Abstract：
:There have been recent developments in the pathology of sporadic ALS. A new filamentous neuronal inclusion body in ALS detected by immunohistochemical localisation of the protein ubiquitin has been characterised at the light microscopic and ultrastructural level and appears specific for the disease. The molecular composition of underlying filaments remains unresolved but the quest for this is a major aim in ALS research. Despite being a progressive degenerative process which primarily affects motor systems, ALS is now recognised to involve several non-motor systems and in long survivors affects many subcortical structures. There is also accumulating evidence that the neurodegenerative process underlying ALS may present as a non-motor clinical syndrome, particularly as a frontal lobe dementia with characteristic inclusions present in the non-motor cortex. Considering ALS as a multisystem disease rather than simply a disease of motor neurones has major implications for research into pathogenesis.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Lowe Jdoi
10.1016/0022-510x(94)90175-9subject
Has Abstractpub_date
1994-07-01 00:00:00pages
38-51eissn
0022-510Xissn
1878-5883pii
0022-510X(94)90175-9journal_volume
124 Supplpub_type
杂志文章,评审abstract：:Under electrophysiological control, a focal demyelinating lesion can be produced in the corpus callosum of the rabbit by slow pressure injection of a 1% solution of lysophosphatidyl choline (LPC). Light- and electron-microscopic examination of the LPC demyelinated corpus callosum indicates that many axons remain struc...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90202-6
更新日期：1980-11-01 00:00:00
abstract：:Impairment of nervous function and thiamine metabolism were studied in 40 patients suffering from pernicious anaemia, 20 of whom had not been treated and the other 20 were on hydroxocobalamin therapy. Of the untreated patients 13 (65%) showed signs of peripheral nerve dysfunction with reduced conduction velocities, as...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90180-x
更新日期：1980-03-01 00:00:00
abstract：BACKGROUND AND PURPOSE:Congestive heart failure is a risk factor for ischemic stroke. Brain natriuretic peptide (BNP) is used as a biological marker of heart failure. We hypothesized that heart failure was associated with the onset of ischemic stroke patients with atrial fibrillation (AF). METHODS:Between June 2006 an...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.02.374
更新日期：2009-06-15 00:00:00
abstract：:A case of central nervous system (CNS) leukemia with normal bone marrow, associated with a novel chromosomal abnormality, is described. A 58 year-old woman complained of hearing disturbance, severe headache and vomiting, and showed signs of meningeal irritation, as well as papilledema and bilateral dysacusis. Immature...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(97)00099-3
更新日期：1997-10-03 00:00:00
abstract：:We report a woman with anti-Yo-associated cerebellar degeneration, severe eye movement abnormalities, and no evidence of malignancy after 3 years of extensive evaluation. The prominent neuro-ophthalmologic findings suggest brainstem involvement, which may be a novel manifestation of anti-Yo associated paraneoplastic s...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2004.07.015
更新日期：2004-10-15 00:00:00
abstract：:The aim of this study was to determine the effectiveness of hypoglossal-facial nerve "side"-to-end (HemiHN-FN) and accessory-facial nerve end-to-end (AN-FN) neurorrhaphy using a predegenerated nerve graft (PNG) for reanimating facial paralysis in a rat FN injury model. A total of 25 rats with complete unilateral facia...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.10.006
更新日期：2014-12-15 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：:A multitude of factors must be overcome following spinal cord injury (SCI) in order to achieve clinical improvement in patients. It is thought that by combining promising therapies these diverse factors could be combatted with the aim of producing an overall improvement in function. Chondroitin sulphate proteoglycans ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.06.011
更新日期：2016-08-15 00:00:00
abstract：:A suspension of kaolin was injected into the cisterna magna of 44 rats at 2 weeks of age. Animals killed at intervals from 5--19 weeks of age showed varying degrees of hydrocephalus. Light microscopy, scanning and transmission electron microscopy revealed stretching and flattening of the ependymal cells but no signifi...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(78)90046-1
更新日期：1978-05-01 00:00:00
abstract：:This study included 40 consecutive patients with athrothrombotic carotid occlusive disease (A-group) and 13 consecutive patients with moyamoya disease (M-group) who had an internal carotid artery occlusion and underwent EC-IC bypass. Flow velocity and diameter of the operated STA on duplex ultrasonography (STDU), as w...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.11.006
更新日期：2013-02-15 00:00:00
abstract：:Autopsy cases of two brothers with bulbo-spinal muscular atrophy associated with gynecomastia, testicular atrophy and sensory neuropathy are reported. The disease started with finger tremor, proximal muscle weakness and facial muscle twitching at the second and fourth decades, accompanied by bulbar signs and glove-sto...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90240-7
更新日期：1988-11-01 00:00:00
abstract：:Thirty patients with acute, unilateral optic neuritis (ON), where re-examination after a mean observation period of 5 years did not reveal any aetiology, were investigated with regard to laboratory abnormalities frequently observed in multiple sclerosis. Eleven patients had oligoclonal IgG in CSF. In 5 of these a meas...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90237-9
更新日期：1976-01-01 00:00:00
abstract：:Deficient activity of human lysosomal hydrolase, acid sphingomyelinase (ASM), results in the neuronopathic (type A) and non-neuronopathic (type B) forms of Niemann-Pick disease (NPD). A deficiency of ASM is known to deprive lymphoblasts of their response to apoptotic induction by X-ray irradiation. To elucidate the ge...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(02)00106-5
更新日期：2002-07-15 00:00:00
abstract：:In order to compare the effects of total catecholamine (CA) or noradrenaline (NA) depletions on cholinergic systems, and the mechanisms of receptor regulation in various brain regions, the regional changes in the levels of acetylcholine (ACh), M1-receptor (M1-R) binding, and M1-R messenger RNA (mRNA) were mainly exami...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(92)90029-k
更新日期：1992-07-01 00:00:00
abstract：BACKGROUND:The relationship between fatigue impact and walking capacity and perceived ability in patients with multiple sclerosis (MS) is inconclusive in the existing literature. A better understanding might guide new treatment avenues for fatigue and/or walking capacity in patients with MS. OBJECTIVE:To investigate t...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jns.2018.02.026
更新日期：2018-04-15 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：:The internodal length of remyelinated internodes was examined by observations on teased CNS nerve fibres following primary demyelination induced by intraspinal injections of lysolecithin into the white matter of cats. A remyelinated internode was identified as a thinly-myelinated internode, where a node of Ranvier was...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(81)90084-8
更新日期：1981-02-01 00:00:00
abstract：:The effects of combined treatment with low-density lipoprotein (LDL)-apheresis, chenodeoxycholic acid (CDCA) and 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) reductase inhibitor were studied in 2 patients with cerebrotendinous xanthomatosis. Patient 1 was initially treated with LDL-apheresis alone: serum cholestanol level...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/j.jns.2003.07.005
更新日期：2003-12-15 00:00:00
abstract：:Hitherto published results on the impact of brain atrophy on the neurological and psychopathological sympion of this problem without risk of complications. We investigated 173 parkinsonian patients (89 men, 84 women) aged 37--83 years. Besides CT in all patients a standardized neurological and psychopathological inves...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90050-9
更新日期：1979-07-01 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：:Myotonic dystrophy type 1 (DM1), an autosomal dominant disease characterized by a CTG expansion in the 3' region of the DMPK gene in chromosome 19, is a highly heterogeneous disease. In this study, we present a family with early onset-classical type DM, and a homogeneous phenotype highlighted by severe neuromuscular s...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(02)00442-2
更新日期：2003-04-15 00:00:00
abstract：BACKGROUND:Current American Stroke Association guidelines recommend initiating intravenous thrombolysis (IVT) for acute ischemic stroke (AIS) within 60min of patient arrival, given the benefits of IVT for AIS are time dependent. This study aimed to identify the delaying factors in door-to-needle time (DTN) in the emerg...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.03.003
更新日期：2017-05-15 00:00:00
abstract：:Wernicke's encephalopathy is often undiagnosed, particularly in non-alcoholics. There are very few reports of non-alcoholic patients diagnosed with Korsakoff syndrome in the absence of a prior diagnosis of Wernicke's encephalopathy and no studies of diffusion tensor imaging in non-alcoholic Korsakoff syndrome. We repo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.09.025
更新日期：2016-11-15 00:00:00
abstract：:Chronic relapsing experimental allergic encephalomyelitis has been induced in juvenile strain 13 guinea pigs with isologous spinal cord in Freund's complete adjuvant. Retention of antigen at the injection site and in the draining lymph nodes was studied by immunocytochemical staining with antiserum to myelin basic pro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(84)90145-x
更新日期：1984-10-01 00:00:00
abstract：:In-situ hybridization with labeled oligonucleotide probes was applied to explore cytokine and chemokine mRNA expression in sections of striated muscle, the target organ in experimental autoimmune myasthenia gravis (EAMG), induced in Lewis rats by immunization with acetylcholine receptor (AChR) and complete Freund's ad...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00181-6
更新日期：1998-11-26 00:00:00
abstract：:Brain myelin was studied in genetically obese (ob/ob) mice and compared with that of normal (+/+) mice from the same strain. The brain from obese mice had a significantly lower amount of myelin, and marked changes in the fatty acid composition of myelin were observed. In contrast, the myelin cholesterol: phospholipid:...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(85)90104-2
更新日期：1985-05-01 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：:Tuberous sclerosis (TS) is caused by point mutations in the TSC1 or TSC2 genes on chromosomes 9q33-34 or 16p13, respectively. Clinical manifestations can be quite variable but are primarily limited to cutaneous, neurologic, and cardiovascular abnormalities. Phenotypes range from neurologically devastated to those with...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2004.02.009
更新日期：2004-06-15 00:00:00
abstract：:The fibre composition of functionally different regions, the superficial and the deep portions, of the human masseter and the biceps brachii muscles of six elderly subjects (mean age 74 years) was studied by morphological and enzyme-histochemical methods. When compared to previous data for young adults, the masseter m...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(97)00208-6
更新日期：1998-01-21 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
