Abstract:
OBJECTIVES:To determine the impact of disease progression on health-related quality of life in amyotrophic lateral sclerosis (ALS). METHODS:A total of 161 patients with ALS were enrolled. Assessments included the revised ALS Functional Rating Scale and the ALS Assessment Questionnaire (ALSAQ-40). Data analysis comprised linear regression and multivariate analyses. RESULTS:ALSFRS-R score (β = 0.75, p < 0.001), depression (β = 0.08, p < 0.001), pain (β = 0.07, p < 0.001), hopelessness (β = 0.07, p = 0.001), and progression rate (β = 0.02, p = 0.02) explained 76% of the ALSAQ-40 summary index variance. Progression rate alone explained 7% of the ALSAQ-40 summary index variance. The subdomains of emotional well-being, followed by ADL, and finally communication and eating were most strongly influenced by progression rate. CONCLUSION:Our study demonstrates the importance of physical health for emotional well-being. In particular, slower disease progression is associated with higher levels of emotional well-being in ALS.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Prell T,Gaur N,Stubendorff B,Rödiger A,Witte OW,Grosskreutz Jdoi
10.1016/j.jns.2018.12.035subject
Has Abstractpub_date
2019-02-15 00:00:00pages
92-95eissn
0022-510Xissn
1878-5883pii
S0022-510X(18)30530-6journal_volume
397pub_type
杂志文章abstract::Idiopathic Parkinson's disease (PD) may possibly be caused by one or more unidentified neurotoxins present in the environment, or formed endogenously, which progressively damage dopaminergic nigrostriatal neurons. N-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) is an experimental neurotoxin which produces biochemi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(87)90106-7
更新日期:1987-11-01 00:00:00
abstract::Using special soft tissue X-ray investigations the authors have found that the increase in volume of the calf musculature in progressive muscular dystrophy does not seem to be in complete accordance with the criteria of pseudohypertrophy. It has been demonstrated that this structural alteration of the musculature does...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(79)90074-1
更新日期:1979-09-01 00:00:00
abstract::We assessed the incidence of hemorrhagic transformation and infarct volume after early intravenous infusion of recombinant human tissue plasminogen activator (rht-PA) in a newly developed rat cerebral embolic model. Male Wistar rats (n=60) were subjected to middle cerebral artery (MCA) occlusion by a single fibrin ric...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00155-5
更新日期:1998-09-18 00:00:00
abstract::The rat double-SAH model is one of the standard models to simulate delayed cerebral vasospasm (CVS) in humans. However, the proof of delayed ischemic brain damage is missing so far. Our objective was, therefore, to determine histological changes in correlation with the development of symptomatic and perfusion weighted...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.03.023
更新日期:2010-06-15 00:00:00
abstract:BACKGROUND:The likelihood of severe disability and death increases with each recurrent stroke. Repeated intravenous (IV) thrombolysis remains one of the therapeutic options when secondary prevention fails; however, its effects after recurrent stroke are largely unknown. The aim of the present review was to assess the r...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2014.07.039
更新日期:2014-10-15 00:00:00
abstract::Peripheral blood lymphocytes from patients with multiple sclerosis (MS), other neurological diseases and healthy controls were investigated for the presence of cell-mediated hypersensitivity to brain gangliosides and myelin basic protein using an active E-rosette assay. Sensitivity to myelin basic protein and ganglios...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(83)90083-7
更新日期:1983-04-01 00:00:00
abstract::Friedreich Ataxia (FRDA) is an autosomal recessive neurodegenerative disorder most commonly caused by guanine-adenine-adenine (GAA) trinucleotide repeat expansions in both alleles of the FXN gene. Although progressive ataxia remains the hallmark clinical feature, patients with FRDA are at high risk of developing cardi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2017.01.027
更新日期:2017-04-15 00:00:00
abstract::In 1977 and 1978 Cook and his associates demonstrated a positive association between ownership of small dogs and both familial and sporadic cases of multiple sclerosis in New Jersey. Because of the far reaching implications of this work, a similar study was carried out and 72 patients with clinically definite multiple...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(82)90133-2
更新日期:1982-09-01 00:00:00
abstract::We studied the relationship between antibody titers to recombinant HTLV-I p40tax protein and gag-env hybrid protein in serum (by an enzyme-linked immunosorbent assay) and HTLV-I proviral DNA load in peripheral blood mononuclear cells (by a quantitative polymerase chain reaction method) in 18 patients with HTLV-I-assoc...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(92)90215-7
更新日期:1992-01-01 00:00:00
abstract:PURPOSE:To prospectively evaluate the usage of intra-voxel incoherent motion (IVIM) magnetic resonance imaging (MRI) parameters in grading and differentiating histological subtypes of meningiomas and in comparing their ability with the traditional diffusion-weighted imaging (DWI) parameter (apparent diffusion coefficie...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.11.037
更新日期:2017-01-15 00:00:00
abstract::We describe the case of a 67-year-old man who exhibited unsteadiness in walking, topographical disorientation, and urinary incontinence. Neurological examination revealed somnolence and mild weakness in the lower limbs with slight rigidity in the upper limbs. Cerebrospinal fluid examination showed pleocytosis with "fl...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.01.035
更新日期:2013-04-15 00:00:00
abstract::Ultrastructural alterations were observed in the sciatic nerve of dystrophic mice. Myelin sheaths were abnormal in shape, abruptly ceased beyond a node of Ranvier, leaving the axon naked. These changes were seen in both afferent and efferent nerve fibres. Apparent embryonal Schwann cells and Schwann cells which were a...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(76)90121-0
更新日期:1976-08-01 00:00:00
abstract:BACKGROUND:Headache burden is not adequately explored in Africa. Here, we measured weighted migraine prevalence from community-based studies in Africa. METHODS:PubMed search was employed using terms 'headache in Africa' AND/OR 'migraine in Africa' for published literature from 1970 until January 31, 2014. PRISMA was a...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,meta分析,评审
doi:10.1016/j.jns.2014.04.019
更新日期:2014-07-15 00:00:00
abstract::Duchenne muscular dystrophy (DMD) is a fatal X-linked recessive disorder of muscle in children. The DMD gene product, "dystrophin", is absent from DMD, while the allelic disease, Becker muscular dystrophy (BMD), exhibits dystrophin of abnormal size and/or quantity. But we are still uncertain about the scenario that in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(91)90039-a
更新日期:1991-02-01 00:00:00
abstract:BACKGROUND:Percutaneous endoscopic gastrostomy (PEG) is offered to amyotrophic lateral sclerosis (ALS) patients with severe dysphagia. Immediate benefits of PEG are adequate food intake and weight stabilization. However, the impact of PEG on survival is still uncertain. In this work we retrospectively evaluated the eff...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.02.016
更新日期:2011-05-15 00:00:00
abstract::We investigated whether the reduced intramembranous particles (IMP) in the muscle plasma membrane in mdx mice reflects a preferential depletion of a particular size of the IMP. The experiments were performed using the freeze-fracture method to analyze the frequency distribution of the size of IMP, the density of ortho...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)05350-6
更新日期:1997-05-29 00:00:00
abstract:OBJECTIVE:Neurodegenerative diseases are often characterized by the presence of intracellular or extracellular protein aggregates in the central nervous system. Mutations of TARDBP gene have been shown to cause Amyotrophic Lateral Sclerosis and have been reported to present with clinical heterogeneity including parkins...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.04.043
更新日期:2018-07-15 00:00:00
abstract:OBJECTIVES:There is insufficient data on the efficacy of intravenous immunoglobulins (IVIg) as maintenance treatment in myasthenia gravis (MG). We therefore examined response to maintenance IVIg therapy in a cohort of MG patients. METHODS:We reviewed all MG patient files treated with IVIg in our neuro-immunology clini...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.10.043
更新日期:2014-03-15 00:00:00
abstract:OBJECTIVE:Examine the relative importance of several risk factors for progression, in a large sample of MS patients. METHODS:Using a retrospective design in a single university-based MS treatment center, we studied 207 patients with relapsing remitting MS diagnosed, treated at our center, evaluated within one year of ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.02.008
更新日期:2010-05-15 00:00:00
abstract::The clinical features, results of nerve conduction studies and sural nerve biopsy findings have been compared in 5 patients with chronic relapsing polyneuropathy in whom plasma exchange was used in treatment. In 2 patients who consistently responded to plasma exchange, the dominant pathological findings was segmental ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(83)90222-8
更新日期:1983-02-01 00:00:00
abstract::To determine the prevalence and subtypes of dementia in southern Taiwan, a two-phase study consisting of a phase I screening survey using the Mini-Mental Status Examination (MMSE) and a phase II diagnostic examination using the CERAD neuropsychological battery and the neurobehavioral examination was conducted. Accordi...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(98)00225-1
更新日期:1998-09-18 00:00:00
abstract::We describe a case of a 50-year-old man with bilateral shoulder girdle weakness caused by anterior subdural hygroma secondary to a previous spontaneous CSF leak. The CSF leak occurred and resolved 16 years prior to presenting with a 6-year progressive painless, asymmetric proximal muscle weakness involving both upper ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.02.029
更新日期:2011-06-15 00:00:00
abstract:OBJECTIVE:Cerebrovascular lesions (CVLs) are known to play important roles in the pathophysiology underlying Alzheimer's disease (AD), especially in elderly AD cases. The present study was conducted to elucidate the relationship between the CVLs and vascular risk factors (VRFs) in elderly Japanese patients with AD. SU...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.07.001
更新日期:2012-11-15 00:00:00
abstract::We describe a 54-year-old diabetic woman who developed ischemic optic neuropathy followed by acute retinal necrosis and multiple areas of focal venous beading. Vitreous fluid contained amplifiable VZV DNA but not HSV-1, CMV or toxoplasma DNA. The clinical presentation was remarkable for jaw claudication and intermitte...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.12.021
更新日期:2013-02-15 00:00:00
abstract::The present study used the nerve pathology, studied by quantitative light microscopy, as the sole basis for classification of peroneal muscular atrophy (PMA). The findings in biopsies of superficial peroneal nerves of 20 patients were compared with normal values obtained from 8 controls. Three homogeneous groups compr...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(83)90173-9
更新日期:1983-10-01 00:00:00
abstract::Several single gene disorders can potentially be overlooked in the differential diagnostic evaluation of patients with multiple sclerosis (MS). Pelizaeus-Merzbacher disease and spastic paraplegia type 2 are allelic X-linked disorders associated with defective myelination of the central nervous system and mutations in ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2017.01.069
更新日期:2017-04-15 00:00:00
abstract::We examined the changes in cognitive function following carotid endarterectomy (CEA) in relation to the cerebral blood flow (CBF) in patients with high-grade carotid stenosis. The subjects consisted of 23 patients who underwent CEA and 17 controls matched by age and education. Single photon emission computed tomograph...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(03)00128-x
更新日期:2003-09-15 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::The effects of long-term exposure to aluminium on the development of Alzheimer-type neuropathological changes have been studied post-mortem in patients with chronic renal failure who did not have dialysis encephalopathy. Administration of aluminium-containing phosphate binding compounds appears to be a major factor in...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(92)90291-r
更新日期:1992-02-01 00:00:00
abstract::Management of myasthenic crisis (MC) requires admission of the patient into a neurological intensive care unit and timely institution of an efficient and safe treatment. Despite the growing clinical experience with disease modifying immunotherapy there is no clinical consensus regarding the use of plasma exchange or h...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2007.04.045
更新日期:2007-10-15 00:00:00