고칼륨혈증 주기성마비 환자의 운동 및 한기유발검사에 의한 전기생리학적 변화

Abstract

Hyperkalemic periodic paralysis and paramyotona congenita share common clinical manifestations, such as autosomal dominant diseases with missense mutations at a gene encoding α-subunit of skeletal muscle voltage sensitive sodium channel (SCN4A). Exercise and cold provocation tests are physiological phenomena of clinical characteristics of these diseases. The authors experienced a case with hyperkalemic periodic paralysis and performed these tests comparing them with a patient with hypokalemic periodic paralysis and a normal person. Significant decremental changes of CMAPs were found by both tests in the case with hyperkalemic periodic paralysis, compared with those in a case of hypokalemic periodic paralysis or normal control. In conclusion, we suggest that exercise and cold provocation tests may be useful for the differential diagnosis between hyperkalemic periodic paralysis and hypokalemic periodic paralysis.