periodic paralysis周期性麻痹.ppt

Periodic Paralysis 周期性麻痹;Ⅰ. Definition of periodic paralysis ;According to the level of serum potassium during attacks, the diseases are divided into three main types: hypokalemic periodic paralysis(HoPP) hyperkalemic periodic paralysis(HyPP) Normokalemic periodic paralysis (NormoPP) Sporadic HoPP is the most commom type in Chinese.;Ⅱ. Etiology and Pathogenesis of Hypokalemic Periodic Paralysis ;Pathogenesis of HoPP;Skeletal muscle calcium channel a1S , CACNA1S, Cav1.1;Ⅲ. Clinical presentation of HoPP ;?The typical attack comes on during the second half of the night or the early morning hours. Excessive hunger or thirst, dry mouth, palpitation, sweating, diarrhea, nervousness, and a sense of weariness or fatigue are prodromata(前驱症状）. Weakness: the paralysis varies from slight weakness of the legs to complete paralysis of all muscles of the trunk and limbs.;Legs are affected earlier than arms; limbs are affected earlier than trunk; proximal muscles are more susceptible than distal ones. Oropharyngeal and respiratory muscles are usually spared 9. ? The attack evolves over minutes to several hours, weakness may last a few hours to several days.;10. Serum potassium is reduced (below the normal level) during attacks 11. ECG shows hypokalemic features: prolonged PR, QRS, and QT intervals and flattening T waves 12. In the interval between attacks, muscle strength is normal, and the serum potassium level is normal 13. Attacks of paralysis tend to occur every few weeks, or every few years, and to lessen in frequency with advancing age ;ECG Changes;Ⅳ. Diagnosis and differential diagnosis ;⑴ Secondary Hypokalemic Periodic Paralysis ;;(2) OtherTypes of Periodic Paralysis ;②Normokalemic Periodic Paralysis (NormoKPP) ;(3) Other Diseases That Cause Acute Paralysis ;Ⅴ. Treatment and management of HoPP ;Ⅴ. Treatment and management of HoPP;周期性麻痹;低钾型周期性麻痹;;每次发作持续数天至1周，发作频度不等，少者一生仅发作一次，多则一个月发作多次， 肌肉瘫痪时，腱反射降低或消失，感觉正常。 血清钾