Abstract：
:Motor neurone disease (MND) is a useful paradigm for many progressive disabling neurological disorders and serves as a particularly opposite model for the study of patients' responses to progressive and irreversible disability. We studied the progression of disability and the patients' perception of their health in a group of MND patients (n = 14) for 6 months from diagnosis or soon after. A comparison group of similar age, gender ratio and initial disability on Barthel index were Parkinson's disease (PD) patients, admitted because of poor response to outpatient drug therapy and increasing disability (n = 22). MND patients showed rapid deterioration in all aspects of self care and mobility, as assessed by Barthel Index. 32% PD patients showed a significant improvement in disability during their admission. For both groups, perception of their physical health on the SF36 was very poor at recruitment compared to age- and sex-matched population norms. However for patients in both groups the SF36 could not be used to monitor changes in perception of health because of floor effects. In those in whom change could be assessed, there was a trend for MND patients to deteriorate and PD patients to improve. We conclude that loss of independence in self care and failing mobility may occur more rapidly than current medical and social services can accommodate. There is a need for planning and proactive intervention to support patients and careers. The patients' perception of their physical health is poor from time of diagnosis as assessed by the SF36, but this scale cannot be used to monitor patients with MND or late stage PD over time.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Young CA,Tedman BM,Williams IRdoi
10.1016/0022-510x(95)00062-7subject
Has Abstractpub_date
1995-05-01 00:00:00pages
50-3eissn
0022-510Xissn
1878-5883pii
0022510X95000627journal_volume
129 Supplpub_type
临床试验,杂志文章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：:The objective of this article has been to describe the presence of a sensory neuronopathy in a patient harbouring ataxia with oculomotor apraxia type 2 (AOA2). A 40 year-old woman, born to consanguineous parents, presented with ataxia, decreased vibration sense, areflexia, indifferent plantar responses, preserved musc...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.09.004
更新日期：2010-11-15 00:00:00
abstract：:In 50 patients with computed tomography-verified small, deep, lacunar, infarcts from a prospective stroke registry, we studied the lesion site in relationship with the clinical syndromes pure motor stroke, sensorimotor stroke and ataxic hemiparesis. Seventy per cent (95% confidence interval: 57-83%) of the lesions wer...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(91)90138-w
更新日期：1991-10-01 00:00:00
abstract：BACKGROUND:Longitudinally extensive transverse myelitis (LETM) is a frequent manifestation of neuromyelitis optica spectrum disorder (NMOSD). However, it can also occur in other immune-mediated diseases of the central nervous system (CNS). Positive aquoporin-4 antibodies (AQP4-ab) predict higher relapse rate after LETM...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.12.033
更新日期：2017-02-15 00:00:00
abstract：:Recently it has been reported that the C57BL mice can be used as a model of benign monoclonal gammopathy (MG). Since experimental models have until now failed to reproduce specific lesions of human dysglobulinemic polyneuropathies, we decided to investigate the peripheral nerve of these mice. The sciatic nerve and the...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90188-2
更新日期：1987-10-01 00:00:00
abstract：:Hyperthermic isolated limb perfusion (HILP) with tumor necrosis factor alpha (TNFalpha) is effective for advanced melanoma and sarcoma of the limbs. Ten patients undergoing HILP with TNFalpha were evaluated by neurological examinations, nerve conduction studies (NCS), sympathetic skin responses (SSR) and conventional ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00098-7
更新日期：1998-06-11 00:00:00
abstract：:We report here the results of a simple and reproducible technique which can be used in semi-routine analysis of peripheral nerve biopsy specimens, so as to have a quantitative analysis of the major lipid classes, i.e. cholesterol, cerebrosides, ethanolamine phospholipids, phosphatidyl-choline, phosphatidyl-serine + ph...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90039-x
更新日期：1979-04-01 00:00:00
abstract：:Duchenne muscular dystrophy (DMD) is an X-linked recessive disease caused by mutations in the dystrophin gene and is characterized by muscle degeneration and death. DMD affects males; females being asymptomatic carriers of mutations. However, some of them manifest symptoms due to a translocation between X chromosome a...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.09.036
更新日期：2014-01-15 00:00:00
abstract：:Despite apparent relationships between ischemic stroke and the acute phase response (APR), considerable variation in the APR exists between individuals. We therefore performed post-hoc analysis of individual APR profiles in 31 patients with ischemic stroke in relation to volume of brain infarction. Patients with ische...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.09.011
更新日期：2006-12-21 00:00:00
abstract：BACKGROUND:Differential diagnosis between idiopathic Parkinson's disease (PD) and multiple system atrophy (MSA) is often difficult in early disease stages. Since MSA is misdiagnosed as PD in more than 20% of the early stages, there is need for methods refining the differentiation of the two disease entities. In PD post...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00279-3
更新日期：2000-04-01 00:00:00
abstract：BACKGROUND:CYP2D6 polymorphisms have been reported to be associated with Parkinson's disease (PD) susceptibility, but the results of these previous studies were inconsistent. OBJECTIVES:To explore whether PD patients with CYP2D6 gene variation have different risk to PD to those with normal function of CYP2D6. METHODS...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,meta分析
doi：10.1016/j.jns.2013.10.030
更新日期：2014-01-15 00:00:00
abstract：:The effect of intravenous infusion of progesterone and Mebumal (ACO, Sweden; penotobarbital, INN) on the frequency and amplitude of the spontaneous interictal spikes from a penicillin focus in the cerebral cortex was investigated in ovariectomized cats anaesthetized with chloralose. The plasma concentration of progest...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(78)90166-1
更新日期：1978-03-01 00:00:00
abstract：BACKGROUND:Fingolimod is approved by the U.S. Food and Drug Administration to reduce relapses and disability progression in relapsing forms of MS. Several screening studies and a first-dose observation (FDO) period are recommended due to adverse effects observed in clinical trials. OBJECTIVE:The objective of this stud...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.09.009
更新日期：2012-12-15 00:00:00
abstract：PURPOSE:Paroxysmal kinesigenic dyskinesia (PKD) is a rare movement disorder. The underlying neural mechanisms have not been fully understood. This study aimed to examine the alteration of resting-state functional connectivity (RSFC) between interhemispheric homotopic regions in PKD using a technique called "voxel-mirro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.02.046
更新日期：2015-04-15 00:00:00
abstract：:Thalamic damage is associated with a variety of neuropsychological dysfunctions, as well as strategic infarct dementia. However, only a limited number of reports in the medical literature have discussed the correlation between the clinical findings and the specific functional changes observed on images. We investigate...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2007.09.016
更新日期：2008-03-15 00:00:00
abstract：PURPOSE:To investigate Blood Oxygen Level Dependent (BOLD) responses to interictal epileptic discharges (IEDs) during EEG-correlated functional MRI (EEG-fMRI) in patients with partial epilepsy. METHODS:We studied eight patients who had a diagnosis of partial epilepsy and active spiking on routine scalp EEG recording. ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2007.11.019
更新日期：2008-05-15 00:00:00
abstract：OBJECTIVE:To identify and characterize cup to disc ratio (CDR) and related optic nerve head abnormalities in multiple sclerosis (MS) using spectral domain optical coherence tomography (OCT). BACKGROUND:While CDR is routinely assessed by ophthalmologists in the evaluation of glaucoma, CDR and related optic nerve head m...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.12.011
更新日期：2011-03-15 00:00:00
abstract：BACKGROUND:Contactin-associated protein 2 (Caspr2) antibody is a neuronal surface antibody (NSAb) capable of causing disorders involving central and peripheral nervous systems (PNS). Thymoma can be found in patients with Caspr2 antibodies and is most frequently associated with PNS symptoms. Myasthenia gravis can be fou...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.03.043
更新日期：2014-06-15 00:00:00
abstract：OBJECTIVES:Though much information exists about the diagnosis, treatment, and epidemiology of myasthenia gravis (MG), a comprehensive data registry and biorepository is critical to better understand disease mechanisms, treatment outcomes, and the impact of treatment strategies. We aimed to design and implement the "Exp...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.116830
更新日期：2020-07-15 00:00:00
abstract：:Systemic inflammatory response syndrome (SIRS) is a medical condition in which the all-organ microcirculation is affected including nervous system. We describe neurological findings in 64 patients with SIRS at Hospital das Clínicas of Sao Paulo University School of Medicine; 45.3% were male and 54.7% female; their age...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.08.015
更新日期：2006-12-01 00:00:00
abstract：:The enzyme-linked immunosorbent assay (ELISA) was used to determine levels of specific IgG antibodies against measles, rubella, vaccinia, corona (OC43) and mumps viruses in cerebrospinal fluid (CSF) and serum of 18 patients with clinically definite multiple sclerosis (MS), 8 patients with optic neuritis (ON), 27 patie...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(82)90031-4
更新日期：1982-12-01 00:00:00
abstract：:We performed post-mortem examinations of three patients with progressive neurogenic amyotrophy of long duration. One patient had been clinically diagnosed as having sporadic amyotrophic lateral sclerosis (ALS) and two had been diagnosed with progressive spinal muscular atrophy (PSMA). The disease durations were 10, 17...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(96)00297-3
更新日期：1997-03-10 00:00:00
abstract：:Neurological injuries produced by explosive blasts are the result of a cascade of events that begin with the initial explosion and evolve from the secondary, tertiary, and quaternary effects that the explosion engenders [Lavonis EJ. Blast Injuries. EMedicine.htm]. Only the results of the primary blast are predictable,...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2006.06.005
更新日期：2006-11-01 00:00:00
abstract：:The authors have shown in a recent paper that survival with amyotrophic lateral sclerosis (ALS) can be increased by the use of non-invasive methods of assisted ventilation (Bipap). However, the progression of muscle weakness was not affected and the quality of life was not positively enhanced. In ALS, reduced physical...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(99)00218-x
更新日期：1999-10-31 00:00:00
abstract：:Cutaneous reflex (CR) was measured in 15 patients with acute lacunar stroke and in 15 age- and sex-matched normal subjects. EMG activities were recorded from the first dorsal interosseous muscle with surface electrodes, rectified and averaged. Intensity of the stimulation on the index finger was four times the sensory...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00142-7
更新日期：1998-07-15 00:00:00
abstract：OBJECTIVE:Report on the kind and distribution of somatotopic sensation loss and its utility in assessing severity of sensation loss in study of a large international cohort of patients with hereditary transthyretin amyloidosis with polyneuropathy (hATTR-PN). METHODS:Smart Somatotopic Quantitative Sensation Testing (S ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2018.08.031
更新日期：2018-11-15 00:00:00
abstract：:Forty-seven spinal cords from subjects between 13 and 95 years of age have been examined; in the majority death occurred suddenly or rapidly from a state of previous good health. The limb motor neurons in the lumbospinal segments have been estimated by counting nucleoli in characeteristic cells in every fifth group of...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(77)90069-7
更新日期：1977-11-01 00:00:00
abstract：:Recent advances in magnetic resonance high-resolution vessel wall imaging (HRVWI), which can detect intramural hematomas (IMH), improve the noninvasive diagnostic accuracy of isolated posterior inferior cerebellar artery dissection (iPICA-D). However, despite the risk of overlooking minute IMH, the utility of T2-weigh...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.116693
更新日期：2020-04-15 00:00:00
abstract：:Post-stroke epilepsy (PSE) is defined as "recurrent seizures following stroke with confirmed diagnosis of epilepsy". Our objective was to describe the epidemiological, clinical and therapeutic PSE aspects at the Yalgado Ouedraogo Teaching Hospital, the main reference centre for neurological conditions in Burkina Faso....
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.06.038
更新日期：2016-09-15 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
