[Chronic progressive external ophthalmoplegia: clinical and electromyographic manifestations in a series of cases]. / Oftalmoplejía externa progresiva crónica: manifestaciones clínicas y electromiográficas en una serie de casos.

INTRODUCTION: Chronic progressive external ophthalmoplegia (CPEO) is a common mitochondrial disease. The different conditions in this group of diseases overlap clinically, enzymatically and genetically. There is no effective treatment. Ptosis improves with corrective surgery involving tarsorrhaphy as a palliative measure. CASE REPORTS: Code numbers were examined in a retrospective study conducted in order to search for patients with ptosis or ophthalmoplegia who had either visited or been admitted to the neurology department over the last 10 years. Data concerning these patients' clinical features and results of complementary tests were collected. Six patients with CPEO were identified, five of whom were females. Ages ranged from 44 to 72 years. All the patients had ptosis, although 50% were asymmetric. Half of them reported mild dysphagia while swallowing liquids. Levels of creatine phosphokinase and acetylcholine antireceptor antibodies were normal. Half the patients showed increased jitter and a muscle biopsy revealed that five of them had ragged red fibres. The most frequent enzyme deficit was complex I and IV deficiency. There were no familial forms; the most common genetic anomaly was single deletion in the mitochondrial deoxyribonucleic acid. CONCLUSIONS: In cases of ptosis and ophthalmoplegia that do not respond to anticholinesterases, knowledge of this condition makes it possible to avoid the use of immunosuppressant drugs, which have important side effects.

Oftalmoplejía externa progresiva crónica: manifestaciones clínicas y electromiográficas en una serie de casos / Chronic progressive external ophthalmoplegia: clinical and electromyographic manifestations in a series of cases

Introducción. La oftalmoplejía externa progresiva crónica(CPEO) es una enfermedad mitocondrial común. Este grupode enfermedades presenta solapamiento clínico, enzimático y genéticoentre las diferentes entidades. No existe un tratamiento eficaz.La ptosis mejora con cirugía correctora de tarsorrafia comouna medida paliativa. Casos clínicos. Estudio retrospectivo en elque se busca por codificación a pacientes con ptosis u oftalmoplejíaen consultas o ingresados en neurología durante los últimos 10años. Se recogieron datos de la clínica y pruebas complementariasde estos pacientes. Se identificó a seis pacientes con CPEO; cincode ellos fueron mujeres. Sus edades estaban comprendidas entrelos 44 y los 72 años. Todos los pacientes presentaban ptosis, aunqueel 50% era asimétrica. La mitad refería disfagia leve paralíquidos. Los niveles de creatinfosfocinasa y de anticuerpos antirreceptoresde acetilcolina fueron normales. Existía un aumentodel jitter en la mitad de los pacientes y fibras rojas rasgadas en labiopsia muscular de cinco de ellos. El déficit enzimático más frecuentefue el de los complejos I y IV. No existieron formas familiares;la anomalía genética más común fue la deleción única en elácido desoxirribonucleico mitocondrial. Conclusión. El conocimientode esta entidad permite, en casos de ptosis y oftalmoplejíaque no responden a anticolinesterásicos, evitar el uso de medicacionesinmunosupresoras con efectos secundarios importantes

Introduction. Chronic progressive external ophthalmoplegia (CPEO) is a common mitochondrial disease. Thedifferent conditions in this group of diseases overlap clinically, enzymatically and genetically. There is no effective treatment.Ptosis improves with corrective surgery involving tarsorrhaphy as a palliative measure. Case reports. Code numbers wereexamined in a retrospective study conducted in order to search for patients with ptosis or ophthalmoplegia who had eithervisited or been admitted to the neurology department over the last 10 years. Data concerning these patients' clinical featuresand results of complementary tests were collected. Six patients with CPEO were identified, five of whom were females. Agesranged from 44 to 72 years. All the patients had ptosis, although 50% were asymmetric. Half of them reported mild dysphagiawhile swallowing liquids. Levels of creatine phosphokinase and acetylcholine antireceptor antibodies were normal. Half thepatients showed increased jitter and a muscle biopsy revealed that five of them had ragged red fibres. The most frequentenzyme deficit was complex I and IV deficiency. There were no familial forms; the most common genetic anomaly was singledeletion in the mitochondrial deoxyribonucleic acid. Conclusions. In cases of ptosis and ophthalmoplegia that do not respondto anticholinesterases, knowledge of this condition makes it possible to avoid the use of immunosuppressant drugs, which haveimportant side effects

Short QT Syndrome: a familial cause of sudden death.

BACKGROUND: A prolonged QT interval is associated with a risk for life-threatening events. However, little is known about prognostic implications of the reverse-a short QT interval. Several members of 2 different families were referred for syncope, palpitations, and resuscitated cardiac arrest in the presence of a positive family history for sudden cardiac death. Autopsy did not reveal any structural heart disease. All patients had a constantly and uniformly short QT interval at ECG. METHODS AND RESULTS: Six patients from both families were submitted to extensive noninvasive and invasive work-up, including serial resting ECGs, echocardiogram, cardiac MRI, exercise testing, Holter ECG, and signal-averaged ECG. Four of 6 patients underwent electrophysiological evaluation including programmed ventricular stimulation. In all subjects, a structural heart disease was excluded. At baseline ECG, all patients exhibited a QT interval

[Physiological approaches to enhancing cardiac resistance in emotional stress]. / Fiziologicheskie podkhody k povysheniiu ustoichivosti serdechnoi deiatel'nosti pri émotsional'nom stresse.

The role of parasympathic effects, graded motor activity, positive emotions, and delta-sleep peptide in enhancing the resistance of cardiac performance in experimental emotional stress was studied in chronic rabbit experiments. The prevalence of parasympathic effects was demonstrated to increase cardiac electric stability and to prevent the elevation of catecholamine levels and the development of myocardial structural damages during stress. A moderate motor activity was found to lead to the session of ventricular extrasystole occurring in stress due to decreases in the myocardial levels of epinephrine with its high levels remaining in blood. Ventricular extrasystole also disappeared after activation of the positive emotional centers of the hypothalamus and after administration of delta-sleep peptide normalizing the electrical stability of the heart in emotional stress.

Differential effects of various oil diets on the risk of cardiac arrhythmias in rats.

BACKGROUND: Independently of the problem of atherogenesis, the amount and type of fat intake influences the risk of cardiac arrhythmias. However, the relative effectiveness of different fats and the underlying mechanisms are controversial. The aim of the present study was to compare the effects of various oil-enriched diets on the risk of ventricular arrhythmias in rat hearts under conditions of ischaemia and reperfusion and to help clarify the mechanisms underlying the differing effects of the oils on the occurrence of arrhythmias. METHODS: Over a 10-week period, we studied five groups of young male Wistar rats given a low-fat chow diet or one enriched with 10% hydrogenated coconut oil, corn oil, linseed oil or sardine oil. Electrocardiograms were recorded from the isolated hearts (Langendorff preparation) perfused with a modified Krebs-Henseleit solution. Ischaemia was induced by a 20 min occlusion of the left anterior descending coronary artery. In another series of experiments, a 10 min occlusion was followed by a 20 min reperfusion period. The times between the first occurrence of extrasystole and the incidence of ventricular tachycardia and fibrillation were determined. The size of the ischaemic zone was assessed using malachite green. The fatty acid composition of the myocardial tissue was analysed using gas chromatography. RESULTS: An increase in the risk of ventricular arrhythmias under conditions of both ischaemia and reperfusion was obvious in the rats that consumed large quantities of saturated fatty acids (coconut oil) and in the group with a very low intake of fat. Polyunsaturated fatty acids (PUFAs), particularly fish oil, exerted a protective effect. The incidence of ventricular fibrillation was 75% in the low-fat group, 67% in the coconut-oil group, 44% in the corn-oil group, 40% in the linseed-oil group and 10% in the fish-oil group. The time until the first occurrence of extrasystole, the incidence of ventricular tachycardia and the incidence of reperfusion-induced ventricular fibrillation were influenced in a similar manner. The size of the ischaemic zone was significantly reduced in the groups given diets enriched with PUFAs. All protective effects were abolished, however, by cyclooxygenase inhibition with aspirin. The fatty acid composition of myocardial tissue, the ratio of n-3 to n-6 fatty acids and the double-bond index were significantly affected by the various diets. CONCLUSION: Whereas saturated fatty acids are obviously proarrhythmic, diets enriched with n-6 or n-3 PUFAs both exert antiarrhythmic effects. Although n-3 fatty acids seem to be more effective, cardioprotection cannot simply be related to the replacement of n-6 by n-3 fatty acids in cardiac membrane phospholipids, given the beneficial effects of corn oil. In any case, replacement of n-3 by n-6 fatty acids is not the underlying mechanism. The overall reduction of prostaglandin formation cannot be the primary mechanism because the beneficial effects of diets rich in PUFAs were abolished by cyclooxygenase inhibition. We conlcude that, besides prostacyclin (PGI2 or PGI3), membrane fluidity and accompanying alterations in functional membrane proteins (e.g. protection from calcium overload) are key factors apart from vascular effects that influence the size of the ischaemic zone.

Children in sauna: electrocardiographic abnormalities.

We studied changes in ECG in 81 children and 20 adolescents and young adults when they were exposed to heat stress in a climatic chamber, which resembled an ordinary Finnish sauna. Changes were similar to those caused by increased sympathetic activity in 57% of the subjects during the bath and in 23% during the recovery period. Three children had extrasystoles during and four after sauna. One previously healthy 5-year-old girl with wandering pacemaker suffered a 3.3 sec sinus arrest and had reversible junctional rhythm during the heat exposure. We conclude that, although these changes were transient and benign, the sinus arrest suggest that heat stress in sauna is considerable and may be risky for those children who have disorders of the sinoatrial node.

High dose caffeine and ventricular arrhythmias.

The effect of high dose caffeine on ventricular arrhythmias was examined in 35 patients with recent myocardial infarctions. All patients received caffeine 450 mg or placebo on separate days using a randomized double-blind study design. Continuous Holter electrocardiographic recordings were performed for 8 h. Caffeine ingestion did not cause any increase in the frequency or complexity of ventricular ectopy with 19 of 35 patients experiencing ventricular arrhythmias after caffeine compared with 24 of 35 with placebo. Serious ventricular ectopic activity (including ventricular couplets and tachycardia) was similar on both study days. Moderately high doses of caffeine do not appear to increase ventricular arrhythmias in this patient population with ischemic heart disease.

[The role of delta-sleep-inducing peptide in the normalization of cardiac electric stability in experimental emotional stress]. / Rol' peptida delta-sna v normalizatsii elektricheskoi stabil'nosti serdtsa pri éksperimental'nom émotsional'nom stresse.

In chronic experiments on rabbits, a delta-sleeping inducing peptide was tested for effects on the cardiac electric stability and onset of ventricular premature beats during experimental emotional stress. The peptide in a dose of 60 nm/kg was found to normalize the thresholds of ventricular fibrillation and its predictors and to decrease or abolish spontaneous ventricular premature beats occurring during the stress. This suggests that the delta-sleep-inducing peptide may be used to prevent cardiac arrhythmias during stresses.

[The anti-arrhythmia action of combinations of N-propylajmaline bromide with anti-arrhythmia agents of different classes]. / Protivoaritmicheskoe deistvie kombinatsii N-propilaimalinbromida s protivoaritmicheskimi preparatami raznykh klassov.

In experiments on 68 dogs with ventricular arrhythmia at the late stage of experimental myocardial infarction caused by the coronary artery branch occlusion it was shown that combinations of N-propylaimaline bromide (IA class of antiarrhythmic agents) with trimecaine (IB class) or anapriline (II class) or phynoptine (IV class) induce a significant increase of the antiarrhythmic effect that is not observed at combination of N-propylaimaline with ethmosine (the both antiarrhythmic drugs belong to IA class). This potentiating effect is thought to be related to differences in the molecular mechanisms of action of antiarrhythmic drugs.

The effect of potassium and potassium plus magnesium supplementation on ventricular extrasystoles in mild hypertensives treated with hydrochlorothiazide.

Eleven mild hypertensives with base-line ventricular extrasystoles underwent a 24-week period of treatment with hydrochlorothiazide 50 mg daily. After 8 weeks of treatment either potassium as hydrochloride 1 g twice daily, or a combination of potassium hydrochloride 1 g and magnesium hydroxide 500 mg twice daily was added to the diuretic therapy in a randomised, double-blind, cross-over design. Each treatment period lasted 8 weeks. Supplementation with potassium or potassium plus magnesium resulted in a clear trend for suppression of ventricular ectopic activity. There was no difference in ventricular extrasystoles between the two supplementation groups. Supplementation with potassium plus magnesium but not with potassium only, resulted in a significant rise in serum potassium values (P less than 0.01). Our study suggests that potassium should be supplemented in thiazide-treated hypertensives who are prone to develop ventricular extrasystoles. Addition of magnesium to potassium supplementation does not result in further improvement in ventricular ectopic suppression.

Cod liver oil does not reduce ventricular extrasystoles after myocardial infarction.

Previous work has shown that in experimental animal models a lower incidence of arrhythmias and sudden death was observed if the animals were fed cod liver oil or fish oil. After a 48-h control period starting, on average, 8 days after the onset of symptoms, 18 men who were recovering from acute myocardial infarction were given 20 ml d-1 cod liver oil for 6 weeks, either immediately after the control period, weeks 0-6 (n = 10), or during weeks 6-12 (n = 8). Forty-eight-hour Holter monitoring was carried out before cod liver oil administration and at the end of weeks 6 and 12. The eicosapentaenoic acid content of plasma phospholipids was increased by 230% during cod liver oil administration. However, no significant change was observed in the 24-h prevalence of ventricular extrasystoles or other arrhythmias during the study period. The mean ln number of ventricular extrasystoles was 2.95 +/- 0.51 (+/- SEM) during cod liver oil ingestion and 2.63 +/- 0.30 when not taking cod liver oil.

A clinical investigation with Holter's monitoring of the antiarrhythmic effect of adonisidum.

The authors studied 24 cases of refractory premature beats by giving 0.1 adonisidum twice daily with Holter's monitoring. It was found that adonisidum was effective in 12 cases (P less than 0.001), or 50%. In another 8 patients adonisidum or mexiletine alone were ineffective for premature beats. However, when these patients were treated with both mexiletine and adonisidum, they responded with no exception (P less than 0.001). All 7 cases monitored with one hour Holter recording responded to adonisidum (P less than 0.001). As adonisidum tends to slow the heart rate, it is especially indicated for patients suffering from premature beats with increased heart rate, and should be used with caution in cases with slow heart rate or A-V block.

[Inhibitory action of intracerebroventricular injections of norepinephrine and clonidine on ventricular extrasystoles induced by stimulating hypothalamus in rabbits].

The effects of bilateral intracerebroventricular (icv) injections of norepinephrine (NE) and clonidine on hypothalamic stimulation-induced ventricular extrasystoles (VE) were investigated in alpha-chloralose and urethane anaesthetized rabbits. NE and clonidine reduced the number of HVE. The effects of NE were antagonized by pretreatment with phentolamine and yohimbine, but unaffected by pretreatment with prazosin. Pretreatment with yohimbine could also abolish the effects of clonidine. It is concluded that the effects of reduction in VE induced by NE and clonidine may be mediated by activating central alpha 2-adrenoceptors rather than alpha 1-adrenoceptors.

[Electroacupuncture and arrhythmia caused by reperfusion of the coronary arteries in rabbits and rats in hypothermia]. / Elektroakupunktura i aritmii, vyzvannye reperfuziei koronarnoi arterii krolikov i krys v usloviiakh gipotermii.

Reperfusion-induced arrhythmias in rats and rabbits are highly temperature-dependent. When the rectal temperature of experimental animals was low, the effects of electroacupuncture at Jian Xi on reperfusion-induced arrhythmias could be observed. The arrhythmias shortened in rabbits, and more ventricular extrasystoles following reperfusion were seen in rats. It is suggested that electroacupuncture at Jian Xi can exert significant influence on reperfusion-induced arrhythmias.

[Effect of hyperbaric oxygenation on the extrasystole in ischemic heart disease patients]. / Vliianie giperbaricheskoi oksigenatsii na ékstrasistoliiu u bol'nykh ishemicheskoi bolezn'iu serdtsa.

A study of the effect of hyperbaric oxygenation (HBO), applied as single sessions or a treatment course, on cardiac rhythm in 82 coronary patients with chronic extrasystole demonstrated positive therapeutic effect to be achieved after the first 5 or 6 HBO sessions in all patients. Further treatment may provoke an aggravation of arrhythmia in some patients. Positive antiarrhythmic effect of a complete HBO course is doubtful, where ectopic activity is increased following the 7th session. There is a correlation between the results of ECG monitoring from the hyperbaric chamber, from session 7 onwards, and 24-hour ECG recording before and after a HBO course. As shown by 24-hour ECG monitoring, positive effect was obtained in 67% of patients with ventricular extrasystole, the number of ventricular extrasystoles remained basically unchanged after a HBO course in 17%, and ectopic activity increased in 15.4%.