abstract：BACKGROUND:Dysarthria may be classified as flaccid, spastic, ataxic, hypokinetic, choreatic, dystonic, or mixed. We hypothesized that in routine neurological practice the reliability and accuracy of perceptual analysis alone in the classification of dysarthria is low and that this classification is mainly based on the ...

journal_title：Journal of neurology

authors： Fonville S,van der Worp HB,Maat P,Aldenhoven M,Algra A,van Gijn J

更新日期：2008-10-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Protein Z (PZ), a vitamin Kdependent protein, plays a role in inhibiting coagulation. Its plasma level or PZ gene polymorphisms have been discussed as risk factors for stroke with conflicting results reported between various studies. Only one of these polymorphisms was studied in a cohort of pati...

journal_title：Journal of neurology

authors： Le Cam-Duchez V,Bagan-Triquenot A,Barbay V,Mihout B,Borg JY

更新日期：2008-10-01 00:00:00

abstract：BACKGROUND:Glatiramer acetate (GA) therapy following brief, low-dose induction with mitoxantrone was safe and more effective than GA alone in suppressing inflammatory disease activity, as determined by a significant reduction in gadolinium (Gd)- enhancing MRI lesions, in a 15- month, randomized, single-blind study of r...

journal_title：Journal of neurology

authors： Arnold DL,Campagnolo D,Panitch H,Bar-Or A,Dunn J,Freedman MS,Gazda SK,Vollmer T

更新日期：2008-10-01 00:00:00

abstract：BACKGROUND:Spinal muscular atrophy (SMA) is caused by a homozygous deletion of the survival motor neuron (SMN)1 gene. The nearly identical SMN2 gene plays a disease modifying role. SMA is classified into four different subtypes based on age of onset and clinical course (SMA types 1-4). The natural history of early onse...

journal_title：Journal of neurology

authors： Piepers S,van den Berg LH,Brugman F,Scheffer H,Ruiterkamp-Versteeg M,van Engelen BG,Faber CG,de Visser M,van der Pol WL,Wokke JH

更新日期：2008-09-01 00:00:00

abstract：:Non motor symptoms (NMS) of PD are a key determinant of health, quality of life and societal cost of PD. Contrary to common perception, many NMS of PD occur early in PD and some may even predate the diagnosis of PD which is based on motor signs. These include olfactory deficit, sleep problems such as REM behaviour dis...

journal_title：Journal of neurology

authors： Chaudhuri KR,Naidu Y

更新日期：2008-09-01 00:00:00

abstract：OBJECTIVE:There exist controversial and discrepant results on the risk of spontaneous abortions and teratogenesis induced by interferon treatment in people with MS.Aim of this study is to evaluate risks of the administration of INFbeta related not only to the foetus, but also to children development up to 12-months dev...

journal_title：Journal of neurology

authors： Patti F,Cavallaro T,Lo Fermo S,Nicoletti A,Cimino V,Vecchio R,Laisa P,Zarbo R,Zappia M

更新日期：2008-08-01 00:00:00

abstract：:Spastic paraplegia type 7 (SPG7) is an autosomal recessive form of hereditary spastic paraparesis (ARHSP) caused by mutations in paraplegin, a subunit of an ATP-dependent AAA-protease located within the inner mitochondrial membrane. We have identified a novel paraplegin mutation, c.1047insC, in a non-consanguineous No...

journal_title：Journal of neurology

authors： Tzoulis C,Denora PS,Santorelli FM,Bindoff LA

更新日期：2008-08-01 00:00:00

abstract：:Movement disorder emergencies include any movement disorder which evolves over hours to days, in which failure to appropriately diagnose and manage can result in patient morbidity or mortality. It is crucial that doctors recognize these emergencies with accuracy and speed by obtaining the proper history and by being f...

journal_title：Journal of neurology

authors： Poston KL,Frucht SJ

更新日期：2008-08-01 00:00:00

abstract：OBJECTIVE:To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6. METHODS:Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched health...

journal_title：Journal of neurology

authors： Lukas C,Hahn HK,Bellenberg B,Hellwig K,Globas C,Schimrigk SK,Köster O,Schöls L

更新日期：2008-08-01 00:00:00

abstract：:Acute porphyrias are a group of inherited metabolic disorders representing overproduction syndromes with the formation of neurotoxic haem precursors. Clinical manifestations consist of acute attacks, which include abdominal pain, dysautonomia, mental symptoms, polyneuropathy and seizures mimicking many other acute neu...

journal_title：Journal of neurology

authors： Pischik E,Kazakov V,Kauppinen R

更新日期：2008-07-01 00:00:00

abstract：:Mutations in the mitofusin 2 (MFN2) gene are a major cause of primary axonal Charcot- Marie-Tooth (CMT) neuropathy. This study aims at further characterization of cerebral white matter alterations observed in patients with MFN2 mutations. Molecular genetic, magnetic resonance imaging (MRI), magnetic resonance spectros...

journal_title：Journal of neurology

authors： Brockmann K,Dreha-Kulaczewski S,Dechent P,Bönnemann C,Helms G,Kyllerman M,Brück W,Frahm J,Huehne K,Gärtner J,Rautenstrauss B

更新日期：2008-07-01 00:00:00

abstract：:Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) are the major immune neuropathies. Although a detailed understanding of the pathogenesis of these conditions is not yet available, the multiple effects of IVIg on the immune and inflamm...

journal_title：Journal of neurology

authors： Hughes R

更新日期：2008-07-01 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：OBJECTIVE:To investigate the efficacy of acupuncture on stroke recovery compared to an inert placebo. DESIGN:Placebo-controlled, randomised, clinical trial. SETTING:Post-stroke rehabilitation wards in five NHS hospitals in the UK. SUBJECTS:Patients between 4 and 10 days after their first stroke. INTERVENTIONS AND O...

journal_title：Journal of neurology

authors： Hopwood V,Lewith G,Prescott P,Campbell MJ

更新日期：2008-06-01 00:00:00

abstract：:Homozygous mutations in the PINK1 gene have been shown to cause early-onset parkinsonism. Here, we describe a novel homozygous mutation (Q126P), identified in two affected German sisters with a clinical phenotype typical for PINK1-associated parkinsonism. We analysed lactate, pyruvate, carnitine and acylcarnitine bloo...

journal_title：Journal of neurology

authors： Prestel J,Gempel K,Hauser TK,Schweitzer K,Prokisch H,Ahting U,Freudenstein D,Bueltmann E,Naegele T,Berg D,Klopstock T,Gasser T

更新日期：2008-05-01 00:00:00

abstract：:Cerebrospinal fluid (CSF) levels of interleukin (IL)-6, IL-11 and leukaemia inhibitory factor (LIF) were evaluated in 43 patients with Alzheimer's disease (AD) and 24 patients with frontotemporal lobar degeneration (FTLD) as compared with 30 agematched controls (CON), and correlated with clinical and demographic data ...

journal_title：Journal of neurology

authors： Galimberti D,Venturelli E,Fenoglio C,Guidi I,Villa C,Bergamaschini L,Cortini F,Scalabrini D,Baron P,Vergani C,Bresolin N,Scarpini E

更新日期：2008-04-01 00:00:00

abstract：INTRODUCTION:The so called "neurodegenerative Langerhans cell histiocytosis" (ND-LCH) is a rare and severe complication of LCH presenting as a progressive cerebellar ataxia associated with pyramidal tract signs, and cognitive impairment. MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known ...

journal_title：Journal of neurology

authors： Ribeiro MJ,Idbaih A,Thomas C,Remy P,Martin-Duverneuil N,Samson Y,Donadieu J,Hoang-Xuan K

更新日期：2008-04-01 00:00:00

abstract：INTRODUCTION:The spinocerebellar ataxias (SCAs), are rare neurodegenerative disorders caused by distinct genetic mutations. Clinically, the SCAs are characterised by progressive ataxia and a variety of other features, including cognitive dysfunction. The latter is consistent with a growing body of evidence supporting a...

journal_title：Journal of neurology

authors： Garrard P,Martin NH,Giunti P,Cipolotti L

更新日期：2008-03-01 00:00:00

abstract：:We examined the effect of bilateral subthalamic nucleus stimulation on levodopa-resistant balance impairment in 14 patients with Parkinson's disease and 18 matched controls. Instability was quantitatively assessed using standardized multidirectional dynamic posturography. Patients were tested after taking a suprathres...

journal_title：Journal of neurology

authors： Visser JE,Allum JH,Carpenter MG,Esselink RA,Speelman JD,Borm GF,Bloem BR

更新日期：2008-02-01 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：:Ataxia with oculomotor apraxia type 1 (AOA1) is a rare autosomal recessive neurodegenerative disease, recently associated with mutations in the aprataxin gene. Main features are early onset cerebellar ataxia, oculomotor apraxia and peripheral neuropathy. The presence of choreoathetosis or dystonia in some patients sug...

journal_title：Journal of neurology

authors： Salvatore E,Varrone A,Criscuolo C,Mancini P,Sansone V,Strisciuglio C,Cicala D,Scarano V,Salvatore M,Pappatà S,De Michele G,Filla A

更新日期：2008-01-01 00:00:00

abstract：:Migraine is a common debilitating neurological disease characterised by attacks of severe headache with or without preceding aura. Its aetiology remains elusive; however it is clear that an interplay of genetic and environmental components play an important role. Familial hemiplegic migraine (FHM) is a rare and severe...

journal_title：Journal of neurology

authors： Ramagopalan SV,Ramscar NE,Cader MZ

更新日期：2007-12-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Stratification of patients with transient ischemic attack (TIA) or ischemic stroke (IS) by risk of recurrent stroke can contribute to optimized secondary prevention. We therefore aimed to assess cardiovascular risk factor profiles of consecutive patients hospitalized with TIA/IS to stratify the r...

journal_title：Journal of neurology

authors： Weimar C,Goertler M,Röther J,Ringelstein EB,Darius H,Nabavi DG,Kim IH,Theobald K,Diener HC,SCALA Study Group.

更新日期：2007-11-01 00:00:00

abstract：:To further evaluate (1) transcranial sonography (TCS) for (pre)clinical diagnosis of Parkinson's disease (PD) and (2) to examine asymptomatic carriers of Parkin mutations we investigated substantia nigra (SN) hyperechogenicity in PD patients and unaffected subjects with and without Parkin mutations. The area (aSN) of ...

journal_title：Journal of neurology

authors： Hagenah JM,König IR,Becker B,Hilker R,Kasten M,Hedrich K,Pramstaller PP,Klein C,Seidel G

更新日期：2007-10-01 00:00:00

abstract：:Cerebellar ataxia can have many genetic causes among which are the congenital disorders of glycosylation type I (CDG-I). In this group of disorders, a multisystem phenotype is generally observed including the involvement of many organs, the endocrine, hematologic and central nervous systems. A few cases of CDG-Ia have...

journal_title：Journal of neurology

authors： Vermeer S,Kremer HP,Leijten QH,Scheffer H,Matthijs G,Wevers RA,Knoers NA,Morava E,Lefeber DJ

更新日期：2007-10-01 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：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：PURPOSE:The role of thalamus and brainstem in generalized epilepsy has been suggested in previous studies. The aim of the present study was to assess regional cerebral blood flow (rCBF) abnormality in juvenile myoclonic epilepsy (JME) patients. METHODS:(99m)Tc-ethylcysteinate dimer brain single photon emission compute...

journal_title：Journal of neurology

authors： Tae WS,Joo EY,Han SJ,Lee KH,Hong SB

更新日期：2007-08-01 00:00:00

abstract：:Biomarkers beyond clinical assessment are needed in patients who suffer from amyotrophic lateral sclerosis (ALS). Here, single-voxel proton magnetic resonance spectroscopy ((1)H MRS) of the gray matter of the motor cortex and the white matter including the pyramidal tracts was used to investigate concentrations of N-a...

journal_title：Journal of neurology

authors： Unrath A,Ludolph AC,Kassubek J

更新日期：2007-08-01 00:00:00

abstract：OBJECTIVE:To assess the association of Medial Temporal lobe Atrophy (MTA) and White Matter Hyperintensities (WMHs) with gray matter perfusion in Mild Cognitive Impairment (MCI). METHODS:56 MCI patients (age = 69.3 +/- 7.0, 32 females) underwent brain MR scan and (99m)Tc ECD SPECT. We evaluated MTA according to Schelte...

journal_title：Journal of neurology

authors： Caroli A,Testa C,Geroldi C,Nobili F,Guerra UP,Bonetti M,Frisoni GB

更新日期：2007-08-01 00:00:00

abstract：:Interferon beta (IFNbeta) therapy for multiple sclerosis (MS) is associated with a potential for the development of neutralising antibodies (NAbs) that negatively affect therapy. Several factors influence the development of NAbs, such as lack of complete sequence homology with the endogenous IFNbeta sequence, frequenc...

journal_title：Journal of neurology

authors： Hartung HP,Polman C,Bertolotto A,Deisenhammer F,Giovannoni G,Havrdova E,Hemmer B,Hillert J,Kappos L,Kieseier B,Killestein J,Malcus C,Comabella M,Pachner A,Schellekens H,Sellebjerg F,Selmaj K,Sorensen PS

更新日期：2007-07-01 00:00:00

abstract：BACKGROUND:Thymoma is frequently associated with paraneoplastic diseases (PDs), most commonly with myasthenia gravis (MG). This association is thought to depend on thymoma's capacity to produce and export T lymphocytes. OBJECTIVE:(1) To determine the frequency and characteristics of thymoma-associated PDs other than M...

journal_title：Journal of neurology

authors： Evoli A,Minicuci GM,Vitaliani R,Battaglia A,Della Marca G,Lauriola L,Fattorossi A

更新日期：2007-06-01 00:00:00

abstract：UNLABELLED:The objective of this study was to investigate the relationship between electrophysiological recordings and clinical as well as radiological findings in patients suggestive to suffer from a lumbar spinal stenosis (LSS). We hypothesise that the electrophysiological recordings, especially SSEP, indicate a lumb...

journal_title：Journal of neurology

authors： Egli D,Hausmann O,Schmid M,Boos N,Dietz V,Curt A

更新日期：2007-06-01 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：:Neuropsychological assessment is one of the main fields of activity of clinical neuropsychologists. Goals of this assessment are verification and description of cognitive and affective impairments as the consequence of brain damage. Included are examinations of basal and higher sensory functions, the intellectual prof...

journal_title：Journal of neurology

authors： Sturm W

更新日期：2007-05-01 00:00:00

abstract：:Cognitive impairment belongs to the core symptoms in MS affecting quality of life, self-esteem, and social as well as occupational functioning. Due to this high impact on patients' well-being efficient treatment concepts are required. Imaging studies on cognition have shown that functional reorganisation takes place s...

journal_title：Journal of neurology

authors： Penner IK,Opwis K,Kappos L

更新日期：2007-05-01 00:00:00

abstract：:Myasthenia gravis patients usually present with fluctuating, asymmetrical stress-dependent weakness in the absence of other neurological disturbances. In weak patients psychopathological disturbances are frequently reported and misdiagnosed as a psychiatric disorder. The question to what extent psychiatric symptoms ar...

journal_title：Journal of neurology

authors： Köhler W

更新日期：2007-05-01 00:00:00

abstract：:Although most patients with spinal muscular atrophy (SMA) are homozygous for deletion of the SMN1 gene, some patients bear one SMN1 copy with a subtle mutation. Detection of such an intragenic mutation may be helpful not only in confirming diagnosis but also in elucidating functional domains of the SMN protein. In thi...

journal_title：Journal of neurology

authors： Kotani T,Sutomo R,Sasongko TH,Sadewa AH,Gunadi,Minato T,Fujii E,Endo S,Lee MJ,Ayaki H,Harada Y,Matsuo M,Nishio H

更新日期：2007-05-01 00:00:00

abstract：:To analyze the in vivo biological effect of anti-interferon beta (IFN-beta) neutralizing antibodies (NABs), blood concentrations of neopterin, beta2microglobulin (Beta2-MG), mRNA-dependent myxovirusresistant protein A (MxA) and dsRNA-dependent protein kinase (PKR) were measured before (predose) and 24 hours after (pos...

journal_title：Journal of neurology

authors： Scagnolari C,Duda P,Bagnato F,De Vito G,Alberelli A,Lavolpe V,Girardi E,Durastanti V,Trojano M,Kappos L,Antonelli G

更新日期：2007-05-01 00:00:00

abstract：:Seventy-seven cases of the optic-spinal form of multiple sclerosis (OSMS) were collected from 6 institutes in 3 cities of Japan, and the clinical and MRI features were analyzed. Two-thirds of the OSMS patients had longitudinally extensive spinal cord MRI lesions (LESL), and had clinical features similar to those of re...

journal_title：Journal of neurology

authors： Nakashima I,Fukazawa T,Ota K,Nohara C,Warabi Y,Ohashi T,Miyazawa I,Fujihara K,Itoyama Y

更新日期：2007-04-01 00:00:00