The diurnal changes of serum myoglobin level and CK activity were investigated in 20 cases of Duchenne dystrophy and 6 normal males under ordinary circumstances in their daily lives. Changes of myoglobin levels were also checked in 25 cases of Duchenne carrier and 11 control females with muscular exercise by ergometer of 70 W for 3 min. In Duchenne dystrophy, the myoglobin level was rather low before waking up, then it abruptly increased thereafter and remained high until retiring. The fluctuation range of myoglobin was greater than that of CK. The myoglobin level of control males was extremely low compared with that in Duchenne dystrophy. In Duchenne carriers, although CK activity did not significantly change after the exercise, 5 of 11 known carriers showed an increased myoglobin level.

Adolescent↗

PubMed1982

Genetic linkage study of Duchenne muscular dystrophy and hemophilia A.

We studied a family in which three boys had both Duchenne muscular dystrophy and hemophilia A. Seven other males had only hemophilia A. Genetic linkage study showed a maximum likelihood estimate of recombination frequency of these two X-linked recessive traits of 0.16 with 95% confidence limits of 0.045 and 0.50. The loci of the two diseases may be closer than previous estimates.

Adolescent↗

PubMed1982

Effects of thyrotropin releasing hormone on cerebellar mutant mice--a kinesiological comparison between rolling mouse Nagoya, weaver and reeler.

Rolling Mouse Nagoya (RMN), weaver and reeler mice were examined for the clinical effectiveness of drugs (thyrotropin releasing hormone (TRH), reserpine, L-dopa) that are reported to facilitate or alter the metabolic system of noradrenaline (NA). Behavioral changes and ataxic gait were recorded by 16 mm cinefilms and EMG of the thigh extensor muscles. In addition, latencies in the reflex discharge of the muscle by tilting load were measured before and after the intraperitoneal injection of each drug and compared to those of normal littermates. TRH injection increased spontaneous behavioral activity in RMN and weaver, and shortened the latency of the reflex discharge by tilting load in RMN. Reserpine injection did not increase behavioral activity, but was accompanied by a slight shortening of latencies in the reflex discharge by tilting load only in RMN. L-dopa did not affect behavioral and EMG activities of these cerebellar mutant mice. No remarkable deficit has yet been in any selective intracortical neurons in RMN and no other characteristic abnormality in central and peripheral nervous, skeleton and muscular systems. It was, therefore, suspected that there was a possibility of metabolic disorders of NA in the cerebellum not only in RMN, but also in patients with certain spinocerebellar degenerations.

Animals↗

PubMed1982