RESUMO
We report a case of a 39 year-old Asian man in whom profound lower limb paralysis, along with severe hypokalemia and electrocardiographic changes, were the presenting features of Graves' disease (GD)-related thyrotoxicosis. Rapid recognition and management of the disorder were the key factors to avoid fatal hypokalemia-induced cardiac arrhythmias and promptly restore patient's capacity to ambulate.
Assuntos
Doença de Graves/complicações , Paralisia Periódica Hipopotassêmica/etiologia , Tireotoxicose/etiologia , Antagonistas Adrenérgicos beta/uso terapêutico , Adulto , Antitireóideos/uso terapêutico , Eletrocardiografia , Emergências , Doença de Graves/diagnóstico , Doença de Graves/tratamento farmacológico , Hong Kong/etnologia , Humanos , Paralisia Periódica Hipopotassêmica/sangue , Paralisia Periódica Hipopotassêmica/tratamento farmacológico , Paralisia Periódica Hipopotassêmica/etnologia , Transporte de Íons , Masculino , Metimazol/uso terapêutico , Exame Neurológico , Potássio/metabolismo , Cloreto de Potássio/uso terapêutico , Propranolol/uso terapêutico , ATPase Trocadora de Sódio-Potássio/metabolismo , Estresse Psicológico/complicações , Tireotoxicose/tratamento farmacológicoRESUMO
Thyrotoxicosis can lead to thyrotoxic periodic paralysis (TPP), an endocrine channelopathy, and is the most common cause of acquired periodic paralysis. Typically, paralytic attacks cease when hyperthyroidism is abolished, and recur if hyperthyroidism returns. TPP is often underdiagnosed, as it has diverse periodicity, duration and intensity. The age at which patients develop TPP closely follows the age at which thyrotoxicosis occurs. All ethnicities can be affected, but TPP is most prevalent in people of Asian and, secondly, Latin American descent. TPP is characterized by hypokalemia, suppressed TSH levels and increased levels of thyroid hormones. Nonselective ß adrenergic blockers, such as propranolol, are an efficient adjuvant to antithyroid drugs to prevent paralysis; however, an early and definitive treatment should always be pursued. Evidence indicates that TPP results from the combination of genetic susceptibility, thyrotoxicosis and environmental factors (such as a high-carbohydrate diet). We believe that excess T(3) modifies the insulin sensitivity of skeletal muscle and pancreatic ß cells and thus alters potassium homeostasis, but only leads to a depolarization-induced acute loss of muscle excitability in patients with inherited ion channel mutations. An integrated etiopathophysiological model is proposed based on molecular findings and knowledge gained from long-term follow-up of patients with TPP.
Assuntos
Paralisia Periódica Hipopotassêmica/epidemiologia , Paralisia Periódica Hipopotassêmica/etiologia , Tireotoxicose/epidemiologia , Tireotoxicose/etiologia , Animais , Humanos , Hipopotassemia/diagnóstico , Hipopotassemia/epidemiologia , Paralisia Periódica Hipopotassêmica/fisiopatologia , Tireotoxicose/fisiopatologiaRESUMO
Paralysis due to hypokalemia results from an acute shift of potassium into cells or excessive potassium deficit. In the absence of potassium deficit, it is observed in Familial Hypokalemic Periodic Paralysis and in Thyrotoxic Hypokalemic Periodic Paralysis (TPP). This report describes the initial presentation of hyperthyroidism as sudden quadriplegia associated with hypokalemia. A healthy 25-year-old Puerto Rican policeman came to the emergency room with sudden paralysis in the four extremities of five hours evolution. He woke up in the morning and could not get up. The day before admission his legs felt weak, and it was hard to get out of bed. He arrived home at 7:00 PM, ate pasta and vegetables, and went to sleep at 10:00 PM. He had no diarrhea or weight loss, no history of medications or illicit drugs. He has a cousin and an aunt with the diagnosis of hypo-thyroidism. The admission temperature was 36.0 degrees C, pulse 96 per minute, respiratory rate 18 per minute, blood pressure 160/70 mmHg. He was alert and oriented as to time, place and person. He could talk properly and was in no respiratory distress. He had no exophtalmos or lid lag. The thyroid was not enlarged or tender. No pseudoclubbing or pretibial edema was found. There was flaccid paralysis of all extremities, 0/5 legs and 1/5 arms. Deep tendon reflexes could not be elicited. The cranial nerves and sensory examination were normal. The hemogram was within normal limits as were the renal and liver functions. Serum sodium was 140 mEq/L, potassium 1.48 mEq/L, phosphorus 1.4 mEq/L. A random glucose was 155 mg/dl and the arterial Ph was 7.41. The urine potassium was 7.04 mEq/L, sodium 60.8 mg/dl. TSH levelwas < 0.03 ug/d], TUP 50.69% (24-40%), T4 17.6 ug/dl (4.7-11.4 ug/dl) Free T4 Index 28.23. He was managed with intravenous potassium chloride, 80 mEq in a period of seven hours with cardiac monitor. The serum potassium level, after the infusion was completed, was 6.70 mEq/L. No cardiac arrhythmia was documented. Muscle strength recovery was gradual and it was complete 4 hours after the infusion was initiated. The next day the potassium level was within normal limits but a wide pulse pressure and tachycardia still persisted.
Assuntos
Paralisia Periódica Hipopotassêmica/etiologia , Tireotoxicose/complicações , Tireotoxicose/diagnóstico , Adulto , Humanos , MasculinoRESUMO
Thyrotoxic hypokalemic periodic paralysis (THPP) is a medical emergency characterized by acute attacks of weakness, hypokalemia, and thyrotoxicosis that resolve with the treatment of hyperthyroidism. Attacks are transient, self-limited, associated with hypokalemia and resemble those of familial hypokalemic periodic paralysis (FHPP), an autosomal dominant neurological channelopathy. This study reviews the clinical features and genetic findings of THPP in 25 Brazilian patients. Most patients had weight loss, taquicardia, goiter, tremor, and ophthalmopathy. Most often attacks arose during the night and recovered spontaneously but some patients evolved to total quadriplegia, and few experienced cardiac arrhythmias. All patients had suppressed TSH and elevated T4 and most had positive anti-thyroid antibodies, indicating autoimmunity thyrotoxic etiology. Potassium was low in all patients during the crisis. Prophylactic potassium therapy has not been shown to prevent attacks; however it was useful for curbing the paralysis during the crisis. We identified the mutation R83H in the KCNE3 gene in one sporadic case, and M58V in the KCNE4 gene in one case with family history. Furthermore, we identified other genetic polymorphisms in the CACNA1S, SCN4A, KCNE1, KCNE2, KCNE1L, KCNJ2, KCNJ8 e KCNJ11 genes. We conclude that THPP is the most common treatable cause of acquired periodic paralysis; therefore, it must be included in the differential diagnosis of acute muscle weakness.
Assuntos
Paralisia Periódica Hipopotassêmica/etiologia , Tireotoxicose/complicações , Adolescente , Adulto , Emergências , Feminino , Humanos , Paralisia Periódica Hipopotassêmica/diagnóstico , Paralisia Periódica Hipopotassêmica/genética , Masculino , Pessoa de Meia-IdadeRESUMO
Paralisia Periódica Hipocalêmica Tireotóxica (PPHT) é uma complicação rara do hipertireoidismo, mais freqüente em orientais que brancos. Apresentamos três pacientes do sexo masculino, brasileiros, cujas idades eram de 28 anos (caso 1), 29 anos (caso 2) e 60 anos (caso 3) com diagnóstico de PPHT. Foram admitidos com tetraparesia das extremidades, tendo o caso 1 relatado crises recorrentes de paralisia e os casos 2 e 3, apenas um episódio. No caso 1, estavam presentes sinais e sintomas de hipertireoidismo como emagrecimento, sudorese, tremor de extremidades, palpitação e bócio difuso discreto, enquanto no caso 2 manifestou-se apenas oftalmopatia, e o terceiro paciente referia diagnóstico de fibrilação atrial pregressa sem avaliação da função tireoidiana. Exames laboratoriais mostraram hipocalemia, TSH suprimido e T4 livre elevado em todos os pacientes. Foram tratados com potássio intravenoso, propranolol e tiamazol via oral, com remissão dos sintomas. O relato dos casos destaca a freqüente dificuldade diagnóstica desta enfermidade que apresenta evolução favorável quando diagnosticada e tratada adequadamente.
Assuntos
Adulto , Humanos , Masculino , Pessoa de Meia-Idade , Hipertireoidismo/complicações , Paralisia Periódica Hipopotassêmica/etiologia , Hipertireoidismo/diagnóstico , Paralisia Periódica Hipopotassêmica/diagnósticoRESUMO
A paralisia periódica hipocalêmica tirotóxica (PPHT) é uma emergência médica caracterizada por ataques agudos de fraqueza muscular, hipocalemia e tirotoxicose, que desaparece com o tratamento do hipertiroidismo. As crises de paralisia são transitórias, auto-limitadas, associadas com hipocalemia e similares àquelas da paralisia periódica hipocalêmica familiar (PPHF), doença neurológica autossômica dominante. Este estudo descreve o quadro clínico e achados genéticos de 25 pacientes brasileiros com PPHT. A maioria dos pacientes apresentava perda de peso, taquicardia, bócio, tremores e oftalmopatia. Os ataques ocorreram, em sua maioria, durante a noite e tiveram recuperação espontânea, apesar de alguns pacientes evoluírem para quadriplegia e arritmias cardíacas. Todos apresentaram TSH suprimido e T4 elevado, e a maioria anticorpos positivos, indicando etiologia auto-imune. O potássio estava baixo em todos durante a crise. A terapêutica profilática com potássio não preveniu os ataques, mas foi útil para diminuir a força da paralisia durante as crises. Identificamos a mutação R83H no gene KCNE3 num caso esporádico e a mutação M58V no gene KCNE4 numa família com PPHT. Além disso, identificamos polimorfismos nos genes CACNA1S, SCN4A, KCNE1, KCNE2, KCNE1L, KCNJ2, KCNJ8 e KCNJ11. Concluímos que a PPHT é a causa mais comum tratável de paralisia periódica adquirida e deve ser lembrada em casos de fraqueza muscular em pacientes jovens.
Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paralisia Periódica Hipopotassêmica/etiologia , Tireotoxicose/complicações , Emergências , Paralisia Periódica Hipopotassêmica/diagnóstico , Paralisia Periódica Hipopotassêmica/genéticaRESUMO
Thyrotoxic hypokalemic periodic paralysis (THPP) is a rare hyperthyroidism complication much more frequent in Asians and Caucasians. We present 3 cases of THPP occurring in Brazilian male patients with 28 years old (y) (Case 1), 29 y (Case 2) and 60 y (Case 3), respectively. They were admitted following an episode of flacid paralysis of extremities. Whereas case 1 reported recurring episodes of paralysis crises, cases 2 and 3 reported only one episode. Signs and symptoms of thyrotoxicosis, such as weigh loss, diaphoresis, extremities tremor, palpitation and mild diffuse goiter were present in the first case; while the second case only presented ophthalmopathy and the third patient referred that 2 years before his admission he presented an episode of cardiac arrhythmia but did not have thyroid function evaluation at that time. Their laboratory findings were hypokalemia, low TSH and raised free T4. They were treated with intravenous potassium, oral propranolol and tiamazol with remission of the symptoms. We report these cases to emphasize the importance of recognizing hyperthyroid periodic paralyses to avoid missing a treatable and curable condition.
Assuntos
Hipertireoidismo/complicações , Paralisia Periódica Hipopotassêmica/etiologia , Adulto , Humanos , Hipertireoidismo/diagnóstico , Paralisia Periódica Hipopotassêmica/diagnóstico , Masculino , Pessoa de Meia-IdadeRESUMO
Periodic paralysis is an uncommon complication of primary aldosteronism in the non-Asian population. We describe the case of a Brazilian woman who presented to the emergency room with proximal symmetric tetraparesis that was later diagnosed as primary aldosteronism. This case report shows that primary aldosteronism should be included in the differential diagnosis of periodic paralysis, especially among hypertensive patients.
Assuntos
Humanos , Feminino , Adulto , Hiperaldosteronismo , Paralisia Periódica Hipopotassêmica/etiologia , Paresia , Diagnóstico Diferencial , Doenças Musculares/diagnóstico , Hipertensão/diagnósticoRESUMO
El diagnóstico etiológico y el manejo de la parálisis flácida aguda representan un reto frecuente para el clínico, quien debe considerar una amplia gama de causas que afectan la función neuromuscular. Se evaluó paciente femenina de 21 años sin antecedentes patológicos con cuadro agudo y rápidamente progresivo de parálisis flácida ascendente con compromiso respiratorio. Se consideró entre las etiologías el síndrome de Guillain-Barré y se solicitó electrolitos y estudio electrofisiológicos. Los estudios electrofisiológicos mostraron disminución de las velocidades y prolongación de las latencias de la conducción motora. El potasio sérico fue de 1.8 mEq/L al ingreso, la CPK fue de 2,277 U/L. La reposición intravenosa de potasio resultó en una rápida mejoría, con recuperación total de la fuerza en las primeras 12 horas de hospitalización. La paciente egresó totalmente recuperada y con pautas de seguimiento. El presente es un ejemplo de canalopatía tipo parálisis hipocalémica periódica con presentación clínica similar a la del síndrome de Guillain-Barré. Las canalopatías deben considerarse en el diagnóstico diferencial de las parálisis flácidas agudas. Se propone un algoritmo de diagnóstico y tratamiento para éstas
Assuntos
Paralisia Periódica Hipopotassêmica , Paralisia Periódica Hipopotassêmica/diagnóstico , Paralisia Periódica Hipopotassêmica/etiologia , Paralisia Periódica Hipopotassêmica/terapia , Paralisia/diagnóstico , Paralisia/etiologia , Paralisia/terapia , Hipotonia Muscular , Doenças NeuromuscularesRESUMO
El diagnóstico etiológico y el manejo de la parálisis flácida aguda representan un reto frecuente para el clínico, quien debe considerar una amplia gama de causas que afectan la función neuromuscular. Se evaluó paciente femenina de 21 años sin antecedentes patológicos con cuadro agudo y rápidamente progresivo de parálisis flácida ascendente con compromiso respiratorio. Se consideró entre las etiologías el síndrome de Guillain-Barré y se solicitó electrolitos y estudio electrofisiológicos. Los estudios electrofisiológicos mostraron disminución de las velocidades y prolongación de las latencias de la conducción motora. El potasio sérico fue de 1.8 mEq/L al ingreso, la CPK fue de 2,277 U/L. La reposición intravenosa de potasio resultó en una rápida mejoría, con recuperación total de la fuerza en las primeras 12 horas de hospitalización. La paciente egresó totalmente recuperada y con pautas de seguimiento. El presente es un ejemplo de canalopatía tipo parálisis hipocalémica periódica con presentación clínica similar a la del síndrome de Guillain-Barré. Las canalopatías deben considerarse en el diagnóstico diferencial de las parálisis flácidas agudas. Se propone un algoritmo de diagnóstico y tratamiento para éstas