ANAESTHESIA AND PROGRESSIVE MUSCULAR DYSTROPHY

[Progressive muscular dystrophy].

This paper describes the case of a young man with progressive muscular dystrophy who had supraventricular arrhythmias and intractable congestive failure. Hemodynamic studies and left cineventriculographic findings were compatible with a diagnosis of severe left ventricular failure. Coronary cinearteriography showed an abrupt ending of the artery to the sino-atrial node. At necropsy, a noninflam...

Progressive Muscular Dystrophy (Erb.)

Genetic-epidemiological studies in progressive muscular dystrophy.

A genetic and epidemiological study is especially important in those hereditary diseases for which no effective treatment is known, since then genetic counselling is the only means of prevention. The progressive muscular dystrophies are typical conditions of this sort. Knowledge of the type of hereditary transmission for a given form of muscular dystrophy is the basis for correct genetic progno...

The electrocardiogram in Duchenne progressive muscular dystrophy.

Interpretation of the electrocardiogram in the Duchenne form of progressive muscular dystrophy (PMD) is still debated in the literature. The group studied consisted of 106 patients with the Duchenne type of PMD, aged 3 to 29 years. The duration of the disease varied from a few months to 15 years. One hundred one patients had abnormal electrocardiograms. These were of four types: type a (64 case...

Moderately progressive Ullrich congenital muscular dystrophy.

OBJECTIVES To describe genetic and clinical features of Ullrich congenital muscular dystrophy (UCMD), and to report the case of a patient diagnosed with UCMD after an exhaustive investigation, which included collagen VI immunohistochemical and genomic analyses. DESCRIPTION This study was based on clinical, immunohistochemical assessment of muscle tissue and genomic analysis of dermal fibrobla...