Longitudinal cognitive and functional changes in primary progressive aphasia.

abstract：:Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating disease of the brain caused by the JC virus that occurs mainly in immunocompromised patients. The prognosis is very poor. As the lesion looks like non- specific leukoencephalopathy, making a diagnosis at the early stage is very difficult. We ...

journal_title：Journal of neurology

authors： Shirai S,Yabe I,Kano T,Shimizu Y,Sasamori T,Sato K,Hirotani M,Nonaka T,Takahashi I,Matsushima M,Minami N,Nakamichi K,Saijo M,Hatanaka KC,Shiga T,Tanaka S,Sasaki H

更新日期：2014-12-01 00:00:00

abstract：OBJECTIVE:To determine the prevalence and characteristics of the cricopharyngeal bar (CPB), defined as marked protrusion with lacking relaxation and stricture of the upper esophageal sphincter on videofluoroscopy, in patients with inclusion body myositis (IBM). METHODS:We conducted a case-control study of comprehensiv...

journal_title：Journal of neurology

authors： Taira K,Yamamoto T,Mori-Yoshimura M,Sajima K,Takizawa H,Shinmi J,Oya Y,Nishino I,Takahashi Y

更新日期：2020-09-26 00:00:00

abstract：BACKGROUND:Diagnostic delay of hereditary transthyretin amyloidosis (ATTRv, v for variant) prevents timely treatment and, therefore, concurs to the mortality of the disease. The aim of the present study was to explore with nerve ultrasound (US) possible red flags for early diagnosis in ATTRv patients with carpal tunnel...

journal_title：Journal of neurology

authors： Salvalaggio A,Coraci D,Cacciavillani M,Obici L,Mazzeo A,Luigetti M,Pastorelli F,Grandis M,Cavallaro T,Bisogni G,Lozza A,Gemelli C,Gentile L,Ermani M,Fabrizi GM,Plasmati R,Campagnolo M,Castellani F,Gasparotti R,Marti

更新日期：2021-01-01 00:00:00

abstract：BACKGROUND:Stroke has a major impact on survivors. Our study was designed to describe the mental status and health-related quality of life (HRQoL) in long-term survivors of TIA or minor ischaemic stroke (MIS) and evaluate associations of mental and physical factors with HR-QoL. METHODS:A random sample of the 10-year s...

journal_title：Journal of neurology

authors： van Wijk I,Gorter JW,Lindeman E,Kappelle LJ,van Gijn J,Koudstaal PJ,Algra A

更新日期：2007-08-01 00:00:00

abstract：:Inborn errors of metabolism in 40 children have been investigated by computed tomography to obtain data on the degree of cerebral involvement in neurodegenerative and storage disorders: 20 children had various mucopolysaccharidoses, 8 sphingolipidoses , 3 mucolipidoses, 2 oligosaccharidoses , 3 ceroidlipofuscinoses an...

journal_title：Journal of neurology

authors： Wende S,Ludwig B,Kishikawa T,Rochel M,Gehler J

更新日期：1984-01-01 00:00:00

abstract：OBJECTIVE:To investigate possible changes in autonomic cardiovascular regulation and cardiopulmonary baroreflex sensitivity in patients with primary cervical dystonia receiving chronic treatment with botulinum toxin type A. METHODS:Short-term power spectral analysis of heart rate and systolic blood pressure variabilit...

journal_title：Journal of neurology

authors： Tiple D,Strano S,Colosimo C,Fabbrini G,Calcagnini G,Prencipe M,Berardelli A

更新日期：2008-06-01 00:00:00

abstract：:The transcranial activation and the conduction properties of corticospinal and callosal neurons were investigated in 12 early-treated adolescents (aged 17.3, SD 3.5 years; range 14-27 years) with phenylketonuria (PKU) by focal transcranial magnetic stimulation (fTMS) of the motor cortex. The patients had no functional...

journal_title：Journal of neurology

authors： Röricht S,Meyer BU,Irlbacher K,Ludolph AC

更新日期：1999-01-01 00:00:00

abstract：:In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing field. First, we highlight the non-motor features at dise...

journal_title：Journal of neurology

authors： Chelban V,Catereniuc D,Aftene D,Gasnas A,Vichayanrat E,Iodice V,Groppa S,Houlden H

更新日期：2020-09-01 00:00:00

abstract：:The diagnosis of Hallervorden-Spatz disease (HSD) has usually been made post mortem, although the recent description of characteristic abnormalities in the globus pallidus has suggested the possibility of an in vivo diagnosis. We present the clinical histories, neurological features and MRI findings of 11 patients, di...

journal_title：Journal of neurology

authors： Angelini L,Nardocci N,Rumi V,Zorzi C,Strada L,Savoiardo M

更新日期：1992-10-01 00:00:00

abstract：:Recent genetic studies suggest a Sardinian type of amyotrophic lateral sclerosis (ALS). Thus, ALS incidence, prevalence and survival were investigated in a large population of Sardinians aimed to disclose population-specific patterns and their temporal changes. This is a population-based incidence and prevalence study...

journal_title：Journal of neurology

authors： Pugliatti M,Parish LD,Cossu P,Leoni S,Ticca A,Saddi MV,Ortu E,Traccis S,Borghero G,Puddu R,Chiò A,Pirina P

更新日期：2013-02-01 00:00:00

abstract：:The objective of the study was to identify differences in treatment strategies for anti-NMDA receptor encephalitis based on specialty of treating physicians, geographic location, and years in practice. We conducted an anonymous worldwide electronic survey through the Practice Current section of Neurology® Clinical Pra...

journal_title：Journal of neurology

authors： Bartolini L,Muscal E

更新日期：2017-04-01 00:00:00

abstract：BACKGROUND:Putaminal iron deposition is a histopathological feature of multiple system atrophy (MSA), which is not observed in patients with idiopathic Parkinson's disease (PD). T2*-weighted magnetic resonance imaging (MRI) gradient echo (GE) sequences are sensitive for paramagnetic susceptibility changes and therefore...

journal_title：Journal of neurology

authors： von Lewinski F,Werner C,Jörn T,Mohr A,Sixel-Döring F,Trenkwalder C

更新日期：2007-09-01 00:00:00

abstract：:Production of reactive oxygen species after cerebral blood flow disruption may enhance tissue damage through multiple molecular pathways. Changes in nitric oxide (NO) metabolism and oxidative stress status were investigated in 47 patients with ischemic stroke by measuring plasma nitric oxide (NO) and peroxynitrite (ON...

journal_title：Journal of neurology

authors： Taffi R,Nanetti L,Mazzanti L,Bartolini M,Vignini A,Raffaelli F,Pasqualetti P,Vernieri F,Provinciali L,Silvestrini M

更新日期：2008-01-01 00:00:00

abstract：:Elevated uric acid levels have recently been found to be associated with slower disease progression in Parkinson's disease, Huntington's disease, multiple system atrophy, and mild cognitive impairment. The aim of this study is to determine whether serum uric acid levels predict survival in amyotrophic lateral sclerosi...

journal_title：Journal of neurology

authors： Paganoni S,Zhang M,Quiroz Zárate A,Jaffa M,Yu H,Cudkowicz ME,Wills AM

更新日期：2012-09-01 00:00:00

abstract：:As there are scarce data regarding the outcomes of acute ischemic stroke (AIS) patients treated with intravenous thrombolysis (IVT) within 60 min from symptom onset ("golden hour"), we sought to compare outcomes between AIS patients treated within [GH(+)] and outside [GH(-)] the "golden hour" by analyzing propensity s...

journal_title：Journal of neurology

authors： Tsivgoulis G,Katsanos AH,Kadlecová P,Czlonkowska A,Kobayashi A,Brozman M,Švigelj V,Csiba L,Fekete K,Kõrv J,Demarin V,Vilionskis A,Jatuzis D,Krespi Y,Liantinioti C,Giannopoulos S,Mikulik R

更新日期：2017-05-01 00:00:00

abstract：:Additional autoimmune diseases in people with multiple sclerosis (MS) and their relatives have been studied many times. Studies have employed different designs, and yielded conflicting results. We performed a systematic review, and calculated overall risk of additional autoimmune diseases in people with MS and their f...

journal_title：Journal of neurology

authors： Dobson R,Giovannoni G

更新日期：2013-05-01 00:00:00

abstract：:Multifocal motor neuropathy (MMN) can be differentiated from motor neuron disease by electrophysiological evidence of conduction block. To increase the probability of recording conduction block, we studied the whole nerve length including proximal segments in 84 patients with pure motor syndromes, using a special stim...

journal_title：Journal of neurology

authors： Jaspert A,Claus D,Grehl H,Neundörfer B

更新日期：1996-10-01 00:00:00

abstract：:The concentrations of glucose, pyruvate and lactate were determined in the blood and lumbar CSF of 63 patients with neuromuscular disorders and 40 controls. The glucose/lactate and lactate/pyruvate ratios were also calculated, in addition to the cytoplasmic NADH/NAD+ (nicotinamide-adenine dinucleotide-H/nicotinamide-a...

journal_title：Journal of neurology

authors： Mechler F,Diószeghy P,Csenkér E,Molnár L

更新日期：1981-01-01 00:00:00

abstract：:Fabry disease (FD) is a rare inherited disorder of the metabolism, associated with renal, cardiac, and cerebrovascular complications. Ischemic and hemorrhagic stroke in FD present with a similar proportion to that observed in the general population, but usually at an early age. Ischemic stroke may result from cardiac ...

journal_title：Journal of neurology

authors： Viana-Baptista M

更新日期：2012-06-01 00:00:00

abstract：:A myasthenia gravis (MG) patient who seems to have recovered can later have recurrence of myasthenic signs. Clearly clinical remission does not always correspond to the normalization of all the factors involved in the pathogenesis of the disease. In ten patients who had apparently recovered from MG, electromyographic ...

journal_title：Journal of neurology

authors： Scoppetta C,Bartoccioni E,David P,Flamini G,Lo Monaco M,Scuderi F,Tonali P

更新日期：1982-01-01 00:00:00

abstract：BACKGROUND:An upper motor neuron (UMN) lesion in amyotrophic lateral sclerosis (ALS) is often difficult to identify because clinical signs may be discrete or masked by severe simultaneous LMN lesions. We compared the diagnostic sensitivity of transcranial magnetic stimulation (TMS) to cranial muscles and limb muscles i...

journal_title：Journal of neurology

authors： Urban PP,Wicht S,Hopf HC

更新日期：2001-10-01 00:00:00

abstract：:Cerebellar ataxias (CAs) represent a heterogeneous group of sporadic or inherited disorders. The clinical spectrum of CAs is continuously expanding. Our understanding of the mechanisms leading to the clinical deficits has improved over these last decades, in particular thanks to progress in genetics, neuroimaging and ...

journal_title：Journal of neurology

authors： Gandini J,Manto M,Bremova-Ertl T,Feil K,Strupp M

更新日期：2020-04-01 00:00:00

abstract：:We report a case of polycythemia vera with chorea in which the brain metabolism and dopamine system were investigated using 2-[(18)F]fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) and (99m)Tc-labeled tropane dopamine transporter ((99m)Tc-TRODAT-1) single photon emission computed tomography (SPECT). Al...

journal_title：Journal of neurology

authors： Huang HC,Wu YC,Shih LY,Lo WC,Tsai CH,Shyu WC

更新日期：2011-11-01 00:00:00

abstract：:We analysed the CACNA1A gene, located on chromosome 19p13, in three unrelated families and one sporadic case with episodic ataxia type 2 (EA-2). In two of the families and the sporadic patient, novel truncating mutations, which disrupt the reading frame and result in a premature stop of the CACNA1A protein, were ident...

journal_title：Journal of neurology

authors： van den Maagdenberg AM,Kors EE,Brunt ER,van Paesschen W,Pascual J,Ravine D,Keeling S,Vanmolkot KR,Vermeulen FL,Terwindt GM,Haan J,Frants RR,Ferrari MD

更新日期：2002-11-01 00:00:00

abstract：:Sexuality in patients with amyotrophic lateral sclerosis (ALS) has received little attention so far. Although sexual function is not directly affected by the disease process, several patients have reported problems within their sexual relationship. We performed a questionnaire survey to ascertain the extent and clinic...

journal_title：Journal of neurology

authors： Wasner M,Bold U,Vollmer TC,Borasio GD

更新日期：2004-04-01 00:00:00

abstract：:Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, e...

journal_title：Journal of neurology

authors： Begeti F,Tan AY,Cummins GA,Collins LM,Guzman NV,Mason SL,Barker RA

更新日期：2013-11-01 00:00:00

abstract：:The association of pathology and neurological deficit with quality of life (QoL) in multiple sclerosis (MS) is not fully understood. In this study, magnetic resonance imaging (MRI) measures of pathology--T1 and T2 lesion volume and ratio; active T2 lesion number; global and regional brain volume and atrophy; magnetiza...

journal_title：Journal of neurology

authors： Hayton T,Furby J,Smith KJ,Altmann DR,Brenner R,Chataway J,Hunter K,Tozer DJ,Miller DH,Kapoor R

更新日期：2012-02-01 00:00:00

abstract：OBJECTIVE:Gluten neuropathy (GN) is the term used to describe peripheral neuropathy that occurs in patients with gluten sensitivity (GS) or coeliac disease (CD) in the absence of other risk factors. We aimed to describe the neurophysiological progression rate of GN across time and look into the potential role of geneti...

journal_title：Journal of neurology

authors： Zis P,Sarrigiannis P,Artemiadis A,Sanders DS,Hadjivassiliou M

更新日期：2021-01-01 00:00:00

abstract：:Knowledge of restless legs syndrome (RLS) has greatly increased in recent years due to the many advances that have been made in diagnosis, management and genetics. Tools have been developed that facilitate the diagnosis and treatment of RLS, in particular the essential diagnostic criteria for RLS have been refined, se...

journal_title：Journal of neurology

authors： Trenkwalder C,Högl B,Winkelmann J

更新日期：2009-04-01 00:00:00

abstract：OBJECTIVE:To explore the value of olfactory identification deficits as a predictor of cerebral β-amyloid status and other markers of brain health in cognitively normal adults aged ~ 70 years. METHODS:Cross-sectional observational cohort study. 389 largely healthy and cognitively normal older adults were recruited from...

journal_title：Journal of neurology

authors： Buchanan SM,Parker TD,Lane CA,Keshavan A,Keuss SE,Lu K,James SN,Murray-Smith H,Wong A,Nicholas J,Cash DM,Malone IB,Coath W,Thomas DL,Sudre C,Fox NC,Richards M,Schott JM

更新日期：2020-11-01 00:00:00