[Cardiac manifestation of normokatemic periodic paralysis in 21 years old male in Kermanshah]

Normokalemic periodic paralysis is an autosomal dominant disorder involving the abnormal function of skeletal muscle's voltage-gated alpha-subunits. It is characterized by paralysis attack of varying severity with concomitant normal serum potassium concentration. Although normokalemic periodic is well established in the literature, but its cardiac manifestations are very rare. We present 21 years old age male, which referred to Imam Ali hospital with severe palpitation, atypical angina and flaccid quadric paralysis. Electrocardiographic manifestation was first-degree AV block. Bijeminated ventricular extrasystole and severe ST-T change in pericardial leads. Initial potassium level was 4 meq/l, CPK and aldolase level was normal. Echocardiography and exercise test were normal. After four days administration of acetazolamide, his weakness was disappeared and ECG became normal. He discharged from hospital with normal neurological examination and effective treatment outcome

Sporadic hypokalaemic periodic paralysis

To describe presentation, laboratory profile and management of five sporadic cases of hypokalaemic periodic paralysis in nationals of four different countries of Asia. Design: Prospective case study with review of literature. Five cases were diagnosed at the department of Medicine at King Abdul Aziz Hospital, Madinah Al-Munawarrah, Saudi Arabia on the basis of episodic reversible flaccid paralysis with hypokalaemia in the absence of other causes of such paralysis. Mean age of these cases was 32 years and all presented as flaccid paralysis with proximal muscles of lower limbs more severely affected than the upper limbs. Most common precipitating factor was use of carbohydrate rich food. Mean initial serum potassium was 1.6 mmol/l. All had complete recovery when infused potassium in 20% urea. Conclusions: The diagnosis of hypokalaemic periodic paralysis should be kept in mind in any patient presenting with sudden flaccid paralysis and hypokalaemia which does not respond to K+ infused in dextrose or saline

Paralisia periódica: estudo anátomo-patológico do músculo esquelético de 14 pacientes / Periodic paralysis: anatomo-pathological study of musculoskeletal of 14 patients

A paralisia periódica é entidade caracterizada por crises de fraqueza muscular relacionadas com alteraçöes do nível sérico de potássio. A biópsia muscular pode mostrar alteraçöes específicas ou inespecíficas. Nosso estudo tem como objetivo a análise de 18 biópsias musculares de 14 pacientes com paralisia periódica (14 hipocalêmica, 2 hipercalêmica). Todas as biópsias mostraram alguma alteraçäo histopatológica. Quatorze biópsias apresentavam vacúolos, que se caracterizavam por serem únicos, de localizaçäo periférica, de aparecimento frequente e preferentemente em fibras do tipo I. Os vacúolos eram mais visualizados naqueles pacientes com longa evoluçäo e sem relaçäo com a frequência de crises. Os agregados tubulares foram encontrados em 10 biópsias principalmente naqueles pacientes com crises frequentes e doença de longa evoluçäo. Em 3 pacientes foram realizadas 2 biópsias, notando-se piora das alteraçöes em 2. Um paciente evoluiu com quadro clínico de miopatia permanente, confirmado pela biópsia muscular. Alteraçöes inespecíficas foram encontradas em graus variáveis em 15 biópsias. Nosso estudo mostra que os vacúolos e os agregados tubulares säo achado frequentes na paralisia periódica, constituindo importante auxílio diagnóstico. Alteraçöes miopáticas evidentes à biópsia sugerem o aparecimento de miopatia permanente, quadro decorrente de doença de longa evoluçäo ou crises severas