Abstract：
:Two siblings who exhibited hereditary parkinsonism with pyramidal signs and cerebellar ataxia are reported. Anticholinergics had a dramatic beneficial effect in both cases, but levodopa did not. This responsiveness, which is similar to that reported in patients with Joseph's disease, suggests dysfunction of an "indirect pathway" involving the globus pallidus and the subthalamic nucleus, in addition to that of the nigrostriatal system. We propose a new hereditary variant of early onset Parkinson's disease distinct from the levodopa sensitive forms of juvenile Parkinson's disease.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Mitsui T,Kawai H,Sakoda S,Miyata M,Saito Sdoi
10.1016/0022-510x(94)90028-0subject
Has Abstractpub_date
1994-09-01 00:00:00pages
153-7issue
2eissn
0022-510Xissn
1878-5883pii
0022-510X(94)90028-0journal_volume
125pub_type
杂志文章abstract：:Amyotrophic lateral sclerosis (ALS) mainly affects the motor neurons but may also include other organs such as the skin. We aimed to determine whether matrix metalloproteinases could provide a link between neuronal degeneration and skin alterations in ALS. We measured CSF, serum and skin tissue MMP-2 and MMP-9 using E...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.05.025
更新日期：2009-10-15 00:00:00
abstract：:Auditory vocal hallucinations are sometimes observed in temporal-lobe epilepsy, but are a frequent sign of psychosis and may rarely be mistaken for the latter. Here we report two patients who suffered from auditory vocal hallucinations, described as unintelligible human voices perceived at their left side during epile...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.03.029
更新日期：2011-06-15 00:00:00
abstract：OBJECTIVE:To study the impact of Guillain-Barré Syndrome (GBS) on the psychosocial functioning of the closest relative and on family functioning during the first year after GBS. METHOD:At 1 (=T1), 3 (=T3), 6 (=T6), and 12 months (=T12) after the onset of GBS, relatives of patients received the General Health Questionn...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.01.002
更新日期：2006-05-15 00:00:00
abstract：:Five patients with acute polyneuritis following treatment with rabies vaccine prepared in suckling mouse brains are reported. These patients had severe and protracted disease. The extensive cranial nerve involvement, high mortality rate, and disabling longterm sequelae were in contrast to cases of the Guillain-Barré s...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90099-2
更新日期：1987-11-01 00:00:00
abstract：BACKGROUND:Hypertensive (HT) patients are at higher risk of cognitive decline than normotensive individuals, because high blood pressure is a risk factor for mild cognitive deterioration. In this study cardio- and cerebrovascular reactivity along with cognitive performance was assessed on newly diagnosed HT patients. ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.07.022
更新日期：2010-12-15 00:00:00
abstract：:The aim of the study was to assess risk factors for vascular dementia (VaD) in elderly psychiatric outpatients without dementia, and to determine to what extent clinical interventions targeted such risk factors. Out of 250 clinical charts, 78 were selected of patients over 60 years old, who showed no signs of dementia...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2007.01.035
更新日期：2007-06-15 00:00:00
abstract：INTRODUCTION:Intracerebral hemorrhage (ICH) associated with pregnancy commonly occurs in the postpartum period in the setting of preeclampsia/eclampsia. We describe the clinical course of two patients with ICH due to postpartum cerebral vasculopathy in the absence of toxemia. METHODS:We reviewed two cases with ICH and...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(02)00245-9
更新日期：2002-12-15 00:00:00
abstract：:Subacute sclerosing panencephalitis (SSPE) is a rare infectious central nervous system disease with a poor prognosis. Nineteen patients, 18 males and one female, ranging in age from 18 to 22, mean 19.6+/-1.5 years with SSPE were evaluated. We treated 9 patients with oral isoprinosine and 10 patients with alpha-interfe...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/j.jns.2008.07.033
更新日期：2008-12-15 00:00:00
abstract：BACKGROUND AND AIMS:Both vascular disease and diabetes type 2 (DM2) decrease cognitive functioning in elderly people. It is uncertain if DM2 affects cognition independent of vascular disease. In patients with symptomatic arterial disease, we studied the effect of DM2 on cognition and identified clinical and radiologica...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.02.337
更新日期：2009-08-15 00:00:00
abstract：:With an Italian case series of 81 Italian patients and 130 controls, we analysed associations between myasthenia gravis (MG) and genetic polymorphisms in the MHC class II/III region. Increases in the frequency of the TNF-B*1, C4A*Q0, C4B*1, DRB1*03 supratype, which is likely part of the 8.1 ancestral haplotype, were m...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(01)00573-1
更新日期：2001-09-15 00:00:00
abstract：OBJECTIVE:To determine the sensitivity and specificity of clonal immunoglobulin heavy chain gene rearrangement (IGHR) analysis in the distinction of benign and malignant lymphoproliferative diseases. METHODS:A retrospective analysis was conducted of patients in whom a malignant lymphoproliferative process was suspecte...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.05.044
更新日期：2006-09-25 00:00:00
abstract：:Proinflammatory cytokines contribute to the regulation of the disease process in inflammatory neuropathies. Cellular localisation of cytokine expression in CIDP nerve biopsies should provide further insight into the pathogenic mechanisms of the disease and the individual cells involved. In this study in situ hybridisa...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(99)00009-x
更新日期：1999-02-01 00:00:00
abstract：:Superficial siderosis (SS) of the central nervous system is a rare disorder caused by chronic or recurrent hemorrhages into the subarachnoid space with hemosiderin and ferritin deposition, which leads to neuronal damage. The source of bleeding remains unknown in 50% of cases. Recently, attention has been focused on fl...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.07.034
更新日期：2012-01-15 00:00:00
abstract：:We herein report a 12-year-old girl with a basal ganglia germinoma who presented with right-sided hemiparesis after a minor head trauma. Magnetic resonance (MR) imaging revealed a minimally enhanced lesion involving the left putamen, thalamus, and corona radiata. The lesion showed low-signal intensity on T1-, and high...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2008.02.006
更新日期：2008-07-15 00:00:00
abstract：:Alzheimer's disease (AD) is a complex disorder and the most common form of neurodegenerative dementia. Several genetic, environmental, and physiological factors, including inflammations and metabolic influences, are involved in the progression of AD. Inflammations are composed of complicated networks of many chemokine...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2017.03.031
更新日期：2017-05-15 00:00:00
abstract：:We have determined various biomarkers in the peripheral blood of Alzheimer, Parkinson and vascular dementia patients by comparing the samples with those of first-degree relatives and control subjects. Our results, together with correlation studies using data from the Mini-Mental State Examination (MMSE), suggest that ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.11.001
更新日期：2012-03-15 00:00:00
abstract：:The reduction in the effectiveness of the blood-brain and blood-spinal cord barriers, previously seen in rats at the height of the acute episode of experimental allergic encephalomyelitis, has now been measured at various stages in the development of the disease up to 60 days after inoculation with guinea pig spinal c...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(83)90147-8
更新日期：1983-08-01 00:00:00
abstract：:Cerebral gliomas may cause a reduction of glucose metabolism in the cerebellum contralateral to the tumor side (crossed cerebellar diaschisis, CCD). We investigated whether CCD is related to tumor localization, histological grade, size and tumor biochemistry. Cerebellar glucose metabolism was measured in 44 glioma pat...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00019-7
更新日期：1998-01-01 00:00:00
abstract：BACKGROUND:There is little information about intracranial atherosclerotic disease (ICAD) and extracranial atherosclerotic disease (ECAD) in patients with acute spontaneous intracerebral hemorrhage (ICH). The purpose of the present study was to elucidate the prevalence of and the factors that correlate with ICAD and ECA...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.06.031
更新日期：2013-09-15 00:00:00
abstract：BACKGROUND:A specific non-motor impairment in Parkinson's disease (PD) concerns difficulties to accurately identify facial emotions. Findings are numerous but very inconsistent, ranging from general discrimination deficits to problems for specific emotions up to no impairment at all. By contrast, only a few studies exi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.12.007
更新日期：2016-02-15 00:00:00
abstract：:Clinical remission in myasthenia gravis after thymectomy and immune suppressive treatment may be reflected by changes of immunoregulatory cytokines. Mononuclear cells from paired blood samples from fifteen patients with myasthenia gravis before and after thymectomy and immunosuppressive therapy were examined by in sit...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(97)00104-4
更新日期：1997-10-03 00:00:00
abstract：:Inherited muscular dystrophy of the chicken is an abnormality affecting the normal development and function of fast-twitch skeletal muscles. Several different strains of dystrophic chickens have been developed by selection for high lipid content in the pectoralis muscle and early onset of the disorder or by outcrossin...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90073-8
更新日期：1980-05-01 00:00:00
abstract：BACKGROUND AND PURPOSE:Supratentorial stroke can cause conjugate eye deviation directed contralateral to the affected side (wrong-way deviation). It is rare and thought to be associated exclusively with hemorrhagic stroke. We prospectively investigated the clinical features and prognostic significance of this wrong-way...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.06.010
更新日期：2011-09-15 00:00:00
abstract：:An accumulation of SCA6 cases has been observed in the Chugoku area of Western Japan. In the Tottori prefecture, located in the northeastern part of the Chugoku district, we observed a cluster of SCA6 families within the eastern area, suggesting that there may be a founder in the Japanese SCA6 population. Genotyping w...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(01)00453-1
更新日期：2001-03-15 00:00:00
abstract：:Five French families with Creutzfeldt-Jakob disease (CJD) were found to have either of 2 different point mutations (at codons 178 and 200) in the amyloid precursor gene (PRNP) on chromosome 20. The ancestry of these and other CJD families outside of France suggests that the codon 178 mutation had a northern European o...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(91)90151-v
更新日期：1991-10-01 00:00:00
abstract：BACKGROUND AND PURPOSE:Pure midbrain stroke can cause isolated unilateral oculomotor paresis. We attempted to determine whether there is a difference in the oculomotor paresis pattern between pure midbrain infarction and midbrain hemorrhage. METHODS:Pure midbrain stroke patients who presented with isolated unilateral ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.03.012
更新日期：2015-04-15 00:00:00
abstract：BACKGROUND:Multiple sclerosis (MS) patients commonly suffer from fatigue and define it as one of their most disabling symptom profoundly disrupting their lives. However, the relationship of fatigue to cognitive functions and its impact on quality of life have not been widely studied. AIM:To define (1) "cognitive fatig...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2005.09.015
更新日期：2006-06-15 00:00:00
abstract：OBJECTIVE:To determine the relative contribution of various risk factors to the development of Parkinson's disease (PD). METHODS:Ten variables that were independently associated with PD in a health system population-based case-control study of epidemiological risk factors for the disease were jointly assessed. Stepwis...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2003.09.014
更新日期：2004-02-15 00:00:00
abstract：BACKGROUND:Although predominantly reported in patients with status epilepticus, periictal MRI abnormalities have been reported in patients with a single or a cluster of seizures. Clinicians are often presented with a dilemma concerning the features of MRI abnormalities induced by a single or a cluster of seizures, as t...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.06.001
更新日期：2014-08-15 00:00:00
abstract：:The stereotyped, episodic abnormal behaviour manifested by a child and her mother on exposure to a particular sort of sunlight is described in detail. The child is the first fully documented patient with this so-called "self-induced" form of photosensitive epilepsy in whom for several years no electroencephalographic ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(83)90076-x
更新日期：1983-04-01 00:00:00
