Letters to the Editor

FETAL PLASMA CARBONIC ANHYDRASE III IN DUCHENNE DYSTROPHY

A highly sensitive sandwich enzyme-immunoassay (EIA) for human muscle carbonic anhydrase isozyme III (CA-III) has been developed using microplate as a solid-phase and peroxidase as a labelled enzyme. The assay can detect levels as low as 2 ng/ml when 20 μl of sample sera were used. Sera from patients with various neurological diseases were studied using this method, and elevated serum CA-III levels were found in patients with Duchenne muscular dystrophy, limb-girdle dystrophy, fascioscapulohumeral dystrophy, polymyositis and amyotrophic lateral sclerosis. The values correlated well with the results of radioimmunoassay (RIA), with a correlation coefficient of 0.92 (P < 0.001). We feel EIA is preferable to RIA for its simple methodology.

An elevation of phosphoglycerate mutase (PMG) has been detected in the blood plasma of the genetically dystrophic chicken and in Duchenne muscular dystrophy (DMD) patients. In the dystrophic chicken, plasma PGM activity reached a peak of about 20-fold the normal level. The content of PGM in the pectoral muscle was simultaneously depressed to less than one-half that of the normal chicken. In a group of 9 DMD patients, plasma PGM activity was found to be significantly raised above the normal range. A survey of a small group of plasma specimens from human fetuses at risk for muscular dystrophy also suggested that PGM merits investigation as a potential adjunct to other diagnostic indices.

Plasma carbonic anhydrase III (CAIII) and creatine kinase (CK) were measured in 44 patients with a variety of neuromuscular disorders.

Markedly elevated CK levels were associated with similarly increased levels of CAIII. In 9 patients, only the CAIII was elevated, but in 2 patients only the CK was raised. The determination of plasma CAIII is thus an important index of muscle disorder and is probably more sensitive than CK.