Familial mydriasis, cardiac arrhythmia, respiratory failure, muscular weakness and hypohidrosis.

OBJECTIVES: To describe a family with some sort of progressive autonomic failure in one generation (2 affected of a sibship of 7 sisters). The main features were: mydriasis, cardiac arrhythmia, cardiomegaly, hypohidrosis, respiratory failure, and muscular weakness. METHODS: Pupillometry, evaporimetry, and isokinetic power measurements were carried out. RESULTS: The autonomic dysfunction pattern (mainly cardiac abnormalities, mydriasis) seems to differ somewhat from that of progressive autonomic failure (Shy-Drager syndrome). "Lewy body-like" inclusions were present, in particular in substantia nigra, but also in locus ceruleus and raphe nuclei (cell loss only in locus ceruleus). There were no oligodendroglial, cytoplasmatic inclusions, apparently a marker in multiple system atrophy. Proper Lewy bodies were also present. Differences seemed to prevail vs the Shy-Drager syndrome. Various traits: muscular weakness pattern (e.g. preferential peroneal distribution), minor elbow contractures, and arrhythmia were reminiscent of Emery-Dreifuss muscle dystrophy (E-D). Distinguishing features included: hereditary pattern, mydriasis, and hypohidrosis. CONCLUSION: Conceivably, this disorder is close to, but still not identical with E-D.

Stroke unit treatment. 10-year follow-up.

BACKGROUND AND PURPOSE: We have previously shown that treatment in our combined acute and rehabilitation stroke unit (SU) improves the outcome during the first 5 years after onset of stroke compared with that for stroke patients treated in general wards (GW). The aim of the present trial was to examine the effects of SU care after 10 years of follow-up. METHODS: In a randomized controlled trial, 110 patients with symptoms and signs of an acute stroke were allocated to the SU and 110 to GW. No significant differences existed in baseline characteristics between the groups. The outcome after 10 years was measured by the proportion of patients at home, the proportion of patients in an institution, the mortality, and the functional state as assessed by the Barthel Index, in which a Barthel Index score of >/=60 was classified as independent or partly independent and a score of >/=95 was classified as independent. RESULTS: After 10 years, 21 (19.1%) of the patients randomized to the SU and 9 (8.2%) of the patients randomized to the GW were at home (P=0.0184). Eighty-three (75.5%) of the patients from the SU and 96 (87.3%) of the patients from the GW were dead (P=0.0082), and 6 (5.4%) and 5 (4.5%), respectively, were in an institution (eg, nursing home; NS). Twenty-two (20.0%) of the SU patients and 9 (8. 2%) of the GW patients had a Barthel Index score of >/=60 (P=0.0118), and 14 (12.7%) and 6 (5.4%), respectively, had a score of >/=95 (P=0.0606). CONCLUSIONS: For the first time it has been shown that SU care improves survival and functional state and increases the proportion of patients able to live at home 10 years after their stroke. Treatment in combined acute and rehabilitation SU seems to have important long-term effects on outcome for stroke patients.

Treatment in a combined acute and rehabilitation stroke unit: which aspects are most important?

BACKGROUND AND PURPOSE: We have previously shown that treatment of acute stroke patients in our stroke unit (SU) compared with treatment in general ward (GWs) improves short- and long-term survival and functional outcome and increases the possibility of earlier discharge to home. The aim of the present study was to identify the differences in treatment between the SU and the GW and to assess which aspects of the SU care which were most responsible for the better outcome. METHODS: Of the 220 patients included in our trial, only 206 were actually treated (SU, 102 patients; GW, 104 patients). For these patients, we identified the differences in the treatment and the consequences of the treatment. We analyzed the factors that we were able to measure and their association with the outcome, discharge to home within 6 weeks. RESULTS: Characteristic features in our SU were teamwork, staff education, functional training, and integrated physiotherapy and nursing. Other treatment factors significantly different in the SU from the GW were shorter time to start of the systematic mobilization/training and increased use of oxygen, heparin, intravenous saline solutions, and antipyretics. Consequences of the treatment seem to be less variation in diastolic and systolic blood pressure (BP), avoiding the lowest diastolic BP, and lowering the levels of glucose and temperature in the SU group compared with the GW group. Univariate analyses showed that all these factors except the level of glucose were significantly associated with discharge to home within 6 weeks. In the final multivariate Cox regression model, shorter time to start of the mobilization/training and stabilized diastolic BP were independent factors significantly associated with discharge to home within 6 weeks. CONCLUSIONS: Shorter time to start of mobilization/training was the most important factor associated with discharge to home, followed by stabilized diastolic BP, indicating that these factors probably were important in the SU treatment. The effects of characteristic features of an SU, such as a specially trained staff, teamwork, and involvement of relatives, were not possible to measure. Such factors might be more important than those actually measured.

Stroke unit treatment improves long-term quality of life: a randomized controlled trial.

BACKGROUND AND PURPOSE: We have previously shown that treatment of acute stroke patients in the combined acute and rehabilitation stroke unit in our hospital improves survival and functional outcome compared with treatment in general wards. The primary aim of the present trial was to examine whether the treatment in our stroke unit had an effect on different aspects of quality of life (QoL) for stroke patients 5 years after the onset of stroke. METHODS: In a randomized controlled trial, 110 patients with symptoms and signs of an acute stroke were allocated to the stroke unit and 110 to general wards. No significant differences existed in baseline characteristics between the two groups. The patients alive after 5 years were assessed by the Nottingham Health Profile (NHP) and the Frenchay Activities Index (FAI), which were the scales used as primary outcome measures for QoL. As secondary outcome measures we used a global score for the NHP and a simple visual analogue scale (VAS). RESULTS: After 5 years, 45 of the patients treated in the stroke unit and 32 of those treated in general wards were alive. All surviving patients were assessed by the FAI. Thirty-seven (82.2%) of the stroke unit patients and 25 (78.1%) of the general wards patients were assessed by the NHP; 38 (84.4%) and 28 (87.5%), respectively, were assessed by the VAS. Patients treated in the stroke unit had a higher score on the FAI (P=0.0142). Assessment with the NHP showed better results in the stroke unit group for the dimensions of energy (P=0.0323), physical mobility (P=0.0415), emotional reactions (P=0.0290), social isolation (P=0.0089), and sleep (P=0.0436), although there was no difference in pain (P=0.3186). The global NHP score and VAS score also showed significantly better results in the stroke unit group (NHP, P<0.01; VAS, P<0.001). Patients who were independent in activities of daily living had significantly better QoL assessed by these scales than patients who were dependent. CONCLUSIONS: Our study shows for the first time that stroke unit care improves different aspects of long-term QoL for stroke patients.

Stroke unit treatment. Long-term effects.

BACKGROUND AND PURPOSE: We have previously shown that treatment in our combined acute and rehabilitation Stroke Unit improves outcome during the first year after onset of stroke compared with stroke patients treated in general wards. The aim of the present trial was to examine the long-term effects of the stroke unit care. METHODS: In a randomized controlled trial, 110 patients with symptoms and signs of an acute stroke were allocated to the Stroke Unit and 110 to general wards. No significant differences existed in baseline characteristics between the two groups. The outcome after 5 years was measured by the proportion of patients at home, the proportion of patients in an institution, the mortality, and the functional state assessed by Barthel Index. RESULTS: After 5 years, 38 (34.5%) of the patients randomized to the Stroke Unit and 20 (18.2%) of the patients randomized to the general wards were at home (P = .006). Sixty-five (59.1%) of the patients from the Stroke Unit and 78 (70.9%) of the patients from the general wards were dead (P = .041), while 7 (6.4%) and 12 (10.9%), respectively, were in an institution (e.g., nursing home) (P = NS). Functional state was significantly better for patients treated in the Stroke Unit. CONCLUSIONS: For the first time it is shown that stroke unit care improves long-term survival and functional state and increases the proportion of patients able to live at home 5 years after the stroke. Combined acute and rehabilitation stroke units appear to be an effective way of organizing treatment for acute stroke patients.

Digital dipyridamole stress echocardiography in silent ischemia after coronary artery bypass grafting and/or after healing of acute myocardial infarction.

This study evaluates dipyridamole stress echocardiography in silent ischemia. Fourteen patients with previous coronary artery bypass grafting (group A) and 16 patients with healed myocardial infarction (group B) were studied. All had > or = 1 mm ST depression without chest pain during bicycle exercise testing. Left ventricular wall motion was analyzed using a computerized display of digital systolic cineloops with a high frame rate. Test results were compared with coronary angiography. Dipyridamole echocardiography accurately identified patients with significant coronary artery stenosis in both groups (3 of 4 in group A, 11 of 14 in group B). Retrograde flow to the occluded native artery was associated with positive results on dipyridamole testing in 6 of 7 patients in group A and all 3 in group B. Sensitivity, specificity and diagnostic accuracy for detecting significant coronary stenosis or occlusions with retrograde flow was 78, 100 and 83%, respectively. Patients with angiographic multivessel disease had a significantly larger increase in wall motion score index during dipyridamole stress than patients with 0- or 1-vessel disease, 0.18 +/- 0.11 versus 0.05 +/- 0.18 (p < 0.05). Two patients developed symptomatic bradycardia and hypotension during dipyridamole infusion. It is concluded that dipyridamole echocardiography accurately identifies myocardial regions with restricted coronary flow. Stress echocardiography is a valuable tool for assessing coronary flow in silent ischemia.

Benefit of a stroke unit: a randomized controlled trial.

In a randomized controlled trial we compared the clinical outcome of acute stroke patients, 110 of whom were allocated to treatment in a stroke unit and 110 to treatment in general medical wards. No significant difference existed between these groups with regard to sex, age, marital status, medical history, or functional impairment on admission. Outcome was measured at 6 and 52 weeks after the stroke by the proportion of patients at home, the proportion of patients in an institution, the mortality, and the functional state. After 6 weeks 56.4% of the patients randomized to the stroke unit and 32.7% of the patients randomized to the general medical wards were at home (p = 0.0004), and after 52 weeks 62.7% and 44.6%, respectively, were at home (p = 0.002). After 6 weeks 36.3% of the patients from the stroke unit and 50.0% from the general medical wards were in an institution (p = 0.02); after 52 weeks 12.7% and 22.7%, respectively, were institutionalized (p = 0.016). After 6 weeks mortality was 7.3% for the stroke unit group and 17.3% for the general medical wards group (p = 0.027). After 52 weeks mortality was 24.6% for the stroke unit group and 32.7% for the general medical wards group (difference not significant). Functional state was significantly better for patients treated in the stroke unit after both 6 and 52 weeks. We conclude that care of patients with acute stroke in a stroke unit improves clinical outcome compared with treatment in general medical wards.

Evolution of infarct size during the early use of nifedipine in patients with acute myocardial infarction: the Norwegian Nifedipine Multicenter Trial.

In a multicenter double-blind study, 227 patients with suspected acute myocardial infarction (AMI) were randomized within 12 hr from onset of symptoms to treatment with nifedipine (112 patients) or placebo (115 patients). AMI was confirmed in 74 patients on nifedipine and in 83 on placebo. Patients with AMI received nifedipine 5.5 +/- 2.9 hr (mean +/- SD) after onset of symptoms. Infarct size was assessed by the release of creatine kinase isoenzyme MB (CK-MB). Infarct size index (CK-MB geq/m2) was 25 +/- 16 (n = 71) in the nifedipine group and 23 +/- 13 (n = 77) in the placebo group (NS). After the first 10 mg of nifedipine systolic blood pressure fell from 147 +/- 30 to 135 +/- 28 mm Hg (p less than .01) and heart rate rose from 75 +/- 18 to 79 +/- 19 beats/min (p less than .01). No change was observed after the first placebo dose. The treatment was continued for 6 weeks. Over this period there were 10 deaths in each group. Early treatment with nifedipine in patients with AMI does not seem to reduce infarct size as determined by enzyme level.

Noninvasive diagnosis and assessment of ventricular septal defect by Doppler ultrasound.

The presence of ventricular septal defect can be diagnosed noninvasively by Doppler ultrasound. Care must be taken to distinguish between VSD and infundibular pulmonary stenosis. VSD was easily differentiated from other cardiac lesions. In 55% of the patients a pressure drop across the VDS, comparable to that present, could be calculated from maximal recorded. In the remaining patients velocity and pressure drop were underestimated, probably due to a too large angle between ultrasound beam and velocity. Pulmonary artery systolic pressure was correctly estimated from Pc-To interval and heart rate, and RPEP(RVET) indicated whether normal or raised diastolic pressure was present in most patients.

Embolism in sinoatrial disease.

Among a series of consecutive patients treated with permanent pacemaker between 1965 and 1976, 43 had sinoatrial disease with paroxysmal tachycardia (group A), 30 sinoatrial disease without tachycardia (group B) and 165 atrioventricular block (group C). A retrospective study showed systemic vascular events compatible with embolism in 35%, 7% and 10% in groups A, B and C, respectively. The groups were comparable as regards age, other diseases and duration of pacemaker therapy. Because of the high incidence of embolism in group A, anticoagulant therapy should be evaluated in patients with the brady-tachycardia syndrome.

Sinoatrial disease in acute myocardial infarction. Long-term prognosis.

Of 32 patients with acute myocardial infarction complicated by sinoatrial disease, 23 survived. All 23 had inferior infarction. During follow-up lasting 4 to 6 years only one patient developed severe chronic sinoatrial disease (sick sinus syndrome) necessitating permanent pacemaker treatment; twelve others died during this time. In 2 of them death was sudden 5 and 6 months after infarction. Atrial pacing studies in 7 of the 11 patients still alive showed no gross abnormalities. A review of 71 patients with chronic sinoatrial disease treated with a permanent pacemaker revealed only 5 with previous documented infarction. The present data suggest that sinus node dysfunction in patients surviving acute infarction is most often only temporary as is atrioventricular block. Occasionally, however, severe chronic sinoatrial disease requiring a permanent pacemaker may develop later, and this course of events is most likely to occur in those patients who had additional complications during the acute infarct.

Sinus arrest in acute myocardial infarction.

The results are given of a 4-year prospective study regarding the occurrence, clinical pattern, and treatment of sinus arrest in patients with acute myocardial infarction. The arrhythmia was observed in 32 of 1665 patients. The detection rate increased from below 1 per cent in the first year to 5 per cent in the last due to better facilities for monitoring. All patients except one had inferior infarction. Paroxysmal atrial fibrillation was observed in 12, corresponding to a frequency approximately similar to that previously reported in patients with sinus arrest unrelated to myocardial infarction. Syncope due to sinus arrest occurred in 7 and hypotension in 19 patients. Small doses of isoprenaline given intravenously were usually effective in restoring normal heart rate, but pacemaker therapy was necessary in 3 patients.