Clinical Neuroscience

[Studies in paroxysmal myoplegia]

BEKÉNY György1, HASZNOS Tivadar1, SOLTI Ferenc1

JUNE 01, 1960

Clinical Neuroscience - 1960;13(06)

[Authors studied a paroxysmal paralysis patient and his son published by Benedek and Angyal in 1943. The hyperkalaemia (7.4 maequ) during spontaneous seizures in the father and the seizure-inducing effect of potassium in the son made the hyperkalaemic nature of paroxysmal paralysis beyond doubt. The noteworthy features of the cases are as follows : in the father, the development of persistent pelvifemoral paresis, which had incapacitated him in recent years, was well documented on the basis of the numerous pathological data available since the age of 18 years. In both cases, muscle biopsy showed pathological changes (mainly vacuolar degeneration) consistent with a hypokalemic form of paroxysmal paralysis. In the father, the EEG showed a proliferation of fast rhythms on the day before the spontaneous seizure and then frontotemporal theta waves on the first day of paralysis. The otherwise normal urinary aldosterone excretion was markedly increased before and during the seizure. The EEG changes and aldosterone hypersecretion preceding and accompanying the seizure suggest that the pituitary, hypothalamus, diencephalon system are involved in the seizure genesis. The central neuroendocrine function changes should be attributed to the seizure initiating and integrating influence. In hyperkalaemic cases, the seizure is probably due to a shift of intracellular (muscle) potassium towards the extracellular space. This may cause paralysis by depolarisation of the muscle cell membrane. Studies to confirm this hypothesis, such as simultaneous arterial and venous K determinations to detect K movement and muscle K determination in seizures, are still lacking. A particularly problematic aspect of the pathomechanism of paralysis is that the hypokalaemic and hyperkalaemic forms are equally affected by muscle work (reducing paresis) and rest (provoking seizures) and that in both forms aldosterone hypersecretion precedes and accompanies the seizure. The authors analyse in detail and summarize in a table the essential differences and identities of the Gamstorp form of adynamia episodica hereditaria and the hypokalemic form. The authors' cases differ in several features from the findings of the Scandinavian authors (onset of severe persistent paresis, paralytic seizures lasting 3-4, exceptionally 8 days, absence of muscle hyperexcitability on EMG during the seizure, reduction in mechanical and electrical excitability during the seizure, etc.). According to the authors, the name adynamia episodica hereditaria does not cover the symptomatology. In any case, the hyperkalaemic form is not a separate disease entity that could require a separate name. For the latter, the name Gamstorp's hyperkalaemic paroxysmal paralysis is recommended.]

AFFILIATIONS

  1. Budapesti Neurológiai Klinika és a Budapesti I. sz. Belklinika

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[Adult toxoplasmosis in the form of solitaeric granuloma causing intracranial vasoconstriction]

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[We report a case of acquired toxoplasmosis in a young adult girl, initially with psychiatric symptoms, occasionally with high fever, which was fatal for two years. She presented with clinical symptoms suggestive of a late-stage intracranial space-occupying process and underwent surgical removal of a tumour-like granuloma from the temporal lobe of the left hemisphere. On tissue examination, the lesion was found to be a granuloma containing toxoplasma pseudocysts, which we termed "granuloma toxoplasmoticum solitarium cerebri". The description of our patient adds to the known neurological aspects of toxoplasmosis, and also opens the possibility to consider toxoplasmosis as a background for chronic meningitis in adolescents or adults, and even for toxic delirosis.]

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