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The text is a report on clinical imaging and electrophysiology in the field of Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Degeneration (FTD). It is authored by a large number of researchers from various institutions. The report provides a comprehensive overview of the current research and findings in the field, covering topics such as imaging techniques, electrophysiological measurements, and their applications in diagnosing and monitoring ALS and FTD. The report is a valuable resource for library patrons interested in understanding the latest advancements in clinical imaging and electrophysiology in ALS and FTD research. [Extracted from the article]

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Delayed diagnosis of amyotrophic lateral sclerosis prevents early entry into clinical trials at a time when neuroprotective therapies would be most effective. Fasciculations are an early hallmark of amyotrophic lateral sclerosis, preceding muscle weakness and atrophy. To assess the potential diagnostic utility of fasciculations measured by high-density surface electromyography, we carried out 30-min biceps brachii recordings in 39 patients with amyotrophic lateral sclerosis, 7 patients with benign fasciculation syndrome, 1 patient with multifocal motor neuropathy and 17 healthy individuals. We employed the surface potential quantification engine to compute fasciculation frequency, fasciculation amplitude and inter-fasciculation interval. Inter-group comparison was assessed by Welch's analysis of variance. Logistic regression, receiver operating characteristic curves and decision trees discerned the diagnostic performance of these measures. Fasciculation frequency, median fasciculation amplitude and proportion of inter-fasciculation intervals <100 ms showed significant differences between the groups. In the best-fit regression model, increasing fasciculation frequency and median fasciculation amplitude were independently associated with the diagnosis of amyotrophic lateral sclerosis. Fasciculation frequency was the single best measure predictive of the disease, with an area under the curve of 0.89 (95% confidence interval 0.81-0.98). The cut-off of more than 14 fasciculation potentials per minute achieved 80% sensitivity (95% confidence interval 63-90%) and 96% specificity (95% confidence interval 78-100%). In conclusion, non-invasive measurement of fasciculation frequency at a single time-point reliably distinguished amyotrophic lateral sclerosis from its mimicking conditions and healthy individuals, warranting further research into its diagnostic applications., (© The Author(s) (2020). Published by Oxford University Press on behalf of the Guarantors of Brain.)

Aims: The objective of this study is to determine the status of major risk factors for coronary heart disease in patients with established coronary heart disease in Croatia and whether the Joint European Societies' recommendations on coronary heart disease prevention are being followed in Croatia and whether secondary prevention practices have improved between 1998 and 2003., Methods: Five surveys were undertaken in 35 centres covering the geographical area of the whole of Croatia between 1 June, 1998 and 31 March, 2003. Consecutive patients of both sexes were identified after coronary-bypass grafting or a percutaneous transluminal coronary angioplasty or a hospital admission with acute myocardial infarction or ischaemia. Data collection was based on a review of medical records and the methodology used was similar to the one used in the EUROASPIRE study., Results: Fifteen thousand, five hundred and twenty patients were enrolled (64.6% men); 35% of patients smoked cigarettes, 66% had raised blood pressure, 69% elevated serum total cholesterol, 69% elevated serum low-density lipoprotein (LDL) cholesterol, 42% low high-density lipoprotein (HDL) cholesterol, 37% elevated triglycerides, 30% diabetes and 34% family history of coronary heart disease. More men were smokers and had low HDL cholesterol, but more women had elevated total and LDL cholesterol, hypertension and diabetes. More men had Q wave acute myocardial infarction, but more women had angina. Over 5 years, the prevalence of hypercholesterolemia decreased substantially from 82.7 to 65%. Eighty-three percent of patients received aspirin and this percentage did not change during the study. The use of diuretics, calcium antagonists and nitrates did not change either. The reported use of statins, angiotensin-converting enzyme inhibitors and beta-blockers increased significantly., Conclusion: This survey shows a high prevalence of modifiable risk factors in Croatian patients with coronary heart disease. Although the higher use of statins, angiotensin-converting enzyme inhibitors and beta-blockers is encouraging, the fact that most coronary heart disease patients are still not achieving the recommended goals remains a concern. There is real potential to reduce the very high coronary heart disease morbidity and mortality in Croatia.

We present a case of meningitis and endocarditis caused by Streptococcus agalactiae (group B streptococcus) in an adult patient with human immunodeficiency virus (HIV) infection. To our knowledge, only four other cases of meningitis, none of which had concomitant endocarditis, have been reported so far. A 45-year-old homosexual patient presented with fever, confusion, and signs of meningeal irritation. Streptococcus agalactiae was cultured from the blood, urine, and cerebrospinal fluid (CSF). Diagnosis of meningitis caused by streptococcus agalactiae was made. On day 35, a heart murmur was noticed, and patient developed cardiac decompensation. Echocardiography revealed vegetations on the mitral and aortic valve. After nine weeks of antibiotic treatment, the patient was discharged from the hospital in good general condition, with improved CSF and echocardiographic findings.

Tolerance to nitroglycerin infusion (NG) can be overridden by dose escalation. The aim of this study was to define for how long it can be done for hypotensive efficacy of NG, in a coronary care setting. A prospective trial with an intra-individual therapeutic comparison was performed in 60 patients with acute myocardial infarction or unstable angina. Initial efficacy of NG was confirmed by a 10% blood pressure decrease (measured by cuff). Seventy-two-hour NG infusion was interrupted, for 30 minutes, every 12 hours. If blood pressure increased by 10% after infusion interruption, the infusion was continued at the previous rate. If blood pressure did not increase (detected tolerance--weakened efficacy of NG), the dose was increased until pressure decreased by 10% and the infusion was continued at the new dose. Failure to achieve hypotensive response, despite a 5-fold dose increase, indicated onset of resistance--completely lost hypotensive efficacy of NG. The majority of patients (49 out of 55) who developed tolerance, developed it during the first 36 hours, while the majority of those who developed resistance (33 out of 40), developed it within 60 hours of the infusion. Tolerance was overridden by dose escalation in 41 out of 55 patients, which was repeated in 31 patients. Complete restoration of NG action was possible over 24 hours in half the patients, and over 48 hours in one third of the patients. Three out of 34 patients who developed tolerance before the 13th hour did not develop resistance during the following 60 hours of dose up-titration. The conclusion is that tolerance to NG can be overridden by dose escalation in the majority of patients for a significant period of time, which is useful in clinical practice.

In this study the incidence and the development of acute renal failure (ARF) in heart transplant recipients is presented. Among the thirteen heart transplant recipients eight of them developed oliguric or nonoliguric ARF. Besides the known factors such as actual condition of the patient, kidney function, peri and post-operative compromised circulation, our results demonstrate the significance of postoperative cyclosporin concentration in combination with the use of other drugs. The results also show the importance of the way cyclosporin has been administrated. The parenteral route of appliance is connected with the greater risk of higher drug concentration in the plasma than the peroral one. In connection with this is the higher incidence of adverse reactions to cyclosporin given parenterally. In addition to ranitidine and captopril, which have been mentioned earlier, the findings of our study indicate that greater attention has to be paid to the treatment with ketoconazol given in combination with cyclosporin, since it results in decreased cyclosporin clearance. Other nephrotoxic drugs like amphotericin also increase the possibility of renal lesions. The incidence of acute renal failure in this group of patients is high. Our data suggest that the reasons for the development of ARF are multifactorial. These data further suggest that a reasonable way to solve nonoliguric form of acute renal failure is to maintain the "internal balance" and that it is not necessary to perform extracorporeal elimination of nitrogen substances, if there are no additional complications (i.e. gastrointestinal bleeding).(ABSTRACT TRUNCATED AT 250 WORDS)

A dynamic model for the estimation of a residual hypotensive efficacy of nitroglycerin (NG) infusion was constructed to distinguish secondary resistance from tolerance to NG, as lost vs weakened efficiency. The model was tested by individual comparison in 82 of 92 patients with acute myocardial infarction or unstable angina. Ten patients were excluded from the study protocol due to complications that required therapy modification. NG infusion (20 micrograms/ml) efficiency was initially confirmed by a 10% decrease in the mean brachial artery (cuff) blood pressure. The infusion (63 hours mean duration) was discontinued every 12 hours for 30 minutes and pressure changes were analyzed. If NG hypotensive efficacy was maintained (as proved by at least 10% pressure increase), infusion was carried on using the initial dose. Lack of 10% pressure increase after 30-minute infusion discontinuity (tolerance) indicated the need for an increase in the NG dose until 10% pressure decrease (not below 105/60) was obtained. Lack of 10% pressure decrease, with a 5-fold increase in NG dose (up to 320 micrograms/ml), was considered to be a sign of secondary resistance. There were no proceeding complications and tolerance was found in 72 patients. Beside tolerance, secondary resistance was simultaneously present in 16 patients, while the NG efficacy was restored by dose increase in the remaining 78% of tolerant patients. "Paradoxical" pressure decrease was noted in 12 tolerant patients after the infusion interruption, while "paradoxical" pressure increase was observed in 3 resistant patients during the infusion acceleration.(ABSTRACT TRUNCATED AT 250 WORDS)

For the evaluation of patients with prosthetic heart valves Doppler echocardiography is superior over other noninvasive techniques because it allows quantitative assessment of valve function. As a noninvasive method Doppler is safe, easily repeatable and provides haemodynamic data that closely correlate with parameters obtained by invasive procedures (Sagar et al., 1986: Simpson et al., 1986; Wilkins et al., 1986; Gibbs, 1987). The purpose of our study was to evaluate Doppler characteristics of mechanical and tissue mitral prostheses; Starr-Edwards, Björk-Shiley, Hall-Medtronic and Hancock in patients with clinically normal valve function.

25 patients are presented with chronic cardiomyopathy (NYHA class IV), who were refractory to conventional digitalis and diuretics treatment which was administered over at least 3 weeks. The patients were split in 2 groups: the first group consisted of 10 patients who were haemodynamically monitored while being administered nitroprusside for 24 hours. After the treatment, the cardiac index significantly increased (by 36%), left ventricular filling pressure decreased (by 48%), and systemic vascular resistance fell (by 47%). The second group, consisting of 15 patients, was administered prazosin in addition to conventional treatment. The effects of prazosin were clinically monitored. After 3 weeks of continual prazosin treatment, the clinical condition of 12 patients was improved to NYHA class II whereas the condition of the other 3 was improved only to NYHA class III. During further treatment this improvement was maintained. The effects of the treatment can be easily followed and assessed on the basis of clinical features, so that other, more complex methods of follow-up are not required. The results of our study show that patients with refractory heart failure can be safely administered prazosin without previous assessment of haemodynamic parameters.