摘要:

A total of 43 patients with multiple sclerosis (MS) consecutively admitted to the Multiple Sclerosis Rehabilitation Hospital in Haslev completed a Danish version of the Laman&Lankhorst Questionnaire (LLQ) on quality of life (qol), and the Beck Depression Inventory (BDI). Twenty‐one of the 43 pts completed the questionnaires at discharge as well. All patients received the standard multidiciplinary rehabilitative treatment. The purpose was to evaluate the LLQ as a measure of qol and as an outcome measure. The 43 MS‐patients had low weighted qol scores on the following items: readily tired, fatigue, dependence on other people, worry about deterioration and walk. Correspondingly, the BDI showed high scores on fatigability, work difficulty and somatic preoccupation. The correlation between LLQ and BDI was statistically significant (0.54, p<0002), but weak. ANOVA with repeated measures (LLQ and BDI at admission and discharge) was applied to evaluate rehabilitation outcome in 21 patients. LLQ: The patients experienced a significant reduction in disability on fatigue and mood; and in the importance of being able to climb stairs and to work. The weighted qol‐scores increased significantly on physical endurance, work and mood. BDI scores showed a significant decrease on pessimism, irritability, loss of libido and total BDI‐score. The changes measured by the LLQ were mainly on behavioral aspects. As the correlation coefficient was only 0.54, the LLQ and the BDI seem to address different aspects of quality

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00512.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The myelin basic protein (MBP) gene is a candidate locus for disease susceptibility in multiple sclerosis (MS). In the present study a part of the tetranucleotide (TGGA)nrepeat polymorphism 5’to the MBP gene was examined in 90 Danish MS patients and 106 controls. Lymphocyte DNA was isolated and used in PCR assay. The PCR fragments produced were separated by agarose and acrylamide electrophoresis. Hereby we found three different bandpattems i.e. a homozygote with a 450 bp fragment, a homozygote with a fragment 375 bp and a heterozygote with both bands present. The 450 bp fragment occurred significantly more often among MS than in the control group and the 375 bp fragment was underrepresented among MS than in the control group. The differences between incidence of the three band pattern in the MS and the control group were significant different at 1% level. Our study thus indicate that there is an association between MS and a length polymorphism of the 5’end to the MBP gene in Danish MS patie

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00513.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

In multiple sclerosis (MS) autonomic cardiovascular dysfunction is an uncommon, but potentially dangerous event, to which studies of spectral analysis of heart rate variability have not been applied, yet. Material and methods – We studied 16 patients with definite MS (11 women and 5 men, mean age 30.3 ± 7.4 yrs., mean EDSS 2.06±1.42) and 16 sex‐ and age‐matched healthy controls. Besides cardiovascular reflex tests (valsalva manoeuvre, deep breathing, lying to standing, Blood Pressure response to standing and sustained handgrip), each underwent spectral analysis of the R‐R interval short‐term variability at rest and after tilting, to detect three components: very low frequency (VLF), low frequency (LF) and high frequency (HF). A recent brain MRI was obtained from patients, to compare plaque characteristics with spectral parameters. Results – At cardiovascular reflexes, only four patients (25%) showed an impairment, mostly of a mild degree. VLF and LF at rest were lower in MS subjects than in controls (p<0.01). No significant correlation was found between spectral parameters and lesion area or localization as detected on MRI. Conclusions – Spectral analysis could usefully flank reflex tests to detect autonomic subclinical cardiovascula

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00514.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

IntroductionIn an attempt to establish the value of MRI, VEP, SEP, and biotesiometry in monitoring disease evolution we undertook a one year follow up study of 70 untreated patients with acute optic neuritis (ON).Material&methods– ON was monosymptomatic in 48 patients (bilateral in 10) and part of clinically definite multiple sclerosis (CDMS) in 22 patients, examined as mentioned below.Results– Results are given at onset and at follow up (in brackets). In monosymptomatic ON, brain MRI was abnormal in 53% (53%), VEP in the eye with acute ON in 79%>(71%), VEP in the clinically unaffected eye in 34% (47%), SEP in 25% (23%), and biotesiometry in 29% (17%). In CDMS, brain MRI was abnormal in 95% (95%), VEP in the eye with acute ON in 86% (77%), VEP in the clinically unaffected eye in 50% (64%), SEP in 55% (50%), and biotesiometry in 63% (53%). Only minor changes in test scores were observed after one year except for significant improvement of VEP in eyes with acute ON. Eight of 32 patients, characterized by at least one abnormal paraclinical test at onset of monosymptomatic ON, had developed CDMS versus none of 16 patients with normal paraclinical results (p = 0.03; Fisher).Conclusion– Patients with monosymptomatic ON with paraclinical signs of multifocal involvement at onset had an increased risk of developing CDMS. No single test predicted the evolution of CDMS, perhaps due to the relatively short follow up

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00515.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Patients with an established diagnosis of epilepsy were included in three groups on the basis of the absence (Group 2) or presence (Group 3) of epileptiform EEG discharges or subtle seizures (Group 4) during the cognitive assessment procedure. A separate age‐matched non‐epileptic control group (Group 1) was formed. Twenty‐five patients were included in each of the four groups. Thus, a total of 100 patients were investigated. The patients were assessed with continuous 21‐channel EEG and video‐monitoring, combined with cognitive testing. The results show consistently lower performance on cognitive tests for Group 4, the group with subtle seizures. The difference with the control group was significant for the intelligence subtests and for the complex information processing test (p<0.05). No transient cognitive impairment was found. The results are discussed in the light of possible factors that may be responsible for the lower test‐scores in the patients of Group 4: both the ictal effects of the seizures themselves, postictal effects and the effects of the epileptiform EEG discharges may have had an impact on cognitive performance. Finally the absence of evidence for transient cognitive impairment in a group with frequent epileptiform EEG discharges is discusse

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00516.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

To evaluate clinical characteristics and outcome of myasthenia gravis (MG) in aged patients (>60yrs), we retrospectively reviewed a continuous series of 122 myasthenic patients observed from January 1968 through December 1994. Patients with congenital, neonatal, or penicillamine‐induced myasthenia were excluded. Twenty‐five subjects (20%) were>60yrs. The male/female ratio was 3:2; 20% of patients had an ocular form and 86% were seropositive. Mediastinum CT scan revealed thymic changes in 14%. During the first five years of disease, 60% of patients with ocular form progressed towards a generalized form and 15% had clinical relapses. At the time of their last visit, 40% of patients were asymptomatic and 60% had improved on medication. No patient died because of myasthenia‐related causes. This study shows that MG in aged patients is characterized by prevalence in males, low frequency of ocular forms, low frequency of positive mediastinum CT which suggests low frequency of thymomas, high frequency of progression of ocular forms, and good response to corticosteroid th

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00517.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

A 43‐year‐old woman with recently diagnosed primary biliary cirrhosis developed antibody‐negative myasthenia gravis. She did not receive D‐penicillamine therapy. Clinical and immunological features of this patient are discussed. In previous reports on an association between primary biliary cirrhosis and myasthenia D‐penicillamine treatment was an obligate causal linkage between both

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00518.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Multimodal evoked potentials (PRVEP, BAEP, mSEP) were recorded in 56 HIV‐1 seropositive outpatients free from opportunistic CNS pathologies and/or overt HIV‐1 encephalopathy. EPs were altered in 17 of 39 (43.6%) seropositive subjects without AIDS (group A) and in 13 of 17 (76.5%) patients with AIDS (group B). A high incidence of subclinical alterations (30.8%) were found in group A patients. Significant BAEP (I‐III, III V, I‐V) interpeak latency and mSEP (N9‐N13, N9‐N20) conduction time prolongations were found in group A and B patients. PRVEP PI00 was significantly prolonged only in group B. An inverse relationship between BAEP interpeak latencies and CD4 count was found. Our findings support the hypothesis of an important role of immunodepression in the development of neurophysiologic abnormalities, together with a preferential involvement of acoustic pathways, in the course of HIV

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00519.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Objectives– The preferential involvement of living categories in naming impairment is well recognised in Herpes Simplex Encephalitis (HSE). In this paper we describe naming, neuropsychological and neuroradiological findings with seven fresh HSE cases.Material&methods– Patients were given a picture naming task that included 60 items belonging to 6 different categories (three living, i.e. fruits, vegetables and animals and three nonliving, i.e. furniture, vehicles and tools). In the statistical analysis several possible sources of bias as the frequency of the target word, the familiarity with the objects to name, the image complexity and other parameters were taken into account.Results– Four out of seven patients were significantly more impaired with living things. We describe their general cognitive profile and discuss the anatomo‐functional aspects of category dissociation.Conclusion– Language impairment, disproportionately severe for the naming of living exemplars, is frequently observed in HSE, is clinically relevant and should be specifically inv

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00520.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Objectives– Oculopharyngeal muscular dystrophy was first described by Taylor in 1915. A substantial number of cases have been reported from North America, the disorder being most frequent in Quebec, Canada.Material– The onset of disease is in middle life, most often presenting with ptosis and a slight degree of ophthalmoplegia, followed some years later by dysphagia and often by proximal limb weakness. The course is slowly progressive, but the dysphagia may become severe and has caused death by starvation in several cases. The disorder is transmitted autosomally dominant in families.Results‐The present report describes a large Norwegian family with 13 affected members of whom we have examined eight. Transmission is autosomally dominant, mode of presentation may be either by ptosis or by dysphagia. In rare cases gait difficulties may be the presenting symptom.Conclusion– Electromyographic and muscle biopsy examinations demonstrate a primary myogenic d

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb00521.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY