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The aim of present study was to analyse possible myopathic changes in the muscles of manifesting carriers of Duchenne Muscular Dystrophy (DMD) using concentric needle emg and macroemg methods. Our material consisted of 10 manifesting carriers aged 9-52 (mean 30 years) and 20 healthy age-matched females. Concentric needle emg (CNEMG) and macroemg was performed in biceps brachii (BB) muscle in both groups: carriers and controls. Myopathic changes were observed in BB muscle in 5 of 10 manifesting carriers using CNEMG and in all cases (10 carriers) using macroemg method. Macro electrode, which reflects total motor unit activity, i.e., the total number and size of component muscle fibres, supplies information about early myogenic lesion of the muscle. Therefore the macroemg seems to be a sensitive and useful electrophysiological diagnostic method in carriers of DMD.

Complex motor unit potentials (CMUPs) with satellites were recorded and analysed in 231 electromyograms of patients with spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD) and a healthy control group. In the control adult group only few CMUPs with satellites were found; no CMUP were found in children. In SMA and DMD patients the CMUPs were present with similar frequency but their morphology, i.e. shape, duration, amplitude of the main and satellite spike components and number of satellites was different. In ALS patients a significant difference between severely involved interosseous muscle and proximal muscles was found. The pathomechanism of CMUPs with satellites is different in myopathic and neurogenic processes, in both however they reflect remodelling of the motor unit. In myopathy they reflect muscle fiber diameter variability and distribution of preserved muscle fibers within the motor unit under study. In neurogenic lesion the CMUPs with satellites are the result of increasing desynchronisation during progressive de- and reinnervation.

The differences in the motor performance during different tasks between 19 subjects suffering from SMA and 10 healthy controls were observed. The simultaneous EMG activity of twelve lower limbs and lower trunk muscles was recorded with surface electrodes. EMG data were automatically reduced and compared with data evaluated from performed by physiotherapist manual testing of muscle strength. Results showed characteristic differences between healthy and spinal muscular atrophy (SMA) subjects: 1. SMA patients display generally more activity occurring in numerous muscle groups and more spinal levels are activated. 2. SMA patients reveal a disturbed functional relation between the posterior and anterior compartments of muscles. 3. EMG activity in SMA patients is spreading out also to the contralateral muscle groups even during slight, unilateral singlejoint movements. Oligosegmental, plurisegmental and brain sources are probably responsible for mentioned phenomena. The reciprocal influences between reduced number of motoneurons (in SMA) and function of central movement generators results in different mode of movement execution in SMA patients.

Examination of the sympathetic skin response (SSR) was performed in 32 patients with systemic sclerosis, morphea and mixed connective tissue disease displaying scleroderma-like features. The control group consisted of 26 healthy subjects and 12 patients with other skin diseases and asymmetrical cutaneous changes. Right and left median and tibial nerves were stimulated successively and the responses were recorded from the palms and soles simultaneously. SSR abnormalities (delayed latency, decrease and/or asymmetry of amplitude, absent response) were observed in 68.8% of the patients, most frequently in linear scleroderma. An amplitude asymmetry of the responses from upper extremities was the most characteristic pattern of abnormalities. There was no correlation between the SSR and the localization, degree and character (inclurated oedema, atrophy, sclerosis) of skin changes, the duration of the disease, symptoms of the disorder of the autonomic nervous system symptoms (vasomotor and/or sudomotor) and the changes in capillaroscopy. All patients with slow motor conduction and sensor conduction velocities (MCV and SCV) had lower SSR amplitude and the patients with prolonged skin sensory chronaxy had more often delayed latency. The results revealed presence of disturbances of the autonomic nervous system in all varieties of scleroderma, both systemic and localized forms, even without any other signs of autonomic dysfunction.

A method for MUAP complexity evaluation is proposed. A complexity coefficient intended for measuring potential complexity is defined, it is a product of filling factor and coefficients measuring the variability of signal parameters. This coefficient has been used to estimate quantitatively the degree of complexity and hence to order a set of potentials. The potential ordering agrees with intuitive expectation. So, it seems that this method may be appropriate for complexity evaluation, and could be applied in the study of remodeling process in neuro- and myopathic diseases.

Our previous paper presenting electromyographic findings in patients with congenital fiber type disproportion myopathy, confirmed the myogenic character of the disease process. That group of patients was however fairly heterogenous regarding both the clinical features and the morphological changes in muscle fibers (e.g. cases with central cores). In the present study we have examined 13 children with hypotonia and muscle fiber type disproportion operated on in childhood for congenital hip dislocation. In all cases CNEMG and SFEMG with FD estimation was performed in biceps brachii and quadriceps femoris muscles. In all muscles examined either slight EMG changes indicative of myopathy or a normal EMG pattern was found. None of the patients demonstrated an evident increase in FD values. Normal FD and the recruitment pattern proportional to the force of contractures indicate that the normal number of motoneurons is preserved. Accordingly, our present findings confirm the conclusions of our previous paper.

In 15 cases with congenital muscle fiber type disproportion, concentric needle EMG (CNEMG) and single fiber EMG (SFEMG) with fiber density (FD) estimation was performed. The aim of the work was to establish the nature of congenital muscle fiber type disproportion. In 11 cases CNEMG revealed interference pattern on maximal effort and low, polyphasic motor unit action potentials (MUAPs) of short duration indicating a myopathic lesion. In four other cases electromyographic changes were less evident. The FD values were slightly increased in 9 cases. This finding could be explained by pronounced muscle fiber splitting with subsequent ephaptic transmission or even secondary denervation and reinnervation. The most important conclusion from our pilot study is the confirmation of the fact that reinnervation with excessive sprouting is not the only mechanism responsible for muscle fiber type disproportion.

The aim of the study is to investigate the motor unit abnormalities in late postpolio muscular atrophy (PPMA) as compared to those found in patients who had polio 20-30 years prior to examination without any new clinical signs. The quantitative concentric needle EMG and a single fiber EMG techniques were employed. Spontaneous activity, the parameters of individual motor units potentials (MUP), number of complex potentials and their stability, jitter and blocking as well as fiber density (FD) have been evaluated. In PPMA patients (5 subjects) we found in newly weakened muscles: spontaneous activity, high percentage of complex potentials, increased jitter, increased FD. The EMG findings in muscles previously affected but without any signs of progression have been similar. In the patients with stable nonprogressing postpolio muscle atrophy (12) all MUP-s parameters indicated changes similar to PPMA but less marked in initially affected muscle with complete or incomplete recovery as well as sometimes in initially clinically unaffected muscles. These findings suggest that the signs of ongoing reinnervation processes persist many years after polio and that PPMA occurring later in life represents disintegration of the previously reinnervated motor units. It is still unclear whether this disintegration depends on decompensation by different factors of fully reinnervated motor units or whether most of the motor units after polio never regained a stable reinnervation.

CN EMG and SF EMG were performed in 12 patients many years after acute poliomyelitis, without symptoms of post-polio syndrome. In 19 muscles with clinical symptoms and in 11 clinically normal muscles chronic neurogenic changes of a similar degree were observed. Fibre density was increased in both groups of muscles. In muscles with clinical symptoms complexes of single fibres with increased jitter and blocking were more frequent than in muscles without clinical manifestations. It seems that the reinnervation after acute polio is a continuing process and thus complete stabilization and integration of the motor unit cannot be achieved.