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.

Arterial blood gas analysis aids early differential diagnosis and treatment of primary and secondary hypokalaemic periodic paralysis.

This study aimed to examine changes in electrolytes and acid-base status in primary and secondary hypokalaemic periodic paralysis (HypoPP), which will help early differential diagnosis of HypoPP. A total of 64 HypoPP patients were enrolled and relevant data from clinical records was collected. Overall, 64 patients (mean age 28.2±7.3 years) of which 58(91%) were males, with 39, 11 and 14 patients, respectively, diagnosed as primary HypoPP, thyrotoxic HypoPP, and other secondary HypoPPs at discharge, were assessed. Those with HypoPP secondary to conditions other than hyperthyroidism were more likely to develop acid-base imbalance (p<0.001); they had higher pH (p=0.046) and HCO3 levels (p=0.014) at baseline, and needed a higher dose of potassium supplement before the serum potassium level returned to normal (p=0.007) and a longer time to regain full muscle strength (p=0.004), compared with those with primary or thyrotoxic HypoPP. Emergent arterial blood gas analysis may aid early differential diagnosis of patients with primary and secondary HypoPP.

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.

Potassium losing, aldosterone producing adrenocortical carcinoma: a rare presentation.

Adrenocortical carcinomas (ACCs) are rare malignancies with an incidence of one to two per million per year. Aldosterone-producing ACCs (APACs) are extremely rare with an incidence less than 1%. We describe a rare case of APAC, presenting with episodic lower-limb weakness and hypertension. Our patient was found to have serum aldosterone levels of 20.8 ng/dL (2.5-15.2) with persistent hypokalaemia and a 9.7×8.3×7.7 cm right adrenal mass, which was suspicious of malignancy on evaluation. He underwent a complete surgical resection which confirmed the diagnosis of ACC and normalised his aldosterone and potassium levels. He was then subjected to postoperative chemotherapy. Postoperative adjuvant chemotherapy with mitotane has a role in preventing recurrence.

[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 periodic paralysis - the importance of patient education.

Hypokalemic periodic paralysis (HOKPP) is a rare neuromuscular disorder caused by altered transport of cellular potassium that leads to significant muscle weakness of the extremities. Paralytic attacks are induced by a drop in the serum potassium level and they have been associated with specific triggers. This case describes a 21-year-old male who has had recurrent presentations of acute paralytic attacks following vigorous physical activity. At presentation, this patient exhibited flaccid paralysis of all skeletal muscles below the neck, but was alert and oriented with stable vital signs. The patient was found to have a potassium level of 2.1 mmol/L and an EKG demonstrating U waves (characteristic of hypokalemia). The patient was treated with potassium supplementation with resolution of symptoms. The mainstay of prevention of long term permanent muscle weakness is avoidance of triggers that can lead to hypokalemia. Through education on disease process and lifestyle modifications, we were able to end the cycle of recurrent hospital readmissions and the subsequent financial burden this generated for the patient and his family.

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.

Thyrotoxic Channelopathies.

Thyrotoxic periodic paralysis (TPP), a disorder most commonly seen in Asian men, is characterized by abrupt onset of hypokalemia and paralysis. The condition primarily affects the lower extremities and is secondary to thyrotoxicosis. Early recognition of TPP is vital to initiating appropriate treatment and to avoiding the risk of rebound hyperkalemia that may occur if high-dose potassium replacement is given. Here we present a case of 31 year old male with thyrotoxic periodic paralysis with diagnostic and therapeutic approach.

Thyrotoxic periodic paralysis: an endocrine cause of paraparesis.

Periodic paralysis is a muscle disorder that belongs to the family of diseases called channelopathies, manifested by episodes of painless muscle weakness. Periodic paralysis is classified as hypokalemic when episodes occur in association with low potassium levels. Most cases are hereditary. Acquired cases have been described in association with hyperthyroidism. Diagnosis is made on clinical and biochemical grounds. Patients may be markedly hypokalemic during the episode and respond well to potassium supplementation. Episodes can be prevented by achieving a euthyroid state. This report describes a young gentleman presenting with thyrotoxic hypokalemic paraparesis. The condition needs to be considered in the differential diagnosis of neuromuscular weakness in the context of hypokalemia by the treating physicians.

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.

Dengue-associated hypokalemic paralysis: causal or incidental?

Dengue-associated hypokalemic paralysis is considered an important but under-emphasized neuromuscular complication of dengue virus infection. Review of the published literature reveals that 35 instances of hypokalemic paralysis associated with dengue have been recorded from the Indian subcontinent and all but two, were males. The median age of presentation is 29 years and moderate to severe grade pure motor quadriparesis is precipitated during the phase of defervescence of moderate to high-grade fever. Recovery starts within 12h of potassium supplementation and is usually complete in a couple of days. Redistribution or increased loss of potassium from the body is speculated as the pathophysiological mechanism involved in the causation of hypokalemia. It is not possible to derive the exact etiopathological correlation from the published literature either due to a lack of comprehensive reporting or inadequate work-up of the patients. Curious is the fact that only 35 patients had manifest-paralysis when more than two-thirds affected with the dengue virus exhibit hypokalemia; whether this indicates a genetically mediated channel disorder or an incidental association remains to be seen.

Iodine-induced thyrotoxic hypokalemic paralysis after ingestion of Salicornia herbace.

A 56-year-old Korean man visited to emergency room due to paroxysmal flaccid paralysis in his lower extremities. There was no family or personal history of periodic paralysis. His initial potassium levels were 1.8 mmol/L. The patient had been taking Salicornia herbacea for the treatment of diabetes and hypertension. Results of a thyroid function test were as follows: T3 = 130.40 ng/dL, TSH = 0.06 mIU/L, and free T4 = 1.73 ng/dL. A thyroid scan exhibited a decreased uptake (0.6%). His symptoms clearly improved and serum potassium levels increased to 4.4 mmol/L by intravenous infusion of only 40 mmol of potassium chloride. Eight months after the discontinuation of only Salicornia herbacea, the patient's thyroid function tests were normalized. Large amounts of iodine can induce hypokalemic thyrotoxic paralysis and it may be necessary to inquire about the ingestion of iatrogenic iodine compounds, such as Salicornia herbacea.

[Thyrotoxic hypokalemic periodic paralysis. A case report]. / Parálisis periódica hipopotasémica tirotóxica: a propósito de un caso.

Thyrotoxic hypokalemic periodic paralysis is an uncommon complication of thyrotoxicosis, characterized by attacks of generalized muscular weakness associated with hypokalemia in patients with hyperthyroidism, most frequently with Graves-Basedow disease. Treatment with antithyroid drugs and potassium supplements reversed the symptoms and the episodes of acute muscular weakness did not reappear.

[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.

Novel etiopathophysiological aspects of thyrotoxic periodic paralysis.

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.

Spectrum of hypokalaemic periodic paralysis in a tertiary care centre in India.

BACKGROUND: Acute flaccid paralysis is a common neurological emergency with diverse causes and variable outcome. There is a paucity of reports documenting the spectrum of hypokalaemic paralysis in neurological practice. OBJECTIVE: To report the clinical features, aetiology, and outcome of patients with hypokalaemic paralysis in a tertiary care teaching hospital in India. METHODS: Consecutive patients with acute flaccid paralysis with hypokalaemia from 2008 to 2010 were included in the study. Patients with Guillain-Barré syndrome, porphyria, polio and non-polio enterovirus infection and myositis were excluded. Detailed clinical examination, urinalysis, renal function tests, arterial blood gas analysis, thyroid hormones, and electrocardiogram were carried out. Patients received intravenous or oral potassium supplementation and their underlying causes were treated. RESULTS: Thirty patients aged 17-52 years, including three females, were included. Secondary causes of hypokalaemic paralysis were present in 13 patients and included thyrotoxic paralysis in five and renal tubular acidosis (RTA) and Gitelman syndrome in four each. All the patients had quadriparesis and 10 had severe weakness (MRC grade <2). Tendon reflexes were reduced in eight and brisk in four patients. Respiratory paralysis was present in six patients and one needed artificial ventilation. Fifteen patients had severe hypokalaemia (<2 mmol/l), four had acidosis, and six had alkalosis. The secondary group had more severe hypokalaemia and needed longer time to recover. CONCLUSION: 43.3% of patients with hypokalaemic paralysis had a secondary cause for their condition. Patients with severe hypokalaemia with acidosis or alkalosis should be investigated for secondary causes as their management differ.