Disturbed benzodiazepine receptor function at the onset of temporal lobe epilepsy--lomanzenil-binding in de-novo TLE.

abstract：:Fifteen patients with Sneddon's syndrome presenting since 1979 were re-examined. After a neurological examination, an orienting test of mental ability as well as an electroencephalogram were performed in all patients. In 10 of the 15 patients computed tomography of the brain was performed, too. Preceding reports and t...

journal_title：Journal of neurology

authors： Weissenborn K,Lubach D,Schwabe C,Becker H

更新日期：1989-01-01 00:00:00

abstract：:Facioscapulohumeral muscular dystrophy (FSHD) is the third most common muscular dystrophy and usually follows an autosomal dominant trait. Clinically, FSHD affects facial muscles and proximal upper limb and girdle muscles, but may present with variable clinical phenotypes even within the same family. Most genetically ...

journal_title：Journal of neurology

authors： Reilich P,Schramm N,Schoser B,Schneiderat P,Strigl-Pill N,Müller-Höcker J,Kress W,Ferbert A,Rudnik-Schöneborn S,Noth J,Lochmüller H,Weis J,Walter MC

更新日期：2010-07-01 00:00:00

abstract：BACKGROUND AND AIMS:Due to common pathophysiological findings of Alzheimer's disease (AD) with diabetes mellitus (DM), insulin has been suggested as a possible treatment of AD or mild cognitive impairment (MCI). A safe alternative of IV insulin is intranasal (IN) insulin. The aim of this systematic review is to investi...

journal_title：Journal of neurology

authors： Avgerinos KI,Kalaitzidis G,Malli A,Kalaitzoglou D,Myserlis PG,Lioutas VA

更新日期：2018-07-01 00:00:00

abstract：OBJECTIVE:We report a clinical description of pure sensory stroke based on data collected from a prospective acute stroke registry. METHODS:From 2500 acute stroke patients included in a hospital-based prospective stroke registry over a 12-year period, 99 were identified as having pure sensory stroke. RESULTS:Pure sen...

journal_title：Journal of neurology

authors： Arboix A,García-Plata C,García-Eroles L,Massons J,Comes E,Oliveres M,Targa C

更新日期：2005-02-01 00:00:00

abstract：:X-linked adrenoleukodystrophy (XALD), a neurological disorder caused by mutations in the peroxisomal membrane protein gene ABCD1, presents as a rapidly progressing, inflammatory cerebral demyelination (cerebral cases) or a slowly progressing, distal axonopathy (non-cerebral cases). Specific ABCD1 defects do not explai...

journal_title：Journal of neurology

authors： Brose RD,Avramopoulos D,Smith KD

更新日期：2012-07-01 00:00:00

abstract：:Epileptic syndromes are distinctive disorders with specific features, which when taken together, permit a specific diagnosis. There is actually a debate on that medial temporal lobe epilepsy with hippocampal sclerosis is an epileptic syndrome. To address this issue, we searched for discriminative semiological features...

journal_title：Journal of neurology

authors： No YJ,Zavanone C,Bielle F,Nguyen-Michel VH,Samson Y,Adam C,Navarro V,Dupont S

更新日期：2017-05-01 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：:Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy with heterogeneous clinical presentation and genetic background. The axonal form (CMT2) is characterised by decreased action potentials indicating primary axonal damage. The underlying pathology involves axonal degeneration which is supposed to ...

journal_title：Journal of neurology

authors： Bansagi B,Antoniadi T,Burton-Jones S,Murphy SM,McHugh J,Alexander M,Wells R,Davies J,Hilton-Jones D,Lochmüller H,Chinnery P,Horvath R

更新日期：2015-08-01 00:00:00

abstract：:During a period of 42 months, we studied the prevalence of epilepsy in a specific health district, composing by four towns with 98,405 inhabitants older than 10 years. This has been accomplished by a two-phase cross-sectional study. The prevalence rate observed was 4.12/1000 inhabitants for all types of epilepsy. No s...

journal_title：Journal of neurology

authors： Luengo A,Parra J,Colás J,Rámos F,Carreras T,Fernández-Pozos MJ,Muñoz A,Hernando V

更新日期：2001-09-01 00:00:00

abstract：:Non-motor symptoms (NMS) of Parkinson's disease (PD) have only recently been increasingly recognized for their impact on a patient's quality of life. In this study, we applied the validated, comprehensive self-completed NMS questionnaire for PD (NMS Quest) to 101 patients with young-onset PD (onset between 21 and 45 y...

journal_title：Journal of neurology

authors： Spica V,Pekmezović T,Svetel M,Kostić VS

更新日期：2013-01-01 00:00:00

abstract：:From a family with essential familial myoclonus, 150 members in eight generations were studied. Twenty-five of them suffered from myoclonus of varying severity. The findings in routine examinations of blood, urine and cerebrospinal fluid, EEG and skull radiographs were normal. Therapeutic trials did not produce lastin...

journal_title：Journal of neurology

authors： Przuntek H,Muhr H

更新日期：1983-01-01 00:00:00

abstract：OBJECTIVE:We aimed to assess the effectiveness of conservative treatments in children with tension-type headaches (TTH). METHOD:We searched Medline, Embase and the Cochrane Library and performed reference checking. Two independent authors assessed trial quality and extracted data. Analysis was carried out according to...

journal_title：Journal of neurology

authors： Verhagen AP,Damen L,Berger MY,Passchier J,Merlijn V,Koes BW

更新日期：2005-10-01 00:00:00

abstract：:Dementia has a reversible cause in some cases, and these should be diagnosed without over-investigating the many patients with irreversible disease. We prospectively studied the prevalence of reversible dementia in a memory clinic, determined the added value of investigations compared with clinical examination and ass...

journal_title：Journal of neurology

authors： Walstra GJ,Teunisse S,van Gool WA,van Crevel H

更新日期：1997-01-01 00:00:00

abstract：:Optic neuritis usually presents with rapid and gradual loss of vision that is either complete or incomplete, and typically associated with retro-orbital pain. To our knowledge there have been no documented reports of optic neuritis presenting with multiple episodes of amaurosis fugax, the sudden and transient loss of ...

journal_title：Journal of neurology

authors： Awad AM,Estephan B,Warnack W,Stüve O

更新日期：2009-12-01 00:00:00

abstract：:Bilateral paramedian thalamic infarctions are usually associated with impaired consciousness, oculomotor disturbances and neuropsychological changes. A 44-year-old healthy woman presented with amaurosis fugax of the right eye immediately after a Valsalva maneuver. Neurological examination, in particular visual acuity,...

journal_title：Journal of neurology

authors： Emond H,Landis T,Perren F

更新日期：2009-06-01 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：:We report a very rare case of Schwartz-Jampel syndrome associated with von Willebrand's disease. This association might be coincidental because of the different modes of inheritance of the two disorders. However, we speculate that there might be some link between the two disorders, for example in the locus of the affe...

journal_title：Journal of neurology

authors： Kuriyama M,Shinmyozu K,Osame M,Kawahira M,Igata A

更新日期：1985-01-01 00:00:00

abstract：:This meta-analysis aims to compare percutaneous transluminal angioplasty (PTA) to medical treatment (MT) for symptomatic vertebral artery stenosis (SVAS) treatment. We searched PubMed, Springer, Google Scholar, Clinical Trials, Cochrane Central, Chinese National Knowledge Infrastructure, and China Biological Medicine ...

journal_title：Journal of neurology

authors： Feng H,Xie Y,Mei B,Liu Y,Li B,Yin C,Wang T,Liu Y

更新日期：2017-05-01 00:00:00

abstract：:Clinical data and the serum and cerebrospinal fluid (CSF) findings of 71 patients with Guillain-Barré syndrome (GBS), 7 with Fisher syndrome and 24 with chronic inflammatory polyradiculoneuropathy (CIP), were analysed. Isoelectric focusing of serum and CSF together with different formulae and diagrams were applied to ...

journal_title：Journal of neurology

authors： Segurado OG,Krüger H,Mertens HG

更新日期：1986-08-01 00:00:00

abstract：:Dementia is reversible in a minority of patients, and these should be diagnosed but without subjecting the majority with irreversible disease to an excessive set of investigations. Should a battery of ancillary investigations be performed routinely in dementia? Or can these tests be carried out as clinically indicated...

journal_title：Journal of neurology

authors： van Crevel H,van Gool WA,Walstra GJ

更新日期：1999-02-01 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：:Twenty-nine cases of both clinically and neuropathologically diagnosed dementia with Lewy bodies (DLB) were retrospectively examined for autonomic symptoms. Twenty-eight cases showed some kind of autonomic dysfunction. Urinary incontinence (97 %) and constipation (83 %) were the two most common. Although urinary reten...

journal_title：Journal of neurology

authors： Horimoto Y,Matsumoto M,Akatsu H,Ikari H,Kojima K,Yamamoto T,Otsuka Y,Ojika K,Ueda R,Kosaka K

更新日期：2003-05-01 00:00:00

abstract：BACKGROUND:Cerebral venous thrombosis (CVT) is associated with intracranial hemorrhage. AIM:To identify clinical and imaging features of CVT-associated intracranial hemorrhage. We hypothesized that higher clot burden would be associated with a higher risk of intracranial hemorrhage. METHODS:We performed a retrospecti...

journal_title：Journal of neurology

authors： Afifi K,Bellanger G,Buyck PJ,Zuurbier SM,Esperon CG,Barboza MA,Costa P,Escudero I,Renard D,Lemmens R,Hinteregger N,Fazekas F,Conde JJ,Giralt-Steinhauer E,Hiltunen S,Arauz A,Pezzini A,Montaner J,Putaala J,Weimar C,

更新日期：2020-11-01 00:00:00

abstract：:Advances in molecular biology have resulted in novel therapy for neurofibromatosis 2-related (NF2) tumours, highlighting the need for robust outcome measures. The disease-focused NF2 impact on quality of life (NFTI-QOL) patient questionnaire was assessed as an outcome measure for treatment in a multi-centre study. NFT...

journal_title：Journal of neurology

authors： Ferner RE,Shaw A,Evans DG,McAleer D,Halliday D,Parry A,Raymond FL,Durie-Gair J,Hanemann CO,Hornigold R,Axon P,Golding JF

更新日期：2014-05-01 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：BACKGROUND:Myelin oligodendrocyte glycoprotein immunoglobulin G associated optic neuritis (MOG-ON) is a recently described entity. Recent studies have shown that MOG-ON has a more severe clinical presentation than classic optic neuritis (ON). OBJECTIVE:This study aimed to define morphological characteristics of MOG-ON...

journal_title：Journal of neurology

authors： Vicini R,Brügger D,Abegg M,Salmen A,Grabe HM

更新日期：2021-01-01 00:00:00

abstract：INTRODUCTION:Mounting evidence supports the existence of an important feedforward cycle between sleep and neurodegeneration, wherein neurodegenerative diseases cause sleep and circadian abnormalities, which in turn exacerbate and accelerate neurodegeneration. If so, sleep therapies bear important potential to slow prog...

journal_title：Journal of neurology

authors： Voysey Z,Fazal SV,Lazar AS,Barker RA

更新日期：2020-12-23 00:00:00

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：:Demographic and clinical features and data on medical history and prior environmental exposure collected during an epidemiological long-term study of multiple sclerosis (MS) were tested for their possible prognostic value. Fifty-two benign MS patients were compared with 29 patients having a malignant course. A primary...

journal_title：Journal of neurology

authors： Lauer K,Firnhaber W

更新日期：1992-02-01 00:00:00

abstract：:Different physiological mechanisms of facilitation of latencies and amplitudes of magnetic motor evoked potentials (MEPs) were evaluated in a cohort of 140 healthy volunteers. The potentials were induced at the vertex and recorded at the abductor pollicis brevis. The aim of the present investigation was to compare phy...

journal_title：Journal of neurology

authors： Hufnagel A,Jaeger M,Elger CE

更新日期：1990-11-01 00:00:00