摘要:

Sera from 90 myasthenia gravis (MG) patients were examined for antibodies against skeletal muscle citric acid extract (CA) antigens by ELISA and indirect haemagglutination (IHA). 31% (ELISA) and 24% (IHA) of the sera were positive, and the results of the two tests were in good agreement. 18 of 28 patients positive in ELISA had IgG CA‐antibodies, 10 had IgM and 3 IgA CA‐antibodies. The patients with IgG antibodies had a high mean age of MG disease onset, and 9/14 thymectomized patients had a thymoma. Patients with IgM CA‐antibodies had a much lower age of onset and 4/5 thymectomized patients had thymus hyperplasia. IgM antibody positive patients had a long disease duration, indicating that they produce IgM antibodies for an indefinite period of time without switching to IgG antibody produ

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04037.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

When cimetidine (CIM) is administered together with the anti‐epileptic drug carbamazepine (CBZ), a drug interaction may cause a rise in plasma concentrations of CBZ, which can result in CBZ‐related toxic symptoms. The aim of this cross‐over study was to investigate whether CIM influences the disposition and kinetics of the new anti‐epileptic oxcarbazepine (OXC) and its metabolites. In 8 healthy volunteers there was no difference in AUC, Cmax or tmax when OXC was administered either with or without CIM. The results of this study suggest that in the treatment of epilepsy OXC offers an important advantage over the established anti‐epileptics, especially when concomitant therapy with CIM is

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04038.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

We examined clinically and electrophysiologically 22 adolescents suffering from hyperphenylalaninemia. Three of 16 treated patients showed slight impairment of proprioception; their examination was otherwise unremarkable. Subclinical deficits were present in the central motor and sensory pathway. In addition, we had evidence of a minor sensory neuropathy: 32% of the 22 patients had subclinical deficits in the visual system. However, in contrast to earlier reports we saw no relation to the degree of metabolic control. Our results suggest that treated adolescent patients with hyperphenylalaninemia show neurological dysfunction that may I be related to morphological, as well as pharmacological changes.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04039.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

To describe the epidemiological characteristics of epilepsy in a northern area of Chile, an investigation was conducted in four localities in the province of Copiapó (population of 17 694). Based on 314 cases of active epilepsy, the prevalence per 1000 at June 30, 1988 was 17.7. The average annual incidence for the period 1984–1988 was 113 per 100000. Partial seizures were the most frequent type of seizure diagnosed (54.1%). Antecedents considered as possible etiological factors were found in 29.9% of cases. According to age of onset, 64.6% had their first attack before 15 years. Middle and low socioeconomic classes had higher prevalence rates of epilepsy. We compare our results with previous Latin‐American stu

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04040.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Five families with late onset autosomal dominant spinocerebellar ataxia, were studied. Linkage between the disease and HLA loci on the short arm of chromosome 6 was shown in the two largest pedigrees. Clinical study of 26 patients and neuropathological study in one are reported. The disease was characterized by cerebellar and pyramidal involvement variably associated with cranial nerve and peripheral nervous system disorders. A remarkable concordance of the main clinical features was observed in patients with similar disease duration. Comparison with previous reports of HLA‐linked spinocerebellar ataxia kindreds showed differences in clinical phenotypes. Although these might be due to genetic variation, the hypothesis is suggested that the phenotype might appear more homogeneous if disease duration is taken into accoun

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04041.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Sixteen former rayon viscose workers were investigated four years after the exposure to carbon disulfide was discontinued. Median age was 58 years (range 43–65 years), median exposure time was 17 years (range 10–35 years). Encephalopathy was diagnosed in altogether 14 workers. To further explore pathophysiological mechanisms, cerebrovascular investigations were employed. Doppler ultrasound examination of the precerebral vessels in 15 workers showed a slight stenosis of the left internal carotid artery in one. Regional cerebral blood flow investigation (rCBF) with single photon emission computerized tomography (SPECT) with Xenon‐133 gas was performed in 14. There was no significant difference from a control group. Regional side‐to‐side asymmetries beyond reference limits were demonstrated in eight workers. The abnormalities were modest, but may indicate a tendency toward focal blood flow disturbances in workers with long‐term exposure to carbo

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04042.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

We evaluated, by using electrophysiological techniques, 29 patients with juvenile Parkinson's disease (JP), who had no known causes or clinical signs of neuropathy. Electromyographic evidence of chronic partial denervation with reinnervation was observed in nine patients (34.6%). Abnormalities of motor conduction in the common peroneal nerve were present in four (13.8%), Sural sensory conduction in nine (31.9%) and sympathetic skin response (SSR) in eleven (37.9%) patients. The symptoms of dysautonomia correlated poorly with changes in SSR. These abnormalities were independent of age at onset, duration or severity of the disease and antiparkinsonian drugs used. This study suggests that the peripheral nervous system is involved in JP in more than 50% of patients. The commonly observed symptoms of dysautonomia in Parkinson's disease may have a peripheral origin.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04043.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

Vitamin B12concentrations were determined in serum and cerebrospinal fluid (CSF) of 32 controls and 102 patients with dementia. The dementias were classified as Alzheimer's disease (AD), senile dementia of Alzheimer type (SDAT) and multi‐infarct dementia (MID). A substantial number of patients (n = 42) could not be assigned to any of these diagnostic groups, as their dementias were of non‐AD/SDAT and non‐MID types. They were instead assigned to a group called non ultra descriptum (NUD). CSF B12correlated significantly with serum B12. There were no statistically significant differences in serum B12levels between the groups. Although with considerable overlap, CSF B12concentrations and CSF/serum B12ratios were significantly lower in the NUD group than in the control group. The NUD group had significantly lower CSF/serum B12ratios than the group of patients with AD SDAT. There was significant male predominance in the group of demented patients that had low CSF/serum B12ratios outside the bivariate reference region. CSF and serum B12levels appear insufficient as measures of the true brain vitamin B12status. It may be a more dynamic approach to use the CSF/serum B12ratio as an indication of transport function across the blood brain barrier, and possibly also across the CSF brain cell ba

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04044.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

The present study analyses one of the largest IDP series published as yet: 266 cases, hospitalised between 1950 and 1983, conventionally treated and uniformly supervised. There was GBS in 84%, chronic IDP in 13%, polyneuritis cranialis in 1.5%, Miller Fisher and predominantly sensory neuropathy in 0.8% each. Numerous features including preceding events, course and outcome have been investigated. Latencies between onset and maximal deficit lacked (expected) bimodality. Sensory involvement was severe, mortality (18%) and maximal disability (20% requiring ventilation) high, autonomic dysfunction (71%) prominent. Statistical comparison of various features confirmed old age, rapid onset and need for ventilation as unfavorable predicting factors. However, preceding infection, bulbar paralysis and onset of paresis in proximal muscles evolved as unfavorable prognostic features as well.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04045.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY

摘要:

The influence of the rheological properties of the blood on cerebral perfusion is still unresolved. Data on normal subjects are lacking and difficulties arise regarding the effect of blood viscosity owing to its close relationship with hematocrit. For these reasons we have studied the relationship between two rheological hematocrit‐independent parameters and CBF in normal subjects of various ages. 36 normal volunteers, aged 20–74, free from risk factors, have been studied. CBF was measured by the Xenon inhalation method. Erythrocyte aggregability was expressed as Mean Erythrocyte Aggregation Index (MEA). Plasma fibrinogen concentration was evaluated by the coagulativc method in 26 subjects. No correlation was found between CBF and MEA or fibrinogen in the subjects under the age of 45. A significant negative correlation was found between CBF and MEA (p = 0.015) and between CBF and fibrinogen (p = 0.011) in the subjects over 45.These data show that cerebral perfusion is influenced by the rheological properties of the microcirculation only with aging. We suggest that a “rheological autoregulation” exists and that it works properly in youth, only to be lost with physiological aging. This finding can be of significance in the pathogenesis of cerebrovascular disease processes in

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1992.tb04046.x

出版商:Blackwell Publishing Ltd    

年代:1992

数据来源: WILEY