摘要:

Introduction‐ The purpose of the study was to evaluate the effect of pregnancy and childbirth on the longterm prognosis for women with multiple sclerosis (MS).Method and material‐ A cohort of 39 women with definite MS were identified on 1.1.1986 using a reproducible selection method. The investigation was initiated in 1986 when handicap was evaluated by Kurtzke Disability Status Score (DSS). At a 5‐year follow up 4 had died and 6 could not participate. There were 29 women in the investigation of whom 7 were childless, 10 had onset of MS at least 6 months after last childbirth, and 12 had onset of MS before or in connection with childbirth. Age and disease duration of the group was uniform.Result‐ At follow up the DSS significantly deteriorated (p = 0.008). The deterioration was seen particularly for childless women (p = 0.03) and women with onset of MS before or in connection with childbirth (p = 0.005).Conclusion‐ On the basis of this prospective investigation and the literature, it may be concluded that it is unlikely that pregnancy and childbirth have an influence on the longterm prognosis for MS. However, the conclusion must be interpreted with caution as the number of patients

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02728.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Seasonal outbreaks of an acute ataxic syndrome occur annually in parts of South‐Western Nigeria, characterized by cerebellar ataxia, nystagmus and varying levels of impaired consciousness following consumption of the roasted larvae ofAnaphe venataButler (Lepidoptera, Notodontidae). An investigation of an epidemic in Ikare, headquarters of the Akoko north‐east local Government in Western Nigeria (pop. 60,000) in the 1993 disease season is reported. The diagnosis of seasonal ataxia was verified in 34 consecutive new admissions (M:F 1: 3.25, median age 29 years, range 2–70 years). All were of low socio‐economic status, and had consumed the larvae ofAnaphe venataprior to the onset of disease. There were 1,126 admissions for the seasonal ataxic syndrome in Ikare in the 1993 season, with an estimated attack rate of 1.87%. The peak incidence was in August, when patients with the syndrome accounted for 71% of all hospital admissions. There was no mortality. Control measures included therapy with high‐potency multivitamins and health

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02729.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Introduction‐ We planned a descriptive study on the incidence of intracranial gliomas spanning a 16‐year period (1976–1991) in the Local Health Service 31 of Ferrara, Northern Italy.Material and methods‐ We used a complete enumeration approach by reviewing all the possible sources of case collection available in the study area.Results‐ The mean annual incidence rate was 5.8 new cases per 100000 population (6.96 for men and 4.78 for women; p<0.05), resulting in 4.7 per 100000 when directly adjusted to the Italian population. The age‐specific incidences showed a small peak in childhood, an increase with age, reaching a maximum in the age group 60 to 64 and then a decline in the elderly. This pattern is similar for both sexes. The adjusted rates increased from 3.94 per 100000 population in the first five‐year period to 5.6 per 100000 in the third (a nonsignificant difference). The distribution of cases within the study area was substantially uniform.Conclusion‐ The incidence rates of Ferrara fell into the middle‐high values so far reported and confirmed the male preponderance found in previous studies. The age‐related pattern is similar to that observed, with few exceptions, in other surveys. Like other authors we did not find a significant temporal trend, although the incidence rates tended to increase with time. The data encourage further, wider epidemiological studies of a

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02730.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Introduction‐ The well‐established relationship between myasthenia gravis (MG) and HLA antigens varies among different ethnic groups. In Caucasians B8 and/or DR3 alleles have been found associated with young MG women without thymoma and with high titres of acetylcholine‐receptor antibody (AChR Ab). An increased frequency of haplotype HLA‐A3, B7 and/or DR2 has been observed in older MG patients with low AChR Ab levels. So far, there is no convincing evidence for an association between a specific haplotype HLA and ocular MG or MG with thymoma. MG subjects often show other concurrent autoimmune disorders suggesting a more general inherited predisposition to autoimmunity. We performed a community‐based study to verify the HLA‐A, B, C, DR and DQ profile on ethnically homogeneous MG patients and with the aim to estimate the frequency of concurrent autoimmune diseases and to compare HLA phenotypes to autoimmune status in different MG patients groups.Methods‐ Forty‐seven patients, living in the province of Ferrara, were followed‐up in our neurologic department and typed for HLA Antigens. In addition a set of immunological laboratory tests was performed.Results‐ We found a trend towards an increased B8 and DR3 frequencies in total affected population; an association between B8 allele and early onset of generalized MG sustained by thymic hyperplasia. The DR3 allele is statistically associated with the presence of additional autoimmune disorders.Conclusions‐ Our data support the hypothesis of a genetically‐based heterogeneity of the disease and show an increased prevalence of associate autoimmune co

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02731.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Introduction‐ Cerebral blood vessels are innervated by sympathetic nerve fibres storing neuropeptide Y (NPY), parasympathetic nerves storing acetylcholine, vasoactive intestinal peptide (VIP) and sensory afferent fibres containing calcitonin gene‐related peptide (CGRP), substance P (SP) and neurokinin A. In experimental studies on subarachnoid haemorrhage (SAH) there are indications that perivascular peptides are involved. In the present study we have in man measured the levels of NPY, VIP, SP and CGRP in brain vessels of patients that have suffered a fatal SAH and compared this with the levels encountered in subjects that died of an extracerebral cause. Material and methods – Vessels from patients who have died from SAH or nonSAH were obtained during autopsy performed within 24 hrs after death. The peptides were extracted and fractionated with reversed phase liquid chromatography (HPLC). The levels of NPY, VIP, SP, and CGRP were measured with radioimmunoassay. Vasomotor responses of human cerebral arteries wereResults– Human cerebral vessels contained NPY, VIP, CGRP and SP which eluted at the same positions as the authentic peptides. The level of CGRP was significantly lower (p<0.01) in arteries removed from SAH patients as compared to control subjects. The level of SP was not changed, if anything it tended to be increased after SAH. The levels of NPY and VIP were not significantly altered after SAH. In isolated brain vessels α‐CGRP was a potent vasodilator of arteries precontracted with whole blood, prostaglandin F2αor endothelin. It had a poor effect on vessels precontracted with 60 mM potassium.Conclusion– The evidence suggest that the trigemino‐cerebrovascular system, storing CGRP and SP, is to a differential degree involved in the pathophysiology of SAH in man and supports the hypothesis of an exhaustion of CGRP as one important factor in the development of late spasm occ

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02732.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Ninety patients with a previous subarachnoid haemorrhage (SAH) were given a set of memory tests comprising immediate free recall of words (indexing long‐term memory, LTM, and short‐term memory, STM), final free recall of words (indexing LTM), final cued recall of words (indexing LTM), and a digit span test (indexing working memory, WM). Patients with a large amount of blood on CT, carried out within 72 h of the bleed, showed LTM as well as STM dysfunction, whereas patients with a small amount of subarachnoid blood evidenced only STM dysfunction. Patients with the ruptured aneurysm located on the anterior cerebral artery, however, constituted an exception with dysfunction of both LTM and STM together with intact WM, independent of the amount of subarachnoid blood. Also, patients with internal carotid artery or middle cerebral artery aneurysms and large volume SAH displayed LTM dysfunction, but differed concerning STM, the former showing intact STM and the latter showing STM dysfunction. Thus, it appears, that the combined information from factors such as the amount of subarachnoid blood and the location of the ruptured aneurysm is of vital importance for explaining the different patterns of memory dysfunctions after

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02733.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

The overall function, pain and mood disturbances of 19 patients with spinal arteriovenous malformations (AVM), treated by embolization between 1983 and 1988, were studied. The after‐care had taken place in different hospitals and clinics. The patients demonstrated markedly poorer physical function (Sickness Impact Profile) and poorer psychological well‐being (Mood Adjective Check List) than control population samples and a comparison group of traumatic incomplete paraplegies. The degree of decrease of mood levels implied possible depressive disorder (Hospital Anxiety and Depression scale) in 16% of the patients and differed from that of the paraplegic comparison group. Furthermore, the AVM patients reported more disturbance of their family and social life than the paraplegics and they were more seldom gainfully employed. Patients recorded a wide range of pain scores, significantly worse than the paraplegics, and their pain was closely linked to overall quality of life (QL) perception. The QL scores were consistently related to all measures of functional and emotional status, but no connection with neurological lesion levels or medical complications was found. Specialised programmes after embolization, such as those offered in spinal injury units, would appear appropriate for AVM patients to improve their physical functioning and to provide a more rewarding social l

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02734.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

It has earlier been claimed that clinical improvement of patients with Parkinson's disease is obtained by treatment with NADH. This has to be verified by double‐blind, clinical studies and measurement of biochemical effects of the treatment. In a double blind study five patients with clinically moderate Parkinson's disease were treated with NADH, 25 mg, given intravenously once a day for four days. Then they were given 25 mg NADH intramuscularly after 2 and 4 weeks. Disability scores were determined before each treatment and two weeks after the final injection. A control group (n = 4) with the same degree of Parkinson's disease obtained sodium chloride with the same schedule. According to the Unified Parkinson's Disease Rating Scale a tendency to clinical improvement was seen after the iv infusions in both treatment and placebo groups. However, the changes were not statistically significant, and no changes occurred during the following weeks. No changes were found neither in the study nor the control group regarding cerebrospinal fluid concentrations of dynorfin, metenkefalin, somatostatin, hydroxy‐methoxy‐phenylglycol, homovanillic acid and 5‐hydroxyindole acetic acid. The results indicate that no great changes are obtained after short‐term treatment of parkinsonian patients with NADH, neither clinically nor bioc

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02735.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Transcranial magnetic stimulation (TMS) is a non‐invasive diagnostic method particularly suited to investigation of the long motor tracts. The clinical value of this method in many cortical and subcortical diseases has been well established, but comparable studies for most spinal cord diseases have still to be made. Forty patients in whom spinal cord disease was established by clinical examination, cerebrospinal fluid examination, and magnetic resonance imaging (MRI) were studied by means of somatosensory evoked potentials (SEP, median and tibial nerve stimulation) and magnetic motor evoked potentials (MEP, first dorsal interosseus and tibialis anterior muscle recordings after transcranial and spinal stimulation). The underlying pathology was neoplastic (n= 16), inflammatory (n= 15) or ischemic (n = 9). Clinical signs and symptoms ranged from slight sensory disturbances to complete paraplegia and had developed within minutes (ischemia) or over many years (benign neoplastic disease). The overall frequency of pathological SEP was slightly higher than that of MEP (78% vs 68%) which was statistically not significant (p>0.05). This was also true for the subgroups, except for pure motor disorders, which gave the same yield for both methods. Decreased amplitudes or absence of MEP were more frequent in neoplastic than in inflammatory lesions (75% vs 33%, p0.05, n. s.). Pathological SEP were found in 75% of patients presenting with pure motor abnormalities, while pathological MEP were found in 30% of patients with pure sensory disturbances. We conclude, in common with the SEP, the MEP are helpful in the examination of spinal cord diseases, even in subclinical disturbances, although the SEP would seem to yield a larger percentage of pathological result

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02736.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

The clinical manifestations and mitochondrial DNA (mtDNA) mutations in a Taiwanese family with a female proband exhibiting mitochondrial myopathy, encephalopathy, lactic acidosis and stroke‐like episodes syndrome are reported. Clinically, the proband had a stroke‐like episode with right hemiparesis, hemianopsia and mental dysfunction as well as short stature, hearing impairments, and elevated lactate levels. Brain magnetic resonance images showed multiple increased signal intensities over the left frontal, parietal and temporal areas. There were no ragged‐red fibers, but paracrystalline inclusion bodies were shown in the muscle biopsies under electron microscopic examination. A deficiency of NADH‐CoQ reductase was also found in biochemical studies of the muscles. The family survey revealed no abnormal findings except for headache and episodic vomiting in her mother. The molecular analysis of mtDNA disclosed a mutation from A to G at the nucleotide pair 3243 of the mitochondrial transfer RNALeugene in the blood, hair follicles and/or muscle of the maternal relatives. A characteristic finding of the MELAS family is variation of percentage of mutated mtDNA in various tissues and individuals. However, a higher proportion of mutated mtDNA was noted in the proband than that in the asymptomatic or oligosymptomatic family members. From the data, the variable clinical phenotypes in this MELAS family can be explained at least partly, by the different proportions of mutant mtDNA in the target tissues of the profound and maternal re

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1994.tb02737.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY