Evaluation of muscle strength and motor abilities in children with type II and III spinal muscle atrophy treated with valproic acid.

abstract：BACKGROUND:The autophagic vacuolar myopathies (AVM) are a group of inherited myopathies defined by the presence of autophagic vacuoles in pathological muscle specimens. AVM can be categorized into three groups: acid maltase deficiency, myopathies characterized by autophagic vacuoles with unique sarcolemmal features, an...

journal_title：BMC neurology

authors： Bokuda K,Sugaya K,Tamura S,Miyamoto K,Matsubara S,Komori T

更新日期：2012-11-21 00:00:00

abstract：BACKGROUND:Glutamic acid decarboxylase (GAD) is the rate-limiting enzyme for producing γ-aminobutyric acid, and it has been suggested that antibodies against GAD play a role in neurological conditions and type 1 diabetes. However, it is not known whether dementia appears as the sole neurological manifestation associate...

journal_title：BMC neurology

authors： Takagi M,Yamasaki H,Endo K,Yamada T,Kaneko K,Oka Y,Mori E

更新日期：2011-12-21 00:00:00

abstract：BACKGROUND:Due to lack of any curative therapy for ALS, symptomatic treatment and maintenance of quality of life (QoL) is very important. We aimed to characterize the affected domains of QoL in ALS patients and to identify factors which are associated with reduced QoL and increased depression. METHODS:159 ALS patients...

journal_title：BMC neurology

authors： Körner S,Kollewe K,Abdulla S,Zapf A,Dengler R,Petri S

更新日期：2015-05-16 00:00:00

abstract：BACKGROUND:Acceptance and Commitment Therapy (ACT), as a type of behavioral therapy, attempts to respond to changes in people's performance and their relationship to events. ACT can affect sleep quality by providing techniques to enhance the flexibility of patients' thoughts, yet maintaining mindfullness. Therefore, fo...

journal_title：BMC neurology

authors： Salari N,Khazaie H,Hosseinian-Far A,Khaledi-Paveh B,Ghasemi H,Mohammadi M,Shohaimi S

更新日期：2020-08-13 00:00:00

abstract：BACKGROUND:Dopaminergic neuronal loss begins years before motor symptoms appear in Parkinson disease (PD). Thus, reliable biomarkers for early diagnosis and prognosis of PD are an essential pre-requisite to develop disease modifying therapies. Inflammation-derived oxidative stress is postulated to contribute to nigrost...

journal_title：BMC neurology

authors： Rathnayake D,Chang T,Udagama P

更新日期：2019-04-06 00:00:00

abstract：BACKGROUND:Nosocomial infection (NI) control is an important issue in neurocritical care due to secondary brain damage and the increased morbidity and mortality of primary acute neurocritical care patients. The primary aim of this study was to determine incidence of nosocomial infections and multidrug-resistant bacteri...

journal_title：BMC neurology

authors： Spatenkova V,Bradac O,Fackova D,Bohunova Z,Suchomel P

更新日期：2018-03-07 00:00:00

abstract：BACKGROUND:Parkinsonian symptoms have been associated with increased morbidity and mortality. Several studies have reported on the prevalence of signs and symptoms. Symptoms questionnaires can identify potential PD cases for further neurological examination to save resources. They can also provide information about how...

journal_title：BMC neurology

authors： Ishihara LS,Khaw KT,Luben R,Bingham S,Welch A,Day N,Brayne C

更新日期：2005-08-24 00:00:00

abstract：BACKGROUND:Friedreich ataxia is a neurological disease originating from an iron-sulfur cluster enzyme deficiency due to impaired iron handling in the mitochondrion, aconitase being particularly affected. As a mean to counteract disease progression, it has been suggested to chelate free mitochondrial iron. Recent years ...

journal_title：BMC neurology

authors： Goncalves S,Paupe V,Dassa EP,Rustin P

更新日期：2008-06-16 00:00:00

abstract：BACKGROUND:The prognostic impact of preadmission use of calcium channel blockers (CCBs) and beta blockers (BBs) on stroke mortality remains unclear. We aimed to examine whether preadmission use of CCBs or BBs was associated with improved short-term mortality following ischemic stroke, intracerebral hemorrhage (ICH), or...

journal_title：BMC neurology

authors： Sundbøll J,Schmidt M,Horváth-Puhó E,Christiansen CF,Pedersen L,Bøtker HE,Sørensen HT

更新日期：2015-03-07 00:00:00

abstract：BACKGROUND:Prediction of clinical outcome in the acute stage of ischaemic stroke can be difficult when based on patient characteristics, clinical findings and on non-contrast CT. CT perfusion and CT angiography may provide additional prognostic information and guide treatment in the early stage. We present the study pr...

journal_title：BMC neurology

authors： van Seeters T,Biessels GJ,van der Schaaf IC,Dankbaar JW,Horsch AD,Luitse MJ,Niesten JM,Mali WP,Kappelle LJ,van der Graaf Y,Velthuis BK,DUST investigators.

更新日期：2014-02-25 00:00:00

abstract：BACKGROUND:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism. CASE PRESENTATION:A 53-year-old man presenting wi...

journal_title：BMC neurology

authors： Hira K,Shimura H,Kamata R,Takanashi M,Hashizume A,Takahashi K,Sugiyama M,Izumi H,Hattori N,Urabe T

更新日期：2019-11-15 00:00:00

abstract：BACKGROUND:'Neuromarketing' is a term that has often been used in the media in recent years. These public discussions have generally centered around potential ethical aspects and the public fear of negative consequences for society in general, and consumers in particular. However, positive contributions to the scientif...

journal_title：BMC neurology

authors： Javor A,Koller M,Lee N,Chamberlain L,Ransmayr G

更新日期：2013-02-06 00:00:00

abstract：BACKGROUND:Spinal cerebrospinal fluid (CSF) leak can lead to intracranial hypotension and is an important differential diagnosis to consider in patients with sudden-onset chronic daily headaches. Pars interarticularis (PI) fracture is a potential rare cause of suspected spinal CSF leak. METHODS:This is a retrospective...

journal_title：BMC neurology

authors： Chan TLH,Cowan R,Hindiyeh N,Hashmi S,Lanzman B,Carroll I

更新日期：2020-04-29 00:00:00

abstract：BACKGROUND:Cardioembolic stroke (CES) due to atrial fibrillation (AF) is associated with high stroke mortality. Oral anticoagulation (OAC) reduces stroke mortality, however, the impact of OAC-administration during hospital stay post ischemic stroke on mortality is unclear. We determined whether the timing of OAC initia...

journal_title：BMC neurology

authors： Palm F,Kraus M,Safer A,Wolf J,Becher H,Grau AJ

更新日期：2014-10-08 00:00:00

abstract：BACKGROUND:An Early Supported Discharge (ESD) and rehabilitation from a coordinated team in the home environment is recommended in several high-income countries for patients with mild to moderate symptoms after stroke. Returning home from the hospital takes place very early in Sweden today (12 days post stroke), thus t...

journal_title：BMC neurology

authors： Nordin Å,Sunnerhagen KS,Axelsson ÅB

更新日期：2015-11-16 00:00:00

abstract：BACKGROUND:Sarcoidosis is a multi-organ disease of unknown etiology characterised by the presence of epithelioid granulomas, without caseous necrosis. Systemic sarcoidosis is rare among children, while neurosarcoidosis in children is even rarer whether it is systemic or not. CASE PRESENTATION:We described the case of ...

journal_title：BMC neurology

authors： Shijo K,Moro N,Sasano M,Watanabe M,Yagasaki H,Takahashi S,Homma T,Yoshino A

更新日期：2018-05-29 00:00:00

abstract：BACKGROUND:Previous epidemiologic studies have examined the association of smoking with amyotrophic lateral sclerosis (ALS) incidence, but their results have been inconsistent. Moreover, limited information exists on the association between smoking and survival in ALS patients. We evaluated the association of smoking w...

journal_title：BMC neurology

authors： Alonso A,Logroscino G,Jick SS,Hernán MA

更新日期：2010-01-14 00:00:00

abstract：BACKGROUND:Seizures are one of the most important neurologic complications of human immuno-deficiency virus (HIV)-negative cryptococcal meningitis. A better understanding of the risk associated factors can help predict those who will require treatment. METHODS:This 22-year retrospective study enrolled 180 patients. Pr...

journal_title：BMC neurology

authors： Hung CW,Chang WN,Kung CT,Tsai NW,Wang HC,Lin WC,Huang CR,Huang CC,Tsai WC,Chang HW,Su YJ,Lin YJ,Cheng BC,Chang YT,Su CM,Lu CH

更新日期：2014-10-13 00:00:00

abstract：BACKGROUND:Patients with ischemic cerebrovascular diseases are more likely to suffer from intracranial aneurysms, and their surgical treatment has a growing controversy in this condition. The current case series was aimed at exploring surgical treatment and perioperative management of intracranial aneurysms in Chinese ...

journal_title：BMC neurology

authors： Zheng Y,Wu C

更新日期：2018-09-14 00:00:00

abstract：BACKGROUND:Machado-Joseph disease (MJD), or spinocerebellar ataxia type 3 (SCA3), is an autosomal dominant neurodegenerative disorder of late onset, which is caused by a CAG repeat expansion in the coding region of the ATXN3 gene. This disease presents clinical heterogeneity, which cannot be completely explained by the...

journal_title：BMC neurology

authors： Bettencourt C,Santos C,Coutinho P,Rizzu P,Vasconcelos J,Kay T,Cymbron T,Raposo M,Heutink P,Lima M

更新日期：2011-10-24 00:00:00

abstract：BACKGROUND:Young ischemic stroke patients are common while classification and analysis based upon imaging characteristics are rarely reported. We intend to compare the clinical and MRI characteristics of cerebral stroke induced by intracranial atherosclerosis between young patients with branch occlusive disease (BOD) a...

journal_title：BMC neurology

authors： Zhao ZN,Li XL,Liu JZ,Jiang ZM,Wang AH

更新日期：2018-06-20 00:00:00

abstract：BACKGROUND:Anti-epileptic drugs (AEDs) are frequently prescribed to persons with HIV/AIDS receiving combination antiretroviral therapy (cART) although the extent of AED use and their interactions with cART are uncertain. Herein, AED usage, associated toxicities and immune consequences were investigated. METHODS:HIV re...

journal_title：BMC neurology

authors： Lee K,Vivithanaporn P,Siemieniuk RA,Krentz HB,Maingat F,Gill MJ,Power C

更新日期：2010-06-17 00:00:00

abstract：BACKGROUND:This study aims to investigate the clinical value of two kinds of vestibular-evoked myogenic potentials in patients with sudden sensorineural hearing loss (SSNHL). METHODS:A total of 82 patients were divided into two groups: vertigo group and non-vertigo group. All patients underwent examinations for pure t...

journal_title：BMC neurology

authors： Wang Y,Gu ST,Bao XL,Guo JL

更新日期：2019-12-21 00:00:00

abstract：BACKGROUND:Primary microcephaly is defined as reduced occipital-frontal circumference noticeable before 36 weeks of gestation. Large amount of insults might lead to microcephaly including infections, hypoxia and genetic mutations. More than 16 genes are described in autosomal recessive primary microcephaly. However, th...

journal_title：BMC neurology

authors： Picher-Martel V,Labrie Y,Rivest S,Lace B,Chrestian N

更新日期：2020-02-15 00:00:00

abstract：BACKGROUND:Heterogeneous disease trajectories of mild cognitive impairment (MCI) and dementia are frequently encountered in clinical practice, but there is still insufficient knowledge to understand the reasons and mechanisms causing this heterogeneity. In addition to correlates of the disorder, patient characteristics...

journal_title：BMC neurology

authors： Liao W,Hamel RE,Olde Rikkert MG,Oosterveld SM,Aalten P,Verhey FR,Scheltens P,Sistermans N,Pijnenburg YA,van der Flier WM,Ramakers IH,Melis RJ

更新日期：2016-11-25 00:00:00

abstract：BACKGROUND:Association of leukoencephalopathy and atypical mycobacteriosis has been rarely reported. We present a case that is relevant for its unusual presentation and because it may shed further light on the pathogenic mechanisms underlying reversible encephalopathies. CASE REPORT:We report the case of a Hispanic 64...

journal_title：BMC neurology

authors： Oliveira MC,Sato DK,Soares-Neto HR,Lucato LT,Callegaro D,Nitrini R,Medeiros RS,Misu T,Fujihara K,Castro LH

更新日期：2015-09-02 00:00:00

abstract：BACKGROUND:The impact of comorbidity on multiple sclerosis (MS) is a new area of interest. Limited data on the risk factors of metabolic syndrome (MetS) is currently available. The aim of this study was to estimate the presence of comorbid conditions and MetS in a sample of adult patients with MS. METHODS:A retrospect...

journal_title：BMC neurology

authors： Sicras-Mainar A,Ruíz-Beato E,Navarro-Artieda R,Maurino J

更新日期：2017-07-17 00:00:00

abstract：BACKGROUND:Deep brain stimulation of the subthalamic nucleus significantly improves motor function in patients with severe Parkinson's disease. However, the effects on nonmotor aspects remain uncertain. The present study investigated the effects of subthalamic nucleus deep brain stimulation on mood and psychosocial fun...

journal_title：BMC neurology

authors： Kaiser I,Kryspin-Exner I,Brücke T,Volc D,Alesch F

更新日期：2008-11-11 00:00:00

abstract：BACKGROUND:Myotonic dystrophy type 1 (MD1) is one of the most prevalent neuromuscular diseases, yet very little is known about how MD1 affects the lives of couples and how they themselves manage individually and together. To better match health care to their problems, concerns and needs, it is important to understand t...

journal_title：BMC neurology

authors： Cup EH,Kinébanian A,Satink T,Pieterse AJ,Hendricks HT,Oostendorp RA,van der Wilt GJ,van Engelen BG

更新日期：2011-07-13 00:00:00

abstract：:The term paraproteinemic neuropathy describes a heterogeneous set of neuropathies characterized by the presence of homogeneous immunoglobulin in the serum. An abnormal clonal proliferation of B-lymphocytes or plasma cells, which may or may not occur in the context of a hematologic malignancy, produces the immunoglobul...

journal_title：BMC neurology

authors： Rison RA,Beydoun SR

更新日期：2016-01-28 00:00:00