Thyrotoxic periodic paralysis presenting with quadriparesis and hyperreflexia.

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism that manifests as painless flaccid paralysis. An East Asian man in his late 20s presented to the emergency department with an acute onset of quadriparesis associated with hypertonia and hyperreflexia. His initial symptoms and signs suggested involvement of the brain and spinal cord; however, MRI of the neuroaxis was normal. His serum potassium concentration was low, and thyroid test results were consistent with hyperthyroidism. The patient was diagnosed with TPP associated with Graves' disease and was treated with potassium supplementation, propranolol and methimazole. Motor strength improved to his baseline level of power; bulk was normal, and tone was increased. Although flaccid paralysis is a typical presentation of TPP, brisk reflexes and muscle spasticity cannot rule out this condition. This case highlights the importance of considering TPP as a possible diagnosis in patients presenting with acute quadriparesis.

Recurrent hypokalemic paralysis in hypothyroidism.

Hypothyroidism, a commonly encountered thyroid disorder, usually manifests with readily recognizable typical features. However, an unusual presentation of a classic thyroid disorder may hinder accurate diagnosis in certain instances. One such rare initial presentation of hypothyroidism is recurrent hypokalemic paralysis, and existing reports in the literature are sparse. It has been more commonly reported in thyrotoxicosis. We report the case details and clinical outcomes of two middle-aged individuals (a 34-year-old male and a 37-year-old female) with recurrent episodes of hypokalemic paralysis. Their clinical examination revealed pure motor hyporeflexia quadriparesis with hypotonia and diminished deep tendon reflexes without any autonomic dysfunction. They had no significant previous medical history. Biochemical findings revealed hypokalemia in both cases (1.4 and 1.9 mEq/L, respectively) with elevated levels of thyroid­stimulating hormone and thyroid­related antibodies in both individuals, thus, confirming the diagnosis of autoimmune hypothyroidism. Immediate treatment with intravenous and oral potassium correction helped in the recovery. Thyroxine supplementation was considered a follow-up treatment, and for a one-year follow-up period there were no complaints of limb weakness reported in both individual.

Refractory familial hypokalaemic periodic paralysis leading to cardiovascular compromise.

Familial hypokalaemic periodic paralysis (FHPP) is a rare neuromuscular disorder that is classified under periodic paralysis (PP), which is characterised by episodes of muscle weakness. Common triggers include intense exercise, fasting or consumption of carbohydrate-rich meals. Hypokalaemic PP has an incidence of 1 in 100 000; despite the temporal association, cardiac manifestations are exceedingly rare. We present a case of FHPP, a channelopathy presenting with severe refractory hypokalaemia. The challenges with our patient were maintaining potassium levels within normal ranges and initiating a close follow-up plan. Due to the lack of clinical guidance in our case, many aspects of care, including surveillance, medications and genetic testing, remain unaddressed. Medical management includes aggressive correction with supplements, potassium-sparing diuretics and carbonic anhydrase inhibitors. Severe cases of dysrhythmias, especially ventricular fibrillation, require electrophysiology evaluation and possible implantation of a defibrillator to prevent sudden cardiac death.

Thyrotoxic periodic paralysis in two sexagenarian men: A case report.

RATIONALE: Thyrotoxic periodic paralysis (TPP) characterized by the triad of muscle paralysis, acute hypokalemia, and the presence of hyperthyroidism is often reported in young adults but rarely reported in age >60 year-old. PATIENT CONCERNS: Two sexagenarian males (age 61 and 62) presenting to the emergency department with progressive muscle paralysis for hours. There was symmetrical flaccid paralysis with areflexia of lower extremities. Both of them did not have the obvious precipitating factors and take any drugs. DIAGNOSIS: Their Wayne scores, as an objective index of symptoms and signs associated with thyrotoxicosis, were <19 (7 and 14, respectively). Their blood pressure stood 162/78 and 170/82 mm Hg, respectively. Their thyroid glands were slightly enlarged. Both of them had severe hypokalemia (1.8 and 2.0 mmol/L). Their presumptive diagnosis of mineralocorticoid excess disorders with severe potassium (K+) deficit were made. However, low urine K+ excretion and relatively normal blood acid-base status were suggestive of an intracellular shift of K+ rather than K+ deficit. Hormone studies confirmed hyperthyroidism due to Graves disease. INTERVENTIONS: A smaller dose of K+ supplementation (only a total of 50 and 70 mmol K+, respectively) were prescribed for the patient. OUTCOMES: After treatment, their serum K+ levels became normal with a full recovery of muscle strength. LESSONS: Our 2 cases highlight the fact that thyrotoxic periodic paralysis must be still kept in mind as the underlying cause of hypokalemia with paralysis and hypertension in elderly patients to avoid missing curable disorders.

Hypokalaemic periodic paralysis secondary to subclinical hyperthyroidism: an uncommon cause of acute muscle paralysis.

Hypokalaemic periodic paralysis secondary to subclinical hyperthyroidism is an uncommon clinical phenomenon characterised by lower limb paralysis secondary to hypokalaemia in the background of subclinical hyperthyroidism. In this article, we report a patient who presented with progressive lower limb muscle weakness secondary to hypokalaemia that was refractory to potassium replacement therapy. He has no diarrhoea, no reduced appetite and was not taking any medication that can cause potassium wasting. Although he was clinically euthyroid, his thyroid function test revealed subclinical hyperthyroidism. His 24-hour urine potassium level was normal, which makes a rapid transcellular shift of potassium secondary to subclinical hyperthyroidism as the possible cause. He was successfully treated with potassium supplements, non-selective beta-blockers and anti-thyroid medication. This case report aimed to share an uncommon case of hypokalaemic periodic paralysis secondary to subclinical hyperthyroidism, which to our knowledge, only a few has been reported in the literature.

[Thyrotoxic hypokalemic periodic paralysis in two African black women]. / Paralysie périodique hypokaliémique thyrotoxique chez deux femmes noires africaines.

Thyrotoxic hypokalemic periodic paralysis is a rare complication of hyperthyroidism. It has been most often reported in Asian subjects while it has been little described in the black population. Its mechanism has been little elucidated, but it would be caused by hyperactivity of the Na+/K+pump. We here report two cases of thyrotoxic hypokalemic periodic paralysis in black African subjects. The clinical manifestation was identical in both patients: proximal muscle paralysis of the lower limbs. Paralysis was associated with severe hypokalemia and occurred in female patients treated for Graves' disease without any other associated disease. Outcome was immediately favorable under potassium supplementation. Treatment of hyperthyroidism prevented recurrences. This study highlights the importance of suspecting the diagnosis of thyrotoxic hypokalemic periodic paralysis despite its rarity in the black African population.

[Hypokalemic Paresis in Adolescent: A Case Report]. / Parésia Hipocaliemica em Adolescente: Um Caso Clínico.

Familial hypokalaemic periodic paralysis is a rare autosomal dominant neuromuscular disease characterized by episodic attacks of flaccid paralysis with concomitant hypokalaemia. We present a case of a 15-year-old male adolescent observed in the pediatric emergency department by flaccid paresis of the 4 limbs of sudden onset and progressive worsening. In the anamnesis, corticosteroid and antihistamine intake were observed on the previous day for urticaria and family history of transient episodes of flaccid paralysis in adolescence, asymptomatic after the fourth decade of life, without an established diagnosis. Diagnostic tests revealed hypokalaemia (K + < 2.4 mEq/L), without hypokaluria and without other changes. Symptomatology resolution after supplementation with potassium was verified until normalization of kaliemia. Flaccid paralysis is a rare form of presentation of hypokalaemia. Several etiologies may be involved in the child or adolescent presenting with acute flaccid paralysis. The description of this case draws attention to the importance of the knowledge of this entity, because if recognized and treated properly, patients usually recover without sequelae.

A paralisia periódica hipocaliémica familiar é uma doença neuromuscular autossómica dominante, rara, caracterizada por crises episódicas de paralisia flácida acompanhadas de hipocaliemia. Apresenta-se o caso de um adolescente do sexo masculino, com 15 anos de idade, observado no Serviço de Urgência de Pediatria por parésia flácida dos quatro membros de início subito e agravamento progressivo. Na anamnese verificou-se a ingestão de corticoesteroide e antihistaminico no dia anterior por urticária e história familiar de episódios transitórios de paralisia flácida na adolescência, assintomáticos após a 4ª década de vida, sem diagnóstico estabelecido. Os exames auxiliares de diagnóstico revelaram hipocaliemia (K+ < 2,4 mEq/L), sem hipocaliúria e sem outras alterações. Verificou-seresolução da sintomatologia após suplementação com potássio até normalização da caliemia. A paralisia flácida é uma forma rara de apresentação da hipocaliemia. Diversas etiologias podem estar envolvidas na criança ou adolescente que se apresenta com com paralisia flácida de início agudo. Com a descrição deste caso alerta-se para a importância do conhecimento desta entidade, porque se reconhecidos e tratados apropriadamente, os doentes geralmente recuperam sem sequelas.

Thyrotoxic hypokalemic periodic paralysis: a life-threatening disorder in Asian men.

Thyrotoxicosis can present as a sporadic form of hypokalaemic periodic paralysis. The condition is associated with massive intracellular shift of potassium, mainly in skeletal muscles. As the total body stores of potassium remain normal, overzealous potassium supplementation targeting serum potassium level results in a poor outcome. We present a fatal case of thyrotoxic hypokalaemic periodic paralysis.

Unilateral renal artery stenosis presenting as acute flaccid paralysis: a rare presentation.

Renovascular hypertension is one of the common causes of secondary hypertension. Here we report a case of patient of renal artery stenosis presenting to the emergency department as a case of acute flaccid paralysis. Renal artery stenosis has been associated with hypokalaemia, but rarely reported to be symptomatic. Initial correction of hypokalaemia leads to improvement of weakness and aetiological work up for hypokalaemia with hypertension revealed hypokalaemia due to hyperaldosteronism secondary to unilateral renal artery stenosis. The patient was managed medically with aldosterone antagonist in the anti hypertensive therapy and weakness did not recur despite withdrawal of potassium supplements. On follow-up, the patient was ambulatory with no signs of weakness, controlled blood pressure and normal potassium level.

Distal renal tubular acidosis in Sjögren's syndrome.

Interstitial nephritis and immune complex-mediated glomerulonephritis are the two common renal manifestations of primary Sjögren's syndrome (SS). Here, we discuss three cases of primary SS where presenting manifestation was distal renal tubular acidosis. The possibility of an underlying autoimmune disorder should be considered in a patient presenting with distal tubular acidosis or recurrent hypokalemic periodic paralysis as treatment of primary disease improves the outcome of illness.

An Unusual Case of Acute Muscle Weakness.

A previously healthy 35-year old man presented to hospital with acute leg weakness following an alcohol binge. On assessment, tachycardia, urinary retention and bilateral upper and lower limb proximal weakness with preserved peripheral power were noted. Biochemistry revealed marked hypokalaemia, which responded to intravenous replacement, and biochemical thyrotoxicosis, leading to the diagnosis of Thyrotoxic Periodic Paralysis (TPP). Anti-thyroid therapy and beta-blockers were commenced and his neurological symptomatology resolved as he became progressively euthyroid. TPP is a rare acquired subtype of hypokalaemic periodic paralysis, typically causing proximal muscle weakness associated with thyrotoxicosis. It is most common in young Asian males. Acute treatment requires cautious oral potassium supplementation, beta-blockade, and anti-thyroid therapy. TPP is prevented by maintaining euthyroidism; otherwise recurrence is likely.

The R900S mutation in CACNA1S associated with hypokalemic periodic paralysis.

Primary hypokalemic periodic paralysis is an autosomal dominant skeletal muscle channelopathy. In the present study, we investigated the genotype and phenotype of a Chinese hypokalemic periodic paralysis family. We used whole-exome next-generation sequencing to identify a mutation in the calcium channel, voltage-dependent, L type, alpha subunit gene (CACNA1S), R900S, which is a rare mutation associated with hypokalemic periodic paralysis. We first present a clinical description of hypokalemic periodic paralysis patients harboring CACNA1SR900S mutations: they were non-responsive to acetazolamide, but combined treatment with triamterene and potassium supplements decreased the frequency of muscle weakness attacks. All male carriers of the R900S mutation experienced such attacks, but all three female carriers were asymptomatic. This study provides further evidence for the phenotypic variation and pharmacogenomics of hypokalemic periodic paralysis.

[A case of thyreotoxic hypokalaemic periodic paralysis in a white male]. / Thyreotoxische hypokaliëmische periodieke paralyse bij een blanke man.

BACKGROUND: Thyreotoxic hypokalaemic periodic paralysis (THPP) is a rare and potentially life-threatening syndrome. It principally affects men of East-Asian origin and has rarely been described in a white person. CASE DESCRIPTION: A 34-year-old Dutch man, suffering from Graves' disease, presented with weakness in his lower limbs. Laboratory investigation showed severe hypokalaemia (1.8 mmol/l) and increased creatinine kinase levels. An electrocardiogram showed atrial fibrillation with a prolonged QTc-interval. The patient was admitted, cardiac rhythm was monitored, and he received potassium supplements. Laboratory investigation of thyroid function showed thyrotoxicosis. The patient was treated with propranolol and thiamazol. At follow-up, thyroid function, potassium levels and muscle strength had normalized. CONCLUSION: Hypokalaemia due to thyrotoxicosis should be considered in cases of unexplained paralysis. The treatment of THPP consists of treating for hyperthyroidism plus propranolol. Since the hypokalaemia is self-limiting, potassium supplementation is only necessary in cases of rhythm disturbances or cardiac-conduction disturbances. Despite adequate treatment, there is a risk of recurrence. Regular monitoring is indicated until euthyroidism is achieved.

Thyrotoxic hypokalaemic periodic paralysis: a rare presentation of Graves' disease in a Hispanic patient.

A 26-year-old Hispanic man with no significant medical history presented to our emergency room with gradual onset weakness of his lower extremities. He was haemodynamically stable and examination revealed loss of motor function in his lower limbs up to the level of hips. Laboratory data revealed hypokalaemia. The patient was started on potassium supplementation and he recovered his muscle strength. Differential diagnosis included familial hypokalaemic periodic paralysis and thyrotoxic periodic paralysis (TPP). Further investigations revealed a low thyroid-stimulating hormone and high free thyroxine levels. Radio iodine 123 scan revealed an enhanced homogeneous uptake in the thyroid suggesting Graves' disease. Thyroid stimulating antibodies were also found to be elevated. The patient was started on methimazole and propranolol and he never had another attack of TPP even at 1 year follow-up.

Acute hypokalemic periodic paralysis possibly precipitated by albuterol.

PURPOSE: An episode of acute hypokalemic paralysis associated with the use of inhaled albuterol is described. SUMMARY: A 34-year-old woman admitted to the emergency department reported the development of pain and diffuse paralysis of the extremities and torso shortly after using an albuterol inhaler. At age 18, she had been diagnosed with hyokalemic periodic paralysis (HPP), a disorder of muscle membrane excitability caused by serum potassium depletion that can lead to life-threatening neuromuscular and cardiovascular complications. After a 15-year period of episodically recurring HPP symptoms despite long-term acetazolamide use, she was switched to spironolactone therapy and had experienced no HPP exacerbations for about 1 year. On her arrival in the emergency department, the patient's serum potassium concentration was 1.8 meq/L and she was mildly tachycardic (heart rate of 125 beats/min). After careful supplementation to gradually increase the serum potassium concentration to 5.4 meq/L, the patient slowly regained movement and strength in her extremities. Application of the adverse drug reaction probability scale of Naranjo et al. to this case yielded a score of 3, indicating that albuterol was possibly the cause of the patient's HPP exacerbation. Beta-2-adrenergic agonists and several other medications can affect serum potassium levels; although the potential risks posed by the use of such drugs in patients with a history of HPP are unclear, cautious use in the context of known HPP is advised. CONCLUSION: A patient previously diagnosed with HPP experienced an exacerbation of HPP possibly induced by inhaled albuterol treatment.

[Thyrotoxic periodic paralysis: a case series of four patients and literature review]. / Paralysie périodique thyréotoxique hypokaliémique : quatre observations et revue de la littérature.

PURPOSE: Periodic thyrotoxic hypokalemic paralysis (TPP) is a neuromuscular complication of hyperthyroidism. It is more common in young Asian males than in Caucasian and African patients. We report four new cases and review the literature. CASE REPORTS: Four consecutive patients were diagnosed with TPP. They were all men with a median age of 34.5 years at presentation. Two patients originated from the Philippines, one was African and one was Caucasian ethnic background. They all presented with a paresis or flaccid paralysis, without respiratory failure. Previous similar episodes in their past medical history, the presence of profound hypokalemia (mean serum potassium level of 2 mmol/L) and the presence of clinical and biological signs of hyperthyroidism led to the diagnosis of TPP. All four patients were diagnosed with Graves' disease. Outcome was favourable in all four patients with the symptomatic treatment of TPP and treatment of Graves' disease. CONCLUSION: TPP is a severe condition, due to a dysfunction of the Na(+)-K(+) ATPase pump. Initial management relies on ß-blocker treatment and careful potassium supplementation. Then, medical or surgical etiological treatment of the thyrotoxicosis is essential to prevent a recurrence. The disease is probably underdiagnosed: it must be suspected when a profound hypokaliema resolves very quickly (<12hours); hyperthyroidism should always be included in the differential diagnosis of a paresis associated with hypokalemia.

Unilateral gynecomastia and hypokalemic periodic paralysis as first manifestations of Graves' disease.

A 39-year-old Chinese man presented to the study hospital with right-sided gynecomastia. Underlying Graves' disease was not diagnosed until recurrent episodes of hypokalemic periodic paralysis were observed. The estradiol (E2) and progesterone levels and the E2-to-testosterone (T) (E2/T) ratio of the patient were elevated before treatment. Immediate intravenous potassium supplementation was started to reverse the paralysis. Additionally, antithyroid drugs were administered to restore a euthyroid state. After treatment, the patient gained strength. Gynecomastia regressed with a return to the euthyroid state; the E2 and progesterone levels normalized and the plasma E2/T ratio declined. In addition to the classic symptoms, some atypical symptoms of Graves' disease may also occur. One of the challenges lies in recognizing the underlying etiology. Early diagnosis and appropriate treatment can avoid unnecessary investigations and serious cardiopulmonary complications.

Complete heart block during potassium therapy in thyrotoxic periodic paralysis.

BACKGROUND: Although cardiac dysrhythmia is common in patients with thyrotoxic periodic paralysis (TPP), high-degree atrioventricular (AV) block complicated by cardiogenic shock, even under KCl supplementation, is rarely described. OBJECTIVES: To present a case of TPP in a patient who developed complete AV block with severe consequences due to paradoxical hypokalemia during KCl therapy. In addition, the management of acute hypokalemia in TPP is reviewed. CASE REPORT: A 41-year-old Chinese man with TPP presented to the Emergency Department with a 2-day history of paralysis in the extremities. He developed complete AV block with cardiogenic shock and respiratory failure, necessitating ventilatory support when plasma K(+) level decreased from 1.7 mmol/L to 1.3 mmol/L during KCl replacement of 30 mmol in 2 h. The administration of another 60 mmol KCl over 3 h achieved a plasma K(+) level of 2.1 mmol/L, resulting in the resolution of AV block and successful weaning. However, rebound hyperkalemia (K(+) 5.6 mmol/L) upon recovery was evident and uneventfully corrected. CONCLUSION: A paradoxical fall in serum K(+) concentration with potentially life-threatening complication is still underappreciated in patients with TPP on KCl supplementation. Early recognition and prompt therapy prevent untoward consequences.

An unexpected cause of proximal myopathy.

A 25-year-old man presented with a short history of profound proximal muscle weakness such that he was unable to stand. Laboratory investigations demonstrated hypokalaemia and mildly elevated serum creatine kinase. He reported a history of 8-10 episodes of less severe weakness in the preceding 8 years and his mother reported similar symptoms. The combination of weakness and hypokalaemia with a probable family history suggested the diagnosis of hypokalaemic periodic paralysis. He was treated with intravenous and oral potassium supplementation, and regained full power within 24 h.

Hypokalemic paralysis in a young obese female.

BACKGROUND: Profound hypokalemia with paralysis usually poses a diagnostic and therapeutic challenge. METHODS: We report on a 28-y-old obese Chinese female presenting with sudden onset of flaccid quadriparesis upon awaking in the morning. There is no family history of hyperthyroidism. She experienced body weight loss of 7 kg in 2 months. RESULTS: The most conspicuous blood biochemistry is marked hypokalemia (1.8 mmol/l) and hypophosphatemia (0.5 mmol/l) associated with low urine K(+) and phosphate excretion. Surreptitious laxatives and/or diuretics abuse-related hypokalemic paralysis were tentatively made. However, her relatively normal blood acid-base status and the absence of low urine Na(+) and/or Cl(-) excretion made these diagnoses unlikely. Furthermore, she developed rebound hyperkalemia (5.7 mmol/l) after only 80 mmol K(+) supplementation. Thyroid function test confirmed hyperthyroidism due to Graves' disease. Control of the hyperthyroidism completely abolished her periodic paralysis. CONCLUSIONS: Thyrotoxic periodic paralysis (TPP) should be kept in mind as a cause of paralysis in female, even with obesity, despite its predominance in adult males.