The nature of the electrophysiological disorder in adynamia episodica.
نویسندگان
چکیده
In 1934 Biemond and Daniels first showed that the serum potassium level fell during paralytic episodes in patients with familial periodic paralysis. Subsequently a normokalaemic form of the disease was recognized (1951), while more recently a hyperkalaemic variety was described by Helweg-Larsen, Hauge, and Sagild (1955). This last type of periodic paralysis was given the name adynamia episodica hereditaria by Gam-storp (1956). In view of the functional relationship between electrical potential and potassium conductance across cell membranes (Hodgkin, 1958), it has been widely anticipated that intracellular recording from muscle fibres in these various conditions might prove a valuable analytical approach. Up to the present time such studies have been carried out on patients with hypokalaemic familial periodic paralysis by while the last authors have also investigated a single patient with the hyperkalaemic variety. Recently we have had an opportunity to make intracellular recordings, between attacks, in a further four patients with hyperkalaemic paralysis and in one with the normokalaemic syndrome. The results to be described indicate that the hyper-and normokalaemic varieties appear to be functionally similar in so far as the resting membrane potential is reduced in both. In addition we have employed intracellular stimulation to investigate the electrical properties of the fibre membranes more fully; the results obtained have been considered in relation to the genesis of the paralytic episodes.
منابع مشابه
Adynamia episodica hereditaria: what causes the weakness?
The cause of weakness was investigated in a patient with adynamia episodica hereditaria without myotonia. A pattern of exercise and rest produced episodes of hyperkalemic periodic paralysis. In addition, local muscle weakness was induced by forearm cooling. Investigations on isolated intercostal muscle demonstrated that a high potassium concentration in the bathing solution triggered a noninact...
متن کاملTwo cases of adynamia episodica hereditaria: in vitro investigation of muscle cell membrane and contraction parameters.
Membrane potentials, current-voltage relationships, and contractile parameters were studied in intact muscle cell bundles obtained from two patients with adynamia episodica hereditaria. In a normal extracellular medium, the cell membranes had resting potentials of about -80 mV and their current-voltage relationships were not significantly different from control curves. In contrast to normal mus...
متن کاملNew classification and treatment for myotonic disorders.
Myotonia is repetitive firing of muscle action potentials causing prolonged muscle contractions even after mechanical stimulations to the muscles have ceased. Most common myotonic disorder is myotonic dystrophy which is now termed DM1, myotonic dystrophy type 1. In Japan, proximal myotonic myopathy, which is now called DM2 has not been reported. Both DM1 and DM2 have Cl channel abnormality whic...
متن کاملADYNAMIA EPlSODlCA HEREDITARIA SODIUM CURRENT AND THE EFFECT OF EXTRACELLULAR pH WITH MYOTONIA: A NON-INACTIVATING
of intact muscle fibers biopsied from a patient who had adynamia episodica hereditaria with electromyographic signs of myotonia. When the potassium concentration in the extracellular medium, [K],, was 3.5 mmol/l, force of contraction, membrane resting potential, and intracellular sodium activity were normal, but depolarizing voltage clamp steps revealed the existence of an abnormal inward curre...
متن کاملElectrophysiological Study of Sciatic Nerve Regeneration Through Tubes Seeded with Schwann Cells
A B S T R A C TIntroduction: Peripheral nerve injury is a common disorder and leads to permanent neurological defects. Schwann cells have been shown to have nerve repair after being transplanted in peripheral nerve injury. The aim of this study was to determine the beneficial effect of allograft Schwann cells on electrophysiological outcome after transection of the sciatic nerve in rats.Methods...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Journal of neurology, neurosurgery, and psychiatry
دوره 31 5 شماره
صفحات -
تاریخ انتشار 1968