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Visual fields and optical coherence tomography (OCT) in neuro-ophthalmology: Structure-function correlation
LauraDonaldsonaEdwardMargolinb
doi : 10.1016/j.jns.2021.118064
Volume 429, 15 October 2021, 118064
Visual field (VF) testing is an essential component of the neurological examination. The differential diagnosis of VF defects depends on relating this measure of afferent visual function to the structure of the visual pathway and optical coherence tomography (OCT) is an invaluable tool for detailed structural evaluation of the optic nerve and retina. This review describes the ways in which interpretation of VF and OCT can be used together to increase the accuracy of the localization of lesions along the visual pathway. Lesions of the anterior visual pathway (originating in ganglion cells or nerve fibre layer of the retina or optic nerve) will typically produce defects that respect the horizontal midline, reflecting the arcuate path of the ganglion cell axons as they travel to the optic nerve. OCT of peripapillary retinal nerve fibre layer and ganglion cell complex (GCC) will typically demonstrate irreversible thinning in compressive and demyelinating lesions affecting anterior visual pathway. Chiasmal lesions produce highly localizable VF defects (junctional scotoma and bitemporal hemianopia) which correspond to the thinning of nasal portion of GCC. Lesions of the optic tract result in incongruous homonymous hemianopia on VF with corresponding hemianopic thinning on GCC developing within months. Lesions affecting optic radiations usually produce more congruous homonymous VF defects and can also produce homonymous thinning on GCC, however, this takes much longer to develop as trans-synaptic degeneration at the lateral geniculate body must occur.
History and current progress of chronic subdural hematoma
RyotaTamuraab1MizutoSatoa1KazunariYoshidaaMasahiroTodaa
doi : 10.1016/j.jns.2021.118066
Volume 429, 15 October 2021, 118066
Chronic subdural hematoma (CSDH) is characterized by an encapsulated collection of old blood. Although CSDH has become the most frequent pathologic entity in daily neurosurgical practice, there are some unresolved research questions. In particular, the causes and recurrent risk factors of CSDH remain as an object of debate. The split of the dural border layer forms a few tiers of dural border cells over the arachnoid layer. Tissue plasminogen activator plays an important role as a key factor of defective coagulation. Historically, CSDH has often been treated via burr hole craniostomy using a closed drainage system. Several different operative strategies and peri-operative strategies such as the addition of burr holes, addition of cavity irrigation, position of drain, or postural position, have been described previously. Although the direction of the drainage tube, residual air, low intensity of T1-weighted images on MRI, and niveau formation have been reported as risk factors for recurrence, antiplatelet or anticoagulant drug use has not yet been verified as a risk factor. Recently, pharmaceutical strategies, including atorvastatin, significantly improved the neurological function in CSDH patients. Many case series, without randomization, have been reported; and given its promising result, several randomized clinical trials using pharmaceutical as well as operative and perioperative strategies were initiated to obtain sufficient data. In contrast, relatively fewer basic studies have achieved clinical applications in CSDH, although it is one of the most common clinical entities. Further scientific basic research may be essential for achieving a novel treatment strategy for CSDH.
The universal brain code a genetic mechanism for memory
Roger N.Rosenberg
doi : 10.1016/j.jns.2021.118073
Volume 429, 15 October 2021, 118073
We do not have an understanding of the fundamental mechanism of how information is stored and retrieved by the brain. A Universal Brain Code utilized for these functions is proposed here. The basic tenent of the Code is that a memory engram is propagated and guided through the connectome by specific proteins/peptides embedded within the pre-synaptic neuronal membrane corresponding to information provided by afferent electrical currents to the pre-synaptic neuron. It is intended to provide a working approach to this central brain activity and begin the process of investigation based on these ideas which are new and unexplored.
Hyperdense middle cerebral artery sign and response to combination of mechanical Thrombectomy plus intravenous thrombolysis in acute stroke patients
AshkanMowlaa1Seyed-MostafaRazaviab1Navdeep S.LailcPegahMohammadidPeymanShiranieKatelyn S.KavakcRobert N.SawyercHarisKamalf
doi : 10.1016/j.jns.2021.117618
Volume 429, 15 October 2021, 117618
Combining intra-arterial mechanical thrombectomy (IAMT) and intravenous thrombolysis (IVT) has shown to have an excellent recanalization rate and better clinical outcome in acute ischemic stroke (AIS) patients. Hyperdense middle cerebral artery sign (HMCAS) on pretreatment non-contrast head CT scan of AIS patients is one of the early ischemic radiological findings in middle cerebral artery territory AIS. We aimed to evaluate whether the presence of HMCAS predicts the outcome of AIS patients receiving combination therapy with IAMT and IVT.
Prestroke cognitive decline in africans: Prevalence, predictors and association with poststroke dementia
AkinOjagbemiacToyinBelloaMayowaOwolabibOlusegunBaiyewuc
doi : 10.1016/j.jns.2021.117619
Volume 429, 15 October 2021, 117619
Pre-existing cognitive decline is a risk factor for stroke onset and poststroke dementia. There is a knowledge gap on prestroke cognitive decline in indigenous Africans. We estimated prevalence and predictors of prestroke cognitive decline, as well as its association with poststroke dementia at one year in Nigerian survivors of a first ever stroke.
Efficacy and safety of modified reduced-dose rituximab in Chinese patients with neuromyelitis optica spectrum disorder: A retrospective cohort study
ShugangCaoad1HaiYue1JingluanTianaYuanyuanLiaYuepingShenfXiaopeiJiaXiaoyuanWangaXiaolingZhouaYanzhengGubcFengZhuaXiaoyuDuanaXinyiXiaoaQiFangabXiangjunCheneQunXueabc
doi : 10.1016/j.jns.2021.117616
Volume 429, 15 October 2021, 117616
To evaluate the long-term efficacy and safety of a modified reduced-dose rituximab (mRTX) regimen compared with azathioprine (AZA) and mycophenolate mofetil (MMF) in Chinese patients with neuromyelitis optica spectrum disorder (NMOSD).
Vocal cord paresis on CTA - A novel tool for the diagnosis of lateral medullary syndrome
ShlomiPeretzaeShiraRosenblatbeMichalZuckermanaEdnaInbarbeHagitShoffel-HavakukceRaniBarneaaeIsraelSteineraeTzippyShochatdInbarZuckerefEitanAurielaeDrorSuhamibe
doi : 10.1016/j.jns.2021.117576
Volume 429, 15 October 2021, 117576
Diagnosis of lateral medullary syndrome (LMS) is often delayed due to elusive clinical presentations and frequently non-revealing neuroimaging tests. We aimed to investigate the use of ipsilateral vocal cord paresis (VCP) identified on neck computed tomography angiography (CTA) as an early diagnostic sign for LMS.
COQ2 V393A confers high risk susceptibility for multiple system atrophy in East Asian population
Kristine JoycePortoaMakitoHiranobJunMitsuiacAyakaChikadaaTakashiMatsukawaacHiroyukiIshiuracThe Japan Multiple System Atrophy Registry ConsortiumTatsushiTodacSusumuKusunokibShojiTsujiad
doi : 10.1016/j.jns.2021.117623
Volume 429, 15 October 2021, 117623
Multiple system atrophy (MSA) is a rare, late-onset, and devastating neurodegenerative disease characterized by autonomic failure, alongside with various combination of parkinsonism, cerebellar ataxia, and pyramidal dysfunction. Since we first identified biallelic mutations in the COQ2 gene in two multiplex MSA families and further reported that heterozygous COQ2 V393A variant confers a susceptibility to sporadic MSA, the results of nearly a decade of investigating this association globally were quite remarkable. COQ2 V393A was virtually absent in the American and European populations but was shown to have varying associations with sporadic MSA in the East Asian populations. In our attempt to clarify the latter and provide a coherent regional conclusion, we conducted two independent case-control series which showed clear association of the V393A variant with sporadic MSA in the Japanese population. We then pooled the results with other studies from the East Asian population and conducted a meta-analysis which broadened and established the association regionally (pooled OR 2.12, 95% CI: 1.35–3.31, PI: 0.63–7.15, p = 0.0047). The subgroup analysis identified a strong association of V393A with MSA-C (pooled OR 2.57, 95% CI: 1.98–3.35; p = 2.56 × 10?12) but not with MSA-P (pooled OR 1.41, 95% CI: 0.88–2.26; p = 0.16). Our results highlighted the importance of investigating region-specific and pan-regional genetic variants that may potentially underlie the pathomechanisms of neurodegenerative diseases. COQ2 V393A variant remains a susceptibility variant rather than causative for MSA particularly, MSA-C subtype, in the East Asian population.
CIDP and hemopathies, an underestimated association
NathalieDeschampsaStéphaneMathisbMathildeDuchesnecKarimaGhorabaGaëlGallouedecaLaurenceRichardaJean-MarcBoulesteixdPhilippeCorciaeLaurentMagyaJean-MichelVallata
doi : 10.1016/j.jns.2021.118055
Volume 429, 15 October 2021, 118055
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated and treatable disease that may be associated with various systemic conditions. Our objective is to describe the clinical, electrophysiological and pathological data of a series of patients with both CIDP and hemopathy. In this retrospective study, we analyzed 21 patients with CIDP and various hemopathies (malignant or not), consecutively observed for almost five years. In this particular context (with a risk of neurological complications of the hemopathy), a nerve biopsy was taken from each patient (after written consent). All the patients fulfilled the EAN/PNS electrodiagnostic criteria (2021) of CIDP: 16 with ‘CIDP’ and 2 with ‘possible CIDP’ (no data for 3 patients). For each patient, pathological analysis of nerve biopsy was compatible with the diagnosis of CIDP, and there was no evidence for hematological complication of the peripheral nervous system. In cases of peripheral neuropathy and malignant hemopathy, the possibility that the peripheral neuropathy is CIDP should not be overlooked because CIDP is clearly accessible to appropriate therapies, with high potential for a positive clinical response. If the diagnosis of CIDP is usually suspected clinically and electrophysiologically, it should be confirmed by pathological study (nerve biopsy) in certain cases. The management of such patients benefits from the collaboration of neurologists, hematologists and oncologists.
Laboratory based assessment of gait and balance impairment in patients with progressive supranuclear palsy
FarwaAliaStacy R.LoushinbHugoBothaaKeith A.JosephsaJennifer L.WhitwellcKentonKaufmanb
doi : 10.1016/j.jns.2021.118054
Volume 429, 15 October 2021, 118054
Gait and balance abnormalities are a significant source of morbidity and mortality in progressive supranuclear palsy (PSP). Gait impairment in PSP is primarily assessed clinically on exam or with the use of rating scales. Three dimensional video based gait and balance analysis performed in a laboratory setting is a highly accurate method of motion analysis (Wren et al., 2020), however limited data is available in patients with PSP.
The Pre-Lumbar puncture Intracranial Hypertension Scale (PLIHS): A practical scale to identify subjects with normal cerebrospinal fluid pressure in the management of idiopathic intracranial hypertension
AlbertoRaggiaStefaniaBianchi MarzolibPaolaCiascabGabriellaCammaratabLisaMelzibLuisaChiapparinicAlessandraErbettacElisaCiceridGiuseppeFaragòeAndreaGioppofSusannaUsaigDomenicoD'Amicog
doi : 10.1016/j.jns.2021.118058
Volume 429, 15 October 2021, 118058
Idiopathic Intracranial Hypertension (IIH) diagnosis requires lumbar puncture to measure cerebrospinal fluid (CSF) pressure. The Pre-Lumbar puncture Intracranial Hypertension Scale (PLIHS) is aimed to detect cases that will show raised or normal CSF opening pressure.
Fist-Edge-Palm (FEP) test has a high sensitivity in differentiating dementia from normal cognition in Parkinson's disease
YeLiuaMeng-YaoQiuaYu-LeiZhangaXiao-JinZhangaDanielTruongbcEng-KingTandYun-ChengWua
doi : 10.1016/j.jns.2021.118060
Volume 429, 15 October 2021, 118060
The Fist-Edge-Palm (FEP) test takes 0.5–3 min to complete and is highly sensitive in differentiating Alzheimer's disease and frontotemporal dementia from normal cognition, but it has not yet been studied in Parkinson's disease (PD).
Phenytoin prophylaxis and functional outcomes following spontaneous intracerebral hemorrhage: A systematic review and meta-analysis
Quincy K.TranadeVeraBzhilyanskayaaTuckerLurieabMatthewFairchildaMehboob A.RehangAsmaRashidcElizabethPowellabAliPourmandf
doi : 10.1016/j.jns.2021.117624
Volume 429, 15 October 2021, 117624
Seizure activity following spontaneous intracerebral hemorrhage (sICH) can worsen patients' comorbidity. However, data regarding whether seizure prophylaxis for sICH is associated with patients' poor functional outcome is inconclusive. We performed a systematic review and meta-analysis to assess the relationship between phenytoin prophylaxis and poor functional outcome after sICH.
Longitudinal risk factors for developing depressive symptoms in Parkinson's disease
TarekAntaraHuw R.MorriscFarazFaghriaHampton L.LeonardabMike A.NallsabAndrew B.SingletonaHirotakaIwakiab
doi : 10.1016/j.jns.2021.117615
Volume 429, 15 October 2021, 117615
Despite the established importance of identifying depression in Parkinson's disease, our understanding of the factors which place the Parkinson's disease patient at future risk of depression is limited.
New-onset seizures in older people: Clinical features, course and outcomes
Sebastian F.GreenabNadineLoeffladcDominic C.HeaneyaSanjeevRajakulendranac
doi : 10.1016/j.jns.2021.118065
Volume 429, 15 October 2021, 118065
The incidence of epilepsy increases with age. With current demographic trends, this presents a healthcare challenge. We investigated the clinical spectrum of first seizures, evaluated neuroimaging and EEG findings, and determined clinical outcomes, including anti-seizure medication (ASM) response in older people. In addition, we sought to understand the relative effects of age and frailty on ASM response.
Clinical and genetic characteristics of 21 Spanish patients with biallelic pathogenic SPG7 mutations
RaquelBaviera-MuñozabcMarinaCampins-RomeuacLidónCarretero-VilarroigbcdIsabelSastre-BatalleracIreneMartínez-TorresacJuan F.Vázquez-CostaabceNuriaMuelasabcTeresaSevillaabcefJuan J.VílchezbcfElenaAllercfgTeresaJaijocfgLuisBatallerabcefCarmenEspinóscfh
doi : 10.1016/j.jns.2021.118062
Volume 429, 15 October 2021, 118062
Spastic paraplegia type 7 (SPG7) is one of the most common hereditary spastic paraplegias. SPG7 mutations most often lead to spastic paraparesis (HSP) and/or hereditary cerebellar ataxia (HCA), frequently with mixed phenotypes. We sought to clinically and genetically characterize a Spanish cohort of SPG7 patients. Patients were recruited from our HCA and HSP cohorts. We identified twenty-one patients with biallelic pathogenic SPG7 mutations. Mean age at onset was 37.4 years (SD ± 14.3). The most frequent phenotype was spastic ataxia (57%), followed by pure spastic paraplegia (19%) and complex phenotypes (19%). Isolated patients presented with focal or multifocal dystonia, subclinical myopathy or ophthalmoplegia. p.Ala510Val was the most frequent pathogenic variant encountered. Compound heterozygous for p.Ala510Val displayed younger onset (p < 0.05) and more complex phenotypes (p < 0.05) than p.Ala510Val homozygotes. Two novel variants were found: p.Lys559Argfs*33 and p.Ala312Glu. In conclusion, spastic ataxia is the most common phenotype found in Spanish patients. Nonetheless, SPG7 analysis should also be considered in patients with less frequent clinical findings such as dystonia or ophthalmoplegia especially when these symptoms are associated with mild spastic ataxia.
Effects of safinamide adjunct therapy on pain in patients with Parkinson's disease: Post hoc analysis of a Japanese phase 2/3 study
YoshioTsuboiaMichinoriKoebisbYukiKogobTakayukiIshidabIppeiSuzukicMasahiroNomotodNobutakaHattorie
doi : 10.1016/j.jns.2021.118070
Volume 429, 15 October 2021, 118070
The non-dopaminergic and dopaminergic actions of safinamide may alleviate pain in patients with Parkinson's disease (PD). We investigated the efficacy of safinamide for pain when administered as an adjunct to levodopa in Japanese patients with PD.
Multiple sclerosis care units in Latin America: Consensus recommendations about its objectives and functioning implementation
EdgardoCristianoaPatricioAbadbJeffersonBeckercAdrianaCarrádeJorgeCorrealefJoséFloresghManuelFrunsiOrlandoGarceajJuanGarcia BónittokFernandoGracialFernandoHamuymnCárlosNavasoLilianaPatruccopVíctorRiveraqMercedVelazquezrJuan IgnacioRojasas
doi : 10.1016/j.jns.2021.118072
Volume 429, 15 October 2021, 118072
Currently, there are several reasons to promote worldwide the concept of multiple sclerosis care units (MSCU) for a better management of affected patients. Ideally, the MSCU should have some human and technical resources that distinguish and improve the care of affected patients; however, local, and regional aspects should be considered when recommending how these units should operate. The objective of these consensus recommendations was to review how MSCU should work in Latin America to improve long-term outcomes in MS patients.
Neurosarcoidosis mimicry of MS: Clues from cases with CNS tissue diagnosis
Thomas F.Scott
doi : 10.1016/j.jns.2021.117621
Volume 429, 15 October 2021, 117621
The clinical picture of neurosarcoidosis (NS) shares many aspects with multiple sclerosis (MS). I examine whether or not clinical measures can reliably distinguish NS mimicking MS from NS coexisting with MS, and the informative role biopsy and autopsy evidence may play in understanding these two disorders. Uniquely challenging, I explore the rare patients presenting with the differential of MS or acute disseminated encephalomyelitis (ADEM) versus NS, including MS or ADEM as an associated illness in patients with systemic sarcoidosis. In most but not all NS patients, red flags against a diagnosis of MS are strong enough to rule out this more common disorder. Biopsy and autopsy findings indicate a tendency of NS granulomatous changes in the CNS to involve the same deep white matter perivascular spaces as expected to occur in MS, and hence correlate with a tendency of NS involving white matter to produce classic MRI findings of MS. The spectrum of NS includes some cases limited to a single anatomical site, including sites classically involved in demyelinating CIS (optic nerve, brainstem, and transverse myelitis). Asymptomatic “non-specific” periventricular MRI changes are described in many studies as “MS-like”. No biopsied or autopsied cases have yet proven associated classic pathological changes of MS in patients with NS.
Wearing-off symptoms during standard and extended natalizumab dosing intervals: Experiences from the COVID-19 pandemic
Gerd HagaBringelandabNelloBlasercdKjell-MortenMyhrabChristian AlexanderVedelerabSoniaGavassoab
doi : 10.1016/j.jns.2021.117622
Volume 429, 15 October 2021, 117622
Natalizumab effectively prevents disease activity in relapsing-remitting multiple sclerosis, but many treated patients report subjective wearing-off symptoms at the end of the 4-week interval between infusions. Extended interval dosing (EID) is a promising strategy to mitigate the risk of natalizumab-associated progressive multifocal leukoencephalopathy, but it is unknown whether EID affects wearing-off symptoms. In this observational study, we evaluated if prevalence or intensity of wearing-off symptoms changed when natalizumab dosing intervals were extended from 4 to 6 weeks in 30 treated patients during the outbreak of COVID-19 in Norway. New or increased wearing-off symptoms during EID were reported by 50%. Symptom increase was more frequent among patients with pre-existing wearing-off symptoms during standard dosing compared to patients without such pre-existing symptoms [p = 0.0005]. Our observations support the need to study the effect of EID on wearing-off symptoms in randomized controlled trials.
Serum neurofilament light chain levels are associated with stroke severity and functional outcome in patients undergoing endovascular therapy for large vessel occlusion
JanRahmiga1KatjaAkgünab1ErikSimonaMatthiasGawlitzacChristianHartmannaTimoSiepmannaLars-PederPallesenaJessicaBarlinnaVolkerPuetzaTjalfZiemssenab1KristianBarlinna1
doi : 10.1016/j.jns.2021.118063
Volume 429, 15 October 2021, 118063
We aimed to analyze serum neurofilament light chain (sNfL) levels in patients undergoing endovascular therapy (EVT) for anterior circulation large vessel occlusion (acLVO).
Transthyretin amyloid-related transitory events (TARTEs): Descriptive analysis of clinical, imagiological, and neurophysiological features
VanessaCarvalhoa1CarlotaCunhabRaquelRochaaJoãoMassanocdFilipeCorreiaaPauloCoelhoaSaraFrançaaVitor TedimCruzaeCatarinaCrutoa
doi : 10.1016/j.jns.2021.118068
Volume 429, 15 October 2021, 118068
Transthyretin-related familial amyloid polyneuropathy (ATTR-FAP) is a multisystemic disorder inherited as an autosomal dominant trait. Transitory events in ATTR-FAP patients are a feature of this disorder and remain poorly depicted in the literature. We aimed to describe a case series of ATTR-FAP patients who presented to our department with transitory events and document the clinical, neuroimaging and neurophysiological characteristics of the events.
Re: Semerano A, Mamadou Z, Desilles JP, Sabben C, Bacigaluppi M, Piotin M, et al. Carotid webs in large vessel occlusion stroke: Clinical, radiological and thrombus histopathological findings. Journal of the neurological sciences. 2021;427:117550
Diogo C.HaussenAlhamza R.Al-BayatiNiravBhattRaul G.Nogueira
doi : 10.1016/j.jns.2021.117620
Volume 429, 15 October 2021, 117620
Histopathologic analysis of retrieved cerebral thrombi in acute ischemic stroke patients with proximal anterior circulation occlusions amenable to endovascular thrombectomy
GloriaMakaJian-QiangLubKanjanaPererac
doi : 10.1016/j.jns.2021.117617
Volume 429, 15 October 2021, 117617
Corrigendum to “Medically unexplained visual loss in a specialist clinic: A retrospective case–control comparison”, [Journal of the Neurological Sciences 361 (2016) 272–276]
Éanna D.O'LearyaBenjaminMcNeillisbSelmaAybekcPaulRiordan-EvadAnthony S.Davida
doi : 10.1016/j.jns.2021.117609
Volume 429, 15 October 2021, 117609
