The cause of some diseases are disorders of ion channels which are identified in the classification nowadays as a separate group as channelopathies. They can be congenital, genetically-conditioned and autoimmune (antibody-mediated) and pertain mainly to muscles incl. neuromuscular junction, perip- heral nerves and the CNS. The author presents a review of sodium, chloride, potassium and calcium channelopathies and some hitherto unknown channelopathies as well as the basic characteristics of different groups. At present the following channelopathies are known: disorders of sodium channels which comprise: hyperkalaemic periodic paralysis, paramyotonia congenita, myotonia fluctuans and permanens, myasthenia gravis, malignant hyperthermia type 2 and the syndrome of a long Q-T interval type 3. Disorders of the chloride channel which cause congenital Thomsen and Becker myotonia. Disorders of the potassium channel with the following units: acquired neuromyotonia, episodic ataxia with myokymias and the syndrome of the long Q-T interval type 1 and 2. Disorders of the calcium channel which produce hypokalaemic periodic paralysis, malignant hyperthermia type 3, episodic ataxia type 2, spinocerebellar ataxia type 6, familial hemiplegic migraine and Lambert-Eaton myasthe- nic syndrome.

        Key words: ion channels, periodic paralysis, myotonia, paramyotonia, neuromyotonia