A frequent occurrence of ophthalmoplegia and muscle fatigability in mitochondrial myopathy (MAM) often makes its differential diagnosis from myasthenia rather difficult. Neuromuscular transmission was investigated in 9 patients with MAM, presenting marked fatigability. The aim of the study was to see whether there were any other causes of muscle fatigability in addition to the metabolic factors. Classical electrostimulation as well as the SFEMG, which is very sensitive in detecting neuromuscular transmission disorders, were used. The findings were far from uniform: we found normal neuromuscular transmission in 5 cases, in 3 patients we observed slight abnormalities of neuromuscular transmission, in 1 case neuromuscular transmission disturbances seemed to be of neurogenic origin. Our results allow an assumption that the causes of muscle fatigability in MAM are of a much more complex nature than it has been anticipated. They might depend not only on the metabolic disorders within the muscle fibre itself but also on the impaired function of the peripheral nerve or of the neuromuscular junction. All the mechanisms combined may also play a role, though in individual patients the contribution of particular factors responsible may vary.

Adolescent↗

PubMedDate not supplied

[Facilitation phenomenon in electrophysiological diagnosis of myasthenia gravis].

A group of 10 patients with atypical result of repetitive stimulation was selected out of the patients examined in our EMG Laboratory. The diagnosis of myasthenia was clinically confirmed. In all those 10 patients no essential amplitude decrement was found on stimulation, so the electrophysiological confirmation of the diagnosis was lacking. On repetitive stimulation (3 Hz) all increment of the response was observed and also post-tetanic facilitation was present. In SFEMG moderate neuromuscular transmission disturbances were found in those patients. It suggests that, sometimes, facilitation may reflect disturbed neuromuscular transmission even despite the absence of the typical amplitude decrement.

Action Potentials↗

PubMedDate not supplied

[A trial of cronassial use in late postpoliomyelitis syndrome].

The results are presented of treatment with gangliosides (Cronassial) in 5 patients with late postpoliomyelitis syndrome. Clinical and electrophysiological examination were done before and after three months of treatment. Only subjective improvement was found. A year-long follow-up failed to fluid progression of the signs and symptoms that, formerly, were progressing slowly. It seems that Cronassial administration (in larger doses and for a longer time) is justified in patients with peripheral motor neuron lesion.

Adult↗

PubMedDate not supplied

[Favorable results of the treatment of the hyperkalemic form of periodic paralysis with salbutamol].

Two cases of hyperkalaemic periodic paralysis with myotonic symptoms were treated successfully with salbutamol. The methods of managing the hyperkalaemic form of periodic paralysis are discussed in brief.

Administration, Oral↗

PubMedDate not supplied

[Atypical clinical image of myopathy with abnormal mitochondria (MAM) in our cases].

Two peculiar cases of mitochondrial myopathy are presented. In the first case the diagnostic difficulties are discussed stressing especially the differentiation of the myopathy from myasthenia. In the second cases polyneuropathy signs were evident, which is extremely rare in this myopathy.

Adult↗

PubMedDate not supplied

[Post-poliomyelitis syndrome. Description of 2 cases].

The authors describe two cases of slowly progressing damage of the peripheral motor neuron which developed in the patients several tens of years following typical acute poliomyelitis anterior in childhood. The clinical features of these cases and the results of electrophysiological investigations are reported in detail. In the light of these cases and a literature review the authors discuss the clinical findings, course, electrophysiological, virological and immunological investigations, and hypotheses concerning the pathological mechanism of the postpoliomyelitis syndrome.

Adult↗