Abstract：
:We followed two cohorts of Amyotrophic Lateral Sclerosis (ALS) patients to examine the survival and prognostic factors of ALS and the impact of selective referral on prognosis of ALS. The first cohort consisted of population-based incident ALS cases from Harris County, Texas, first diagnosed between 1985 and 1988. The second was a clinical series from a tertiary care center in Houston, Texas, diagnosed between 1977 and 1989. The overall 3-year survival was 29% in the incidence cohort and 32% in the referral cohort; however, the 5-year survival was much lower for the incidence cohort than the referral cohort (4% vs 21%). The large difference in 5-year survival was not explained by the distributions of prognostic factors in the two cohorts but due to stronger unfavorable effects of prognostic factors in the incidence cohort than the referral cohort. In both cohorts, older age at diagnosis, bulbar onset, and positive family history of ALS were unfavorable prognostic factors while blacks had better survival than whites or hispanics. We confirmed that longer duration from onset to diagnosis was a favorable prognostic factor in both cohorts but the effect was more pronounced in the referral series.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Lee JR,Annegers JF,Appel SHdoi
10.1016/0022-510x(95)00154-tsubject
Has Abstractpub_date
1995-10-01 00:00:00pages
207-15issue
2eissn
0022-510Xissn
1878-5883pii
0022510X9500154Tjournal_volume
132pub_type
杂志文章abstract：:Primary adult-onset dystonia is thought to be partly genetic, but families large enough for a genome wide search are difficult to find. We examined the first-degree relatives of 76 primary adult-onset dystonia patients to assess the feasibility of model-free nonparametric methods that allow either screening of candida...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.08.009
更新日期：2006-12-21 00:00:00
abstract：:A sensitive and specific enzyme linked immunosorbent assay (ELISA) utilizing human recombinant acetylcholinesterase has been employed for the detection of human antibodies to human acetylcholinesterase. The method can detect allogenic antibodies to the Yt(a) form of human erythrocyte AChE. Adaptation of this ELISA met...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00304-x
更新日期：2000-05-01 00:00:00
abstract：:We describe a 56-year-old man who had a progressive pseudobulbar palsy, spastic tetraparesis, forced laughing and disturbance of voluntary eyelid closure, and was clinically compatible with chronic progressive spinobulbar spasticity. Magnetic resonance images (MRI) revealed atrophy of the bilateral motor cortices and ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(90)90131-6
更新日期：1990-05-01 00:00:00
abstract：BACKGROUND AND AIMS:Subacute combined degeneration (SACD) of the spinal cord, characterized by degeneration of lateral and posterior columns, is often found in vitamin B12 deficiency. Our aim was to look for sensitivity of imaging in depicting the spinal cord abnormality in vitamin B12 deficient patients and to find an...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.05.020
更新日期：2014-07-15 00:00:00
abstract：:The purpose of the study was to determine the relationship between static postural control parameters to fear of falling and falling history in people with multiple sclerosis (MS) without mobility assistive devices. One-hundred and seven relapsing-remitting patients diagnosed with MS, 62 women aged 42.8 (S.D.=12.0), p...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.09.029
更新日期：2013-12-15 00:00:00
abstract：:Long-term trials have demonstrated the continued efficacy of interferon (IFN) beta treatment in patients with relapsing-remitting (RR) multiple sclerosis (MS) during prolonged administration. The objective of the work was to evaluate the effects of reducing IFN beta administration frequency and total weekly dose in pa...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/j.jns.2004.03.023
更新日期：2004-07-15 00:00:00
abstract：BACKGROUND:Fatigue is a common and debilitating symptom in multiple sclerosis (MS). Over the past decade, a growing body of research has focussed on the pathophysiological mechanisms underlying central (cognitive and physical) fatigue in MS. The precise mechanisms causing fatigue in MS patients are complex and poorly u...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2019.03.007
更新日期：2019-05-15 00:00:00
abstract：:The analysis of human skeletal muscle mitochondria revealed a progressive decline in mitochondrial respiratory chain function with age. The activities affected to the greatest extent were those of complexes I and IV which were decreased by 59% and 47% respectively between the ages of 20-30 years and 60-90 years of age...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(92)90270-u
更新日期：1992-11-01 00:00:00
abstract：:A Finnish family is described with 9 cases of presenile dementia in 3 generations. The mean age at onset was 52 years (range 46--62 years). Progressive dementia, upper motor neuron signs, muscular rigidity, and twitching, irregular tremors were consistent features in the 6 clinically investigated patients and were ass...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90171-0
更新日期：1979-08-01 00:00:00
abstract：:Sera from 40 patients with idiopathic Parkinson's disease and their age- and sex-matched controls were assayed for immunoglobulin G (IgG) antibodies against herpes simplex virus (HSV) type 1-induced cell surface antigens with the indirect immunofluorescent test. An increased level of HSV antibodies was found among the...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(78)90017-5
更新日期：1978-02-01 00:00:00
abstract：:A method for preparative separation of cerebrospinal fluid specific proteins by affinity chromatography and isoelectric focusing is described. It has been tested on CSF from a group of human patients suffering from TIA. The advantage of the method is the production of a protein pattern easy to survey by eye. The sensi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90132-x
更新日期：1980-01-01 00:00:00
abstract：:Somatoform Disorders (SFMD) were recently described in Parkinson Disease (PD) and Dementia with Lewy Bodies (DLB). The present paper updates the observations in our cohort of patients and further details clinical phenomenology. Of 3178 patients consecutively referred to our Institutions from 1999, 1572 subjects had ne...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.07.010
更新日期：2011-11-15 00:00:00
abstract：:HS1-associated protein X-1 (HAX-1) and presenilin-associated rhomboid-like protein (PALR) were reported to play an important role in the activation of HtrA2/Omi, which is also designated PARK13, in the mitochondria. To elucidate the role of HAX-1 and PARL in patients with Parkinson's disease (PD) and dementia with Lew...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.116928
更新日期：2020-08-15 00:00:00
abstract：:A 90-year-old man with a longstanding bilateral essential tremor presented with right-sided weakness of sudden onset. The CT scan of the brain showed a hypodense area in the left side of the pons consistent with an infarction. The paralysis disappeared without recurrence of the tremor on the right, but the tremor pers...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(97)05397-5
更新日期：1997-08-01 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：: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：PURPOSE:To determine conditions which influence transfer of iopamidol from lumbosacral cerebrospinal fluid (CSF) to blood. METHODS:Iopamidol transfer was measured in 32 patients over 180 min after radiculography and compared with patient variables. RESULTS:Iopamidol transfer began early in 12 patients, more slowly in...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(02)00340-4
更新日期：2003-01-15 00:00:00
abstract：:Thymomas are associated with paraneoplastic autoimmune diseases at a high frequency. It is rare that four paraneoplastic autoimmune disorders co-occur in a single patient. We describe a thymoma patient with diagnoses of myasthenia gravis, vitiligo, alopecia areata, and oral lichen planus associated with a thymoma. Aft...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.05.038
更新日期：2011-09-15 00:00:00
abstract：:The study is designed to assess the oxidative stress intensity in erythrocytes obtained from patients in different clinical phenotypes of neuroinflammation, defined as clinically isolated syndrome (CIS) and relapsing-remitting multiple sclerosis (RRMS). Advanced oxidation protein products (AOPP), malondialdehyde (MDA)...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.11.006
更新日期：2014-02-15 00:00:00
abstract：:Guillain-Barré syndrome (GBS) is considered a monophasic disorder yet recurrences occur in up to 6% of patients. We retrospectively studied an Italian-Japanese population of 236 GBS and 73 Miller Fisher syndrome (MFS) patients and searched for factors which may be associated with recurrence. A recurrent patient was de...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.02.053
更新日期：2016-06-15 00:00:00
abstract：:Fatiguability is a determining characteristic of different muscle fibre types. An important aspect, indirectly related to fatiguability, gluconeogenesis, was investigated by observing fructose-1,6-diphosphatase (FDP) activity in experimental models prepared in rabbits by cross-reinnervation of the fatigue-resistant m....
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90250-1
更新日期：1976-11-01 00:00:00
abstract：:Low 25-hydroxy vitamin D (25-[OH]-D) serum concentrations have been associated with higher disease activity in multiple sclerosis (MS) patients. In a large cross-sectional study we assessed the vitamin D status in MS patients in relation to seasonality and relapse rate. 415 MS-patients (355 relapsing-remitting MS and ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.01.059
更新日期：2017-04-15 00:00:00
abstract：:Clinical awakening can be seen just before or after seizure onsets. In this study we determined the time between onset of seizures and awakening in patients with frontal lobe epilepsy (FLE) and temporal lobe epilepsy (TLE). Sixty-eight patients who underwent video-EEG monitoring with simultaneous PSG were retrospectiv...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.12.009
更新日期：2012-04-15 00:00:00
abstract：BACKGROUND:Global cerebral ischemia followed by reperfusion, leads to extensive neuronal damage, particularly the neurons in the hippocampal CA region. Recent studies have demonstrated that pharmacological agents, such as Nigella sativa L. (Ranunculaceae) that is an annual herbaceous flowering plant, given at the time ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.11.019
更新日期：2014-02-15 00:00:00
abstract：:The gut microbiome is composed of an enormous number of microorganisms, generally regarded as commensal bacteria. Resident gut bacteria are an important contributor to health and significant evidence suggests that the presence of healthy and diverse gut microbiota is important for normal cognitive and emotional proces...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2019.03.027
更新日期：2019-05-15 00:00:00
abstract：:A point mutation at codon 210 (GTT to ATT) of the prion protein gene on chromosome 20 was found in a 48-year-old CJD-affected woman of a Chinese family. This affected woman had an early onset and long-duration form of CJD. Serial magnetic resonance image (MRI) analysis of this woman showed severe brain atrophy, promin...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(96)00198-0
更新日期：1996-11-01 00:00:00
abstract：:Phenylketonuria (PKU) is a recessive autosomal disorder caused by a severe deficiency of phenylalanine-4-hydroxilase activity which leads to the accumulation of L-phenylalanine (Phe) in the tissues and plasma of patients. The main clinical features are retarded development and intellectual impairment. Recent studies h...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.01.016
更新日期：2010-05-15 00:00:00
abstract：:Antibodies against myelin oligodendrocyte glycoprotein (MOG) mediate demyelination in experimental autoimmune encephalomyelitis (EAE) in different animal species and are implicated in the immunopathogenesis of multiple sclerosis (MS). In order to evaluate the anti-MOG response, we have analyzed the cerebrospinal fluid...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(03)00066-2
更新日期：2003-07-15 00:00:00
abstract：BACKGROUND:The effects of smoking on clinical outcomes following acute stroke remain controversial. METHODS:We evaluated the influence of smoking on 90-day outcomes after acute atherothrombotic stroke in 292 Japanese men extracted from the database of the Edaravone and Argatroban Stroke Therapy for Acute Ischemic Stro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.jns.2013.09.023
更新日期：2013-12-15 00:00:00
abstract：:Selenoprotein N-related myopathy (SEPN1-RM) is an early-onset muscle disorder that can manifest clinically as congenital muscular dystrophy with spinal rigidity and can result in specific pathological entities such as multiminicore disease, desmin-related myopathy with Mallory body-like inclusions, and congenital fibe...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.09.011
更新日期：2011-01-15 00:00:00
