Patterns of cortical activity differ in ALS patients with limb and/or bulbar involvement depending on motor tasks.

abstract：:Frailty is known to predict dementia. However, its link with neurodegenerative alterations of the central nervous system (CNS) is not well understood at present. We investigated the association between the biomechanical response of the CNS and frailty in older adults suspected of normal pressure hydrocephalus (NPH) pr...

journal_title：Journal of neurology

authors： Vallet A,Del Campo N,Hoogendijk EO,Lokossou A,Balédent O,Czosnyka Z,Balardy L,Payoux P,Swider P,Lorthois S,Schmidt E

更新日期：2020-05-01 00:00:00

abstract：:The short-term benefits of levodopa/carbidopa intestinal gel (LCIG) in patients with advanced Parkinson's disease (PD) are well documented, but the long-term benefits are still uncertain. The aim of this study was to investigate the motor and cognitive outcome of LCIG treatment in advanced PD after a follow-up period ...

journal_title：Journal of neurology

authors： Cáceres-Redondo MT,Carrillo F,Lama MJ,Huertas-Fernández I,Vargas-González L,Carballo M,Mir P

更新日期：2014-03-01 00:00:00

abstract：:We hypothesize that cerebral microbleeds (CMB) in patients with different neuropsychological profiles (amnestic or non-amnestic) and MRI features of vascular damage could provide important information on the underlying pathological process in early Alzheimer's disease. The study was performed at two trial sites. We st...

journal_title：Journal of neurology

authors： Poliakova T,Levin O,Arablinskiy A,Vasenina E,Zerr I

更新日期：2016-10-01 00:00:00

abstract：:We investigated the levels of transforming growth factor beta 1 (TGF-beta 1) in cerebrospinal fluid (CSF) in children with meningitis, with a view to prognostic relevance. CSF TGF-beta 1 levels on admission were measured by a sandwich enzyme immunoassay in children with bacterial meningitis (n = 16), aseptic meningiti...

journal_title：Journal of neurology

authors： Huang CC,Chang YC,Chow NH,Wang ST

更新日期：1997-10-01 00:00:00

abstract：BACKGROUND:Rapid eye movement sleep behavior disorder (RBD) is highly comorbid with Parkinson's disease (PD). Emerging evidence suggests that dopamine-replacement therapies (DRTs) for PD may modify the course of RBD, yet the nature of the association between DRTs and RBD remains unclear. To begin addressing this issue,...

journal_title：Journal of neurology

authors： Meloni M,Bortolato M,Cannas A,Laccu I,Figorilli M,Congiu P,Defazio G,Meloni F,Puligheddu M

更新日期：2020-10-01 00:00:00

abstract：:We assessed the prevalence of vertebral artery (VA) stenosis or occlusion and its influence on outcome in patients with acute basilar artery occlusion (BAO). We studied 141 patients with acute BAO enrolled in the Basilar Artery International Cooperation Study (BASICS) registry of whom baseline CT angiography (CTA) of ...

journal_title：Journal of neurology

authors： Compter A,van der Hoeven EJ,van der Worp HB,Vos JA,Weimar C,Rueckert CM,Kappelle LJ,Algra A,Schonewille WJ,BASICS Study Group.

更新日期：2015-02-01 00:00:00

abstract：:After head injuries, particularly mild or moderate ones, a range of post-concussion symptoms (PCS) are often reported by patients. Such symptoms may significantly affect patients' psychosocial functioning. To date, no measure of the severity of PCS has been developed. This study presents the Rivermead Post Concussion ...

journal_title：Journal of neurology

authors： King NS,Crawford S,Wenden FJ,Moss NE,Wade DT

更新日期：1995-09-01 00:00:00

abstract：:The International Cooperative Ataxia Rating Scale (ICARS) is a 100-point semiquantitative scale designed primarily to assess cerebellar dysfunction. However, little is known of the metric properties of this scale. We assessed the ICARS by rating the severity of cerebellar dysfunction in 27 patients with spinocerebella...

journal_title：Journal of neurology

authors： Wang PS,Liu RS,Yang BH,Soong BW

更新日期：2007-06-01 00:00:00

abstract：:The experience of music is difficult to study objectively. Here we describe a detailed analysis of musical hallucinations developing after a probable brainstem stroke in an 83 year old musician who was able to describe and notate the hallucinations. The hallucinations comprised simple, repetitive melodic and rhythmic ...

journal_title：Journal of neurology

authors： Warren JD,Schott GD

更新日期：2006-08-01 00:00:00

abstract：:Neurological manifestations in pandemics frequently cause short and long-term consequences which are frequently overlooked. Despite advances in the treatment of infectious diseases, nervous system involvement remains a challenge, with limited treatments often available. The under-recognition of neurological manifestat...

journal_title：Journal of neurology

authors： Valerio F,Whitehouse DP,Menon DK,Newcombe VFJ

更新日期：2020-10-26 00:00:00

abstract：:Recent reports suggest the possible beneficial effects of haemopoietic stem cell transplantation (HSCT) in autoimmune diseases such as multiple sclerosis (MS). The definition of the risk/benefit ratio for such a treatment is perceived as a major issue for the neurological community worldwide. The First Consensus Confe...

journal_title：Journal of neurology

authors： Comi G,Kappos L,Clanet M,Ebers G,Fassas A,Fazekas F,Filippi M,Hartung HP,Hertenstein B,Karussis D,Martino G,Tyndall A,van der Meché FG

更新日期：2000-05-01 00:00:00

abstract：:Risk of relapse after natalizumab (NAT) cessation and switch to dimethyl fumarate (DMF) is unknown. The objective of this paper is to identify the risk and associated risk factors for relapse after switching from NAT to DMF in relapsing-remitting multiple sclerosis. Patients (n = 30) were treated with NAT for ≥12 mont...

journal_title：Journal of neurology

authors： Zurawski J,Flinn A,Sklover L,Sloane JA

更新日期：2016-08-01 00:00:00

abstract：:Vitamin B(12) (VitB(12), cobalamin) deficiency has been associated with various neuropsychiatric conditions, such as peripheral neuropathy, subacute combined degeneration, affective disorders, and cognitive impairment. Current assays analyze vitamin B(12), of which only a small percentage is metabolically active. Meas...

journal_title：Journal of neurology

authors： Schrempf W,Eulitz M,Neumeister V,Siegert G,Koch R,Reichmann H,Storch A

更新日期：2011-03-01 00:00:00

abstract：OBJECTIVE:The variability of the severity and regional distribution of pathological process in basal ganglia (BG) and brainstem-cerebellar systems results in clinical heterogeneity and represents the motor subtype of multiple system atrophy (MSA). This study aimed to quantify spatial patterns of multimodal MRI abnormal...

journal_title：Journal of neurology

authors： Lee MJ,Kim TH,Mun CW,Shin HK,Son J,Lee JH

更新日期：2018-07-01 00:00:00

abstract：:Adolescent-onset spastic ataxia is a proposed novel phenotype in compound heterozygous carriers of an intronic mutation (c.1909 + 22G > A) in the POLR3A gene. Here, we present ten new cases of POLR3A-related spastic ataxia and discuss the genetic, clinical and imaging findings. Patients belonged to six pedigrees with ...

journal_title：Journal of neurology

authors： Infante J,Serrano-Cárdenas KM,Corral-Juan M,Farré X,Sánchez I,de Lucas EM,García A,Martín-Gurpegui JL,Berciano J,Matilla-Dueñas A

更新日期：2020-02-01 00:00:00

abstract：:We evaluated cyclosporine A (CsA) treatment in 9 patients (6 female and 3 male), 16-63 years old, with severe myasthenia gravis (MG) for a mean period of 2 years (range 16-36 months). All of the patients had been previously treated either with corticosteroids or by combined immunotherapy, and 5 needed periodic plasma ...

journal_title：Journal of neurology

authors： Bonifati DM,Angelini C

更新日期：1997-09-01 00:00:00

abstract：:The pharmacokinetics of free and total valproic acid (VPA) in plasma and whole blood after oral administration during steady state was investigated in seven infants (mean age 10.7 months) receiving monotherapy. The VPA concentrations in whole blood closely followed those in plasma but at a reduced level. A positive co...

journal_title：Journal of neurology

authors： Herngren L,Lundberg B,Nergårdh A

更新日期：1991-09-01 00:00:00

abstract：:This observational study examined the outcome of two different therapeutic strategies in the treatment of chronic neuropathic pain by including pregabalin (PGB) as mono- or add-on therapy in one of two treatment options. Patients with a pain score of > or =4, refractory to usual care for neuropathic pain for at least ...

journal_title：Journal of neurology

authors： Lampl C,Schweiger C,Haider B,Lechner A

更新日期：2010-08-01 00:00:00

abstract：BACKGROUND:Therapy of autoimmune diseases of the central and peripheral nervous system with intravenous IgG immunoglobulin (IVIg) is well established. Since IVIg is produced from pooled human plasma, autoantibodies can be found in IVIg products and, accordingly, in patient sera after transfusion. The de novo evidence o...

journal_title：Journal of neurology

authors： Grüter T,Ott A,Meyer W,Jarius S,Kinner M,Motte J,Pitarokoili K,Gold R,Komorowski L,Ayzenberg I

更新日期：2020-03-01 00:00:00

abstract：:The aim of this work was to determine whether survival changed during 2002-2009 at a French amyotrophic lateral sclerosis (ALS) center. We included all patients with ALS who were seen consecutively at the center from January 2002-May 2009. Participants were followed from date of first visit through death, date of cens...

journal_title：Journal of neurology

authors： Gordon PH,Salachas F,Bruneteau G,Pradat PF,Lacomblez L,Gonzalez-Bermejo J,Morelot-Panzini C,Similowski T,Elbaz A,Meininger V

更新日期：2012-09-01 00:00:00

abstract：:Evaluating freezing of gait (FOG) and quantifying its severity in patients with Parkinson's disease (PD) is challenging; objective assessment is not sufficiently established. We aimed to improve the ability to objectively evaluate FOG severity by investigating the value of measuring the duration of the test and its co...

journal_title：Journal of neurology

authors： Herman T,Dagan M,Shema-Shiratzky S,Reches T,Brozgol M,Giladi N,Manor B,Hausdorff JM

更新日期：2020-09-01 00:00:00

abstract：:Several viruses have the capacity to cause serious infections of the nervous system in patients who are immunosuppressed. Individuals may be immunosuppressed because of primary inherited immunodeficiency, secondary immunodeficiency due to particular diseases such as malignancy, administration of immunosuppressant drug...

journal_title：Journal of neurology

authors： Kennedy PGE

更新日期：2020-10-13 00:00:00

abstract：:Limb-girdle muscular dystrophies (LGMDs) are genetically heterogeneous and the diagnostic work-up including conventional genetic testing using Sanger sequencing remains complex and often unsatisfactory. We performed targeted sequencing of 23 LGMD-related genes and 15 genes in which alterations result in a similar phen...

journal_title：Journal of neurology

authors： Kuhn M,Gläser D,Joshi PR,Zierz S,Wenninger S,Schoser B,Deschauer M

更新日期：2016-04-01 00:00:00

abstract：:Alexander disease (AD) is a rare genetic disorder of the central nervous system due to a dysfunction of astrocytes. The most common infantile form presents as a progressive leukodystrophy with macrocephalus. Recently, heterozygous de novo mutations in the gene encoding glial fibrillary acidic protein (GFAP) have been ...

journal_title：Journal of neurology

authors： Brockmann K,Dechent P,Meins M,Haupt M,Sperner J,Stephani U,Frahm J,Hanefeld F

更新日期：2003-03-01 00:00:00

abstract：:This short review summarizes epilepsy papers published in the Journal of Neurology in 2009, covering pathophysiology (inflammation; propagation pathways), new treatments (oxcarbazepine in paediatrics; levetiracetam in status epilepticus), and non-seizure outcomes (violence; foetal/pregnancy). ...

journal_title：Journal of neurology

authors： Lozsadi DA,Von Oertzen J,Cock HR

更新日期：2010-11-01 00:00:00

abstract：:An 8-year-old boy with galactose-1-phosphate uridyl transferase (GALT) deficiency presented with hypotonia, muscle hypotrophy, hepatomegaly, bilateral cataract and mild mental retardation. Two brothers showed a GALT activity consistent with a homozygotic condition and both parents were found to be heterozygotes for th...

journal_title：Journal of neurology

authors： Bresolin N,Comi GP,Fortunato F,Meola G,Gallanti A,Tajana A,Velicogna M,Gonano EF,Ninfali P,Pifferi S

更新日期：1993-05-01 00:00:00

abstract：:The pathogenesis of myopathies occurring in critically ill patients (critical illness myopathy, CIM) is poorly understood. Both local and systemic responses to sepsis and other severe insults to the body are presumed to be involved but the precise mechanisms by which muscle function is impaired are far from clear. To ...

journal_title：Journal of neurology

authors： Friedrich O,Hund E,Weber C,Hacke W,Fink RH

更新日期：2004-01-01 00:00:00

abstract：BACKGROUND:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that still lacks reliable diagnostic biomarkers. This study aims to evaluate the diagnostic and prognostic potential of CSF total Tau (t-Tau), phospho-Tau (p-Tau) and p-Tau/t-Tau ratio in ALS patients using CSF neurofilament light (NFL)...

journal_title：Journal of neurology

authors： Scarafino A,D'Errico E,Introna A,Fraddosio A,Distaso E,Tempesta I,Morea A,Mastronardi A,Leante R,Ruggieri M,Mastrapasqua M,Simone IL

更新日期：2018-10-01 00:00:00

abstract：:Recently, a frequent prion protein gene (PRNP) polymorphism consisting of a methionine (M) for valine (V) substitution at codon 129 has been associated with cognitive impairment in elderly individuals. Down syndrome (DS) is associated with mental retardation and development of Alzheimer-like brain abnormalities. In th...

journal_title：Journal of neurology

authors： Del Bo R,Comi GP,Giorda R,Crimi M,Locatelli F,Martinelli-Boneschi F,Pozzoli U,Castelli E,Bresolin N,Scarlato G

更新日期：2003-06-01 00:00:00

abstract：OBJECTIVE:The main aim was to explore the changes in hand-grip strength in patients with Duchenne muscular dystrophy (DMD) aged 5-29 years. Secondary aims were to test the effect of mutation, ambulatory status and glucocorticoid use on grip strength and its changes over time and to compute the number of subjects needed...

journal_title：Journal of neurology

authors： Hogrel JY,Decostre V,Ledoux I,de Antonio M,Niks EH,de Groot I,Straub V,Muntoni F,Ricotti V,Voit T,Seferian A,Gidaro T,Servais L

更新日期：2020-07-01 00:00:00