[Cardiac involvement in Kugelberg-Welander disease. A prospective study of 8 cases]

Kugelberg-Welander disease is a juvenile form of slowly progressive spiral amyotrophy in which the incidence of cardiac involvement is difficult to appreciate as cases are sporadic. Classically, it presents with atrial hyperexcitability with variable degrees of atrioventricular block. In order to assess the prevalence of cardiac involvement in this condition, the authors undertook a prospective study in 8 patients with Kugelberg-Welander disease. All underwent clinical and paraclinical examinations to assess cardiac function and rhythm. Doppler echocardiography, myocardial scintigraphy with Thallium 201 and Technetium99m, resting ECG. 24 hour Holter monitoring and signal averaged ECG were performed in all cases. This unique study detected one patient with infraclinical disease of the conduction system who became symptomatic during the follow-up period and required definitive cardiac pacing. During the study, one 20 year old man with Kugelberg-Welander disease was admitted to hospital with dilated cardiomyopathy with a fatal outcome. Because of the small number of patients and after a review of the literature, this study is to be continued to obtain a more accurate evaluation of cardiac involvement in this neuromuscular disease.