Clinical Neuroscience

[Tests for paroxysmal myoplegia. III. Permanent lesions of the muscular system. Development of metabolic myopathy. ]

BEKÉNY György1

DECEMBER 01, 1960

Clinical Neuroscience - 1960;13(12)

[The author reviews the literature on cases of paroxysmal paralysis in which irreversible musculoskeletal lesions have been demonstrated. He describes the relevant data of two hypokalemic and two hyperkalemic cases published with Hasznos and Solti. A significant proportion of paroxysmal paralysis cases develop persistent paresis and atrophy. The almost regular development of muscle lesions is confirmed by muscle biopsy findings. Muscle biopsy showed significant pathological changes in three of our cases where permanent paresis and atrophy were not present. Permanent muscle paralysis develops in the same muscles most affected by the seizure. The weakness of the pelvifemoral distribution and the hypervoluminal leg cramps are highly reminiscent of the muse, progr. pseudohypertrophic form of dystrophy. This is the reason why the association of these two pathologies has been frequently reported in the literature. After a critical analysis of these cases, the author concludes that there is no known case of paroxysmal paralysis and dystrophia muse, progr. in the same patient. The decisive evidence on this point is provided by the difference in the pathophysiological lesions. The muscle pathological lesion of paroxysmal paralysis is essentially vacuolar degeneration. The histopathological picture of hypokalemic and hyperkalemic cases is identical. The permanent muscular lesions of paroxysmal paralysis are the result of ion shifts and colloid chemistry changes associated with seizures. The paroxysmal osmotic changes initially result in reversible vacuolar formation. Later, irreversible degeneration of the loosened vacuolar centre of the muscle fibre is initiated. A metabolic myopathy associated with periodic recurrent potassium (and sodium ?) metabolism disturbances is therefore involved. The pathogenesis and pathomechanism of the muscle lesions just outlined are also likely to be due to a potassium-deficient diet and to myopathies induced by the administration of various glucocorticoids. ]

AFFILIATIONS

  1. Budapesti Orvostudományi Egyetem Neurológiai Klinika

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