Abstract：
:Cases of adult-onset idiopathic motor neuron disease (MND) identified from January 1970 through December 1986 were studied in a defined area of California. The patients were followed prospectively throughout the illness in 99% of cases. Among 708 cases aged 25-74 years at onset, the most common type (86%) was typical, sporadic amyotrophic lateral sclerosis (SporALS). The risk of bulbar onset and shorter survival times increased with age in both men and women. About 4%, mainly younger men, experienced unusually long courses with milder paralysis, but could not be identified early in the illness. They probably represent one extreme of the ALS spectrum rather than a distinct subtype. Familial ALS (FamALS) was diagnosed in 7%. It developed earlier in life but ran a slightly longer course, which suggests a different disease process. Overall there was a statistically significant predominance of males, especially in 17 cases (2%) of progressive muscular atrophy (PMA). There were 26 cases (4%) classified as primary lateral sclerosis (PLS). Progressive bulbar palsy was not found; that diagnosis usually denotes merely the bulbar onset of ALS.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Norris F,Shepherd R,Denys E,U K,Mukai E,Elias L,Holden D,Norris Hdoi
10.1016/0022-510x(93)90245-tsubject
Has Abstractpub_date
1993-08-01 00:00:00pages
48-55issue
1eissn
0022-510Xissn
1878-5883pii
0022-510X(93)90245-Tjournal_volume
118pub_type
杂志文章abstract：:The ability to accurately navigate within an environment (known or new) is not fully understood but involves a number of highly complicated cognitive process related to both central and peripheral nervous system structures and neuronal networks. We describe a patient who developed loss of his spatial navigational skil...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.06.026
更新日期：2011-09-15 00:00:00
abstract：:The process of vascularisation was studied in transplanted astrocytomas in BD-IX rats. The development of blood vessels was followed from the earliest signs of angiogenesis throughout tumour growth. On the basis of tumour vasculature, 3 consecutive stages of tumour growth could be distinguished; avascular, early vascu...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(81)90188-x
更新日期：1981-01-01 00:00:00
abstract：:This paper describes a histochemical and histographic analysis of the masticatory muscles obtained from 78 early autopsy samples from subjects from 4 days to 87 years old. Five groups of muscles have been stuied: the temporalis, the medial and lateral pterygoid, the superficial bundle of the masseter and the mylohyoid...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90163-x
更新日期：1980-03-01 00:00:00
abstract：:Here we report a case with positive serum anti-aquaporin 4 (AQP4) antibody who presented with hypersomnolence, symmetrical hypothalamic lesions and a reduced CSF orexin (hypocretin) level without optic nerve and spinal cord lesions on MRI. All of the symptoms, MRI finding and CSF orexin level improved simultaneously a...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.08.031
更新日期：2012-01-15 00:00:00
abstract：:An immunocytochemical study of surface and cytoplasmic markers of the monocyte/macrophage lineage was undertaken on peripheral blood (PB) and cerebrospinal fluid (CSF) cells in patients with multiple sclerosis (MS) or other neurological diseases (OND) and in three healthy controls. Size and granularity were assessed b...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90274-3
更新日期：1993-12-15 00:00:00
abstract：:Findings in a sural nerve biopsy from a patient with rheumatoid neuropathy are reported. Inflammatory changes in different stages were observed in epineurial arterioles. Arterioles with fibrinoid necrosis contained IgG, IgM, rheumatoid factor and complement. The same substances were found at the inner site of the peri...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(77)90128-9
更新日期：1977-09-01 00:00:00
abstract：OBJECTIVE:To identify the main determinants of health-related quality of life (HRQoL) in Brazilian stroke survivors. METHODS:Patients were assessed with the NIH Stroke Scale, Barthel index (BI), Lawton scale, modified-Rankin scale, Cumulative Illness Rating Scale for Geriatrics, Mini-Mental State Examination, Hospital...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.04.008
更新日期：2009-09-15 00:00:00
abstract：:High plasma homocysteine (tHcy) is a risk factor for cardiovascular disease and stroke and Alzheimer's disease (AD). An inverse relationship has been reported between tHcy and plasma B12 and folate levels. Seventy-nine AD patients and 156 controls from three Arab villages in northern Israel participated. Plasma tHcy, ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2004.08.011
更新日期：2004-12-15 00:00:00
abstract：:Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoproliferative disorder characterized by massive intravascular growth of lymphoma cells with a predilection for the central nervous system (CNS). Diagnosis is generally delayed by variable clinical presentation and nonspecific laboratory findings. Brain biops...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.10.012
更新日期：2013-02-15 00:00:00
abstract：:Nitric oxide (NO) may be involved in myelin and oligodendrocyte injury associated with multiple sclerosis (MS), a demyelinating disease of unknown etiology. The cerebrospinal fluid (CSF) from MS patients may provide an important signal inducing a pathologic process within the central nervous system (CNS). To investiga...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(96)00164-5
更新日期：1996-10-01 00:00:00
abstract：:Muscle biopsies were obtained from the vastus lateralis of 14 male subjects: 3 long distance runners, 2 world class power lifters and 9 active, although not highly trained, individuals used as controls. The fibers were investigated by electron microscopy and the mitochondrial volume percent, lipid volume percent and Z...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(81)90076-9
更新日期：1981-02-01 00:00:00
abstract：:Baló's concentric sclerosis (BCS) and tumefactive demyelination (TD) are considered atypical forms of multiple sclerosis (MS). Baló lesions are characterized by concentric rings corresponding to alternating bands of demyelination and relatively preserved myelin (Hu and Lucchinetti, 2009). Tumefactive lesions are pseud...
journal_title：Journal of the neurological sciences
pub_type： 信件
doi：10.1016/j.jns.2014.11.024
更新日期：2015-01-15 00:00:00
abstract：BACKGROUND:Intracranial hemorrhage (ICH) is the most common neurologic complication of reversible cerebral vasoconstriction syndrome (RCVS). In this study, we compared hemorrhagic and non-hemorrhagic RCVS with an aim to evaluate the risk factors and short-term clinical outcomes of hemorrhagic lesions. METHODS:We used ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2021.117312
更新日期：2021-01-07 00:00:00
abstract：:Low levels of muscle carnitine have been found in patients with Duchenne dystrophy, a case possibly of Becker dystrophy, and limb-girdle syndrome as well as in patients with the recently described muscle carnitine deficiency syndrome. Tissues of the mouse, hamster, and chicken were analyzed to determine whether tissue...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(78)90251-4
更新日期：1978-08-01 00:00:00
abstract：:Ophthalmological examination and visual evoked responses (VERs) were repeated at 6-120 (mean 46) months after the first attack of acute optic neuritis in 80 patients who had abnormal VERs in 98 symptomatic eyes at the initial examination. The wide field VER returned to within the normal range in 19/98 (19%) symptomati...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(86)90075-4
更新日期：1986-10-01 00:00:00
abstract：:Lower limb neuropathic pain in HIV patients is a common manifestation of sensory neuropathy (HIV-SN), but can be seen in patients who do not meet standard definitions of HIV-SN. The drug stavudine is a risk factor for HIV-SN, but some patients treated without stavudine experience HIV-SN, and the prevalence and risk fa...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2018.12.041
更新日期：2019-02-15 00:00:00
abstract：BACKGROUND:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by the involvement of cerebral small arteries. Although cerebral large artery disease has been reported in CADASIL patients, the prevalence and location of relevant cerebral arterial disease ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.11.029
更新日期：2015-12-15 00:00:00
abstract：:In boys with Duchenne muscular dystrophy (DMD) plasma myoglobin levels remained approximately constant with age while creatine kinase (CK) activity progressively decreased. For carrier detection, plasma myoglobin level was found to be less reliable than CK activity. The myoglobin level was raised only in some of those...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(84)90194-1
更新日期：1984-02-01 00:00:00
abstract：BACKGROUND:Insulin-like growth factor-I (IGF-I) has been shown to protect against stroke in rats when administered intracerebroventricularly. However, this invasive method of administration is not practical for the large number of individuals who require treatment for stroke. Intranasal (IN) delivery offers a noninvasi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(01)00532-9
更新日期：2001-06-15 00:00:00
abstract：:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by loss of motor neuron and various cognitive deficits including writing errors. (11)C-flumazenil (FMZ), the positron emission tomography (PET) GABA(A) receptor ligand, is a marker of cortical dysfunction. The objective of this study was...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.11.039
更新日期：2012-04-15 00:00:00
abstract：:In a prospective study, we analysed the presence of monoclonal immunoglobulin (moIg) in the serum from 30 patients with amyotrophic lateral sclerosis (ALS) and 30 matched controls using a sensitive Western blot technique. The incidence of serum moIg was 60% in the ALS group and 13.3% in the control group. Most ALS ser...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(91)90220-2
更新日期：1991-07-01 00:00:00
abstract：:Histone deacetylases (HDACs), or lysine deacetylases (KDAC), are epigenetic regulators that catalyze the removal of acetyl moieties from the tails of lysine residues of histones and other proteins. To date, eighteen HDAC family members (HDAC1-11 and SIRT1-7) have been identified and grouped into four classes according...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2011.02.017
更新日期：2011-05-15 00:00:00
abstract：BACKGROUND:Intravenous rt-PA is effective in hyperacute ischemic stroke (HAIS) but is administered only in few patients. OBJECTIVES:To report the thrombolysis rate in our stroke unit using a stroke code (SC) protocol with a prenotification system and to analyze the SC impact on the thrombolysis rate in a systematic re...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,meta分析
doi：10.1016/j.jns.2011.10.009
更新日期：2012-03-15 00:00:00
abstract：BACKGROUND:Unilateral spatial neglect (USN) is frequently found in ischemic stroke patients. Because USN is related to poor functional outcomes, evaluating recovery from USN after stroke is critical. METHODS:Patients with acute ischemic stroke with lesions in the right cerebral hemisphere on MRI and exhibiting left US...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.06.021
更新日期：2017-08-15 00:00:00
abstract：:We study a patient with extrapontine myelinolysis (EPM), without central pontine myelinolysis (CPM), who exhibited changes in abnormalities observed with MRI. MRI in the acute stage of the bilateral basal ganglia showed a high signal intensity on T2-weighted images, and a low signal on T1-weighted images. The low sign...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00275-5
更新日期：1998-12-11 00:00:00
abstract：:The aim of the present study was to determine whether a cognitive decline, related to multiple sclerosis (MS), also involves deficits in semantic memory. Semantic memory function was evaluated by studying the conscious understanding of conceptual meanings. A group of MS patients with cognitive decline was presented wi...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(98)00314-1
更新日期：1999-01-15 00:00:00
abstract：:Vitamin B1- and B-deficient encephalopathy was produced in adult Wistar rats and the effect of methylcobalamin was studied. These experiments were performed in vitamin B1- or B-deficient rats with or without the administration of guanidine, a magnesium antagonist. These encephalopathic rats showed symmetrical lesions ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90210-3
更新日期：1987-01-01 00:00:00
abstract：:Controversy exists regarding the apolipoprotein E (ApoE) epsilon 4 allele association with vascular dementia (VaD). The results range from increased epsilon 4 frequency, similar to that found for Alzheimer's disease (AD), to no association at all. Our objective was to clarify the relationship between ApoE epsilon 4 al...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(02)00287-3
更新日期：2002-11-15 00:00:00
abstract：OBJECTIVE:We carried out a meta-analysis focusing on the relationships between rs1137070 C>T and rs1799836 A>G polymorphisms in the MAO gene as a modifier of Parkinson disease (PD) susceptibility. METHOD:A literature search of the Cochrane Library Database, Web of Science, PubMed, CINAHL, EMBASE, and the Chinese Biome...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,meta分析
doi：10.1016/j.jns.2014.07.016
更新日期：2014-10-15 00:00:00
abstract：:Common pathogenic mechanisms may be involved in the prevalence of ischemic stroke and coronary heart disease. Recently, genome-wide association (GWA) studies identified a chromosome region (9p21) that confers the risk of coronary heart disease. In a hospital-based case-control study conducted in Chinese Hans, we teste...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.04.013
更新日期：2009-09-15 00:00:00
