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Frequency of dementia in Parkinson's disease: A systematic review and meta-analysis
CatarinaSeveriano e SousaabJoanaAlarcãoacIsabelPavão MartinsbdJoaquim J.Ferreiraabe
doi : 10.1016/j.jns.2021.120077
Volume 432, 15 January 2022, 120077
There is a considerable variation in the reported frequency of dementia in Parkinson's disease (PDD). The aim of this study was to evaluate the frequency of PDD reported in published studies and to examine the different methodological, clinical, and demographic factors that may contribute to this variation.
Clinical milestones in Parkinson's disease: Past, present, and future
LucaMarsiliaAbhimanyuMahajanb
doi : 10.1016/j.jns.2021.120082
Volume 432, 15 January 2022, 120082
CNS and PNS manifestation in immune checkpoint inhibitors: A systematic review
ErumKhanaAshish K.ShresthabMahmoudElkhoolycHannahWilsondMichaelEbberteSamikshaSrivastavafSijinWengStevenRollinsgShitizSriwastavadefh
doi : 10.1016/j.jns.2021.120089
Volume 432, 15 January 2022, 120089
Immunomodulatory therapies, including the use of immune checkpoint inhibitors (ICIs), have made a profound impact on treatment of advanced cancers in recent decades. Neurologic immune-related adverse events (irAEs) related to use of these agents are rare but potentially fatal sequelae. This systematic reviewed aimed to describe onset, clinical features, treatment, and outcome of neurological irAEs following ICI usage.
Different K+-release in distal myogenic and neurogenic muscular weakness during non-ischemic exercise
PhilippBurowaSusanne A.GöbelaJaninaRichteraSteffenNaegelaFritzMarkwardtbStephanZierza
doi : 10.1016/j.jns.2021.120070
Volume 432, 15 January 2022, 120070
In myotonic dystrophy, an increased potassium release upon ischemic forearm exercise has been previously described. However, it remains unclear whether this is specific for myotonic dystrophies or just due to distal muscular weakness.
Characteristics of myotonic dystrophy patients in the national registry of Japan
MarikaSugimotoaSatoshiKurubHirotoTakadacRihoHorieaKosukeYamauchiaTomoyaKubotaaTsuyoshiMatsumuradHarumasaNakamuraeEnKimuraeMasanori P.Takahashia
doi : 10.1016/j.jns.2021.120080
Volume 432, 15 January 2022, 120080
Myotonic dystrophies (DM) are inherited autosomal dominant disorders affecting multiple organs. Currently available therapeutics for DM are limited; therefore, a patient registry is essential for therapeutic development and success of clinical trials targeting the diseases. We have developed a nationwide DM registry in Japan under the Registry of Muscular Dystrophy (Remudy). The registration process was patient-initiated; however, physicians certified the clinical information. The dataset includes all Naarden and TREAT-NMD core datasets and additional items covering major DM clinical features. As of March 2020, we enrolled 976 patients with genetically confirmed DM. The majority (99.9%) of these patients had DM1, with 11.4% having the congenital form. However, 1 patient had DM2. Upon classifying 969 symptomatic DM1 patients based on their age at onset, an earlier onset was associated with a longer CTG repeat length. Myotonia was the most frequent symptom, followed by hand disability, fatigue, and daytime sleepiness. The frequency of hand disabilities, constipation, and visual disturbances was higher for patients with congenital DM. According to a multiple regression analysis of objective clinical measurements related to prognosis and activities of daily living, CTG repeat length strongly influenced the grip strength, forced vital capacity, and QRS time in an electrocardiogram. However, the grip strength was only modestly related to disease duration. This report will shed light on the Japanese national DM registry, which has recruited a significant number of patients. The registry will provide invaluable data for planning clinical trials and improving the standard of care for patients.
Lessons learned in stroke care during COVID-19 pandemic and preparing for future pandemics in the MENA+ region: A consensus statement from the MENA+-SINO
AmalAl HashmiaStephanvon BandemerbAshfaqShuaibcOssama YassinMansourdMohammedWassyeAtilla OzcanOzdemirfMehdiFarhoudigHosamAl JehanihAdnanKhaniSebyJohnjMaherSaqqurklOn the behalf of MENA-SINO Organization1
doi : 10.1016/j.jns.2021.120060
Volume 432, 15 January 2022, 120060
COVID-19 pandemic has negatively impacted stroke care services at multiple levels. There was a decline in acute stroke admissions. Fewer interventions have been performed. Increased “door-to-needle times and “door-to-groin puncture” during this pandemic. These factors combined have led to declining in the favoured outcomes of stroke patients' globally. Yet this pandemic permits an opportunity for higher preparedness for future pandemics.
Basilar artery curvature is associated with migraine with aura in the Northern Manhattan Study
DixonYangaCenZhangbSetareh S.OmrancBrettCucchiaradTatjanaRundekeClinton B.WrightfRalph L.SaccoeMitchell S.V.ElkindagJoseGutierreza
doi : 10.1016/j.jns.2021.120073
Volume 432, 15 January 2022, 120073
Genetic and imaging studies demonstrate a link between vascular morphology and migraine with aura (MA). We examined the relationship between basilar artery (BA) curvature and MA in a population-based cohort of stroke-free participants.
Phenotypic categorisation of individual subjects with motor neuron disease based on radiological disease burden patterns: A machine-learning approach
PeterBedeabAizuriMuradaJasminLopeaStaceyLi Hi ShingaEoinFineganaRangariroyashe H.ChipikaaOrlaHardimanaKai MingChangac
doi : 10.1016/j.jns.2021.120079
Volume 432, 15 January 2022, 120079
Motor neuron disease is an umbrella term encompassing a multitude of clinically heterogeneous phenotypes. The early and accurate categorisation of patients is hugely important, as MND phenotypes are associated with markedly different prognoses, progression rates, care needs and benefit from divergent management strategies. The categorisation of patients shortly after symptom onset is challenging, and often lengthy clinical monitoring is needed to assign patients to the appropriate phenotypic subgroup. In this study, a multi-class machine-learning strategy was implemented to classify 300 patients based on their radiological profile into diagnostic labels along the UMN-LMN spectrum. A comprehensive panel of cortical thickness measures, subcortical grey matter variables, and white matter integrity metrics were evaluated in a multilayer perceptron (MLP) model. Additional exploratory analyses were also carried out using discriminant function analyses (DFA). Excellent classification accuracy was achieved for amyotrophic lateral sclerosis in the testing cohort (93.7%) using the MLP model, but poor diagnostic accuracy was detected for primary lateral sclerosis (43.8%) and poliomyelitis survivors (60%). Feature importance analyses highlighted the relevance of white matter diffusivity metrics and the evaluation of cerebellar indices, cingulate measures and thalamic radiation variables to discriminate MND phenotypes. Our data suggest that radiological data from single patients may be meaningfully interpreted if large training data sets are available and the provision of diagnostic probability outcomes may be clinically useful in patients with short symptom duration. The computational interpretation of multimodal radiology datasets herald viable diagnostic, prognostic and clinical trial applications.
Effect of natalizumab treatment on the rate of No Evidence of Disease Activity in young adults with multiple sclerosis in relation to pubertal stage
ShayMenascuabAvivaFattal-ValevskibcAdiVaknin-DembinskydRonMiloefKerenGevabiDavidMagalashviliabMarkDolevabShlomoFlectheragAlonKalronabShmulikMironabChenHoffmannbhRoyAloniabMichaelGurevichab1AnatAchironab1
doi : 10.1016/j.jns.2021.120074
Volume 432, 15 January 2022, 120074
Approximately 40% of young-onset multiple sclerosis (MS) patients experience breakthrough disease, which carries a high risk for long-term disability, and requires using therapies beyond traditional first-line agents. Despite the increasing use of newer disease-modifying treatments (DMTs) in this population, data are not available to guide the need for escalating DMTs and there is a scarcity of data on the effects of natalizumab in children and young adults with active disease. We performed a retrospective analysis of the rate of No Evidence of Disease Activity (NEDA), tolerability, and safety of natalizumab in a multi-center cohort of 36 children and young adults with highly active MS. All patients had active disease and initiated treatment with natalizumab. The primary endpoint was the rate of achieving NEDA-3 status, within two years of natalizumab treatment. To examine a possible effect of age on the outcome of treatment, outcomes were also analyzed by pre-pubertal (n = 13 children aged 9–13 years) and pubertal subgroups (n = 23 young adolescents aged 14–20 years). The NEDA-3 status of the pre-pubertal group was 92% in the first and second year and in the pubertal group - 96% in the first year and 92% in the second year. Natalizumab reduced the number and volume of brain lesions in both pre-pubertal and pubertal groups. Treatment was well-tolerated, only 8 patients (22.2%) had adverse events during the 2-year study period. Our analysis shows that natalizumab is effective and well-tolerated in pre-pubertal and pubertal MS patients.
Development of the SMA independence scale–upper limb module (SMAIS–ULM): A novel scale for individuals with Type 2 and non-ambulant Type 3 SMA
DylanTrundellaAnneSkalickybHannahStauntonaAshaHareendrancStephanieLe ScouilleraLouiseBarrettdOwenCoopercKsenijaGornieTimSeabrookfSangeetaJethwafStefanCanod
doi : 10.1016/j.jns.2021.120059
Volume 432, 15 January 2022, 120059
The amount of assistance required to perform daily activities for individuals with Type 2 and non-ambulant Type 3 spinal muscular atrophy (SMA) is often cited as meaningful for quality of life, and important to routinely assess.
Neurologic and cognitive sequelae after SARS-CoV2 infection: Different impairment for ICU patients
FlaviaMattiolia1SimonePivabe2ChiaraStampatoria1FrancescaRighettia1IlariaMegaa1ElenaPelib2EmmaSalacCesareTomasic2Anna MariaIndelicatoe3NicolaLatronicobde24GiuseppeDe Palmae34
doi : 10.1016/j.jns.2021.120061
Volume 432, 15 January 2022, 120061
The exact incidence of neurological and cognitive sequelae of COVID-19 in the long term is yet unknown. The aim of this research is to investigate the type of neurological and cognitive impairment in COVID-19 cases of different severity. Two hundred fifteen patients, who had developed COVID-19, were examined 4 months after the diagnosis by means of neurological exam and extensive cognitive evaluation, investigating general cognition, memory, verbal fluency, visuospatial abilities and executive functions. Fifty-two of them were treated in intensive care unit (ICU patients), whereas 163 were not hospitalized (non-ICU patients). Neurological deficits were found in 2/163 (1.2%) of non-ICU and in 7/52 (13.5%) of the ICU cases, all involving the peripheral nervous system. ICU patients performed significantly worse in all the neuropsychological tests and showed a worse age- and education-corrected cognitive impairment: Cognitive Impairment Index (CII) was higher in ICU than in non-ICU patients (median ICU 3 vs 2, p = .001). CII significantly correlated with age in both groups, was unrelated to length of follow- up, diabetes and hypertension and - only for ICU patients- to PaO2/FiO2 at ICU admission. Obtained results support the greater susceptibility of COVID-19 patients, treated in ICU, to develop neurological deficits and cognitive impairment at a four-month follow up, as compared to cases with mild/moderate symptoms.
Influence of single pass recanalization in acute ischemic stroke with large vessel occlusion in patients of Asian ethnicity
SoichiroNumaa1KazutakaUchidaa1NobuyukiSakaibHiroshiYamagamicManabuShirakawaaHirotoKageyamaaTakeshiMorimotodShinichiYoshimuraaRecovery by Endovascular Salvage for Cerebral Ultra-acute Embolism Japan Registry 2 (RESCUE-Japan Registry 2) Investigators
doi : 10.1016/j.jns.2021.120076
Volume 432, 15 January 2022, 120076
A previous report revealed single pass recanalization was associated with better functional outcome for patients with acute ischemic stroke with large vessel occlusion in real-world settings. However, the effect of single pass recanalization in acute large vessel occlusion based on the largest registry in real-world settings in Asian population are not well scrutinized.
Outcomes after reperfusion therapies in patients with ACA stroke: A multicenter cohort study from the EVATRISP collaboration
A.Filiogloa1N.Simaana1A.HonigaM.R.HeldnerbR.von RennenbergcA.PezzinidV.PadjeneA.RentzosfV.L.AltersbergergP.BaumgartnerhA.ZiniiI.GrisendijS.AladdinaJ.M.GomorikS.M.Pilgram-PastorlJ.F.ScheitzcM.MagonimI.BerisavaceA.NordanstignM.PsychogiosoA.LufthM.GentileiF.AssenzajM.ArnoldbC.H.NoltepM.GambalM.ErcegovaceK.JoodnS.T.EngeltergqS.WegenerhS.ForlivesiiM.ZeddejH.GensickegqT.TatlisumaknJ.E.CohenrR.R.Lekera
doi : 10.1016/j.jns.2021.120081
Volume 432, 15 January 2022, 120081
Patients with stroke secondary to occlusions of the anterior cerebral artery (ACA) often have poor outcomes. The optimal acute therapeutic intervention for these patients remains unknown.
Cerebrospinal fluid neurofilament light predicts the rate of executive function decline in younger-onset dementia
N.WaliaabD.EratnebcdS.M.LoibcQ.-X.LieS.VargheseeC.B.MalpasfgM.WalterfangbcdA.H.EvansgS.ParkerabS.J.CollinsehC.L.MasterseD.Velakoulisbcd
doi : 10.1016/j.jns.2021.120088
Volume 432, 15 January 2022, 120088
Determining disease severity and predicting prognosis in younger onset-dementia (YOD) remains challenging. Whether CSF biomarkers neurofilament light (NfL), tau and amyloid? 42 (A?42) can help provide such information has been underexplored.
Efficacy and safety of istradefylline in patients with Parkinson's disease presenting with postural abnormalities: Results from a multicenter, prospective, and open-label exploratory study in Japan
MakioTakahashiaToshioShimokawabJinsooKohcTakaoTakeshimadHirofumiYamashitaeYoshinoriKajimotofAkihisaMorigHidefumiItoc
doi : 10.1016/j.jns.2021.120078
Volume 432, 15 January 2022, 120078
Postural abnormalities in Parkinson's disease (PD) can devastatingly impair the quality of life, especially in patients with advanced disease, and are generally refractory to dopaminergic agents. The objective of this exploratory study was to investigate the efficacy and safety of istradefylline for the treatment of postural abnormalities in PD. In this open-label, 24-week, single-arm prospective trial, PD patients with postural abnormalities experiencing the wearing-off phenomenon on levodopa-containing therapies were enrolled and received a starting dose of 20 mg/day istradefylline orally for 4 weeks, which was then increased to 40 mg/day. The primary endpoint was the change from baseline to week 24 in the 14-item Unified Dystonia Rating Scale (UDRS) total score. Pivotal secondary endpoints were changes in the sub-items of UDRS, Movement Disorder Society-Unified Parkinson's Disease Rating Scale (MDS-UPDRS) part III, and adverse drug reactions (ADRs). Overall, 24/31 enrolled patients completed the study; mean (standard deviation) age and duration of motor complications were 73.3 (7.7) years and 3.2 (4.4) years, respectively. Mean (95% confidence interval [CI]) change in the UDRS total score was 4.84 (1.97, 7.71; P = 0.002), with significant improvements in the neck, right distal arm and hand, and trunk severity scores. Mean (95% CI) change in the MDS-UPDRS part III score was 7.84 (4.34, 11.34; P < 0.001). The most common ADRs were malaise, dyskinesia exacerbation, and visual hallucinations in 2 (6.5%) patients each. This exploratory study demonstrated that istradefylline could be efficacious for postural abnormalities and was generally well tolerated in patients with PD experiencing the wearing-off phenomenon with levodopa-containing therapies.
Clot evaluation and distal embolization risk during mechanical thrombectomy in anterior circulation stroke
FabioPilatoaIacopoValentebRosalindaCalandrellibAndreaAlexandrebVincenzoArenacMarcoDell'AquilacAldobrandoBroccolinidGiacomoDella MarcadRobertaMorosettidGiovanniFrisullodValerioBrunettidMarisaDistefanodAlessandroPedicellibCesareColosimobeVincenzoDi Lazzaroa
doi : 10.1016/j.jns.2021.120087
Volume 432, 15 January 2022, 120087
Clot features along with patients' clinical features may influence thrombus compactness predisposing at distal embolization during thrombectomy. The aim of this study was to evaluate thrombus features assessed by radiological and histopathological analysis along with patient-related features to predict distal embolization during thrombectomy.
Looking beyond the opioid receptor: A desperate need for new treatments for opioid use disorder
Yu KyungLeeaMark S.GoldbBrian S.Fuehrleinc
doi : 10.1016/j.jns.2021.120094
Volume 432, 15 January 2022, 120094
The mainstay of treatment for opioid use disorder (OUD) is opioid agonist therapy (OAT), which modulates opioid receptors to reduce substance craving and use. OAT maintains dependence on opioids but helps reduce overdose and negative sequelae of substance abuse. Despite increasing availability of OAT, its effectiveness is limited by difficulty in initiating and maintaining patients on treatment. With the worsening opioid epidemic in the United States and rising overdose deaths, a more durable and effective treatment for OUD is necessary. This paper reviews novel treatments being investigated for OUD, including neuromodulatory interventions, psychedelic drugs, and other novel approaches. Neuromodulatory interventions can stimulate the addiction neural circuitry involving the dorsolateral prefrontal cortex and deeper mesolimbic structures to curb craving and reduce use, and multiple clinical trials for interventional treatment for OUD are currently conducted. Similarly, psychedelic agents are being investigated for efficacy in OUD specifically. There is a resurgence of interest in psychedelic agents' therapeutic potential, with evidence of improving mood symptoms and decreased substance use even after just one dose. Exact mechanism of their anti-addictive effect is not fully elucidated, but psychedelic agents do not maintain opioid dependence and some may even be helpful in abating symptoms of withdrawal. Other potential approaches for OUD include targeting different parts of the dopamine-dependent addiction pathway, identifying susceptible genes and modulating gene products, as well as utilizing vaccines as immunotherapy to blunt the addictive effects of substances. Much more clinical data are needed to support efficacy and safety of these therapies in OUD, but these proposed novel treatments look beyond the opioid receptor to offer hope for a more durably effective OUD treatment.
Comparison of fixed cell-based assay to radioimmunoprecipitation assay for acetylcholine receptor antibody detection in myasthenia gravis
ArioMirianaMichael W.NicolleaPamelaEdmondbAdrianBudhramab
doi : 10.1016/j.jns.2021.120084
Volume 432, 15 January 2022, 120084
To compare specificity and sensitivity of a commercially available fixed cell-based assay (F-CBA) to radioimmunoprecipitation assay (RIPA) for acetylcholine receptor antibody (anti-AChR) detection in myasthenia gravis (MG).
Integrating neurology and pharmacy through telemedicine: A novel care model
HanlinLiaImama A.NaqvibSarah E.TomcBarbaraAlmeidabYuliyaBarattaChristina M.Ulaneb
doi : 10.1016/j.jns.2021.120085
Volume 432, 15 January 2022, 120085
Teleneurology had been best studied in acute stroke care, but the Coronavirus (COVID)-19 pandemic has highlighted applicability in outpatient practice. Telepharmacy is a convenient method for pharmacists to provide medication management to enhance care. Studies in the outpatient space suggest non-inferiority of teleneurology to increase access to specialized care for patients in rural locations. The role of telemedicine based interdisciplinary collaborations in a metropolitan and under-resourced setting has not been explored. We describe our approach to a teleneurology-telepharmacy collaboration at an urban academic medical center. Since its implementation pre-COVID, the program has expanded and transformed to serve the community further.
Reassessing accuracy of blood cell correction factor for traumatic lumbar puncture
George T.KannarkatJacquelineDarrowAbhayMoghekar
doi : 10.1016/j.jns.2021.120097
Volume 432, 15 January 2022, 120097
This study assessed the utility of correction formulas for procedural peripheral blood admixture with cerebrospinal fluid (CSF) in adult patients using in vitro addition of peripheral blood.
Out with the old, in with the new: Could plasma exchange be used to fill a therapeutic gap in neurology?
Jonathan I.Spencerab1MaximillianCraneb1MarcoPisabAlex D.WaldmanbGabriele C.DeLucab
doi : 10.1016/j.jns.2021.120056
Volume 432, 15 January 2022, 120056
The global tally of neurological disorders is exponentially rising and yet effective therapies for most remain evasive. There is a great deal of research into novel small molecules, immunotherapies and gene therapies to fill this therapeutic gap. We believe greater focus on plasma exchange as a research and clinical tool may provide useful insight into pathological mechanisms and effective treatment strategies. Plasma exchange has been traditionally used to treat antibody-mediated neurological diseases, such as myasthenia gravis and neuromyelitis optica, but there could be much wider future potential uses in neurology. Plasma exchange is not antibody specific, as it also removes a variety of other plasma-soluble factors, including age-related and disease-associated neurotoxic proteins, such as fibrinogen and amyloid. As research develops into the role of blood-brain barrier and immunological alterations in diseases not typically regarded as immune-driven, interest in neurotoxic plasma proteins grows. Here, we highlight that plasma exchange may have uses outside of antibody-mediated neurological diseases, by removing neurotoxic proteins from the systemic circulation.
Paraneoplastic myelopathy with amphiphysin autoantibodies and lobular breast carcinoma in situ
Christina A.AlexandresaMatthew A.TremblaybChristine L.KlassencAmyDegnimdEoin P.Flanaganef
doi : 10.1016/j.jns.2021.120086
Volume 432, 15 January 2022, 120086
A one-year longitudinal evaluation of cerebrospinal fluid and blood neurochemical markers in a patient with cryptococcal meningitis complicated by ischemic stroke.
SamirAbu-Rumeilehab1TamaraGaribashvilia1Jürgen BenjaminHagemanncVeronikaStillaFranziskaBachhuberaMarkusOttoabHayrettinTumaniadMakbuleSenela
doi : 10.1016/j.jns.2021.120090
Volume 432, 15 January 2022, 120090
Pain insensitivity in frontally-predominant dementia
Mario F.Mendezab
doi : 10.1016/j.jns.2021.120027
Volume 432, 15 January 2022, 120027
Statin treatment and cerebral microbleeds: A risk assessment
TomoyukiKawada
doi : 10.1016/j.jns.2021.120038
Volume 432, 15 January 2022, 120038
Authors' reply to “Statin treatment and cerebral microbleeds: A risk assessment”
panelAristeidis H.KatsanosaMagdy H.SelimbAshkanShoamanesha
doi : 10.1016/j.jns.2021.120037
Volume 432, 15 January 2022, 120037
Exposure to TNF inhibitors is rare at MOGAD presentation
VyankaRedenbaughaEoin P.FlanaganabValentinaFlorisefPietroZaraefM. TariqBhattiacFranciscoSanchezacMatthewKosterdSaraMariottohSean J.PittockabJohn J.ChenacAlbertoCauligPaoloSollaeEliaSechie
doi : 10.1016/j.jns.2021.120044
Volume 432, 15 January 2022, 120044
The peripheral view: Neuromuscular diagnosis, care and surveillance in global health
MichelleKvalsundabGretchen L.BirbeckacDavid N.Herrmanna
doi : 10.1016/j.jns.2021.120071
Volume 432, 15 January 2022, 120071
Neurochondrin immunoglobulin G – Associated myelopathy – Ataxia syndrome
LisetteDominguezaIris Vanessa MarinCollazoaEliaSechibParkerBohmaSebastian LopezChiribogaa
doi : 10.1016/j.jns.2021.120058
Volume 432, 15 January 2022, 120058
Low-dose statin therapy for intracranial artery stenosis: A single center preliminary study
HitoshiAizawaTakeshiTaguchiNobuhiroIdoHaruhisaKatoHirooTerashi
doi : 10.1016/j.jns.2021.120075
Volume 432, 15 January 2022, 120075
