A comprehensive rehabilitation programme tailored to the needs of adults with muscular dystrophy.

OBJECTIVE: To assess if activities of daily living (ADL), coping and quality of life could be improved in adults with muscular dystrophy through a comprehensive rehabilitation programme. DESIGN: Quasi-experimental, controlled clinical study comparing patients with similar age and disease aspects. SETTING: Two different counties in Sweden, being either study or control setting. SUBJECTS: The study group comprised 37 adults (21 women, 16 men; mean age 50 years), while the control group comprised 39 people (25 women, 14 men; mean age 46 years). INTERVENTIONS: Four rehabilitation sessions tailored to different medical, physical and psychosocial needs of the patients, comprising a total of 10 days over a period of 18 months. MAIN MEASURES: ADL, the Mental Adjustment to Cancer Scale measuring coping strategies, the Sickness Impact Profile measuring health-related quality of life, the Hospital Anxiety and Depression Scale, and the Psychosocial Well-being Questionnaire. RESULTS: No significant differences were found between groups with regard to the outcome measures. There was increased dependence on others in ADL after 18 months in both groups, but it was more pronounced in the control group. Furthermore, a clear trend was observed in the data with regard to coping patterns, the control group using more coping strategies such as 'Helplessness/hopelessness' (P= 0.057), 'Anxious preoccupation' (P = 0.085) and 'Fatalistic' (P= 0.073) when being compared to the study group. CONCLUSIONS: No apparent effects on ADL were found from the rehabilitation programme, although there was a tendency of reduction of maladaptive coping patterns in the study group. This initial study may provide the rationale and basis for a randomized controlled trial.

Muscle involvement in juvenile idiopathic arthritis.

OBJECTIVE: An observational study of changes in muscle structure and the relation to muscle strength in juvenile idiopathic arthritis (JIA). METHODS: Fifteen children and teenagers (eight girls and seven boys) with JIA, aged 9-19 yr (mean age 16.1), were studied. Muscle biopsies were obtained from the anterior tibial muscle and were examined using histopathological and immunohistochemical methods. Muscle fibre types were classified and fibre areas measured. As markers of inflammation, the major histocompatibility complex (MHC) class I and class II and the membrane attack complex (MAC) were analysed. Results were compared with biopsies from the gastrocnemius muscle in 33 young (19-23 yr) healthy controls. Isometric and isokinetic muscle strengths were measured in ankle dorsiflexion. Strength was compared with reference values for healthy age-matched controls. Nerve conduction velocities were recorded in the peroneal and sural nerves. RESULTS: Four of the 15 muscle biopsies were morphologically normal. Eleven biopsies showed minor unspecific changes. Two of these also showed minor signs of inflammation. MHC class II expression was found in 4/15 patients, which was significantly more than in the healthy controls (P = 0.0143). The expression of MHC class I and MAC did not differ from that in the controls. The mean area of type I fibres was lower than that of type IIA fibres in 12/13 biopsies. Muscle strength was significantly reduced in the patient group. There was a significant positive correlation between muscle fibre area and muscle strength. Nerve conduction studies were normal in all cases. CONCLUSIONS: Changes in leg muscle biopsies appear to be common in children and teenagers with JIA. The presence of inflammatory cells in the muscle and expression of MHC class II on muscle fibres may be a sign of inflammatory myopathy. There are no findings of type II muscle fibre hypotrophy or neuropathy, as in adults with RA.

The expression of adhesion molecules in muscle biopsies: the LFA-1/VLA-4 ratio in polymyositis.

OBJECTIVES: The expression of three pairs of adhesion receptors and ligands was examined in 22 consecutive muscle biopsies showing morphological signs of inflammation. MATERIAL AND METHODS: The following groups were studied: patients with polymyositis (PM) (n=7), patients with myositis that did not fulfil criteria for PM, i.e. suspected PM (n=5), patients with other diseases, with no clinical signs of inflammatory myopathy (n=6), and a small group of non-PM inflammatory myopathies (n=4). The endothelial expression of ICAM-1, VCAM-1 and E-selectin was evaluated, as was the cellular expression of LFA-1, VLA-4 and SLex. In addition, the expression of MHC class I and II was studied. RESULTS: The ratio between the number of cells expressing LFA-1 and VLA-4 showed significant differences between the groups, with the lowest values in PM. CONCLUSION: The LFA-1/VLA-4 ratio should be suitable for diagnostic purposes. Our findings also indicate that the VLA-4/VCAM-1 system is important for chronic T cell inflammation in muscle, in line with findings in other "hidden" organs like joints and the central nervous system.

Stenting in small coronary arteries (SISCA) trial. A randomized comparison between balloon angioplasty and the heparin-coated beStent.

OBJECTIVES: The purpose of this study was to assess the clinical and angiographic benefits of elective stenting in coronary arteries with a reference diameter of 2.1 to 3.0 mm, as compared with traditional percutaneous transluminal coronary angioplasty (PTCA). BACKGROUND: The problems related to small-vessel stenting might be overcome using modern stents designed for small vessels, combined with effective antiplatelet therapy. METHODS: In five centers, 145 patients with stable or unstable angina were randomly assigned to elective stenting treatment with the heparin (Hepamed)-coated beStent or PTCA. Control angiography was performed after six months. The primary end point was the minimal lumen diameter (MLD) at follow-up. Secondary end points were the restenosis rate, event-free survival and angina status. RESULTS: At follow-up, there was a trend toward a larger MLD in the stent group (1.69 +/- 0.52 mm vs. 1.57 +/- 0.44 mm, p = 0.096). Event-free survival at follow-up was significantly higher in the stent group: 90.5% vs. 76.1% (p = 0.016). The restenosis rate was low in both groups (9.7% and 18.8% in the stent and PTCA groups, respectively; p = 0.15). Analyzed as treated, both the MLD and restenosis rate were significantly improved in patients who had stents as compared with PTCA. CONCLUSIONS: In small coronary arteries, both PTCA and elective stenting are associated with good clinical and angiographic outcomes after six months. Compared with PTCA, elective treatment with the heparin-coated beStent improves the clinical outcome; however, there was only a nonsignificant trend toward angiographic improvement.

Changes in a rat facial muscle after facial nerve injury and repair.

This study describes changes in a rat facial muscle innervated by the mandibular and buccal facial nerve branches 4 months after nerve injury and repair. The following groups were studied: (A) normal controls; (B) spontaneous reinnervation by collateral or terminal sprouting; (C) reinnervation after surgical repair of the mandibular branch; and (D) chronic denervation. The normal muscle contained 1200 exclusively fast fibers, mainly myosin heavy chain (MyHC) IIB fibers. In group B, fiber number and fiber type proportions were normal. In group C, fiber number was subnormal. Diameters and proportions of MyHC IIA and hybrid fibers were above normal. The proportion of MyHC IIB fibers was subnormal. Immediate and delayed repair gave similar results with respect to the parameters examined. Group D rats underwent severe atrophic and degenerative changes. Hybrid fibers prevailed. These data suggest that spontaneous regeneration of the rat facial nerve is superior to regeneration after surgical repair and that immediacy does not give better results than moderate delay with respect to surgical repair. Long delays are shown to be detrimental.

Women react with more myocardial ischemia and angina pectoris during elective percutaneous transluminal coronary angioplasty.

BACKGROUND: Women have been considered to be at higher risk of complications relating to percutaneous transluminal coronary angioplasty (PTCA) than are men. One reason for this sex-related difference could be the ischemic response of myocardium during the procedure. OBJECTIVE: To investigate whether there are sex-related differences in ischemic response of myocardium during elective PTCA. METHODS: Consecutive patients (n = 192, of whom 48 were women), were subjected to vectorcardiography during the PTCA procedure. Vectorcardiographic variables, magnitude of ST-segment vector (ST-VM), and magnitude of ST-segment vector change (STC-VM) were studied. RESULTS: Women were older (63 +/- 10 versus 56 +/- 10 years, P< 0.001) than men in our study and more often had diabetes mellitus and hypertension. Women less often had stents implanted (24 versus 50%, P < 0.01) and they were subjected to fewer balloon inflations (P < 0.001), with a total inflation time shorter than that for men (P< 0.001). Maximum STC-VM was 25% greater for women (P < 0.05). Women reported greater maximum pain (P < 0.05) and nitroglycerine was more frequently used for them during PTCA (P < 0.05). Occurrence of episodes of residual ischemic STC-VM (the difference between total number of episodes and number of balloon inflations) was more common for women (3 +/- 5 versus 1 +/- 3, P< 0.01). Duration of residual ischemic STC-VM episodes (the difference between total duration of episodes and duration of balloon inflations) was longer for women than it was for men (242 +/- 275 versus 148 +/- 233 s, P < 0.05). In a stepwise multivariate analysis and for a matched case-control group, episodes of residual STC-VM and duration of residual STC-VM episodes still indicated that there was an independent sex-related difference (P < 0.01 and P < 0.01, respectively). CONCLUSIONS: Women more commonly develop vectorcardiographic signs of severe myocardial ischemia, more frequently experience episodes of ischemia and report more severe angina pectoris during elective PTCA than do men.

Prognostic role of on-line vectorcardiography as regards repeat revascularization after successful coronary angioplasty.

This study evaluated the prognostic significance of continuous on-line vectorcardiography (VCG) during elective coronary angioplasty (percutaneous transluminal coronary angioplasty, PTCA). Patients (n = 192, mean age 58 +/- 10), treated with elective and initially successful PTCA, were included. VCG monitoring was started before start of the PTCA procedure and was carried out during the entire procedure. ST vector magnitude (ST-VM) was monitored. A 6-month follow-up was obtained. Main outcome measures were the frequency of cardiac events and revascularization during follow-up. During follow-up, 1 patient died, 6 suffered a nonfatal myocardial infarction and 50 were revascularized. Angiography revealed restenosis in 88% of the patients who had a revascularization. In the total patient group, the VCG predictor of revascularization was the total ischemic time of all ST-VM episodes (p = 0.05). Clinical predictors of revascularization were diabetes mellitus (p < 0.01), a more severe type of lesion (type B; p < 0.01), percent post-PTCA stenosis (p < 0.05), nominal balloon size (p < 0.01), maximum balloon pressure (p < 0.05) and no stent implanted (p < 0.001). In a multivariate analysis all the above significant univariate variables of revascularization were entered. Total ischemic time of ST-VM (p < 0.01) was the best variable giving independent prognostic information. In the nonstent group, total ischemic time of ST-VM (p < 0.01) was the only independent predictor of a further revascularization. In conclusion, VCG monitoring during elective PTCA gives on-line information that identifies patients at an increased risk of a revascularization during 6 months after the initial procedure.

Randomized intergroup trial of cisplatin-paclitaxel versus cisplatin-cyclophosphamide in women with advanced epithelial ovarian cancer: three-year results.

BACKGROUND: A randomized trial conducted by the Gynecologic Oncology Group (GOG, study #111) in the United States showed a better outcome for patients with advanced ovarian cancer on the paclitaxel-cisplatin regimen than for those on a standard cyclophosphamide-cisplatin regimen. Before considering the paclitaxel-cisplatin regimen as the new "standard," a group of European and Canadian investigators planned a confirmatory phase III trial. METHODS: This intergroup trial recruited 680 patients with broader selection criteria than the GOG #111 study and administered paclitaxel as a 3-hour instead of a 24-hour infusion; progression-free survival was the primary end point. Patient survival was analyzed by use of the Kaplan-Meier technique. Treatment effects on patient survival were estimated by Cox proportional hazards regression models. All statistical tests were two-sided. RESULTS: The overall clinical response rate was 59% in the paclitaxel group and 45% in the cyclophosphamide group; the complete clinical remission rates were 41% and 27%, respectively; both differences were statistically significant (P =.01 for both). At a median follow-up of 38.5 months and despite a high rate of crossover (48%) from the cyclophosphamide arm to the paclitaxel arm at first detection of progression of disease, a longer progression-free survival (log-rank P =.0005; median of 15.5 months versus 11.5 months) and a longer overall survival (log-rank P =. 0016; median of 35.6 months versus 25.8 months) were seen in the paclitaxel regimen compared with the cyclophosphamide regimen. CONCLUSIONS: There is strong and confirmatory evidence from two large randomized phase III trials to support paclitaxel-cisplatin as the new standard regimen for treatment of patients with advanced ovarian cancer.

On-line vectorcardiography during elective coronary angioplasty indicates procedure-related myocardial infarction.

BACKGROUND: Increased creatine kinase concentrations after elective percutaneous transluminal coronary angioplasty (PTCA) have been shown to be associated with increased late cardiac mortality. OBJECTIVE: To evaluate the potential of continuous on-line vectorcardiography during elective PTCA to identify procedure-related myocardial infarction. METHODS: Patients (n = 192, ages 58 +/- 10 years), treated with elective and initially successful PTCA, were studied using vectorcardiogram (VCG) recordings. VCG monitoring was started 5 min before start of the PTCA and was carried out during the entire procedure, for at least 30 min after the first balloon inflation. ST-segment vector magnitude (ST-VM) and ST-segment change vector magnitude (STC-VM) were monitored. RESULTS: Fifteen (7.8%) procedure-related myocardial infarctions occurred. Indicators of procedure-related myocardial infarction were maximum value of ST-VM (P < 0.001) and STC-VM (P < 0.001), total ischemic time of all ST-VM episodes (P < 0.001) and STC-VM episodes (P < 0.001). The variable most closely related to a procedure-related myocardial infarction was the maximum STC-VM value during the procedure. With an optimized cutoff value, maximum STC-VM predicts a procedure-related myocardial infarction with a sensitivity of 93%, a specificity of 59% and a negative predictive value of 99%. Patients who had a stent implanted had significantly greater VCG values (P < 0.05-P < 0.001) than the group without a stent. There was a trend (P < 0.06) to a relation between increased creatine kinase concentration and stent implantation. In patients both with and without an implanted stent, greater STC-VM values were associated with procedure-related myocardial infarction (P < 0.01). CONCLUSION: Continuous VCG monitoring during elective PTCA is a promising method for immediate detection of patients at increased risk of procedure-related myocardial infarction.

Comparison of diabetic and non-diabetic patients referred for coronary angiography.

AIM: To evaluate whether diabetic patients differ from non-diabetic patients when referred for coronary angiography regarding previous history, indication for and findings at coronary angiography, use of medication, exercise test results and mortality. METHODS: Data were prospectively collected on patients referred for consideration of coronary revascularization to seven of the eight public Swedish heart centers that performed approximately 92% of all bypass operations in Sweden in 1994. RESULTS: 2762 patients were included of whom 406 (15%) had a history of diabetes mellitus. There was no difference in age or sex in the two groups. Chronic stable angina was the most common indication (73% in both groups) and only 3% were admitted due to silent ischemia. Diabetic patients had more severe symptoms (Canadian Cardiovascular Society III-IV) than non-diabetic patients (66% vs. 58%, p<0.01). They more frequently used ACE-inhibitors (33% vs. 19%, p<0.0001) and calcium channel blockers (47% vs. 40%, p<0.01) and more often had a diagnosis of arterial hypertension than non-diabetic patients (50% vs. 33%, p<0.0001). Diabetic patients more often had depressed myocardial function (EF<35%); 12% and 8%, respectively (p<0.01), and more extensive coronary artery disease (left main/3-VD; 48% vs. 37%, p<0.001). The mortality during the subsequent 21 months was 7.9% among diabetic patients and 3.6% among non-diabetic patients (p<0.001). CONCLUSION: Among patients being referred for coronary angiography in Sweden, 15% were patients with a history of diabetes. They differed from patients without such a history by more often having severe symptoms and a higher prevalence of left main/triple vessel disease. Coronary angiography may thus be underused in diabetic patients with chest pain.

CABG in chronic stable angina pectoris patients: indications and outcomes (SECOR/SBU). Swedish Societies for Cardiology, Thoracic Radiology and Thoracic surgery/Swedish Council for Technology Assessment in Health Care.

OBJECTIVE: In order to ascertain the reasons why coronary revascularisation is performed, the appropriateness of these procedures and their outcomes, a national collaborative study encompassing 7 of 8 hospitals performing CABG in Sweden was undertaken. This article presents the indications and outcomes in the largest intervention group, chronic stable angina pectoris treated by first time CABG. METHODS: A prospective multi-centre study was carried out during a 3.5 month period in each centre. Patients (1039) with chronic stable angina pectoris undergoing first time CABG were enrolled. Patients' quality of life was recorded at the time they underwent angiography and again 6 months post-operatively. Mortality and serious peri-and post-operative complications were recorded. The study was designed and carried out by an independent government agency, the Swedish Council for Technology Assessment in Health Care (SBU) with a project group of representatives for the Swedish Societies for Cardiology, Thoracic Radiology and Thoracic Surgery. RESULTS: Median age was 66 years. A total of 80% were males. The mortality rates at 30 days and 6 months were 1.0 and 1.9%, respectively. A balloon pump was used for 0.9% of patients and 1.4% of them were on a respirator for more than 24 h. At recruitment, 43.9% of the patients reported having severe angina (CCS II/IV), 70.1% had angina > or = 3 times per week, and 53.1% used sublingual nitrates > or = 3 times per week. The corresponding results at 6 months were 6.7, 10.5 and 3.3%, respectively. Patient satisfaction with operative treatment was high. In comparison with the pre-operative status, quality of life was markedly improved after surgery. CONCLUSION: This study shows that CABG in chronic stable angina pectoris yields good outcomes with a low mortality and morbidity and a high degree of patient satisfaction and quality of life.

Age-standardized incidence and prevalence of Parkinson's disease in a Swedish community.

Parkinson's disease (PD) shows a geographical variation. All prescriptions for anti-parkinsonian drugs were recorded for a half-year in a region with low L-dopa consumption. Hospital and outpatient records were studied and physicians were asked to supply details of PD patients in the region, with 147,777 inhabitants. The crude prevalence was 115 PD per 100,000 inhabitants, based on 170 cases. In contrast to other studies we report an age-standardized prevalence, which was 76 per 100,000, using the European Standard Population as reference. The corresponding approximate incidences were 11.0 (crude) and 7.9 (age-standardized) per 100,000 person-years. Male preponderance appeared in all age groups. Mean age at onset was 65.6 years, the highest figure reported. Variation between studies for age at onset, differences in prevalence, and male preponderance suggest environmental risk factors to be of importance for PD.

Treatment of Parkinson's disease with NADH.

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 biochemically.

Dopa-responsive dystonia with depigmentation of the substantia nigra and formation of Lewy bodies.

The patient, who died at 23 years of age, was first diagnosed when she was 12 year old as having a dopa-responsive dystonia with decreased concentrations of monoamine metabolites in the cerebrospinal fluid. Also the concentrations of other neurotransmitters including somatostatin, substance P and metenkefalin were lowered indicating a more widespread damage of the cerebral neurotransmitter systems. At autopsy the brain was essentially intact except for pronounced gliosis and extreme loss of melanotic nerve cells in the substantia nigra. Several remaining nerve cells showed the presence of Lewy bodies.

Neuropathy and myopathy in primary Sjögren's syndrome: neurophysiological, immunological and muscle biopsy results.

Seventeen consecutive patients with primary Sjögren's syndrome (PSS) received neurophysiological examination, analysis of antibodies against peripheral nerve-myelin (PNM) and muscle biopsy, to show the prevalence and characteristics of peripheral neuropathy (PN) and myopathy; 3 PSS cases showed a clinical mild sensorimotor polyneuropathy, 1 of them had been treated with cytostatic drugs; 1 had mononeuropathia multiplex; and 1 clinical carpal tunnel syndrome. In these 5 patients neurophysiological investigation verified the clinical diagnosis of peripheral nerve involvement; 2 with PN had serum-antibodies against PNM; 1 of IgM-, and 1 of IgA-isotype. Muscle biopsies were taken from 15 PSS patients; 11 showed inflammatory myositis or inflammatory perivascular infiltrates and 3 showed signs of denervation. A combination of inflammation and morphological signs of myopathy, compatible with the biopsy diagnosis of polymyositis, was seen in 4, 1 of whom showed clinical signs of polymyositis. We conclude that the peripheral nervous and muscular systems are often involved in PSS, but commonly with relatively mild symptoms and laboratory findings. The common findings of inflammatory myopathy indicate an immune reaction in muscles in addition to other autoimmune manifestations of the disease.

Combined cervicothoracic approach in thymectomy for myasthenia gravis.

Thymectomy was performed for myasthenia gravis on 30 patients, using a new approach with a collar incision which gave full exposure of the retrothyroid space and was directly connected to a median sternotomy. The thymus was removed en bloc without pleural incision. There was no perioperative mortality and the only complications were transient respiratory insufficiency in two cases. The postoperative hospital stay was 3-9 (mean 5.8) days. The effect of thymectomy was evaluated after 2-8 years at the Department of Neurology, when changes in symptoms (stages I-IV) or medication (need for cholinesterase inhibitors) were registered. The total clinical improvement rate was 97%, with 3% of the patients improved three stages, 33% two stages and 60% one stage compared with the preoperative classification. Twenty patients (67%) were asymptomatic at follow-up and six (20%) also required no medication. The medication need was reduced in 70% of cases (mean reduction 42%). Our cervicothoracic approach resulted in the same rate of improvement as in studies using more extensive transsternal procedures, but the morbidity was lower, with no complications requiring prolonged hospital stay. The morbidity was also less than after only transcervical procedures aiming to perform total thymectomy--a prerequisite for maximal and lasting benefit from surgery. Moreover, as this cervicothoracic approach is simple and safe, it can be recommended as an option in the surgical management of myasthenia gravis.