摘要:

ABSTRACT—In 10 patients with amyotrophic lateral sclerosis (ALS), the CSF content of the neuropeptides vasoactive intestinal polypeptide (VIP) and cholecystokinin (CCK) as well as neural cell adhesion molecule (NCAM) was investigated. Compared with normal controls, no deviations were found in CCK or NCAM, while the values of VIP were significantly lower in ALS patients. This finding may reflect a loss of motor neuron

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03735.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—Familial amyloidotic polyneuropathy (FAP) is characterized by both sensimotor and autonomic dysfunction. Autonomic disturbance involving the gastrointestinal tract, the urinary bladder, the cardiac conduction system, and the peripheral circulation has been described. In this study simple, non‐invasive tests of autonomic function based on heart rate variability were applied to 12 patients with FAP and 12 healthy volunteers. The heart rate variation during normal breathing, deep breathing and during tilt from recumbent to standing position was measured. All tests showed significantly less heart rate variation in patients than in controls and the heart rate variation decreased with increasing severity of neurological disability, but the small number of patients in our study does not allow any further comparison between subgroups. Our study thus indicates impaired cardiovascular autonomic function in patients with FAP and we believe that these findings might also be of importance in other forms of systemic amyloido

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03736.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—Neurotrophic activities in human serum and post‐mortem muscle and spinal cord of possible relevance to pathophysiological mechanisms in amyotrophic lateral sclerosis (ALS) were studied. Tests includedin vitroassays for nerve fibre outgrowth from sympathetic ganglia and for survival promotion of dissociated ciliary neurons, both types of neurons, of chicken embryo origin. Extracts of postmortem biceps muscle promoted survival of ciliary neurons in a dose‐dependent manner. Half‐maximum effect was found at a protein concentration of about 450 μg/ml for both ALS and control muscle. Ventral horn extracts were about 5 times as efficient as muscle in promoting neuron survival, again with no differences seen between control and ALS samples. Sera from patients suffering from ALS as well as normal sera did not enhance survival of ciliary neurons to any considerable extent, nor did they induce fibre outgrowth from sympathetic ganglia. Both groups of sera, if present above 5% in the medium, suppressed fibre outgrowth induced by added nerve growth factor (NGF). Sera from some of the ALS patients impaired survival in dissociated ciliary neurons supported by a trophic activity in choroid extract. The results do not indicate major neurotrophic deficits as the cause of ALS disease but suggest that a neurotoxic mechanism may be

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03737.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—Two patients, in whom visual disturbance (Case 1) and sudden hemiparalysis due to a hemorrhagic lesion (Case 2) had led to craniotomy and histological diagnosis of giant cell glioblastoma, each had an unexpectedly long survival period of 7 and 9 years, respectively. Radiologically, the tumours were well demarcated, but without any distinguishing features, by comparison with glioblastomas in general. The tumours, to a great extent, consisted of cells with large, bizarre multiple nuclei. The highly pleomorphic cells displayed strong cytoplasmic GFAP immunopositivity, which suggested an astroglial origin. Thus, these tumours were considered a variant of glioblastoma (“giant cell glioblastoma”) with a more favourable prognosis than experienced by most patients with gliobla

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03738.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—Two cases of myoclonic encephalopathy due to bismuth salts intoxication are reported. In both, treatment with dimercaprol led to clinical recovery. This therapy was shown to enhance bismuth clearance. We also present data on the CSF metabolites dopamine, norepinephrine and serotonin of one patien

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03739.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—The long‐term prognosis and quality of life of 201 patients admitted to hospital with reversible ischemic attacks (RIA) were estimated in a prospective study. The median follow‐up time was 58 months. Further RIAs were reported by 91 patients (45%) and 48 (24%) suffered a stroke. The risk of stroke was markedly higher in the first 6 months after RIA, after which the annual stroke rate was rather constant with an average of 4.8%, about 8 times higher than expected. The average annual mortality rate for the RIA patients was 5.9%, which is significantly higher than expected. Cardiovascular deaths accounted for more than half of all deaths, stroke for one fourth. Life‐table analysis of subgroups disclosed a much more favorable prognosis for women under 60 years. High systolic blood pressure, diabetes, and previous myocardial infarction were identified as risk factors. The occurrence of RIA had significantly influenced the quality of life and occupational status for the majority of the patients, even for those who did not suffer a subsequent stroke. Decreased working capacity, general asthenia and fatigue and impaired memory were the most common complaints. We conclude that RIA may be a more serious vascular event than generally believed. Apart from carrying a substantial risk of stroke and death, even a single RIA can cause permanent psychological dysfunction influencing the quality

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03740.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—A large Japanese family with probable Machado‐Joseph disease (MJD) is described. Detailed neurological examination in 12 patients from 3 generations revealed variable combinations of cerebellar ataxia, ocular paresis, difficulty in eye‐lid opening, bulging eyes, facial “myokymia”, facial dystonia, pyramidal signs, extrapyramidal signs, and peripheral neuropathy. Mode of inheritance was in all likelihood autosomal dominant. Blood components were typed for 19 conventional chromosome markers. Although association of the affected members with the PGM1system was high, linkage analysis failed to reveal any markers studied with a lod score higher than 3. The unique constellation of symptoms appeared sufficient to rule out other types of spinocerebellar degeneration. When there is a typical case in a given family, MJD appears to be a clinically recognizab

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03741.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—The release of leukotriene C4(LTC4), an important 5‐lipoxygenase product of the arachidonic acid metabolism from polymorphonuclear leucocytes (PMNLs) of guinea pigs with experimental allergic encephalomyelitis (EAE), the animal model of MS, has been found to be significantly increased compared with healthy animals. Subsequently, the dual cyclo‐oxygenase and 5‐lipoxygenase inhibitor BW755C was applied to 15 guinea pigs with EAE. Two control groups (15 each) were treated with the cyclo‐oxygenase inhibitor indomethacin or physiological saline, respectively. In the BW755C treated group, no animal developed symptoms of the disease in contrast to, respectively, 5 and 3 animals in the 2 other groups. Histological examination of the CNS revealed a highly significantly lower inflammation score in the BW755C treated animals, and the release of LTC4from PMNLs was highly significantly decreased in this group compared with each of the others. The findings suggest that the vascular permeability enhancing LTC4plays a pathogenetic role in EAE and indicate that inhibition of this sulfidopeptide leukotriene suppresses the disease. Therefore, the application of leukotriene inhibitors could contribute to the future treatm

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03742.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—A new technique for quick objective and quantitative determination of important aspects of the motor handicap in movement disorders is presented. A compound, but natural, test movement was used to find out if the degree of dysfunction in postural, locomotor and manual motor functions differed among the patients and if medication influenced these functions differently. After 12 h without medication, 16 patients with Parkinson's disease showed a movement time between 1.5 and 13.6 times that of an age‐matched normal subject and a greater performance variability on repeated examination. In some patients the increase of test movement time was caused mainly by the locomotion component while in others the time for the postural or manual part of the movement was more markedly augmented. Thus, a specific motor disability profile was found for each patient and expressed in quantitative terms. The effects of 1‐dopa treatment were quantified in each pa

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03743.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT—The action of vinpocetine (14–ethoxycarbonyl‐(3α, 16α‐ethyl)‐14, 15 eburnamine) on calcifications in liver, kidney, and CNS tissues from cholesterolinduced atherosclerosis in 16 experimental rabbits was studied. Three of 4 groups (4 rabbits each group) feeding on a cholesterol‐rich diet developed atherosclerosis after 3 months. Two of the 4 groups were also fed vinpocetine supplements and analysis by neuturon activation method showed a decrease in calcium content in CNS tissues and in liver and kidney. Results suggest that vinpocetine acts to decalcify cholesterolinduced atherosclerotic lesions in

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1989.tb03744.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY