[Attempts at treatment of amyotrophic lateral sclerosis].
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Biomedical subjects
Publications and source records attributed to T Domzal.
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The authors investigated the electromyographic response to mechanical stimulation of the palmar skin (palmo-mental reflex) and plantar skin (planto-mental reflex) in the mentalis muscle. The palmo-mental reflex was present in EMG in all 22 healthy subjects an in 51 cases of 51 cases of limited cerebral lesions. The planto-mental reflex was absent in healthy subjects, but it was found in 14 out of 31 cases of focal brain injury and in 2 out of 20 cases with diffuse lesions. The planto-mental reflex is a pathological reflex appearing mainly in severe focal cerebral lesions. No description of this reflex was found in the available literature.
The author reviews the pertinent literature and the results of own investigations in migraine. EEG changes in migraine are observed in nearly 50% of cases during attacks as well as in the periods free of pains. Most investigations were done in the periods between attacks. The H response characteristic of migraine was found by the author in 25% of cases only. Focal changes were present in 30% of cases. They were not related to the side of the pain, its duration and the form of migraine. Seizure activity was never observed. The author regards isolation of the so-called dysrhythmic form of migraine as not justified. EEG changes suggest--according to the author--that migraine is a primary cerebral and only secondarily a vascular disorder.
The authors report a family in which the mother and her one son had typical eipsodes of flaccid muscular paralysis with raised serum potassium level during the episode. In the period between these episodes only myotonia was observed. Histological examinations and EMG confirmed the diagnosis. Myotonia was present in two other siblings.
In Huntington's chorea the biochemical disturbances are to some degree a reverse of those observed in Parkinson's disease and a failure of the cholinergic system is prevalent. Former attempts at treatment were based on blockade of the dopaminergic system. The author suggests that the general line of treatment should be -- similarly as in Parkinson's disease -- not blockade of the predominant system but enhancing the cholinergic activity by administration of acetylcholine precursors and agents blocking cholinesterase. Eight patients were treated in this way and significant improvement was achieved in half of them. Further therapeutic trials along these lines are justified theoretically and the main problem will be to find substances crossing the blood-brain barrier and acting more strongly.
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