Neurological assessment (including MRC scale) at the time of myosonology showed clinical features of myopathic syndrome more pronounced for distal leg muscles in all patients. Normal conduction velocities of the fibular, tibial, median nerves and myogenic changes of distal calves and hand muscles were found by electromyography. An advanced muscular dystrophy was proved by muscle biopsy, performed in the patients with VCPDM and TMD buy VE-822 ( Table 1). Triceps surae muscles were evaluated in a lying position by using a special probe for 3D/4D real time

imaging (Logic 7, GE). The transverse diameter of both TS heads in longitudinal MG-132 in vivo plan, the angle of inclination of the muscle fibers towards the surface of the aponeurosis and 3D/4D imaging of calf architectonics were evaluated in rest and during maximal plantar flexion (PF). The results were compared to myosonograms of 3 age- and sex-matched healthy controls. The normal TS myosonogram is demonstrated in Fig. 1.

The whole muscle is enveloped by hyperechoic epimysium. The muscle fibers are hypoechoic and grouped in fascicles, divided by hyperechoic septs of fibrous and fat tissue of the perimysium. In a longitudinal B-mode image the perimysium is depicted as oblique parallel hyperechoic lines. The PF causes calf muscle contraction that increases the transverse muscle diameter and the angle of muscle fiber towards the aponeurosis. The 4D ultrasound imaging shows a reticular TS architectonics despite the muscle activity, age and sex of healthy controls. Its hypoechoic areas increase during PF, very due to thickening

of the contracted muscle fibers. Compared to healthy controls all patients with DM had a reduced transverse TS diameter and decreased muscle contractility. The muscle fibers were inclined and their orientation was under a smaller angle towards the aponeurosis during rest and PF (Fig. 2). The normal reticular muscle structure was replaced by granular myoarchitectonics – a combination of spot-like hypo- and hyperechoic areas on 4D ultrasound imaging was found in association with the degree of muscle atrophy, fat tissue infiltration and fibrosis. The hyperechoic areas had a tendency of fusing in the patient with HIBM2 (Fig. 3). Distal myopathies are a group of genetically and clinically heterogeneous disorders classified into one broad category, due to the presentation of weakness involving distal skeletal muscles of upper and lower limbs.