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COENZYME Q IN DUCHENNE MUSCULAR DYSTROPHY
Author(s) -
SÖVIK ODDMUND,
STRÖMME JOHAN H.,
FOLKERS KARL
Publication year - 1971
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1971.tb06681.x
Subject(s) - duchenne muscular dystrophy , medicine , muscular dystrophy , aldolase a , identical twins , coenzyme a , endocrinology , pediatrics , enzyme , biochemistry , biology , reductase
Summary The effect of hexahydrocoenzyme Q 4 (CoQ) has been studied in 4 cases of Duchenne muscular dystrophy, including a pair of identical twins. CoQ was given by the oral route in doses increasing from 50 mg to 1 g daily. In the case of the twins one received CoQ, whereas the other was given corn oil as a control. During a period of treatment of more than one year no improvement in muscular strength was observed. On the contrary, there was a steady progression of the disease in 3 of the 4 cases, with an identical clinical course in the twins. No overall consistent decrease in CPK or aldolase activities was observed during the period of treatment, but there was a statistically significant decrease of CPK and aldolase for one boy. The absence of overall beneficial changes during this particular trial might have been because (a) hexahydrocoenzyme Q 4 is intrinsically inactive in Duchenne dystrophy; (b) the dose level was too low; (c) the disease in these particular boys was too advanced. Besides these limitations, this trial also does not exclude the possibility that other homologs of the coenzyme Q group may have therapeutic benefit in human muscular dystrophy.