THE DIAGNOSTIC SIGNIFICANCE OF SERUM ENZYMES AND ELECTROCARDIOGRAM IN VARIOUS MUSCULAR DYSTROPHIES

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Abstract
Clinical differentiation of Duchenne MD (DMD), Becker MD, Iimbgirdle (LGMD) and benign congenital MD (BCMD), in the early stages is not always possible and may require observation for several years before the correct diagnosis can be reached. DMD, with its rapid downhill course, has at the age of 10 years reached the stage which is far beyond that seen usually in patients affected with one of the other three MDs. Becker MD, LGMD, and BCMD, at that age, may still present similar clinical features. Helpful differential diagnostic criteria discussed in this article are summarized in Table 8. If the patient is a girl, it would be unlikely for her to have X-linked MD. She most likely represents a case of LGMD or BCMD, which may still not be distinguishable from each other in the early stages. Highly elevated CPK and Ald levels are typical for early X-linked MDs and BCMD and uncommon in LGMD. LDH and SGOT are very high in the early stages of DMD, high in Becker MD and BCMD and distinctly less elevated in LGMD. Thus serum enzyme levels are helpful in distinguishing LGMD from the other three MDs. Electrocardiographic abnormalities are common in the X-linked MDs. Tall right precordial R-waves with or with out deep Q waves in the left precordial leads are found in the majority of patients with X-linked MD. It is, however, not a constant finding of X-linked MD. Prescence of the EGG abnormalities appears to exclude MD with autosomal inheritance, notably LGMD and BCMD.