Oxidative stress biomarkers in mitochondrial myopathies, basally and after cysteine donor supplementation.

abstract：:We have investigated how the abnormal head posture and motility in spasmodic torticollis interferes with ecological movements such as combined eye-to-foot whole-body reorientations to visual targets. Eight mildly affected patients and 10 controls voluntarily rotated eyes and body in response to illuminated targets of ...

journal_title：Journal of neurology

authors： Anastasopoulos D,Ziavra N,Pearce R,Bronstein AM

更新日期：2013-08-01 00:00:00

abstract：:Natalizumab, an anti-alpha4 integrin antibody, significantly reduces the number of visibly enhancing multiple sclerosis (MS) lesions. In this substudy of a 2-year trial of natalizumab monotherapy versus placebo, contrast-enhanced imaging investigated for subtle blood brain barrier (BBB) leakage in relapsing remitting ...

journal_title：Journal of neurology

authors： Soon D,Altmann DR,Fernando KT,Giovannoni G,Barkhof F,Polman CH,O'Connor P,Gray B,Panzara M,Miller DH

更新日期：2007-03-01 00:00:00

abstract：:Autonomic dysfunction is frequently observed in patients with multiple sclerosis (MS), but clinical studies disagree on the frequency and type of abnormalities in autonomic function tests. Orthostatic dizziness (OD) has been reported in up to 49% of patients, but the pathophysiological mechanisms are poorly understood...

journal_title：Journal of neurology

authors： Flachenecker P,Wolf A,Krauser M,Hartung HP,Reiners K

更新日期：1999-07-01 00:00:00

abstract：:Spinocerebellar ataxia (SCA) type 7 is an autosomal dominant disorder characterized by neural loss, mainly in the cerebellum and regions of the brainstem and particularly the inferior olivary complex. This neurodegeneration disease is associated with expansion of unstable CAG repeats within the 5'-translated region of...

journal_title：Journal of neurology

authors： Hsieh M,Lin SJ,Chen JF,Lin HM,Hsiao KM,Li SY,Li C,Tsai CJ

更新日期：2000-08-01 00:00:00

abstract：:Several reports in the literature describe the effects of low-dose (5 mg/kg/day) idebenone in significantly reducing cardiac hypertrophy in patients with Friedreich ataxia. However, the effects of idebenone on neurological function have not been reliably determined in these studies; when neurological parameters were r...

journal_title：Journal of neurology

authors： Schulz JB,Di Prospero NA,Fischbeck K

更新日期：2009-03-01 00:00:00

abstract：:Spinal muscular atrophy (SMA) is an autosomal recessive disorder that affects motor neurons. It is caused by mutations in the survival motor neuron gene 1 (SMN1). The SMN2 gene, which is the highly homologous SMN1 copy that is present in all the patients, is unable to prevent the disease. An SMN2 dosage method was app...

journal_title：Journal of neurology

authors： Cuscó I,Barceló MJ,Rojas-García R,Illa I,Gámez J,Cervera C,Pou A,Izquierdo G,Baiget M,Tizzano EF

更新日期：2006-01-01 00:00:00

abstract：:Little is known about the pattern of subacute cognitive domain impairments after ischaemic stroke, nor the temporal evolution of such impairments. Our objective was to investigate the pattern of cognitive impairment in different neuropsychological domains up to a year after ischaemic stroke. We included prospectively ...

journal_title：Journal of neurology

authors： Hurford R,Charidimou A,Fox Z,Cipolotti L,Werring DJ

更新日期：2013-01-01 00:00:00

abstract：:This study aimed at identifying gender-specific differences in stroke knowledge, stroke risk perception and the educational effects of a multimodal educational intervention. We performed computer-assisted telephone surveys among an average sample of 500 members of the general public (44.0% male, 56.0% female), before ...

journal_title：Journal of neurology

authors： Marx JJ,Klawitter B,Faldum A,Eicke BM,Haertle B,Dieterich M,Nedelmann M

更新日期：2010-03-01 00:00:00

abstract：OBJECTIVE:To identify factors that impact on the ability of patients with multiple sclerosis (MS) to remain in work in order to make recommendations for future clinical management. METHODS:Cross-sectional studies using qualitative (phase one) and quantitative (phase two) methods. In phase one, 62 patients were intervi...

journal_title：Journal of neurology

authors： O'Connor RJ,Cano SJ,Ramió i Torrentà L,Thompson AJ,Playford ED

更新日期：2005-08-01 00:00:00

abstract：:Autosomal recessive cerebellar ataxia type 3 (ARCA3) is a rare inherited disorder caused by mutations in the ANO10 gene. The disease is characterized by slowly progressive spastic ataxia variably associated with motor neuron involvement, epilepsy, and cognitive decline. We performed mutational screening in 80 patients...

journal_title：Journal of neurology

authors： Nanetti L,Sarto E,Castaldo A,Magri S,Mongelli A,Rossi Sebastiano D,Canafoglia L,Grisoli M,Malaguti C,Rivieri F,D'Amico MC,Di Bella D,Franceschetti S,Mariotti C,Taroni F

更新日期：2019-02-01 00:00:00

abstract：:The aims of this study are to investigate whether auditory dysfunction is part of the spectrum of neurological abnormalities associated with Leber's hereditary optic neuropathy (LHON) and to determine the perceptual consequences of auditory neuropathy (AN) in affected listeners. Forty-eight subjects confirmed by genet...

journal_title：Journal of neurology

authors： Rance G,Kearns LS,Tan J,Gravina A,Rosenfeld L,Henley L,Carew P,Graydon K,O'Hare F,Mackey DA

更新日期：2012-03-01 00:00:00

abstract：:The neurochemical abnormalities underlying vascular parkinsonism (VP) have not been well characterised. A better understanding may help to optimize pharmacological interventions. Since VP patients generally have a poorer response to l-Dopa than Parkinson's disease (PD) patients, we investigated whether levels of relev...

journal_title：Journal of neurology

authors： Herbert MK,Kuiperij H,Bloem BR,Verbeek MM

更新日期：2013-12-01 00:00:00

abstract：:Corticospinal lesions cause impairments in voluntary motor control. Recent findings suggest that some degree of voluntary control may be taken over by a compensatory pathway involving the reticulospinal tract. In humans, evidence for this notion mainly comes from StartReact studies. StartReact is the acceleration of r...

journal_title：Journal of neurology

authors： van Lith BJH,Coppens MJM,Nonnekes J,van de Warrenburg BPC,Geurts AC,Weerdesteyn V

更新日期：2018-11-01 00:00:00

abstract：:Several studies have reported infection as a possible risk factor for cervical artery dissection (CAD). We retrospectively analyzed several inflammatory parameters of CAD patients with the aim of detecting differences between spontaneous (n = 25) and traumatic (n = 18) CAD. In this case-control study, we observed sign...

journal_title：Journal of neurology

authors： Forster K,Poppert H,Conrad B,Sander D

更新日期：2006-06-01 00:00:00

abstract：:Intrathecal synthesis of immunoglobulin G (IgG) specific to Treponema pallidum, ssp. pallidum, or to the human immunodeficiency virus 1 (HIV-1) was investigated in patients with contemporary treponemal and HIV-1 infections. Using a T. pallidum and an HIV-1 IgG enzyme-linked immunosorbent assay, specific antibody units...

journal_title：Journal of neurology

authors： Müller F,Moskophidis M,Schmitz H

更新日期：1988-03-01 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：:It is well known that neurorehabilitation can reduce disability or improve handicap of people with multiple sclerosis (MS). The aim of this study was to evaluate the effectiveness of a short period (6 weeks) of a tailored, individualised outpatient rehabilitation program in people with progressive MS. A randomised-con...

journal_title：Journal of neurology

authors： Patti F,Ciancio MR,Cacopardo M,Reggio E,Fiorilla T,Palermo F,Reggio A,Thompson AJ

更新日期：2003-07-01 00:00:00

abstract：: ...

journal_title：Journal of neurology

authors： Argaet EC,Young AS,Bradshaw AP,Welgampola MS

更新日期：2019-01-01 00:00:00

abstract：OBJECTIVES:To estimate the socio-economic impact of multiple sclerosis (MS) in Italy. METHODS:Outpatients with MS were enrolled at 44 centres across Italy. Socio-demographic, clinical and resource utilization data were collected using a validated questionnaire. Each patient completed a weekly diary of expenses due to ...

journal_title：Journal of neurology

authors： Amato MP,Battaglia MA,Caputo D,Fattore G,Gerzeli S,Pitaro M,Reggio A,Trojano M,Mu. S. I. C. Study Group.

更新日期：2002-02-01 00:00:00

abstract：:In Parkinson's disease (PD), nonmotor symptoms manifest before motor symptoms. In this report, we present a remarkable case of a semiprofessional painter with PD whose painting style dramatically changed from abstract painting to realism before he developed motor, psychiatric, and autonomic nerve disorders. This case ...

journal_title：Journal of neurology

authors： Shimura H,Tanaka R,Urabe T,Tanaka S,Hattori N

更新日期：2012-05-01 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：:Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown etiology that affects multiple organs. We report 6 cases of ECD with neurological involvement and neuroradiological abnormalities on brain MRI. A literature review revealed 60 other cases of ECD with neurological involvement. We t...

journal_title：Journal of neurology

authors： Lachenal F,Cotton F,Desmurs-Clavel H,Haroche J,Taillia H,Magy N,Hamidou M,Salvatierra J,Piette JC,Vital-Durand D,Rousset H

更新日期：2006-10-01 00:00:00

abstract：:Vertigo is frequently associated with migraine, and sometimes it is the cardinal symptom. This type of migraine is called "vestibular migraine", "migrainous vertigo", or "migraine-associated vertigo". Earlier findings on effective prophylactic medication for such migraine attacks and their clinical features are few an...

journal_title：Journal of neurology

authors： Baier B,Winkenwerder E,Dieterich M

更新日期：2009-03-01 00:00:00

abstract：:Three cases of Fahr's syndrome are described. All patients had disturbances of calcium metabolism and had had a meningoencephalitis in childhood. It is suggested that gliovascular changes, induced by cerebral inflammation, can later facilitate the occurrence of calcification of the striopallidodentate system when abno...

journal_title：Journal of neurology

authors： Morgante L,Vita G,Meduri M,Di Rosa AE,Galatioto S,Coraci MA,Di Perri R

更新日期：1986-02-01 00:00:00

abstract：:KIF1A gene encodes the kinesin 1a protein, an axonal motor protein working in cargo transport along neurites. Variants in KIF1A were identified in different forms of neurodegenerative diseases with dominant and recessive inheritance. Homozygous recessive mutations were found in the hereditary sensory and autonomic neu...

journal_title：Journal of neurology

authors： Citterio A,Arnoldi A,Panzeri E,Merlini L,D'Angelo MG,Musumeci O,Toscano A,Bondi A,Martinuzzi A,Bresolin N,Bassi MT

更新日期：2015-12-01 00:00:00

abstract：:Regional cerebral glucose metabolism (rCMRGlc) and dopamine D2 receptor binding were measured in a 31-year-old, severely affected, untreated patient with Wilson's disease of 3 years' duration using positron emission tomography and 18F-deoxyglucose and 18F-methylspiperone ([18F]MSP), respectively. There was a severe re...

journal_title：Journal of neurology

authors： Schlaug G,Hefter H,Nebeling B,Engelbrecht V,Weiss P,Stöcklin G,Seitz RJ

更新日期：1994-10-01 00:00:00

abstract：:REM sleep behavior disorder (RBD) is known to be observed more frequently in patients with an α-synucleinopathy such as Parkinson's disease (PD) than in the general population. The precise prevalence of RBD in Japanese PD patients is not known. Therefore, we investigated the prevalence and the clinical characteristics...

journal_title：Journal of neurology

authors： Nihei Y,Takahashi K,Koto A,Mihara B,Morita Y,Isozumi K,Ohta K,Muramatsu K,Gotoh J,Yamaguchi K,Tomita Y,Sato H,Seki M,Iwasawa S,Suzuki N

更新日期：2012-08-01 00:00:00

abstract：:The posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of (sub)acute onset characterized by varied neurological symptoms, which may include headache, impaired visual acuity or visual field deficits, disorders of consciousness, confusion, seizures, and focal neurological deficits. In a major...

journal_title：Journal of neurology

authors： Fischer M,Schmutzhard E

更新日期：2017-08-01 00:00:00

abstract：:Four cases of mild botulinus type B intoxication are reported with the clinical neurophysiological studies. Electromyographically tetanic stimulation of the N. ulnaris initially led to tetanic potentiation of the muscle action potential. After an interval without any symptoms tetanic reduction became apparent between ...

journal_title：Journal of neurology

authors： Hagenah R,Müller-Jensen A

更新日期：1978-02-14 00:00:00

abstract：BACKGROUND AND PURPOSE:The use of early prognostic data provided by various scores in critically ill stroke patients remains unclear. We tested the performance of the Simplified Acute Physiology Score (SAPS) II in prediction of mortality of acute stroke patients in the NeuroCriticalCareUnit (NCCU). METHODS:During one ...

journal_title：Journal of neurology

authors： Handschu R,Haslbeck M,Hartmann A,Fellgiebel A,Kolominsky-Rabas P,Schneider D,Berrouschot J,Erbguth F,Reulbach U

更新日期：2005-10-01 00:00:00