Paralisia Periódica Hipocalêmica Tireotóxica: relato de caso / Thyrotoxic Hypokalemic Periodic Paralysis: case report

A paralisia periódica hipocalêmica tireotóxica é uma complicação rara do hipertireoidismo. Caracteriza-se por episódios de fraqueza muscular recorrente, associado à tireotoxicose e hipocalemia. Ocorre frequentemente em pacientes do sexo masculino e de origem asiática. Nesse contexto, o objetivo deste estudo é descrever o relato de caso de um paciente acometido por paralisia periódica hipocalêmica tireotóxica com redução acentuada da qualidade de vida e internações recorrentes devido a quadro agudo de tetraparesia flácida ascendente associado a hipocalemia grave por não adesão ao tratamento do hipertireoidismo. A paralisia periódica hipocalêmica tireotóxica apresenta evolução favorável quando reconhecida e tratada com controle inicial dos sintomas para normalização sérica do potássio e posterior resolução do quadro tireotóxico.

Thyrotoxic hypokalemic periodic paralysis is a rare complication of hyperthyroidism. The issue has been characterized by episodes of recurrent muscle weakness associated with thyrotoxicosis and hypokalemia. It occurs most often in male patients of Asian origin. This study aims on describing the case report of a patient affected by thyrotoxic hypokalemic periodic paralysis with intense reduction in life quality and recurrent hospitalizations due to ascending acute flaccid tetraparesis associated with severe hypokalemia due to non-adherence to treatment of hyperthyroidism. Thyrotoxic hypokalemic periodic paralysis presents a favorable evolution when identified and treated with initial symptom control for serum potassium normalization and subsequent resolution of the thyroid toxicity.

Síndrome de Sjögren primario que debuta con polimiositis mitocondrial, neuropatía axonal y parálisis hipopotasémica: reporte de caso / Onset of primary Sjögren syndrome with mitochondrial polymyositis, axonal neuropathy, and hypokalaemic paralysis: Case report

RESUMEN El síndrome de Sjögren es una entidad multisistémica de naturaleza autoinmune, clásicamente considerada una exocrinopatía debido a la alta frecuencia de síntomas secos (queratoconjuntivitis seca, xerostomía) como resultado de infiltración poliglandular por linfocitos autorreactivos. Sin embargo, menos del 10% de estos pacientes puede iniciar con manifestaciones extraglandulares severas, traducidas en peores desenlaces a largo plazo. Se presenta el caso de una gestante que inició con síndrome de debilidad aguda proximal relacionada con miositis con enfermedad mitocondrial e hipopotasemia severa, en el contexto de acidosis tubular renal distal, como manifestación extraglandular de síndrome de Sjögren primario. Se discuten brevemente manifestaciones neurológicas de esta entidad, incluyendo aquellas secundarias a trastornos metabólicos precipitados por compromiso autoinmune.

ABSTRACT Sjögren's syndrome is a multisystemic autoimmune disorder. It is classically considered as an exocrine disease, given the high frequency of dry symptoms (keratoconjunctivitis sicca, xerostomia) as a result of poly-glandular infiltration by autoreactive lymphocytes. However, less than 10% of these patients can onset with severe extra-glandular manifestations, resulting in worse long-term outcomes. The case of a pregnant woman is presented, who debuted with acute proximal weakness syndrome related to myositis with mitochondrial pathology and severe hypokalaemia in the context of distal renal tubular acidosis, as an extra-glandular manifestation of primary Sjögren's syndrome. Neurological manifestations of this condition are briefly discussed, including those secondary to metabolic disorders precipitated by autoimmune compromise.

Reconhecimento da paralisia periódica hipocalêmica tireotóxica na emergência: relato de caso e revisão da literatura / Recognizing thyrotoxic hypokalemic periodic paralysis on emergency settings: a case report and literature revie

A paralisia periódica hipocalêmica tireotóxica é uma complicação inusitada do hipertireoidismo, porém é considerada urgência endocrinológica e ainda frequentemente subdiagnosticada. Sua apresentação clínica consiste na tríade de défice de potássio, tireotoxicose e fraqueza muscular ­ sendo esse último sintoma comum em diversas patologias. Realizamos uma revisão bibliográfica e destacamos, por meio do relato de caso, a importância do diagnóstico precoce dessa doença, possibilitando uma evolução favorável ao paciente, independente de sua etnia, sexo ou região geográfica. Atentamos ainda ao tratamento da doença, que, apesar de sua simplicidade, acarreta muitos equívocos.

The thyrotoxic hypokalemic periodic paralysis is a rare complication of hyperthyroidism, but is considered an endocrinological urgency, and yet frequently underdiagnosed. Its clinical presentation consists of potassium deficit, thyrotoxicosis, and muscular weakness, with the latter symptom being very common in several pathologies. We performed a bibliographic review and highlight, through a case report, the importance of the early diagnosis of this disease to allow favorable progression to the patient, regardless of ethnicity, sex, or geographical region. We also reinforce the importance of the disease treatment which, despite its simplicity, leads to many mistakes.

Parálisis periódica hipopotasémica familiar, Instituto Hondureño de Seguridad Social / Family Hypokalemic Periodic Paralysis, Social Security Institute of Honduras, Internal Medicine Service

La parálisis periódica hipopotasémica , es una enfermedad congénita autosómica dominante; predomina en sexo masculino, con concentraciones sanguíneas de potasio bajas. Objetivo: identificar la parálisis hipopotasémica familiar como un reto clínico para el diagnóstico y tratamiento del paciente. Presentación del caso clínico: masculino, en la tercera década de la vida; sin antecedentes personales, se presenta a emergencia con tetraparesia que inicia en manos, de distal a proximal, con posterior dificultad para la deambulación. Antecedentes familiares de su madre y hermana que habían padecido cuadro similar; exámenes de laboratorio: química sanguínea, Creatin fosfocinasa 1 481, Transaminasa glutámica oxaloacetica 46 U/L, Transaminasa glutámica pirúvica 36 U/L, Sodio 143 mg/dl, Potasio 2.6 mg/dl, Calcio 9.3 mg/dl. Tratamiento al ingreso 40 mEq de cloruro de potasio y 250 cc de solución salina normal 0.9%. Se realizó prueba terapéutica con cloruro de potasio y ver recuperación progresiva de la fuerza muscular, pruebas tiroideas descartan otras causas. Conclusión: la parálisis hipopotasémica se ha vuelto un diagnóstico poco sospechado en el ingreso de pacientes con tetraparesia, aunque el tratamiento rápidamente instaurado puede mejorar los síntomas y permite un estudio posterior de la etiología...(AU)

Acetazolamide-Induced Type II Renal Tubular Acidosis and Muscle Weakness

No abstract available.

Thyrotoxic hypokalemic periodic paralysis due to Graves' disease in 2 adolescents

Thyrotoxic periodic paralysis (TPP) is a notable and potentially lethal complication of thyrotoxicosis, and Graves' disease is the most common cause of TPP. TPP is commonly reported in Asian males between 20–40 years of age, but it is rare in children and adolescents. We report 2 Korean adolescents (a 16-year-old male and a 14-year-old female) with episodes of TPP who were previously diagnosed with Graves' disease. These 2 patients presented with lower leg weakness in the morning after waking up. They were diagnosed with TPP-associated with thyrotoxicosis due to Graves' disease. After they were initially treated with potassium chloride and antithyroid drugs, muscle paralysis improved and an euthyroid state without muscle paralytic events was maintained during follow-up. Therefore, clinicians should consider TPP when patients have sudden paralysis and thyrotoxic symptoms such as goiter, tachycardia, and hypertension.

Caso Clínico: Parálisis Periódica Hipopotasémica / Case Report: Hypokalemic Periodic Paralysis

INTRODUCCIÓN: La parálisis periódica hipopotasémica es una enfermedad poco frecuente. Se caracteriza por episodios de debilidad muscular o plejia, reversible con la normalización de los niveles de potasio. Al ser una entidad poco común, el reporte del presente caso será de utilidad para el diagnóstico diferencial de la debilidad muscular aguda. CASO CLÍNICO: Paciente masculino de 22 años de edad, que posterior a ejercicio físico extenuante e ingesta moderada de hidratos de carbono y alcohol, presenta debilidad muscular aguda de miembros superiores e inferiores. EVOLUCIÓN: Al ingreso se realizó el diagnóstico de polirradiculoneuropatía desmielinizante aguda, administrándose una dosis de inmunoglobulina humana. Sin embargo, una vez obtenidos los resultados de laboratorio, se evidenció un potasio sérico de 2.4 mEq/L. Se inició la reposición con cloruro potásico en infusión. El paciente fue valorado por neurología y genética, con base en la anamnesis, examen físico, laboratorios y cuadro clínico del paciente, se realizó el diagnóstico de parálisis periódica hipopotasémica. Paciente presentó una evolución favorable, recibiendo el alta al cuarto día de hospitalización. CONCLUSIONES: La parálisis periódica hipopotasémica es una entidad poco frecuente, raramente incluida en el diagnóstico diferencial de la debilidad muscular aguda. La identificación oportuna y la consejería apropiada son esenciales para la prevención de complicaciones potencialmente mortales para el paciente.

BACKGROUND: Hypokalemic periodic paralysis is a rare disease, characterized by episodes of limb muscle weakness, reversible with the normalization of potassium levels. Being a rare entity, this report will be useful for the differential diagnosis of acute muscle weakness. CASE REPORT: A 22-year-old male patient, who after strenuous physical activity and a moderate intake of carbohydrates and alcohol, presented acute muscle weakness of the upper and lower limbs. EVOLUTION: On admission, the patient was diagnosed of acute demyelinating polyradiculoneuropathy, administering a dose of human immunoglobulin. However, once the laboratory results were available, a serum potassium of 2.4 mEq/L was evidenced. The replacement was started with potassium chloride in infusion. The patient was evaluated by neurology and genetics. Based on the anamnesis, physical examination, laboratories and clinical picture of the patient, the diagnosis of hypokalemic periodic paralysis was made. Patient presented a favorable evolution, receiving discharge on the fourth day of hospitalization. CONCLUSIONS: Hypokalemic periodic paralysis is an uncommon disease, rarely included in the differential diagnosis of acute muscle weakness. Timely identification and appropriate counseling are essential for the prevention of life-threatening complications.

Inusual forma de presentación de hipertiroidismo asociado a parálisis hipokalémica / An unusual presentation of hyperthyroidism associated with hypokalemic paralysis

La parálisis periódica hipokalémica tirotóxica (PPHT) constituye un raro trastorno caracterizado por debilidad muscular severa asociado a hipokalemia en pacientes hipertiroideos. Se reporta en este artículo un paciente masculino de 28 años de edad quien ingresó a un centro de salud de primer nivel, por debilidad muscular generalizada con subsecuente caída desde su altura sin pérdida de la conciencia. Es remitido posteriormente a un Hospital regional (en Colombia), en donde se identifica la presencia de bocio. Se solicitaron pruebas de función tiroidea y niveles de hormona estimulante de la tiroides (TSH) los cuales fueron anormales. Este caso clínico busca difundir el conocimiento de una patología considerada infrecuente en la región, con el fin de resaltar algunos aspectos clínicos y fisiopatológicos, destacando la importancia de tener presente el diagnostico de esta enfermedad y lograr hacer un manejo oportuno teniendo en cuenta sus complicaciones potencialmente fatales.

Hypokalemic thyrotoxic periodic paralysis is a rare disorder characterized by severe muscle weakness with low serum potassium levels in hyperthyroid patients. We describe a 28- years-old man who was admitted to a first level health center with a history of muscle weakness and a fall from his height without loss of consciousness. He was transferred to a regional Hospital (in Colombia) where, in a physical examination, was noticed an enlarged size of thyroid gland. Thyroid function tests and levels of thyroid stimulating hormone (TSH) were requested, which were abnormal. This case report attempts to spread the knowledge of a disease considered rare in the region, in order to highlight some clinical and pathophysiological aspects, the importance of always be considered in patients with this clinical presentation and making an appropriate treatment and diagnosis.

Parálisis periódica hipocalémica: reporte de caso y revisión del tema / Hypokalemic periodic paralysis: A case report and a topic review

Las parálisis periódicas primarias son canalopatías poco frecuentes, de las cuales hacen parte: la parálisis periódica hipocalémica, la parálisis periódica hipercalémica y el síndrome Andersen-Tawil; son caracterizadas por ataques de debilidad muscular generalizada, con recuperación de la fuerza entre los ataques. Presentamos el caso de una mujer de 21 años con el segundo episodio de parálisis y documentación de hipocalemia, sin antecedentes de importancia, sin factores clínicos o paraclínicos que expliquen el trastorno electrolítico, con recuperación posterior de la fuerza al realizar tratamiento de la hipocalemia. Posteriormente se lleva a cabo una revisión del tema.

Primary periodic paralyses are rare channelopathies which include: hypokalemic periodic paralysis, hyperkalemic periodic paralysis, and Andersen-Tawil Syndrome. These entities are characterized by attacks of generalized muscle weakness and recovery of muscle strength between attacks. A case is presented of a 21-year-old woman who presented a second episode of paralysis and documented hypokalemia, with no important antecedents, with no clinical or diagnostic test factors which explain her electrolyte disorder, with recovery of muscle strength after receiving treatment for hypokalemia. A review of the topic was conducted.

Paralisia periódica hipocalêmica tireotóxica: relato de caso / Thyrotoxic hypokalemic periodic paralysis: a case report

Relata-se neste artigo o caso de um paciente de ascendência negra com quadro agudo e progressivo de paralisia flácida ascendente, sem comprometimento de musculatura respiratória ou facial, em vigência de hipertireoidismo em tratamento e hipocalemia desencadeados por quadro infeccioso. Foi realizado o diagnóstico de paralisia periódica hipocalêmica tireotóxica e controle inicial dos sintomas por adequação sérica do potássio e posterior resolução do quadro após tratamento com iodoterapia.

It is reported in this paper the case of a black ancestry patient with acute and progressive framework ascending flaccid paralysis without impairment of respiratory and facial muscles, in effect in treatment of hyperthyroidism and hypokalemia triggered by infection. The diagnosis of thyrotoxic hypokalemic periodic paralysis was accomplished and initial symptoms were controlled for adequacy of serum potassium and subsequent resolution after treatment with radioiodine.

Parálisis periódica hipocalémica tirotóxica. Aporte del laboratorio en un nuevo desafío diagnóstico / Hypokalemic periodic paralysis thyrotoxic Contribution of the laboratory in a new diagnostic challenge

La parálisis periódica tirotóxica hipocalémica es una patología de muy baja frecuencia en la Argentina; no obstante, es muy importante tenerla en cuenta como urgencia, ya que puede ser fatal si el diagnóstico y el tratamiento correctos no son oportunos. El objetivo de esta investigación bibliográfica es destacar el aumento de la incidencia de una enfermedad potencialmente fatal y, a la vez, fácilmente reversible con el tratamiento adecuado y oportuno, en las Salas de Guardia de nuestro país, a fin de que se la tenga en cuenta en las sospechas diagnósticas de emergencia y el aporte que puede hacer el bioquímico desde un Servicio de Urgencia, para ayudar al diagnóstico en forma rápida y certera.(AU)

Hypokalemic thyrotoxic periodic paralysis is a disease of very low frequency in Argentina; however, it is very important to take it into account as an emergency, because it can be fatal without a timely diagnosis and treatment. The objective of this research is to highlight its increased incidence in the emergency rooms of our country (it is a potentially fatal disease, but at the same time it is easily reversible with a proper and timely treatment), in order to include it in the emergency diagnostic suspicion and the biochemist contribution in an emergency service to reach a quick and accurate diagnosis.(AU)

rare case of paralysis in an endemic area

Thyrotoxicosis mostly presents with tachycardia, tremor, weight loss and other hypermetabolism signs. However, there are other unusual signs of thyrotoxicosis such as paralysis. This unusual clinical presentation may postpone prompt diagnosis and treatment. In this case report, we present a 27-years-old woman, who presented with quadriparesis at the emergency department

A Case Report of Thyrotoxic Periodic Paralysis; Serial Nerve Conduction Studies before and after Recovery / 대한임상신경생리학회지

No abstract available.

Paralisia periódica hipocalêmica tireotóxica em paciente de ascendência africana / Thyrotoxic hypokalemic periodic paralysis in patients of African descent

A paralisia periódica hipocalêmica tireotóxica (PPHT) é uma urgência endocrinológica caracterizada por ataques recorrentes de fraqueza muscular, associados à hipocalemia e à tireotoxicose. Essa patologia ocorre mais frequentemente em pacientes do sexo masculino, de origem asiática, sendo rara sua apresentação em pessoas de ascendência africana. O caso descrito mostra um paciente afrodescendente que apresentou tetraparesia associada à hipocalemia. Durante esse episódio foi realizado o diagnóstico de hipertireoidismo. A PPHT, embora rara, é potencialmente letal. Portanto, em casos de crises de paresias flácidas, essa hipótese diagnóstica deve ser sempre considerada, principalmente se associada à hipocalemia. Nessa situação, se não houver diagnóstico prévio de hipertireoidismo, este também deve ser considerado.

Thyrotoxic hypokalemic periodic paralysis (THPP) is an endocrine emergency marked by recurrent attacks of muscle weakness associated with hypokalemia and thyrotoxicosis. Asiatic male patients are most often affected. On the other hand, African descents rarely present this disease. The case described shows an afrodescendant patient with hypokalemia and tetraparesis, whose diagnosis of hyperthyroidism was considered during this crisis. The THPP, although rare, is potentially lethal. Therefore, in cases of flaccid paresis crisis this diagnosis should always be considered, especially if associated with hypokalemia. In this situation, if no previous diagnosis of hyperthyroidism, this should also be regarded.

Glucocorticoid Triggers an Attack of Periodic Hypokalemic Paralysis during Treatment for Diffuse Large B-cell Lymphoma / 대한내과학회지

Hypokalemic periodic paralysis is a rare disorder characterized by sudden onset of weakness and low serum potassium levels. We report a case provoked by combination chemotherapy including prednisolone. A 23-yr-man, diagnosed with diffuse large B-cell lymphoma, received chemotherapy. He developed significant weakness in upper and lower extremities during chemotherapy, and his serum potassium level was 1.7 mmol/L. Potassium replacement restored the weakness. Further workup revealed that prednisolone had provoked hypokalemic paralysis. As prednisolone triggered an attack of hypokalemic periodic paralysis, it should be administered with caution, particularly in patients with periodic paralysis.

Glucocorticoid Triggers an Attack of Periodic Hypokalemic Paralysis during Treatment for Diffuse Large B-cell Lymphoma / 대한내과학회지

Hypokalemic periodic paralysis is a rare disorder characterized by sudden onset of weakness and low serum potassium levels. We report a case provoked by combination chemotherapy including prednisolone. A 23-yr-man, diagnosed with diffuse large B-cell lymphoma, received chemotherapy. He developed significant weakness in upper and lower extremities during chemotherapy, and his serum potassium level was 1.7 mmol/L. Potassium replacement restored the weakness. Further workup revealed that prednisolone had provoked hypokalemic paralysis. As prednisolone triggered an attack of hypokalemic periodic paralysis, it should be administered with caution, particularly in patients with periodic paralysis.

Channelopathies / 소아과

Channelopathies are a heterogeneous group of disorders resulting from the dysfunction of ion channels located in the membranes of all cells and many cellular organelles. These include diseases of the nervous system (e.g., generalized epilepsy with febrile seizures plus, familial hemiplegic migraine, episodic ataxia, and hyperkalemic and hypokalemic periodic paralysis), the cardiovascular system (e.g., long QT syndrome, short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), the respiratory system (e.g., cystic fibrosis), the endocrine system (e.g., neonatal diabetes mellitus, familial hyperinsulinemic hypoglycemia, thyrotoxic hypokalemic periodic paralysis, and familial hyperaldosteronism), the urinary system (e.g., Bartter syndrome, nephrogenic diabetes insipidus, autosomal-dominant polycystic kidney disease, and hypomagnesemia with secondary hypocalcemia), and the immune system (e.g., myasthenia gravis, neuromyelitis optica, Isaac syndrome, and anti-NMDA [N-methyl-D-aspartate] receptor encephalitis). The field of channelopathies is expanding rapidly, as is the utility of molecular-genetic and electrophysiological studies. This review provides a brief overview and update of channelopathies, with a focus on recent advances in the pathophysiological mechanisms that may help clinicians better understand, diagnose, and develop treatments for these diseases.

The large-conductance calcium-activated potassium channel holds the key to the conundrum of familial hypokalemic periodic paralysis / 소아과

PURPOSE: Familial hypokalemic periodic paralysis (HOKPP) is an autosomal dominant channelopathy characterized by episodic attacks of muscle weakness and hypokalemia. Mutations in the calcium channel gene, CACNA1S, or the sodium channel gene, SCN4A, have been found to be responsible for HOKPP; however, the mechanism that causes hypokalemia remains to be determined. The aim of this study was to improve the understanding of this mechanism by investigating the expression of calcium-activated potassium (KCa) channel genes in HOKPP patients. METHODS: We measured the intracellular calcium concentration with fura-2-acetoxymethyl ester in skeletal muscle cells of HOKPP patients and healthy individuals. We examined the mRNA and protein expression of KCa channel genes (KCNMA1, KCNN1, KCNN2, KCNN3, and KCNN4) in both cell types. RESULTS: Patient cells exhibited higher cytosolic calcium levels than normal cells. Quantitative reverse transcription polymerase chain reaction analysis showed that the mRNA levels of the KCa channel genes did not significantly differ between patient and normal cells. However, western blot analysis showed that protein levels of the KCNMA1 gene, which encodes KCa1.1 channels (also called big potassium channels), were significantly lower in the membrane fraction and higher in the cytosolic fraction of patient cells than normal cells. When patient cells were exposed to 50 mM potassium buffer, which was used to induce depolarization, the altered subcellular distribution of BK channels remained unchanged. CONCLUSION: These findings suggest a novel mechanism for the development of hypokalemia and paralysis in HOKPP and demonstrate a connection between disease-associated mutations in calcium/sodium channels and pathogenic changes in nonmutant potassium channels.

A Case of Thyrotoxic Periodic Paralysis in 17-Year-Old Adolescent

Thyrotoxic periodic paralysis (TPP) is a rare disease of muscle paralysis associated with hypokalemia and thyrotoxicosis. Hypokalemic periodic paralysis can be caused by potassium channelopathy or thyroid dysfunction. Thyrotoxic hypokalemic periodic paralysis is far more common in Asian males aged between 20 to 40 years and rare in children and adolescents. Clinical symptoms with motor paralysis were recovered just after treatment of potassium, propranolol, and methimazole. However, thyroid function tests were not normalized until 3 month treatment of methimazole and propranolol. We report a 17-year-old patient diagnosed with Graves' disease with TPP. Thyroid function test should including in periodic paralysis patient's evaluation.

Etiology of Hypokalemic Paralysis in Korea: Data from a Single Center

Recognizing the underlying causes of hypokalemic paralysis seems to be essential for the appropriate management of affected patients and their prevention of recurrent attacks. There is, however, a paucity of documented reports on the etiology of hypokalemic paralysis in Korea. We retrospectively analyzed 34 patients with acute flaccid weakness due to hypokalaemia who were admitted during the 5-year study period in order to determine the spectrum of hypokalemic paralysis in Korea and to identify the differences in clinical parameters all across the causes of hypokalemic paralysis. We divided those 34 patients into 3 groups; the 1st group, idiopathic hypokalemic periodic paralysis (HPP), the 2nd, thyrotoxic periodic paralysis (TPP), and the 3rd group, secondary hypokalemic paralysis (HP) without TPP. Seven of the patients (20.6%) were diagnosed as idiopathic HPP considered the sporadic form, and 27 patients (79.4%) as secondary HP. Among the patients diagnosed as secondary HP, 16 patients (47.1%) had TPP. Patients of secondary hypokalemic paralysis without TPP required a longer recovery time compared with those who had either idiopathic HPP or TPP. This is due to the fact that patients of secondary HP had a significantly negative total body potassium balance, whereas idiopathic HPP and TPP were only associated with intracellular shift of potassium. Most of the TPP patients included in our study had overt thyrotoxicosis while 3 patients had subclinical thyrotoxicosis. This study shows that TPP is the most common cause of hypokalemic paralysis in Korea. And we suggest that doctors should consider the presence of TPP in patients of hypokalemic paralysis even if they clinically appear to be euthyroid state.