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Peak negative myocardial velocity gradient and wall-thickening velocity during early diastole are noninvasive parameters of left ventricular diastolic function in patients with Duchenne's progressive muscular dystrophy.

Myocardial velocity gradient and wall-thickening velocity were measured in the interventricular septum and left ventricular posterior wall (LVPW) by color-coded M-mode Doppler tissue echocardiography in patients with Duchenne's progressive muscular dystrophy (DMD) with a normal shortening fraction (n = 14) and age-matched control subjects (n = 40). In the LVPW, peak myocardial velocity gradients during systole and early diastole were significantly lower for patients with DMD than in control subjects (P <.0005, and P <.0001, respectively). Peak myocardial wall-thickening velocities of the LVPW during systole and early diastole were also lower for patients with DMD (P <.0005 and P <.0001, respectively). Mitral peak atrial to early filling velocity ratio was not significantly different between the 2 groups. The cut-off values of peak myocardial velocity gradients and wall-thickening velocities of the LVPW during early diastole for differentiation between patients with DMD and control subjects were -5.8/s and -6.0 cm/s, respectively (sensitivity/specificity: 93%/93% and 93%/85%, respectively). In conclusion, wall thinning during early diastole is frequently abnormal in patients with DMD, even when conventional echocardiographic findings are normal.

Adolescent↗

Serum carbonic anhydrase III in progressive muscular dystrophy.

Serum carbonic anhydrase III (CA-III) levels were determined by means of an enzyme immunoassay method and compared with serum creatine kinase (CK) and muscle-specific enolase (MSE) levels in 143 patients with four types of progressive muscular dystrophy (PMD), namely, Duchenne muscular dystrophy (DMD), limb-girdle dystrophy, facioscapulohumeral dystrophy and congenital dystrophy. Serum CA-III levels were raised in the majority of patients, especially in those with DMD. In DMD patients, the gradual decline in the CA-III level was observed with age. High correlations were found between CA-III, CK and MSE levels. The frequency of cases with elevated CA-III levels was the same as or greater than that of elevated CK or MSE levels in four types of PMD. These results suggest that serum CA-III may be a useful marker of muscle disease.

Adolescent↗

[A case of ophthalmic manifestations in progressive muscular dystrophy].

A case of bilateral eyelid ptosis was observed in a woman aged 35. She had also some weakness of the muscles of the upper extremities and periodic dysphagia. These symptoms progressed gradually during 20 years. The clinical manifestations and the results of other investigations (EMG, muscle biopsy) made possible the diagnosis of sporadic ophthalmic form of progressive muscular dystrophy.

Adult↗

[Status of connective tissue in progressive muscular dystrophies].

60 patients with different forms of neuromuscular disorders were examined. Morphological studies of skeletal muscles and of diurnal excretion with urine of acetic GAG were carried out. It was established that the changes in the stromal connective tissue in the progressive muscular dystrophia appear in the early stages of the disease and affect both the essential substance and the fibrillar structures. The excretion with the urine of acetic GAG was increased. In denervative amyotrophy the changes became apparent against the background of marked clinical symptoms. The data obtained may be important for a differential diagnosis of progressive muscular dystrophy and denervative amyotrophy, and for the development of differential drug therapy.

Adolescent↗

Skeletal muscle pathology in ovine congenital progressive muscular dystrophy. 2. Myofiber morphometry.

Fiber-type proportions were determined in 12 skeletal muscles (peronaeus tertius, tibialis cranialis, tensor fascia lata, psoas major, extensor digitorum lateralis, tensor fascia antibrachii, vastus intermedius, soleus, anconaeus, and flexor digitorum superficialis of fore and hind limb) from infantile (20 weeks), juvenile (16 months) and adult (2.5 and 3.5 years) Merino sheep with ovine congenital progressive muscular dystrophy and in age-matched controls. Although confined to type I fibers, lesions were severe not only in type I fiber dominant muscles (vastus intermedius, soleus and anconaeus) but also in the type II dominant medial triceps brachii and in the superficial digital flexors which had approximately equal proportions of both fiber types. The frequency distribution curves of myofiber diameter in dystrophic anconaeus and superficial digital flexor of the fore limb altered as the disease progressed. In young sheep (20 weeks) type I fibers increased in size, then in juvenile and adult sheep the curves became flatter and broader and lost symmetry due to the presence of hypertrophic and atrophic fibers. The fiber diameter profiles were similar to those seen in Duchenne dystrophy in humans.

Animals↗