Miopatije i poremećaji funkcije mišića

Abstract

Myopathic affections are generally looked upon, in accordance with accepted concept, as separate nosologic units. However, successive accumulation of facts combined with clinical experience inevitably lead to the belief ­ in the author's opinion ­ that such affections are only clinical pictures of a general derangement of the organism bringing about first of all certain disturbances in different parts of the central nervous system which are then followed ­ as a consequence of the above­named primary disturbances ­ by various symptoms, forms and processes of the disorders in question.

According to clinical pictures and other related disorders in myopathy, the author classifies all myopathic affections sorting them into two basic groups. The first group is represented by Myotonia congenita (Thomsen), Myotonia dystrophica (Steinert­Curschmann), Dystrophia musculorum progressiva (Erb), and also Dystrophia musculorum climacterica, which may be considered as belonging here. The second group comprises Paralysis familiaris periodica, Myasthenia gravis pseudopara1ytica an d Myatonia congenita (Oppenheim).

After a detailed exposition of relevant facts, the author elaborates a novel conception of the motor plate as a peripheric neuron, thus inferring the uniform plan of the make­up of all peripheric nerves.

On the basis of clinical manifestations and specific disorders in myopathy the author draws the conclusion that the first group of myopathic affections is concerned with primary disorders of higher neurons and centers, the myoneural disfunction here being only due to the freeing of the motor end plate from the inhibitory action of upper neurons. The second group, however, is concerned with a primary disorder of higher neurons and centers as well as a contemporaneous primary disorder of the motor end plate as a peripheric neuron which has remained at a lower phylogenetic degree of growth.

After a final analysis of clinical manifestations, specific disturbances of metabolism, hormonal and vegetative disorders as well as hereditary peculiarities, the author sums up by concluding that all myopathic affections can be proved to possess a common feature, i. e. all of them exhibit early changes with manifestations showing their location to be predominantly in formations of the central nervous system around the central canal of the spinal cord and cerebral chambers. On to these primary lesions of the central nervous system link consecutively disorders of the central regularization and those of metabolism with contemporaneous primary disturbances of the motor end plate as a peripheric neuron, bringing about ­ as a consequence of all these disorders ­ corresponding changes in skeleton muscles.

As to the nature itself of these lesions of the central nervous system in myopathy, the author is of the opinion that in all probability it is the primary disorder of the cytodenderogenesis that is in question here, predominantly of those formations round the central canal of the spinal cord and cerebral chambers. The resulting clinical pictures of myopathic affections vary according to the nature and the particular spot of the central nervous system where the disorder of the cytodendrogenesis takes place, but in all cases the primary disorder is m the process of cytodendrogenesis itself all the rest of the symptoms being, of a secondary nature.

Adopting this basic conception as the best, the author lays down the mam lines of therapy in myopathy. In this connection careful consideration is given in the first instance to means and methods conducive to speeding up and regulating the retarded cytodendrogenesis and thus causally influencing and promoting recovery. With reference to this, of all conventional remedies and methods of therapy the author gives preference in the first place to current preparations of glutamic acid, and in the next place to methods of treatment (insulin, antibiotics, and the like) influencing primarily the diencephalic hypothalamus region.