.Results:Acute stroke, tumor and central nervous system infection were the most common etiologies, accounting for 26%, 12% and 12% of cases respectively. Acute hemorrhage and traumatic brain injury combined accounted for another 12%. Previously unreported etiologies included posterior reversible encephalopathy syndrome (PRES), familial hemiplegic migraine and cerebral amyloidosis. There were 9 cases of chronic PLEDs attributable to underlying cortical dysplasia or severe remote cerebral injury, all with an accompanying partial seizure disorder. A prominent role for alcohol withdrawal was noted, and in 6 cases was the sole etiological factor. Fever was present as a potential contributing factor in 40% of cases, and significant metabolic abnormalities in 35%. Seizure activity occurred in 85% of patients overall, but in 100% of patients with PLEDs Plus and BiPLEDs Plus. The overall mortality rate was 27%. Mortality among patients with BiPLEDs however was almost twice that, at 52%.Conclusions:This case series demonstrates the wide variety of potential PLED etiologies. It also emphasizes that despite advances in neurocritical care, the morbidity and mortality associated with PLEDs has changed little since their recognition four decades ago.
Postural Sway and Effect of Levodopa in Early Parkinson's Disease
Authors: Beuter Anne , Hernández Roberto , Rigal Robert , Modolo Julien , Blanchet Pierre J. 
Published: December 2014

Objective:To characterize postural stability control and levodopa responsiveness in early Parkinson's disease (PD).Methods:Postural sway was studied during quiet stance in ten patients within six years of PD onset, both before (OFF) and after (ON) regular oral levodopa dosing. Postural sway was recorded using a force platform during 30 sec with eyes open, and six dependent variables were examined.Results:Mild baseline subclinical changes in postural sway were recorded in our patients. Clear benefit was observed in five out of six characteristics (mean sway, transversal sway, sagittal sway, sway intensity, and sway area) in the ON condition.Conclusion:Postural control mechanisms are affected early in PD and modulated by dopamine.
The Learning Curve of Minimally-Invasive Lumbar Microdiscectomy
Authors: McLoughlin Gregory S. , Fourney Daryl R. 
Published: December 2014

Object:The safe integration into practice of a new surgical technique requires an appreciation of the learning curve. The object of this study was to assess the learning curve for minimally invasive microdiscectomy (MIM) utilizing a tubular retractor system.Methods:A prospective evaluation of a single surgeon's first 52 consecutive MIM cases for radiculopathy secondary to single-level posterolateral lumbar disc herniation was performed. The learning curve was assessed using operative time, conversion to open rate, complications, and length of hospitalization.Results:The duration of operative time decreased over the course of the study (range, 49-151 min). By case 15, operative time was typically 60 min or less. There was only one conversion to an open procedure (Case 2). Complications occurred in three cases. All but nine patients were discharged home on the day of surgery.Conclusion:The learning curve for MIM was demonstrated. Further assessment of this curve for a large group of surgeons is necessary before a randomized controlled trial comparing standard microdiscectomy to MIM can be conducted.
ONF Trainee Awards Contribute to Capacity Building in Neurotrauma
Authors: Turner Jane , Turner Dan , Riopelle Richard J. , Bassett-Spiers Kent , Kagan Corinne 
Published: December 2014

Objective:Injury to the brain and spinal cord is one of the most catastrophic and costly occurrences in the Ontario health system. The objective of the present study was to evaluate the impact of past Ontario Neurotrauma Foundation (ONF) studentships and fellowships in terms of capacity building in the neurotrauma field in Ontario.Method:An online, cross sectional survey amongst past recipients of studentships and fellowships that terminated prior to July 2005. Explicit data were collected on various aspects of career development including current activity, awards and publications.Results:Thirty-six out of 42 (86%) eligible past trainees responded; 12 (33%) were Masters students, 12 (33%) were PhD students and 12 (33%) were Post-Doctoral students. A majority of the recipients (61%) are currently involved in neurotrauma-related activities (clinical, research and teaching) in more than 20% of their time, with no substantial differences between the degree groups. Half the recipients are currently involved in neurotrauma-related research in more than 20% of their time. The awardees published 1.5 peer-review manuscripts/person-year and received multiple awards. A high majority of our recipients (86%) feel that the ONF award had a substantial impact on their career.Conclusions:A high proportion of past award recipients remain involved in neurotrauma activities, especially in research. These results may lead to a cautious conclusion of the positive impact of the ONF studentships and fellowships on neurotrauma capacity building. These results should be considered in strategic planning of funding agencies similar to ONF.
Ataxias with Autosomal, X-Chromosomal or Maternal Inheritance
Authors: Finsterer Josef 
Published: December 2014

Heredoataxias are a group of genetic disorders with a cerebellar syndrome as the leading clinical manifestation. The current classification distinguishes heredoataxias according to the trait of inheritance into autosomal dominant, autosomal recessive, X-linked, and maternally inherited heredoataxias. The autosomal dominant heredoataxias are separated into spinocerebellar ataxias (SCA1-8, 10-15, 17-23, 25-30, and dentato-rubro-pallido-luysian atrophy), episodic ataxias (EA1-7), and autosomal dominant mitochondrial heredoataxias (Leigh syndrome, MIRAS, ADOAD, and AD-CPEO). The autosomal recessive ataxias are separated into Friedreich ataxia, ataxia due to vitamin E deficiency, ataxia due to Abeta-lipoproteinemia, Refsum disease, late-onset Tay-Sachs disease, cerebrotendineous xanthomatosis, spinocerebellar ataxia with axonal neuropathy, ataxia telangiectasia, ataxia telangiectasia-like disorder, ataxia with oculomotor apraxia 1 and 2, spastic ataxia of Charlevoix-Saguenay, Cayman ataxia, Marinesco-Sjögren syndrome, and autosomal recessive mitochondrial ataxias (AR-CPEO, SANDO, SCAE, AHS, IOSCA, MEMSA, LBSL CoQ-deficiency, PDC-deficiency). Only two of the heredoataxias, fragile X/tremor/ataxia syndrome, and XLSA/A are transmitted via an X-linked trait. Maternally inherited heredoataxias are due to point mutations in genes encoding for tRNAs, rRNAs, respiratory chain subunits or single large scale deletions/duplications of the mitochondrial DNA and include MELAS, MERRF, KSS, PS, MILS, NARP, and non-syndromic mitochondrial disorders. Treatment of heredoataxias is symptomatic and supportive and may have a beneficial effect in single patients.**Please see page 424 for abbreviation list.
Seizure Attacks While Driving: Quality of Life in Persons with Epilepsy
Authors: Tiamkao Somsak , Sawanyawisuth Kittisak , Towanabut Somchai , Visudhipun Pongsak ,   Thai QOL Epilepsy Investigators
Published: December 2014

Objective:To study the effect on quality of life (QOL) of a seizure attack while driving in persons with epilepsy (PWE).Methods:From four provincial and eight university hospitals in Thailand, we enrolled epileptic patients who drove a car or motorcycle or used to drive. The SF-36 questionnaire was used to evaluate QOL. The mean SF-36 score for all dimensions was calculated and compared with patients who either had or did not have a seizure attack while driving and in those who either had or had not been involved in a traffic accident while driving.Results:We had 245 adult PWE who drove a car or motorcycle or used to drive. Of these, 69 cases (28%) had a seizure attack whilst driving. Over half (36/69; 57%) had had seizure-related accidents, most of which were mild but about 20% needed hospitalization. PWE having a seizure attack while driving had a significantly lower QOL in four of the eight categories compared with patients who had not. PWE who had a seizure-related accident had a significantly lower mean value in the vitality category than those who did not.Conclusions:Seizure attacks while driving diminished QOL in PWE even though they only suffered minor injuries. Driving as a QOL issue should be discussed with patients. A good public transportation system would ease the need to drive.
Safety and Yield of Early Cessation of AEDs in Video-EEG Telemetry and Outcomes
Authors: Moien-Afshari Farzad , Griebel Robert , Sadanand Venkat , Vrbancic Mirna , Hernandez-Ronquillo Lizbeth , Lowry Noel , Téllez Zenteno José F. 
Published: December 2014

Background:Video-electroencephalography (VEEG) telemetry is the simultaneous recording of ictal and interictal EEG pattern and paroxysmal behavior to investigate the nature of paroxysmal events.Methods:This is a prospective study performed to asses the safety and yield of early discontinuation of antiepileptic drugs (AEDs) in the telemetry unit. Over a 2.5-year period, 50 patients that met the indications for VEEG monitoring were admitted by an epileptologist to neuro-observation units with continuous monitoring, nursing coverage and EEG technicians support during working hours and on-call thereafter. In most cases AEDs (except Phenobarbital) were discontinued in 24h. We prospectively assessed the yield and safety of the telemetry investigation as well as epilepsy surgery outcomes.Results:Our monitoring answered the study question in 88% of the patients. The question was not answered in 12% of cases due to the lack of recorded events. Our results changed the management in 74% of cases and potentially improved quality of life by decreasing the AEDs consumption and number of seizures per month. Over all, 22% received epilepsy surgery and became either seizure free or their seizures became non-disabling. Our method significantly decreased the duration of hospital admission for monitoring and minimal complications occurred only in 8% of patients.Conclusions:In conclusion, our method for short VEEG monitoring has a high yield for diagnosis, minimal complications and is cost effective. These qualities, together with good surgery results validate our method for the investigation and treatment of refractory seizure cases.
Underlying Venous Pathology Causing Perimesencephalic Subarachnoid Hemorrhage
Authors: Lee Jaejoon , Koh Eun-Mi , Chung Chin-Sang , Hong Seung-Chul , Kim Yong-Bum , Chung Pil-Wook , Suh Bum-Chun , Moon Heui-Soo 
Published: December 2014

Background:Perimesencephalic subarachnoid hemorrhage (PSH) is a relatively benign clinical entity with a low risk of recurrent bleeding. The precise etiology of PSH has not yet been determined. We report here three cases of PSH with clinical and radiological features that support a venous system as a cause.Case Presentation:The first patient, a 72-year-old woman, had PSH and venous hemorrhagic infarct in the left thalamus on non-contrast CT. Subsequent cerebral angiography revealed widespread thrombosis in the cerebral venous system, a potential cause for reflux overflow hemorrhage. The second patient, a 55-year-old man with an established diagnosis of neuro-Behçet's disease, a well-known cause for cerebral venulitis, presented with PSH one year later. The third patient, a 39-year-old female, with incomplete Behçet's disease was admitted with PSH.Discussion:Current concepts on the anatomic origin and the possible pathophysiologic mechanism leading to PSH are discussed. The underlying pathological conditions in the venous system in our cases provide theoretical clues to the anatomic origin of PSH in general.
The Metabolic Epicenter of Supratentorial Gliomas: A1H-MRSI Study
Authors: Sankar Tejas , Kuznetsov Yevgeniy E. , Ryan Robert W. , Caramanos Zografos , Antel Samson B. , Arnold Douglas L. , Preul Mark C. 
Published: December 2014

Background:Assessing the impact of glioma location on prognosis remains elusive. We approached the problem using multivoxel proton magnetic resonance spectroscopic imaging (1H-MRSI) to define a tumor “metabolic epicenter”, and examined the relationship of metabolic epicenter location to survival and histopathological grade.Methods:We studied 54 consecutive patients with a supratentorial glioma (astrocytoma or oligodendroglioma, WHO grades II-IV). The metabolic epicenter in each tumor was defined as the1H-MRSI voxel containing maximum intra-tumoral choline on preoperative imaging. Tumor location was considered the X-Y-Z coordinate position, in a standardized stereotactic space, of the metabolic epicenter. Correlation between epicenter location and survival or grade was assessed.Results:Metabolic epicenter location correlated significantly with patient survival for all tumors (r2= 0.30, p = 0.0002) and astrocytomas alone (r2= 0.32, p = 0.005). A predictive model based on both metabolic epicenter location and histopathological grade accounted for 70% of the variability in survival, substantially improving on histology alone to predict survival. Location also correlated significantly with grade (r2= 0.25, p = 0.001): higher grade tumors had a metabolic epicenter closer to the midpoint of the brain.Conclusions:The concept of the metabolic epicenter eliminates several problems related to existing methods of classifying glioma location. The location of the metabolic epicenter is strongly correlated with overall survival and histopathological grade, suggesting that it reflects biological factors underlying glioma growth and malignant dedifferentiation. These findings may be clinically relevant to predicting patterns of local glioma recurrence, and in planning resective surgery or radiotherapy.
Cost-Effectiveness: Cholinesterase Inhibitors and Memantine in Vascular Dementia
Authors: Wong Camilla L. , Bansback Nick , Lee Philip E. , Anis Aslam H. 
Published: December 2014

Background:Several randomized controlled trials of cholinesterase inhibitors and memantine in mild to moderate vascular dementia have demonstrated the efficacy of these treatments. However, given these drugs incur considerable cost, the economic argument for their use is less clear.Objective:To determine the incremental cost-effectiveness of cholinesterase inhibitors and memantine for mild to moderate vascular dementia.Design:A decision analysis model using a 24-28 week time horizon was developed. Outcomes of cholinesterase inhibitors and memantine and probabilities of adverse events were extracted from a systematic review. Costs of adverse events, medications, and physician visits were obtained from local estimates. Robustness was tested with probabilistic sensitivity analysis using a Monte Carlo simulation.Interventions:Donepezil 5 mg daily, donepezil 10 mg daily, galantamine 16-24 mg daily, rivastigmine flexible dosing up to 6 mg twice daily, or memantine 10 mg twice daily versus standard care.Main Outcome Measures:Incremental cost-effectiveness ratio (ICER) expressed as cost per unit decrease in the Alzheimer's Disease Assessment Scale-cognitive (ADAS-cog) subscale.Results:Donepezil 10 mg daily was found to be the most cost-effective treatment with an ICER of $400.64 (95%CI, $281.10-$596.35) per unit decline in the ADAS-cog subscale. All other treatments were dominated by donepezil 10 mg, that is, more costly and less effective.Conclusion:From a societal perspective, treatment with cholinesterase inhibitors or memantine was more effective but also more costly than standard care for mild to moderate vascular dementia. The donepezil 10 mg strategy was the most cost-effective and also dominated the other alternatives.