Abstract:
:Thirty-seven Japanese autopsy cases with diffuse Lewy body disease (DLBD) were reviewed from a clinicopathological viewpoint. Based on the neuropathological finding of whether or not many concomitant senile plaques (SPs) and/or neurofibrillary tangles (NFTs) are present. DLBD is divided into two forms: a common form and a pure form. In the common form not only numerous Lewy bodies but also many SPs and/or NFTs are found in the cerebral cortex, whereas in the pure form there are no or few senile changes. Of the 37 cases, 28 cases had the common form, and 9 had the pure form of DLBD. In the common form all cases had shown progressive cortical dementia in the presenile or senile period. About 60% of the cases began with memory disturbance, while 25% showed Parkinson's or Shy-Drager syndrome initially. Parkinson's syndrome, consisting mainly of muscular rigidity and akinesia, was usually marked in the later stage, although there were also 8 cases (28.6%) in which no parkinsonian symptoms were detected even in the terminal stage. On the other hand, almost all cases with the pure form of DLBD showed juvenile Parkinson's syndrome, followed by progressive cortical dementia, although there was one presenile case with mild dementia and Parkinson's syndrome. These Japanese cases are compared with cases reported in Western countries.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Kosaka Kdoi
10.1007/BF00314594subject
Has Abstractpub_date
1990-06-01 00:00:00pages
197-204issue
3eissn
0340-5354issn
1432-1459journal_volume
237pub_type
杂志文章,评审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
pub_type: 杂志文章
doi:10.1007/s00415-008-0847-1
更新日期:2008-07-01 00:00:00
abstract::To determine the epidemiological, clinical, and genetic characteristics of congenital hypomyelinating leukodystrophies, including Pelizaeus-Merzbacher disease (PMD), we conducted a nationwide epidemiological survey in Japan. A two-step survey targeting all medical institutions specializing in pediatric neurology and c...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7263-5
更新日期:2014-04-01 00:00:00
abstract::Neurological manifestations in pandemics frequently cause short and long-term consequences which are frequently overlooked. Despite advances in the treatment of infectious diseases, nervous system involvement remains a challenge, with limited treatments often available. The under-recognition of neurological manifestat...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-10261-3
更新日期:2020-10-26 00:00:00
abstract::Hemichorea-hemiballism (HCHB) was infrequently related to cortical lesions such as tumor or infarction. Although functional derangement of the basal ganglia (BG) or the thalamus (Th) was suggested, pathomechanism of HCHB secondary to cortical lesions remains uncertain. We recruited the patients with HCHB secondary to ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7096-7
更新日期:2013-12-01 00:00:00
abstract:BACKGROUND:Recent research has convincingly shown that the ability to work mainly depends on the cognitive status in multiple sclerosis (MS). An international committee of experts recommended a brief neuropsychological battery to evaluate cognitive performance in MS. BICAMS comprises three tests, the Symbol Digit Modal...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-9034-1
更新日期:2018-11-01 00:00:00
abstract:BACKGROUND:One of the goals in treating subarachnoid hemorrhage patients is to prevent or minimize vasospasm-induced ischemia. Intracerebral microdialysis is a rapidly developing tool to monitor physiological and pathophysiological changes in chemical processes associated with ischemia. OBJECTIVE:To determine the diag...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-003-1079-z
更新日期:2003-07-01 00:00:00
abstract::Cerebral palsy (CP) continues to be a major problem in India. The present study provides an insight into the various clinical and neuroradiological correlates of CP. The study included 102 children with CP and was subjected to magnetic resonance imaging (MRI) of the brain. Forty-seven (46%) patients belonged to the 1-...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5782-2
更新日期:2011-03-01 00:00:00
abstract::The frequency of malignant diseases among 1866 living and 340 deceased multiple sclerosis (MS) patients was investigated in Finland. The study revealed a low prevalence (0.64%) and mortality (0.07%) rate of cancer among MS patients. The difference between MS patients and general population was significant. The highest...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313623
更新日期:1977-10-07 00:00:00
abstract::Magnetisation transfer (MT) imaging provides indirect information on tissue structure abnormalities in areas that otherwise may appear normal on conventional MRI. We determined the evolution of MT changes in normal appearing white matter (NAWM) and lesion on serial examination of 9 multiple sclerosis (MS) patients and...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-003-1115-z
更新日期:2003-08-01 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
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-008-0790-1
更新日期:2008-06-01 00:00:00
abstract:: ...
journal_title:Journal of neurology
pub_type: 信件
doi:10.1007/s00415-018-9103-5
更新日期:2019-01-01 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
pub_type: 杂志文章
doi:10.1007/s00415-014-7429-1
更新日期:2014-10-01 00:00:00
abstract::A boy was suspected of having meningoencephalitis probably of viral origin, although all microbiological, including virological, tests were negative. Postmortem examination revealed a dissecting aortic aneurysm and disseminated intravascular coagulation involving the central nervous system exclusively. The clinical ev...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313465
更新日期:1981-01-01 00:00:00
abstract::The history of our understanding of the pathogenesis and pathophysiology of multiple sclerosis are reviewed in the context of Charcot's contribution. The implications for treatment of the new knowledge gained from studies during life of pathology and pathogenesis (by MRI) and pathophysiology (by evoked potentials) are...
journal_title:Journal of neurology
pub_type: 传,历史文章,杂志文章
doi:10.1007/BF00838443
更新日期:1993-01-01 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
pub_type: 杂志文章
doi:10.1007/s004150050484
更新日期:1999-10-01 00:00:00
abstract::Little is known about the relationship between cognitive dysfunctions and the non-motor complex in subjects with newly diagnosed untreated Parkinson's disease (PD). The aim of this study was to explore the association between non-motor symptoms (NMS) and cognitive dysfunctions in an incident cohort of de novo, drug-na...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6407-0
更新日期:2012-09-01 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
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-006-0400-z
更新日期:2007-04-01 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
pub_type: 杂志文章,评审
doi:10.1007/BF03161074
更新日期:2000-12-01 00:00:00
abstract::Treatment of multiple sclerosis (MS) with interferon beta (IFNbeta) can be associated with the development of binding antibodies (BAbs) and neutralizing antibodies (NAbs). NAbs are a subset of BAbs that prevent IFNbeta from effectively binding to or activating its receptor, thereby blocking its biologic effects and in...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-004-1206-5
更新日期:2004-06-01 00:00:00
abstract:AIM:To investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) spectrum of diseases. METHODS:Comprehensive assessment was taken on 21 patients with PLS and results were compared to patients diagnosed with pure motor ALS (n = 27) and ALS...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8917-5
更新日期:2018-08-01 00:00:00
abstract::A man, aged 63, had an illness which lasted 11 months from onset with pain under the left costal margin which radiated to the epigastrium, until his death from cardiac failure. His symptoms consisted principally of parasthesias and proximal weakness of both upper and lower extremities with atrophy of the shoulder and ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314720
更新日期:1978-04-14 00:00:00
abstract::Brains from 54 patients with organic dementia were examined systematically. As in previous investigations a predominance of Alzheimer type changes was observed. Seventeen patients showed Lewy bodies in the nucleus basalis, the substantia nigra and the locus coeruleus as well as other lesions of parkinsonian type. In 5...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313858
更新日期:1986-04-01 00:00:00
abstract::Frequent serial EEG investigations of three patients with neuropathologically confirmed Creutzfeldt-Jakob disease lasting 13, 24 and 68 weeks revealed typical periodic activity of short duration with stereotyped bilateral sharp waves at the 7th, 8th, and 12th week, respectively, after the onset of symptoms. During the...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00328506
更新日期:1989-12-01 00:00:00
abstract:OBJECTIVES:The mechanisms of early neurologic deterioration (END) and prevention strategies for END are not completely understood. The aim of this study was to investigate the association between CYP2C19*2 variants and END, and the effectiveness of antiplatelet therapy for prevention of END according to CYP2C19*2 genot...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-018-8998-1
更新日期:2018-10-01 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
pub_type: 杂志文章
doi:10.1007/s00415-008-0737-6
更新日期:2008-04-01 00:00:00
abstract::Leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate (LBSL) is a rare, autosomal recessive disorder caused by mutations in the gene encoding a mitochondrial aspartyl-tRNA synthetase, DARS2. The disease is characterized by progressive spastic ataxia and magnetic resonance imaging (MRI) sh...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6176-9
更新日期:2012-02-01 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
pub_type: 杂志文章
doi:10.1007/s00415-006-0310-0
更新日期:2007-06-01 00:00:00
abstract::Early identification of cardiogenic vertigo (CV) is necessary to prevent serious complications of cardiovascular diseases. However, the literature is limited to case reports without detailed clinical features or diagnostic criteria. The aim of this study was to define characteristics of CV and propose diagnostic crite...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10252-4
更新日期:2020-10-06 00:00:00
abstract::Although the beneficial effect of subcutaneous injections of botulinum toxin type A (BTX-A) is well known in both blepharospasm and hemifacial spasm, the position of the injection sites around the orbicularis oculi may influence the effectiveness and side effects. Here we report results of preseptal and pretarsal BTX-...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/pl00007849
更新日期:2002-01-01 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
pub_type: 杂志文章
doi:10.1007/s00415-014-7357-0
更新日期:2014-08-01 00:00:00