Clinical characteristics and outcomes between children and adults with anti-N-Methyl-D-Aspartate receptor encephalitis.

abstract：:Disability in multiple sclerosis (MS) patients is associated with white matter (WM) and gray matter (GM) pathology, and both processes contribute differently over the disease course. Total and regional GM volume loss can be imaged via voxel-based morphometry (VBM). Here, we retrospectively analyzed a group of 213 MS p...

journal_title：Journal of neurology

authors： Grothe M,Lotze M,Langner S,Dressel A

更新日期：2016-06-01 00:00:00

abstract：:Bcl2-associated athanogene 3 (BAG3) gene mutations cause dilated cardiomyopathy and myofibrillar myopathy. Recently, a novel c.625C>T (p.Pro209Ser) mutation in BAG3 was reported to cause axonal Charcot-Marie-Tooth (CMT) disease in three families. Here, we describe two patients with adult-onset and moderate CMT in a Ch...

journal_title：Journal of neurology

authors： Fu J,Ma M,Song J,Pang M,Li G,Zhang J

更新日期：2020-04-01 00:00:00

abstract：OBJECTIVES:There are varying reports on whether monoclonal gammopathy of undetermined significance-associated neuropathy (MGUSN) patients are distinguishable from those with chronic inflammatory demyelinating polyneuropathy (CIDP) and whether specific MGUSN subclasses are associated with specific clinical phenotypes. ...

journal_title：Journal of neurology

authors： Alkhawajah NM,Dunnigan SK,Bril V

更新日期：2014-08-01 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：BACKGROUND:More research is needed to understand the contribution of comorbidities to MS symptomatology. OBJECTIVES:To examine the dose-response relationship between the number of comorbidities and severity of MS symptoms and to assess the relative contribution of comorbidity groups and individual comorbidities to the...

journal_title：Journal of neurology

authors： Lo LMP,Taylor BV,Winzenberg T,Palmer AJ,Blizzard L,van der Mei I

更新日期：2020-09-02 00:00:00

abstract：BACKGROUND:Nusinersen recently became available as the first treatment for Spinal Muscular Atrophy (SMA) and data on its effectiveness and safety in adult SMA patients are still scarce. METHODS:We evaluated the effectiveness and safety of nusinersen treatment during 14 months in 16 adult patients with SMA types 3 and ...

journal_title：Journal of neurology

authors： De Wel B,Goosens V,Sobota A,Van Camp E,Geukens E,Van Kerschaver G,Jagut M,Claes K,Claeys KG

更新日期：2020-09-15 00:00:00

abstract：:Cerebello-thalamo-cortical circuit has been indicated important for tremor in Parkinson's disease (PD), but the role of dentate nucleus (DN) in parkinsonian tremor remains unclear. To investigate whether DN plays a role in PD tremor, we recruited 50 PD and 29 age-matched health controls (HC). The patients were divided...

journal_title：Journal of neurology

authors： Ma H,Chen H,Fang J,Gao L,Ma L,Wu T,Hou Y,Zhang J,Feng T

更新日期：2015-10-01 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：:In 40 rabbits cerebral ischaemia was induced by autologous blood clot emboli injected into the middle cerebral artery. Autologous blood clot formed spontaneously (within 2 h) in a catheter fixed in the internal carotid artery. The effects of embolization were investigated on arterial blood pressure, respiration, local...

journal_title：Journal of neurology

authors： Fekete I,Hegedüs K,Molnár L

更新日期：1988-05-01 00:00:00

abstract：:Acute mountain sickness (AMS) can occur during climbs to high altitudes and may seriously disturb the behavioral and intellectual capacities of susceptible subjects. During a Himalayan expedition 32 mountaineers were examined with electroencephalography (EEG) and transcranial doppler sonography (TCD) to assess relativ...

journal_title：Journal of neurology

authors： Feddersen B,Ausserer H,Neupane P,Thanbichler F,Depaulis A,Waanders R,Noachtar S

更新日期：2007-03-01 00:00:00

abstract：:Recessive mutations in GJC2, the gene-encoding connexin 47 (Cx47), cause Pelizaeus-Merzbacher-like disease type 1, a severe dysmyelinating disorder. One recessive mutation (p.Ile33Met) has been associated with a much milder phenotype--hereditary spastic paraplegia type 44. Here, we present evidence that a novel Arg98L...

journal_title：Journal of neurology

authors： Abrams CK,Scherer SS,Flores-Obando R,Freidin MM,Wong S,Lamantea E,Farina L,Scaioli V,Pareyson D,Salsano E

更新日期：2014-10-01 00:00:00

abstract：:Two siblings with multiple intracranial haemangiomatosis are reported, each having psychomotor retardation, epilepsy and gyriform type calcification in the occipital and frontal lobes. There were haemangiomas in one autopsy case with calcification in the deep layers of the cortex and subcortical white matter. ...

journal_title：Journal of neurology

authors： Tateno A,Matsui A,Sakuragawa N,Nonaka I,Arima M

更新日期：1985-01-01 00:00:00

abstract：:Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are often clinically confused with each other. Moreover, the discrepancy between clinical and pathological diagnoses of CBD and PSP are still controversial. We report here two atypical cases of PSP and CBD. A 73-year old woman was admitted with r...

journal_title：Journal of neurology

authors： Mizuno T,Shiga K,Nakata Y,Nagura J,Nakase T,Ueda Y,Takanashi Y,Urasaki K,Oyamada Y,Fushiki S,Nishikawa J,Yasuhara M,Nakajima K,Nakagawa M

更新日期：2005-06-01 00:00:00

abstract：:In multiple sclerosis (MS), randomly located demyelination lesions may involve a large part of the central nervous system and disturb oculomotor activity, including impairment of saccadic and pursuit systems. The aim of the work was to determine the frequency of smooth pursuit disturbances in MS patients and assess th...

journal_title：Journal of neurology

authors： Jozefowicz-Korczynska M,Pajor AM

更新日期：2011-10-01 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：: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：:In contrast to other neurodegenerative diseases, sporadic Creutzfeldt-Jakob disease (sCJD) is rarely diagnosed in patients older than 75 years. Data describing the characteristics of sCJD in the very old are rare and inconclusive. Therefore, a historical cohort study was designed to evaluate clinical, cerebrospinal fl...

journal_title：Journal of neurology

authors： Karch A,Raddatz LM,Ponto C,Hermann P,Summers D,Zerr I

更新日期：2014-05-01 00:00:00

abstract：:The objective of this study was to investigate cognitive dysfunction in 24-60-year-old neuromyelitis optica (NMO) patients, demographically matched healthy subjects, and MS patients. We conducted a comprehensive literature review of the PubMed, Medline, EMBASE, CNKI, Wan Fang Date, Web of Science, and Cochrane Library...

journal_title：Journal of neurology

authors： Meng H,Xu J,Pan C,Cheng J,Hu Y,Hong Y,Shen Y,Dai H

更新日期：2017-08-01 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：:A man, aged 46, who had been taking Clioquinol in high doses for a long period, developed a characteristic neurological syndrome of subacute myelo-optic neuropathy rather abruptly. Electron microscopical examination of the muscle biopsy, obtained five months after the onset of the disease, revealed severe degenerative...

journal_title：Journal of neurology

authors： Otte G,de Coster W,Thiery E,de Reuck J,vander Eecken H

更新日期：1977-05-13 00:00:00

abstract：:Atrial fibrillation (AF) is considered a predictor for severe stroke and poor outcome. The aim was to evaluate whether AF is associated with poor outcome in acute ischemic stroke (IS) patients treated with intravenous thrombolysis (IVT). In a retrospective study, 157 consecutive IS patients (98 males, mean age 67.3 +/...

journal_title：Journal of neurology

authors： Sanák D,Herzig R,Král M,Bártková A,Zapletalová J,Hutyra M,Skoloudík D,Vlachová I,Veverka T,Horák D,Kanovský P

更新日期：2010-06-01 00:00:00

abstract：:Mutations in the SPG3A gene cause a form of pure, early-onset autosomal dominant hereditary spastic paraplegia linked to chromosome 14q. The encoded protein, atlastin, is a putative member of the dynamin superfamily of large GTPases involved in cellular trafficking patterns. We report a new atlastin mutation causing s...

journal_title：Journal of neurology

authors： Scarano V,Mancini P,Criscuolo C,De Michele G,Rinaldi C,Tucci T,Tessa A,Santorelli FM,Perretti A,Santoro L,Filla A

更新日期：2005-08-01 00:00:00

abstract：:Motor neuron disease (MND) is a group of disorders in which there is degeneration of upper and lower motor neurons to a variable degree. Amyotrophic lateral sclerosis is the most frequent form of the disease, presenting with both upper and lower motor neuron involvement. Frequently, especially in the early stages of t...

journal_title：Journal of neurology

authors： Bouche P,Le Forestier N,Maisonobe T,Fournier E,Willer JC

更新日期：1999-07-01 00:00:00

abstract：:Horizontal eye-movement responses of four patients with Alzheimer-type dementia were recorded using the infrared oculographic technique. Abnormally frequent saccadic intrusions occurred during the fixation and slow, smooth pursuit tasks in all four patients. Saccadic intrusions have previously been reported in the lit...

journal_title：Journal of neurology

authors： Jones A,Friedland RP,Koss B,Stark L,Thompkins-Ober BA

更新日期：1983-01-01 00:00:00

abstract：OBJECTIVE:Crossed aphasia (CA), usually referred to as an acquired language disturbance, is caused by a lesion in the cerebral hemisphere ipsilateral to the dominant hand, and the exact mechanism is not clear. The development of handedness is influenced by education and training and the impact of habitualization, while...

journal_title：Journal of neurology

authors： Tan X,Guo Y,Dun S,Sun H

更新日期：2018-07-01 00:00:00

abstract：:Clinical diagnosis of amyotrophic lateral sclerosis (ALS) in patients presenting with cramps and fasciculations may not be evident at the first consultation. Sequential reviews, clinical and neurophysiological, form an important part of clinical practice in such cases. Recent attempts to delineate a more benign group ...

journal_title：Journal of neurology

authors： Singh V,Gibson J,McLean B,Boggild M,Silver N,White R

更新日期：2011-04-01 00:00:00

abstract：:The pathophysiology of the striatum and cerebral cortex were studied from the pharmacological aspect. Investigation of the dopamine content in the cerebral cortex revealed that the premotor and motor area showed the highest level (61+/-6.2 ng/g). Intravenous injection of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (M...

journal_title：Journal of neurology

authors： Nomoto M,Kaseda S,Iwata S,Shimizu T,Fukuda T,Nakagawa S

更新日期：2000-09-01 00:00:00

abstract：:Cerebellar syndrome is one of the most disabling developments in multiple sclerosis (MS). In neurodegenerative disorders, cerebellar syndrome is thought to be related to a neurochemical deficit of 5-hydroxytryptamine (5-HT). Previous studies found that a levorotatory form of 5-hydroxytryptophan, a 5-HT precursor, and ...

journal_title：Journal of neurology

authors： Monaca-Charley C,Stojkovic T,Duhamel A,De Seze J,Ferriby D,Vermersch P

更新日期：2003-10-01 00:00:00

abstract：:Cerebral amyloid angiopathy (CAA) was found in 57% of 123 autopsy brains removed from patients aged 59-101 years. The incidence of CAA increased with age. CAA was seen most frequently in the occipital cortex. Immunohistochemically, amyloid of CAA was positive for amyloid P component and negative for human AA protein a...

journal_title：Journal of neurology

authors： Yamada M,Tsukagoshi H,Otomo E,Hayakawa M

更新日期：1987-08-01 00:00:00

abstract：:Two siblings with Lafora disease (LD) are described: one with epilepsy, myoclonus, EEG abnormalities, severe dementia and many Lafora bodies (LBs) in muscle and skin tissue; the other with myoclonus, epilepsy, EEG abnormalities and LBs in muscle and in skin tissue, without dementia. The findings suggest that the diagn...

journal_title：Journal of neurology

authors： Iannaccone S,Zucconi M,Quattrini A,Nemni R,Comola M,Taccagni L,Smirne S

更新日期：1991-07-01 00:00:00