bluuebunny/crossref_metadata_2025_split huggingface dataset:
Spinocerebellar Ataxia Type 12 was not Found in Korean Parkinsonian Patients
Authors: Cho Jin Whan , Kim Sung Yeon , Park Sung Sup , Jeon Beom S. 
Published: December 2014

Background:Parkinsonism (PD) is occasionally seen in several types of spinocerebellar ataxia (SCA). Mutations in SCA gene have been reported in the patients of parkinsonism without ataxia.Methods:We examined spinocerebellar ataxia type 12 mutation in 877 PD and 199 multiple system atrophy (MSA) patients.Results and Conclusions:No patients showed abnormal SCA12 expansion. It suggests that PD and MSA are not associated with SCA12 and it is not necessary to screen SCA12 in PD and MSA patients.
The Vestibulo-ocular Reflex During Active Head Motion in Chiari II Malformation
Authors: Salman Michael S. , Sharpe James A. , Lillakas Linda , Dennis Maureen , Steinbach Martin J. 
Published: December 2014

Background:Chiari type II malformation (CII) is a developmental anomaly of the cerebellum and brainstem, which are important structures for processing the vestibulo-ocular reflex (VOR). We investigated the effects of the deformity of CII on the angular VOR during active head motion.Methods:Eye and head movements were recorded using an infrared eye tracker and magnetic head tracker in 20 participants with CII [11 males, age range 8-19 years, mean (SD) 14.4 (3.2) years]. Thirty-eight age-matched healthy children and adolescents (21 males) constituted the control group. Participants were instructed to ‘look’ in darkness at the position of their thumb, placed 25 cm away, while they made horizontal and vertical sinusoidal head rotations at frequencies of about 0.5 Hz and 2 Hz. Parametric and non-parametric tests were used to compare the two groups.Results:The VOR gains, the ratio of eye to head velocities, were abnormally low in two participants with CII and abnormally high in one participant with CII.Conclusion:The majority of participants with CII had normal VOR performance in this investigation. However, the deformity of CII can impair the active angular VOR in some patients with CII. Low gain is attributed to brainstem damage and high gain to cerebellar dysfunction.
Intraspinal Neurenteric Cysts in Children
Authors: Cai Chunquan , Shen Changhong , Yang Weidong , Zhang Qingjiang , Hu Xiaoli 
Published: December 2014

Background:Neurenteric cysts are rare congenital epithelium-lined cysts of the central nervous system. They are found predominantly in the spinal cord, with lower incidence in the intracranial compartment, and may be associated with various other congenital spinal anomalies. Seven patients with symptomatic intraspinal neurenteric cysts are presented.Materials and Methods:Seven patients with intraspinal neurenteric cysts aged from nine months to ten years treated at this hospital from May 2000 to July 2006 were reviewed. The clinical manifestations, imaging and surgical findings of patients were analyzed retrospectively. All patients underwent operation. One patient's cervical neurenteric cyst was resected using the lateral cervical approach, and the other six resections were performed with posterior approach.Results:All seven patients presented with neurological involvement. One patient had an intramedullary cyst, while the other six cysts were situated ventrally. Three patients' cysts occurred in the cervical region, two in the cervicothoracic region, one in the thoracic region and one in the lumbar region. One patient had bony anomalies, and one had a lumbar posterior occult spinal dysraphism. Five patients' symptoms improved rapidly after surgery.Conclusions:Intraspinal neurenteric cysts in children are rare and most occur ventral to the spinal cord. Magnetic resonance imaging (MRI) is the most effective imaging modality. Earlier diagnosis and surgical resection of spinal neurenteric cysts improves prognosis.
Circulating Endothelial Cells as Potential Markers of Atherosclerosis
Authors: Gao Yang , Liu Chunyan , Zhang Xiangjian , Gao Jian , Yang Chunyan 
Published: December 2014

Background and Purpose:Vascular endothelial cell (VEC) injury represents a major initiating step in the process of atherosclerosis, which may lead to cerebral infarction. “Circulating endothelial cell” (CEC) is an index of ongoing endothelial injury, while intimal-medial thickness (IMT) detected by sounography was used to evaluate the severity of atherosclerosis. However, to our knowledge, there is no study that investigated the relationship of these two determinations. Our study was designed to address correlate CEC with IMT.Methods and Results:The study population consisted of 30 patients with acute cerebral infarction (ACI) and 30 age-and sex-matched volunteers as controls. The CEC counts were determined using Hladovec's method. All subjects underwent a 2-dimensional ultrasound examination of both carotid arteries to measure IMT. CEC counts in ACI group were significantly increased compared with control group (4.88±2.14 cells /0.9μl vs 2.73±1.95/0.9μl, P0.01); IMT in ACI patients was also significantly thicker compared with volunteers (2.72±1.07 mm vs 1.73±0.99 mm, P<0.01). There was positive correlation between CEC counts and maximal carotid artery IMT in both groups (r=0.522, P<0.01 in ACI patients and r=0.395, P<0.05 in healthy volunteers).Conclusions:Circulating endothelial cell counts can directly reflect the vascular injury. CEC counts parallel IMT. The CEC may be an independent predictor of cerebral infarction.
Multisystem Disorder in Late-Onset Chronic Progressive External Ophthalmoplegia
Authors: Pfeffer Gerald , Sirrs Sandra , Wade N. Kevin , Mezei Michelle M. 
Published: December 2014

Abstract:Introduction:Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial syndrome on a disease spectrum with Kearns-Sayre syndrome (KSS). Clinical presentation is variable and our experience suggested that phenotypic differences exist in CPEO with onset after age 20.Methods:This descriptive study is a retrospective chart review of 40 patients with late-onset CPEO. Clinical features, laboratory and neurophysiology results were reviewed.Results:Multisystem dysfunction was very common in this series. Gastrointestinal dysfunction was more common than expected (60%) as was migraine headache (40%). Clinical characteristics on the KSS disease spectrum were uncommon in this series with only 2.5% having pigmentary retinopathy, 5% with cardiac conduction abnormality, and 22.5% having endocrinopathy (most often thyroid dysfunction rather than diabetes). Neurophysiology abnormalities included length-dependent axonal polyneuropathy in 44% (sometimes subclinical) and myopathic EMG changes in 26%. Exposure to sources of acquired mitochondrial toxicity including cigarette use and hepatitis C infection were more common than expected in this series.Discussion:Phenotype was different in this late-onset series compared with previous reports in CPEO patients. In this series of late-onset patients, multi-organ dysfunction was more common than previously reported in CPEO, and some classical mitochondrial manifestations, such as pigmentary retinopathy were rare. We suggest that acquired mitochondrial toxicity may have a role in the pathogenesis of adult-onset CPEO.
Effect of Lentiviral shRNA of Nogo Receptor on Rat Cortex Neuron Axon Outgrowth
Authors: Xu Shengming , Liu Mingyuan , Zhang Tao , Lv Bitao , Liu Baifeng , Yuan Wen 
Published: December 2014

Abstract:Background and Aims:Axon growth is crucial for injured neural tissue to recover; however it is difficult to achieve in general. Axon outgrowth is inhibited by the activation of the Nogo receptor (NgR) by one of three different ligands. The present study aimed to suppress the inhibitory effect of the three inhibitory proteins to facilitate axon outgrowth.Methods:A lentiviral vector, siNgR199 (that has the capacity to interfere with the gene of NgR expression), was constructed for suppressing the gene transcription of NgR. Rat cortex neurons and oligodendrocytes were prepared to observe the effect of siNgR199 on facilitating axon outgrowth.Results:After transfection, the lentiviral siRNA of NgR remained in target neurons for almost two weeks whereas the conventional siRNA of NgR remained in neurons less than five days. Lentivirus-mediated delivery of exogenous small interfering RNA (siNgR199) targeting NgR significantly reduced the expression of this receptor and promoted axon outgrowth. In contrast, provision of naked siRNA targeting NgR (NgRsiRNA) showed less inhibitory effect on NgR protein expression and did not affect axon outgrowth.Conclusions:Lentiviral siRNA of NgR effectively suppresses the expression of NgR in cultured neurons that facilitates the axon outgrowth. The data implicate that lentiviral siRNA of NgR has therapeutic potential in facilitating the recovery of injured neural tissue.
Blood Pressure and Early Clinical Outcome Among Acute Ischemic Stroke Patients
Authors: Zhang Jintao , Peng Ying , Fan Huanqing , Chen Mei , Xu Tan , Zhang Yonghong 
Published: December 2014

ABSTRACT:Objectives:The association between blood pressure (BP) and short-term clinical outcome of acute ischemic stroke is inconclusive. We investigated the association between BP in the first 72 hours following admission and death in-hospital and neurologic deficiency at discharge among patients with acute ischemic stroke.Methods:A total of 2675 acute ischemic stroke patients confirmed by a computed tomography scan or magnetic resonance imaging were included in the present study. Blood pressure in the first 72 hours after admission and other study variables were collected for all ischemic stroke patients. Neurological functions National Institute of Health Stroke Scale (NIHSS) were evaluated by trained neurologists at discharge. The study outcome was defined as death in-hospital and neurologic deficiency (NIHSS≥10) at discharge.Results:Systolic and diastolic BP were significantly and positively associated with odds of study outcome in acute ischemic stroke. For example, compared to those with a systolic BP<140 mmHg, multiple-adjusted odds ratio (95% confidence interval) of study outcome was 3.29(1.22, 8.90) among participants with systolic BP of 180-219 mmHg,P<0.05; compared to those with a diastolic BP<90 mmHg, multiple-adjusted odds ratio of study outcome was 7.05(1.32, 37.57) among participants with diastolic BP ≥ 120 mmHg,P<0.05.Conclusion:Systolic BP≥180 and diastolic BP≥120 were significantly and positively associated with death in-hospital or neurologic deficiency at discharge among patients with acute ischemic stroke.
Carotid Endarterectomy Versus Stenting: A Meta-Analysis of Randomized Trials
Authors: Yavin Daniel , Roberts Derek J. , Tso Michael , Sutherland Garnette R. , Eliasziw Misha , Wong John H. 
Published: December 2014

Background:A meta-analysis of randomized controlled trials (RCTs) was conducted to update the available evidence on the safety and efficacy of carotid endarterectomy (CEA) versus carotid artery stenting (CAS) in the treatment of carotid artery stenosis.Methods:A comprehensive search was performed of MEDLINE, EMBASE, CENTRAL, bibliographies of included articles and past systematic reviews, and abstract lists of recent scientific conferences. For each reported outcome, a Mantel-Haenszel random-effects model was used to calculate odds ratios (ORs) and 95% confidence intervals (CI). The I2statistic was used as a measure of heterogeneity.Results:Twelve RCTs enrolling 6,973 patients were included in the meta-analysis. Carotid artery stenting was associated with a significantly greater odds of periprocedural stroke (OR 1.72, 95% CI 1.20 to 2.47) and a significantly lower odds of periprocedural myocardial infarction (OR 0.47, 95% CI 0.29 to 0.78) and cranial neuropathy (OR 0.08, 95% CI, 0.04 to 0.16). The odds of periprocedural death (OR 1.11, 95% CI 0.56 to 2.18), target vessel restenosis (OR 1.95, 95% CI 0.63 to 6.06), and access-related hematoma were similar following either intervention (OR 0.60, 95% CI 0.30 to 1.21).Conclusions:In comparison with CEA, CAS is associated with a greater odds of stroke and a lower odds of myocardial infarction. While the results our meta-analysis support the continued use of CEA as the standard of care in the treatment of carotid artery stenosis, CAS is a viable alternative in patients at elevated risk of cardiac complications.
Frontal Assessment Battery to Evaluate Frontal Lobe Dysfunction in ALS Patients
Authors: Ahn Suk-Won , Kim Su-Hyun , Kim Jee-Eun , Kim Sung-Min , Kim Seung Hyun , Sung Jung-Joon , Lee Kwang-Woo , Hong Yoon-Ho 
Published: December 2014

Background:Assessment of frontal lobe impairment in amyotrophic lateral sclerosis (ALS) is a matter of great importance, since it often causes ALS patients to decrease medication and nursing compliance, thus shortening their survival time.Methods:The frontal assessment battery (FAB) is a short and rapid method for assessing frontal executive functions. We investigated the applicability of the FAB as a screening method for assessing cognitive impairments in 61 ALS patients. Depending on the results of the FAB, we classified patients into two subgroups: FAB-normal and FAB-abnormal. We then performed additional evaluations of cognitive function using the Korean version of the mini-mental state examination (K-MMSE), a verbal fluency test (COWAT), and a neuropsychiatric inventory (NPI). Results of these tests were compared between the two groups using Mann-Whitney U-tests, and Spearman correlation analyses were used to investigate the relationships between FAB score and disease duration and severity.Results:Of the 61 sporadic ALS patients included in this study, 14 were classified as FAB-abnormal and 47 were classified as FAB-normal. The FAB-normal and FAB-abnormal patients performed significantly differently in all domains of the COWAT. There was no difference in behavioral disturbance, as assessed by the NPI, between the two groups. The FAB scores were found to significantly correlate with both disease duration and severity.Conclusions:The FAB shows promise as a method of screening for frontal lobe dysfunction in ALS, as it is not only quick and easy, but also reliable. Additional studies should examine how FAB performance changes as ALS progresses.
Early Treatment of a Progressive Rasmussen's Like Syndrome with Ganciclovir
Authors: McLachlan Richard S. , Diosy David , Levin Simon 
Published: December 2014

Background:Escalating focal sensorimotor seizures, progressive neurologic deficit and cognitive decline with associated typical magnetic resonance imaging (MRI) findings or pathological confirmation constitute the syndrome of Rasmussen's encephalitis.Methods:Three patients with this clinical scenario had long-term follow-up after being given the antiviral drug ganciclovir 10 mg/kg/day IV for ten days within one to three months of disease onset.Results:Seizures occurred at least hourly and were localized to the Rolandic region. The MRIs were normal in one patient and in two showed changes consistent with ongoing seizures. Two patients, one whose short-term outcome was reported previously, had immediate and sustained cessation of seizures and resolution of their neurologic deficit. One patient with seizures from both hemispheres did not respond and went on to hemispherectomy that confirmed chronic encephalitis. Seizures continued in five other patients treated 6 to 72 months after disease onset.Conclusion:The sustained seizure control after ganciclovir in two of three patients suggests this drug may be effective when given early in the course of this subacute intractable focal epilepsy syndrome.
Adherence to Multiple Sclerosis Disease-Modifying Therapies in Ontario is Low
Authors: Wong Janice , Gomes Tara , Mamdani Muhammad , Manno Michael , O'Connor Paul W. 
Published: December 2014

Background/Objective:Differences in patient adherence to various disease-modifying drugs (DMDs) in the treatment of multiple sclerosis (MS) are not well understood. The goal of this study was to evaluate adherence of adult MS patients in Ontario with public drug plan coverage to various DMDs: intramuscular interferon beta-1a (i.m. IFNβ-1a, Avonex), subcutaneous interferon beta-1a (s.c. IFNβ-1a, Rebif), subcutaneous interferon beta-1b (IFNβ-1b, Betaseron) or glatiramer acetate (Copaxone).Methods:In this retrospective cohort study, Ontario Public Drug Plan beneficiaries aged 15 or older who were newly treated with i.m. IFNβ-1a, s.c. IFNβ-1a, IFNβ-1b or glatiramer acetate between April 2006 and March 2008 were followed forward until treatment discontinuation, switch to another DMD or a maximum two year follow-up period. Cumulative persistence rates were analyzed by the Kaplan-Meier method. The proportion of patients reaching the study endpoints after the two year follow-up period was also calculated.Results:Cumulative persistence rates for all four DMDs were similar over time (p=0.80), ranging from 73.6-79.1% at six months, 59.1-63.1% at one year and 41.5-47.4% at two years. After two years, the proportion of patients who had discontinued treatment, switched to another DMD or died was similar among DMDs (p=0.79, Fisher's exact test). Switching between DMD types was low and occurred in 3.4-6.5% of new DMD users.Conclusions:Adherence to DMDs in adult MS patients in Ontario is poor, which is consistent with previously reported adherence rates to MS DMDs in other regions. No significant differences in adherence exist between the DMDs evaluated in this study.
Lumbar Microdiscectomy: A Clinicoradiological Analysis of Outcome
Authors: Rahme Ralph , Moussa Ronald , Bou-Nassif Rabih , Maarrawi Joseph , Rizk Tony , Nohra Georges , Samaha Elie , Okais Nabil 
Published: December 2014

Background:The long-term outcome after lumbar microdiscectomy (LMD) may be affected by low back pain (LBP) and segmental instability, the determinants of which remain unclear. We sought to analyze the interaction between clinical, functional, and radiological variables and their impact on patient outcome.Methods:All patients who underwent LMD in 2004-2005 were invited to participate in this retrospective cohort study. Patients were re-evaluated clinically and radiologically after a three to five year follow-up.Results:Forty-one of 97 eligible patients were enrolled. Twelve patients (29.3%) reported moderate-to-severe sciatica, 12 (29.3%) had moderate LBP, and 13 (31.7%) exhibited clinical evidence of segmental instability. Thirty-eight patients (92.7%) had minimal disability and 3 (7.3%) had moderate disability. Twenty-three patients (56.1%) were fully satisfied, while 18 (43.9%) had only partial satisfaction, having expected a better outcome. Thirty-three patients (80.5%) returned to full-time work. Median disc space collapse (DSC) was 20% (range 5-66%) and L4-L5 was particularly affected. Prevalence of Modic changes increased from 46.3% to 78% with type 2 predominance. Multivariate logistic regression analysis identified the following negative prognostic factors: female sex, young age, lack of regular exercise, and chronic preoperative LBP. There was no correlation between the course of Modic changes, DSC, and patient outcome.Conclusion:Although many patients may be symptomatic following LMD, significant disability and dissatisfaction are uncommon. Female sex, young age, lack of exercise, and chronic preoperative LBP may predict a worse outcome. Disc collapse is a universal finding, particularly at L4-L5. Neither DSC nor Modic changes seem to affect patient outcome.
Pain Management in Post-Craniotomy Patients: A Survey of Canadian Neurosurgeons
Authors: Hassouneh Basil , Centofanti John E. , Reddy Kesava 
Published: December 2014

Introduction:Despite the growing recognition for analgesic needs in post-craniotomy patients, this remains a poorly studied area in neurological surgery. The class and regimen of analgesia that is most suitable for these patients remains controversial. The objective of this study is to examine the current beliefs and practices of Canadian neurosurgeons when managing post-craniotomy pain.Methods:A survey was sent to all practicing Canadian neurosurgeons to examine the following aspects of analgesia in craniotomy patients: type of analgesics used, common side effects encountered, satisfaction with current regimen and the rationale for their practice.Results:Of 156 potential respondents, 103 neurosurgeons (66%) completed the survey. Codeine (59%) was the most prescribed firstline analgesic followed by morphine (38%). The use of a second-line opioid was significantly higher among codeine prescribers compared to morphine, 53% compared to 28% (p < 0.001). Nausea, constipation and neurologic depression were reported as common side effects by 76%, 66% and 27% of respondents respectively. Of the respondents, 90% reported a high level of satisfaction with their current choice of analgesia; nonetheless, they predominantly described their practice as personal preference or protocol driven rather than evidence-based.Conclusions:Codeine - a weak opioid - is the most common first-line analgesic prescribed to craniotomy patients. This practice is associated with substantially increased reliance on potent opioids for rescue analgesia. Whether novel regimens can provide optimal pain control while minimizing neurologic and gastrointestinal side effects remains to be addressed by future trials.
ERK5: A Novel IKKα-Kinase in Rat Hippocampal Neurons
Authors: Goalstone Marc L. 
Published: December 2014

Background:Alzheimer's Disease (AD) is characterized in part by the increased presence of neurofibrillary tangles and amyloid beta (Aβ) plaques. Alzheimer's Disease is considered an inflammatory disease and, as such, nuclear factor-kappaB (NFκB) plays an important role in the pathophysiology of AD. Insulin acts as a neurotrophic factor. Yet, in the context of insulin resistance, concomitant hyperinsulinemia may contribute to the pathogenesis of AD.Methods:Rat Primmary Hippocampal Neurons (RPHN) were treated with insulin in the absence and presence of Wortmannin and ERK5 small inhibitory RNA and assayed for downstream effectors of activated ERK5.Results:Here we demonstrate that genetic inhibition of ERK5 blocks insulin stimulated (1) activation and translocation of ERK5 and NFκB, (2) phosphorylation of IKKα via association with ERK5, (3) increases in Aβ1-40 and Aβ1-42 soluble proteins 3-fold and 2.2-fold, respectively, and (4) increases in tau phosphorylation in RPHN.Conclusions:ERK5 plays an active role in insulin signaling in neurons and may be a potential therapeutic target for neurodegenerative diseases.
Causes for Treatment Delays in Dystonia and Hemifacial Spasm: A Canadian Survey
Authors: Jog Mandar , Chouinard Sylvain , Hobson Doug , Grimes David , Chen Robert , Bhogal Meetu , Simonyi Susan 
Published: December 2014

Background:Dystonia must be accurately diagnosed so that treatment can be administered promptly. However, dystonia is a complex disorder, with variable presentation, which can delay diagnosis.Methods:Data were gathered by questionnaire from 866 patients with dystonia or hemifacial spasm (HFS) treated in 14 movement disorders centres in Canada injecting botulinum toxin, to better understand the path to diagnosis, wait times and obstacles to treatment.Results:Most participants were female (64.1%), mean age was 58 years, and patients consulted an average of 3.2 physicians before receiving a dystonia or HFS diagnosis. Many patients (34%) received other diagnoses before referral to a movement disorders clinic, most commonly “stress” (42.7%). A variety of treatments were often received without a diagnosis. The mean lag time between symptom onset and diagnosis was 5.4 years. After the decision to use botulinum toxin, patients waited a mean of 3.1 months before treatment. The most common diagnoses were cervical dystonia (51.6% of patients), HFS (20.0%) and blepharospasm (9.8%).Conclusions:Survey results show that diagnosis of dystonias or of HFS, and therefore, access to treatment, is delayed. An educational program for primary care physicians may be helpful to decrease the time to diagnosis and referral to a specialist centre for treatment.