Key emerging issues in progressive supranuclear palsy and corticobasal degeneration.

abstract：:Genetic evidence indicates a central role of cerebral accumulation of beta-amyloid (Abeta) in the pathogenesis of Alzheimer's disease (AD). Beside presenilin 1 and 2, three other recently discovered proteins (Aph 1, PEN 2 and nicastrin) are associated with gamma-secretase activity, the enzymatic complex generating Abe...

journal_title：Journal of neurology

authors： Sala Frigerio C,Piscopo P,Calabrese E,Crestini A,Malvezzi Campeggi L,Civita di Fava R,Fogliarino S,Albani D,Marcon G,Cherchi R,Piras R,Forloni G,Confaloni A

更新日期：2005-09-01 00:00:00

abstract：BACKGROUND:Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a rare early onset neurodegenerative disease that typically results in ataxia, upper motor neuron dysfunction and sensorimotor peripheral neuropathy. Dysarthria and dysphagia are anecdotally described as key features of ARSACS but the natu...

journal_title：Journal of neurology

authors： Vogel AP,Rommel N,Oettinger A,Stoll LH,Kraus EM,Gagnon C,Horger M,Krumm P,Timmann D,Storey E,Schöls L,Synofzik M

更新日期：2018-09-01 00:00:00

abstract：:The use of a dopamine agonist with a long duration of action has theoretical advantages in attempting to reduce the motor fluctuations in Parkinson's disease. We report the results of a double-blind controlled study of adding cabergoline, an ergot derivative with potent long-lasting high affinity for the D2 receptor, ...

journal_title：Journal of neurology

authors： Steiger MJ,El-Debas T,Anderson T,Findley LJ,Marsden CD

更新日期：1996-01-01 00:00:00

abstract：:The diagnosis and management of lesions of the brachial roots and of the brachial plexus is improved by appropriate investigation, both in acute and chronic disorders. The choice of investigation should be determined by the clinical problem. Since they are relatively non-invasive, electrophysiological investigations a...

journal_title：Journal of neurology

authors： Swash M

更新日期：1986-06-01 00:00:00

abstract：PURPOSE:Epileptogenic foci exhibit disturbed function at the level of the benzodiazepine receptor. The aim of our study was to investigate the incidence of focal reductions of temporal benzodiazepine receptor binding (BRB) as assessed by scintigraphy with 123I-iomazenil in patients with denovo temporal lobe epilepsy (T...

journal_title：Journal of neurology

authors： Matheja P,Lüdemann P,Kuwert T,Weckesser M,Kellinghaus C,Weitemeyer L,Diehl B,Schuierer G,Ringelstein EB,Schober O

更新日期：2001-07-01 00:00:00

abstract：:In refractory temporal lobe epilepsy (TLE) temporal lobe structures and functions are continuously or intermittently affected by abnormal brain electrical events, noxious neurochemical agents, and metabolic disturbances. There is conflicting evidence regarding the relationship between the duration of refractory mesial...

journal_title：Journal of neurology

authors： Jokeit H,Ebner A,Arnold S,Schüller M,Antke C,Huang Y,Steinmetz H,Seitz RJ,Witte OW

更新日期：1999-10-01 00:00:00

abstract：BACKGROUND AND PURPOSE:Mechanical thrombectomy (MT) improves early clinical outcome in patients with acute ischemic stroke but insights on determinants of long-term outcome after MT treatment are scarce. METHODS:Data from stroke patients with anterior circulation large vessel occlusion of a prospective MT registry (01...

journal_title：Journal of neurology

authors： Fuhrer H,Forner L,Pruellage P,Weber S,Beume LA,Schacht H,Egger K,Bardutzky J,Weiller C,Urbach H,Niesen WD,Meckel S

更新日期：2020-04-01 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

abstract：:In Huntington's disease (HD) atrophy of the caudate nucleus and putamen has been described many years before clinical manifestation. Volume changes of the pallidum, thalamus, brainstem, accumbens nucleus, hippocampus, and amygdala are less well investigated, or reported with contradicting results. The aim of our study...

journal_title：Journal of neurology

authors： van den Bogaard SJ,Dumas EM,Acharya TP,Johnson H,Langbehn DR,Scahill RI,Tabrizi SJ,van Buchem MA,van der Grond J,Roos RA,TRACK-HD Investigator Group.

更新日期：2011-03-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which was thought to be untreatable. However, recent evidence in both experimental animals and men indicates that antiglutamatergic strategies are the first to have an influence on its pathogenesis and slow down the disease process. Since the eff...

journal_title：Journal of neurology

authors： Ludolph AC

更新日期：2000-12-01 00:00:00

abstract：BACKGROUND:Classifying and diagnosing peripheral vestibular disorders based on their symptoms is challenging due to possible symptom overlap or atypical clinical presentation. To improve the diagnostic trajectory, gadolinium-based contrast-enhanced magnetic resonance imaging of the inner ear is nowadays frequently used...

journal_title：Journal of neurology

authors： van der Lubbe MFJA,Vaidyanathan A,Van Rompaey V,Postma AA,Bruintjes TD,Kimenai DM,Lambin P,van Hoof M,van de Berg R

更新日期：2020-12-01 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: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：:Aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorder (NMOSD) is a rare but often severe autoimmune disease with median onset around 40 years of age. We report characteristics of three very-late-onset NMOSD (including complete NMO) patients >75 years of age, in whom this diagnosis initially se...

journal_title：Journal of neurology

authors： Krumbholz M,Hofstadt-van Oy U,Angstwurm K,Kleiter I,Jarius S,Paul F,Aktas O,Buchholz G,Kern P,Straube A,Kümpfel T

更新日期：2015-05-01 00:00:00

abstract：:We report the familial occurrence and apparent autosomal dominant inheritance of Sneddon's syndrome with variable clinical expression. The proband, a 40-year-old woman, presented with livedo reticularis and progressive neurological deterioration following a stroke. The diagnosis was confirmed by cerebral angiogram and...

journal_title：Journal of neurology

authors： Lossos A,Ben-Hur T,Ben-Nariah Z,Enk C,Gomori M,Soffer D

更新日期：1995-02-01 00:00:00

abstract：:Applying microarray technology to identify new diagnostic and prognostic markers in peripheral blood cells (PBC) after therapeutic intervention opens great perspectives regarding patient subclassification. Three recombinant products of the pleiotropic agent interferon beta (rIFN-beta) are available for disease modifyi...

journal_title：Journal of neurology

authors： Goertsches RH,Hecker M,Zettl UK

更新日期：2008-12-01 00:00:00

abstract：:Left-handedness is most often genetic, but may also follow early, localised damage to the developing brain. This so-called "pathological" left-handedness syndrome is often associated with right hemisphere speech dominance. To find out whether verbal laterality or handedness were affected by congenital, intracranial ar...

journal_title：Journal of neurology

authors： Wester K,Hugdahl K

更新日期：2003-01-01 00:00:00

abstract：:In order to learn more on the occurrence of pains and motor deficit in severe diabetic polyneuropathy we reviewed the data of a series of 30 diabetic patients with an uncommonly severe length-dependent diabetic polyneuropathy (LDDP). Extensive sensory loss predominated with pains and temperature sensations and affecte...

journal_title：Journal of neurology

authors： Said G,Baudoin D,Toyooka K

更新日期：2008-11-01 00:00:00

abstract：:Multiple Sclerosis (MS) is an inflammatory disease of the Central Nervous System with multifocal areas of demyelination. Although its etiology and pathogenesis remain controversial, several lines of evidence indicate that MS is mediated by a misdirected immune response against one or several myelin proteins. The invol...

journal_title：Journal of neurology

authors： Correale J,de los Milagros Bassani Molinas M

更新日期：2002-04-01 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：: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：BACKGROUND:Conflicting findings have been reported on the prognostic significance of serum 25-hydroxyvitamin D level in patients with stroke. The objective of this meta-analysis was to evaluate the prognostic utility of serum 25-hydroxyvitamin D in stroke patients. METHODS:PubMed and Embase databases were systematical...

journal_title：Journal of neurology

authors： Liu H,Wang J,Xu Z

更新日期：2020-11-01 00:00:00

abstract：:This paper summarizes the methods we devised for the treatment of psychosis, orthostatic hypotension, and mood disorders among the various non-motor complications of Parkinson's disease. Psychosis may not manifest when a patient believes in his/her delusions. If left untreated over a prolonged period, however, the del...

journal_title：Journal of neurology

authors： Fujimoto K

更新日期：2009-08-01 00:00:00

abstract：BACKGROUND:The significance of neutrophil-to-lymphocyte ratio (NLR) has been explored in different diseases. Few studies addressed its role in patients with multiple sclerosis (MS), with promising results regarding its association with disease activity or disability. OBJECTIVES:We aimed at confirming the role of NLR a...

journal_title：Journal of neurology

authors： Gelibter S,Pisa M,Croese T,Dalla Costa G,Orrico M,Preziosa P,Sangalli F,Martinelli V,Furlan R,Filippi M

更新日期：2021-01-03 00:00:00

abstract：:Somatostatin levels were measured in cerebrospinal fluid of patients with Alzheimer's disease, multi-infarct dementia and normal pressure hydrocephalus and compared with levels from a normal control group. All pathological groups showed a statistically significant decrease of somatostatin with respect to the control g...

journal_title：Journal of neurology

authors： Molins A,Catalán R,Sahuquillo J,Castellanos JM,Codina A,Galard R

更新日期：1991-06-01 00:00:00

abstract：OBJECTIVE:We characterised the clinical and neuro-otological characteristics of patients with Susac syndrome. METHODS:The medical records of 30 patients with Susac syndrome were reviewed for details of their clinical presentation and course, neuro-otological symptoms, investigation results including audiology and vest...

journal_title：Journal of neurology

authors： Hardy TA,Taylor RL,Qiu J,O'Brien B,Gopinath S,Trewin B,Spring PJ,Shaffi M,Bolitho SJ,Garsia RJ,Roxburgh R,Mason DF,Ip J,Chan F,Chen L,Wilson I,Beadnall HN,Barnett MH,Parratt JDE,Watson JDG,Welgampola MS,Reddel S

更新日期：2020-12-01 00:00:00

abstract：:The spinocerebellar ataxias (SCAs) with autosomal dominant inheritance are a clinically and genetically heterogeneous group of neurological disorders with overlapping as well as highly variable phenotypes primarily affecting the cerebellum. To date, 28 different loci have been identified. Nine SCAs are caused by repea...

journal_title：Journal of neurology

authors： Edener U,Kurth I,Meiner A,Hoffmann F,Hübner CA,Bernard V,Gillessen-Kaesbach G,Zühlke C

更新日期：2009-11-01 00:00:00

abstract：:We conducted an open-labeled clinical trial of interferon beta-1b (IFNB) treatment in 20 patients with primary progressive multiple sclerosis (PPMS) and longitudinally monitored autoantibodies against double-stranded DNA (dsDNA), thyroid peroxidase (TPO),myelin basic protein (MBP), myelin oligodendrocyte glycoprotein ...

journal_title：Journal of neurology

authors： Bitsch A,Dressel A,Meier K,Bogumil T,Deisenhammer F,Tumani H,Kitze B,Poser S,Weber F

更新日期：2004-12-01 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：:A haplotype marker consisting of three biallelic restriction fragment length polymorphism (RFLP) loci from the VH-2 variable gene family was examined in 124 families with sibling pairs concordant for multiple sclerosis, 178 unrelated patients and 159 unaffected controls to investigate the role of the immunoglobulin he...

journal_title：Journal of neurology

authors： Wood NW,Sawcer SJ,Kellar-Wood HF,Holmans P,Clayton D,Robertson N,Compston DA

更新日期：1995-10-01 00:00:00