Genetic analysis of 37 cases with primary periodic paralysis in Chinese patients.

BACKGROUND: Primary periodic paralysis (PPP) is an inherited disorders of ion channel dysfunction characterized by recurrent episodes of flaccid muscle weakness, which can classified as hypokalemic (HypoPP), normokalemic (NormoPP), or hyperkalemic (HyperPP) according to the potassium level during the paralytic attacks. However, PPP is charactered by remarkable clinical and genetic heterogeneity, and the diagnosis of suspected patients is based on the characteristic clinical presentation then confirmed by genetic testing. At present, there are only limited cohort studies on PPP in the Chinese population. RESULTS: We included 37 patients with a clinical diagnosis of PPP. Eleven (29.7%) patients were tested using a specific gene panel and 26 (70.3%) by the whole-exome sequencing (WES). Twenty-two cases had a genetic variant identified, representing a diagnostic rate of 59.5% (22/37). All the identified mutations were either in the SCN4A or the CACNA1S gene. The overall detection rate was comparable between the panel (54.5%: 6/11) and WES (61.5%: 16/26). The remaining patients unresolved through panel sequencing were further analyzed by WES, without the detection of any mutation. The novel atypical splicing variant c.2020-5G > A affects the normal splicing of the SCN4A mRNA, which was confirmed by minigene splicing assay. Among 21 patients with HypoPP, 15 patients were classified as HypoPP-2 with SCN4A variants, and 6 HypoPP-1 patients had CACNA1S variants. CONCLUSIONS: Our results suggest that SCN4A alleles are the main cause in our cohort, with the remainder caused by CACNA1S alleles, which are the predominant cause in Europe and the United States. Additionally, this study identified 3 novel SCN4A and 2 novel CACNA1S variants, broadening the mutation spectrum of genes associated with PPP.

Hypokalemic Periodic Paralysis Type 2 Due to SCN4A Val1105Met Mutation: A Case Study.

Hypokalemic Periodic Paralysis Type 2 (HOKPP2) is a rare autosomal dominant disorder characterized by recurrent episodes of muscle weakness, paralysis, and hypokalemia. In this case report, we present the clinical details of a 49-year-old female diagnosed with HOKPP2. Genetic testing revealed a heterozygous mutation in the Sodium Voltage-Gated Channel Alpha Subunit 4 (SCN4A) gene, confirming the diagnosis of HOKPP2. Management strategies, including potassium supplementation and lifestyle modifications, were implemented, resulting in a significant decrease in the frequency of symptomatic episodes. This case highlights the importance of considering HOKPP2 in patients with recurrent muscle weakness, particularly those with a familial history of similar symptoms. Furthermore, it underscores the crucial role of genetic testing in guiding patient management and facilitating genetic counseling.

Physiotherapy Strategies in Hypokalemic Periodic Paralysis: A Case Report.

The rare neuromuscular disease known as hypokalemic periodic paralysis (hypoKPP), which results in severe muscle weakness in the extremities, is brought on by abnormalities in potassium transport within cells. Laboratory testing is confirmatory, which reveals notably low potassium levels, causing paralysis, which improves once the low potassium is restored. The patient generally complains of muscle weakness with difficulty in performing activities of daily living and impaired participation in functional tasks, with few suffering from coexisting sensory impairments. Physiotherapy generally plays a symptomatic role with motion exercises for the affected muscle groups. There is no standardized physiotherapy protocol for disease-specific impairments. A 46-year-old man complained of bilateral upper and lower limb muscular weakness and was admitted to the neurology ward. The patient also complained of having tingling numbness throughout their entire limbs and had experienced similar episodes of symptoms six months prior. During laboratory evaluation, a significantly low potassium level was found, leading to a diagnosis of hypoKPP. Following medical management, neurophysiotherapy was initiated. Physiotherapy strategy shows significant improvement in muscular strength and functional activities. Thus, this case report concludes that physiotherapy plays a vital role in managing hypoKPP by enhancing muscular strength, functional activities, and quality of life.

A Rare Case of Hypokalemic Periodic Paralysis With Acute Urinary Retention: Diagnosis and Management.

Hypokalemic periodic paralysis (hypoPP) is a rare channelopathy caused by mutations in skeletal muscle ion channels that usually occurs in young individuals and adolescents. The etiology can be attributed to various factors, such as idiopathic or secondary causes. It is characterized by episodes of sudden flaccid muscle weakness. Timely detection may mitigate the risk of severe complications. Secondary causes of hypoPP, such as hyperthyroidism, should be ruled out, as this could lead to thyrotoxic periodic paralysis. We report the case of a 19-year-old boy who presented to the ED with severe weakness in both the upper and lower extremities. The weakness rapidly progressed to his trunk and was accompanied by acute urinary retention. The physical examination was significant for bilateral upper and lower extremity weakness. Subsequent laboratory investigations revealed markedly low serum potassium levels. The patient's symptoms resolved after the replacement of potassium, and he was discharged without neurological deficits. Although rarely accompanied by acute urinary retention, hypoPP must be differentiated from other causes of weakness and paralysis so that the proper treatment can be initiated quickly. The rarity of hypoPP, a condition seldom encountered in clinical practice, and the added rarity of its coexistence with acute urinary retention further underscore the uniqueness of this case report.

Prevalence and risk factors of low vitamin D levels in children and adolescents with familial hypokalemic periodic paralysis.

Patients with familial hypokalemic periodic paralysis (HOKPP) experience episodes of reversible immobility and are at an increased risk of limited sunlight exposure, potentially leading to vitamin D deficiency. However, there is a lack of data on vitamin D levels in this population. We investigated serum vitamin D levels and their associated factors in children with HOKPP. This study included 170 genetically-confirmed children with HOKPP, aged 3-18 years, and 170 age-, sex-, and body mass index (BMI)-matched healthy controls from the Korean Channelopathy Study, a prospective controlled investigation. Anthropometric and clinical characteristics were recorded, and serum levels of calcium, ionized calcium, phosphorus, alkaline phosphatase, 25-hydroxyvitamin D, and intact parathyroid hormone (PTH) were analyzed. Vitamin D deficiency (< 20 ng/mL) was observed in 87.0% of the patients compared to 45.5% of the controls (P < 0.05) during the summer-fall season. During the winter-spring season, 91.7% of the patients and 73.4% of the controls were deficient (P < 0.05). A strong positive correlation was found between onset age of the first paralytic attack and vitamin D levels (r = 0.78, P < 0.01). Conversely, the frequency and duration of paralytic attacks were negatively correlated with vitamin D levels (r = -0.82 and r = -0.65, P < 0.01, respectively). Age, BMI, age at onset, frequency and duration of attacks, and PTH levels were independently associated with vitamin D levels (ß = -0.10, -0.12, 0.19, -0.27, -0.21, and -0.13, P < 0.05, respectively). CONCLUSIONS: Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics. We recommend routine screening for vitamin D levels in these patients to address this prevalent deficiency. Considering the high prevalence of vitamin D deficiency observed, further research on other diseases characterized by reversible immobility is warranted. WHAT IS KNOWN: • A correlation between immobility and low serum vitamin D levels has been established. However, the vitamin D status of patients with familial hypokalemic periodic paralysis (HOKPP) who experience periods of reversible immobility remains unknown. WHAT IS NEW: • Vitamin D deficiency was highly prevalent in children with HOKPP, and vitamin D levels correlated with various disease characteristics.

A Thyrotoxic Periodic Paralysis Case Study: From Weakness to Wellness.

Hypokalaemic periodic paralysis (HPP) is a rare disorder characterized by episodic attacks of muscle weakness and hypokalaemia. Numerous factors contributing to HPP have been identified, encompassing both hereditary and familial origins as well as acquired factors. In this context, we highlight thyrotoxicosis causing HPP. We present a case of a 40-year-old Asian individual who presented with episodes of sudden onset bilateral proximal limb weakness and palpitations. Laboratory investigations revealed severe hypokalaemia (serum potassium: 1.8 mmol/L). Immediate potassium replacement therapy alleviated symptoms. Further evaluation revealed a new diagnosis of hyperthyroidism, with subsequent treatment initiated (carbimazole and propranolol) preventing recurrence of symptoms. This case highlights the importance of recognizing HPP as a potential manifestation of thyroid dysfunction, particularly in individuals of Asian ethnicity.

Thyrotoxic Periodic Paralysis as an Ongoing Diagnostic Challenge: A Case Report and Literature Review.

Thyrotoxic periodic paralysis (TPP) is a rare condition that presents with episodic periodic paralysis due to hypokalemia that develops from hyperthyroidism. Timely diagnosis is still an ongoing challenge due to lack of awareness, self-resolving episodes, and the fact that it clinically mimics familial hypokalemic periodic paralysis (FHPP), which is more common in the West. TPP is more commonly seen among Asians but has been emerging in Western countries due to globalization. We present a case of a 24-year-old Hispanic male who presented with bilateral lower extremity weakness. He had five such episodes in the past year, which resolved on their own. The current episode of weakness was worse, and he required a wheelchair to ambulate. Despite extensive work, it took over four months to make a definitive diagnosis and treat his hyperthyroidism. A literature review reported that most cases of TPP are usually diagnosed after multiple episodes, and the causes of diagnostic error were studied. Through this review, we present a case of TPP with diagnostic delay, a literature review discussing the etiology, pathogenesis, clinical manifestations, and management, with an emphasis on the diagnostic challenge of TPP. Awareness of this condition, timely evaluation for hyperthyroidism as a cause for hypokalemic periodic paralysis, and understanding the factors that contribute to its diagnostic challenge will aid in timely recognition and treatment.

Newly Diagnosed Hypokalemic Periodic Paralysis Triggered by COVID-19.

Hypokalemic periodic paralysis (HypoPP) is a rare genetic disorder characterized by low potassium levels and episodic periods of muscle weakness. HypoPP has previously been attributed to numerous viral infections; however, cases related to coronavirus disease 2019 (COVID-19) are extremely limited. The current case is thus unique and involves a healthy 23-year-old male who presented to the emergency department after several uncharacteristic falls and three days of upper and lower extremity weakness. Initial labs revealed a potassium level of 1.1 mmol/L as well as being COVID-19 positive. Potassium supplementation helped stabilize his levels and relieved all of his symptoms. Based on an extensive clinical workup and significant family history of the mother and maternal grandmother with weakness in the setting of hypokalemia, a diagnosis of HypoPP was made. Upon discharge, he was placed on potassium-sparing diuretics to help prevent further symptom relapse and advised to complete genetic testing. With the high likelihood of the virus being endemic for years to come, clinicians should remember to consider HypoPP with patients with muscle weakness, especially in patients with concurrent COVID-19 infection, to minimize unnecessary workup and prevent potentially life-threatening symptoms of hypokalemia.

A novel CACNA1S gene variant in a child with hypokalemic periodic paralysis: a case report and literature review.

BACKGROUND: The CACNA1S gene encodes the alpha 1 S-subunit of the voltage-gated calcium channel, which is primarily expressed in the skeletal muscle cells. Pathogenic variants of CACNA1S can cause hypokalemic periodic paralysis (HypoPP), malignant hyperthermia susceptibility, and congenital myopathy. We aimed to study the clinical and molecular features of a male child with a CACNA1S variant and depict the molecular sub-regional characteristics of different phenotypes associated with CACNA1S variants. CASE PRESENTATION: We presented a case of HypoPP with recurrent muscle weakness and hypokalemia. Genetic analyses of the family members revealed that the proband had a novel c.497 C > A (p.Ala166Asp) variant of CACNA1S, which was inherited from his father. The diagnosis of HypoPP was established in the proband as he met the consensus diagnostic criteria. The patient and his parents were informed to avoid the classical triggers of HypoPP. The attacks of the patient are prevented by lifestyle changes and nutritional counseling. We also showed the molecular sub-regional location of the variants of CACNA1S which was associated with different phenotypes. CONCLUSIONS: Our results identified a new variant of CACNA1S and expanded the spectrum of variants associated with HypoPP. Early genetic diagnosis can help avoid diagnostic delays, perform genetic counseling, provide proper treatment, and reduce morbidity and mortality.

Case report: A novel CACNA1S mutation associated with hypokalemic periodic paralysis.

Background: Hypokalemic periodic paralysis (HypoKPP) is a rare neuromuscular genetic disorder causing recurrent episodes of flaccid paralysis. Most cases are associated with CACNA1S mutation, causing defect of calcium channel and subsequent impairment of muscle functions. Due to defined management approaches early diagnosis is crucial for promptly treatment and prevention new attacks. Materials and methods: We report a case of HypoKPP associated with previously unreported mutation in CACNA1S gene (p.R900M). Molecular modeling of CaV1.1 was applied to evaluate its pathogenicity. Results: As a patient referred between attacks neurological status, laboratory and neurophysiological examination were unremarkable. Molecular modeling predicted that the p.R900M mutation affects the process of calcium channels activation. Conclusion: Novel CACNA1S mutation, associated with HypoKPP was identified. Monte-Carlo energy minimization of the CaV1.1 model supported the association of this mutation with this disease.

Hypokalemic periodic paralysis: a 3-year follow-up study.

BACKGROUND AND OBJECTIVES: Primary hypokalemic periodic paralysis (HypoPP) is an inherited channelopathy most commonly caused by mutations in CACNA1S. HypoPP can present with different phenotypes: periodic paralysis (PP), permanent muscle weakness (PW), and mixed weakness (MW) with both periodic and permanent weakness. Little is known about the natural history of HypoPP. METHODS: In this 3-year follow-up study, we used the MRC scale for manual muscle strength testing and whole-body muscle MRI (Mercuri score) to assess disease progression in individuals with HypoPP-causing mutations in CACNA1S. RESULTS: We included 25 men (mean age 43 years, range 18-76 years) and 12 women (mean age 42 years, range 18-76 years). Two participants were asymptomatic, 21 had PP, 12 MW, and two PW. The median number of months between baseline and follow-up was 42 (range 26-52). Muscle strength declined in 11 patients during follow-up. Four of the patients with a decline in muscle strength had no attacks of paralysis during follow-up, and two of these patients had never had attacks of paralysis. Fat replacement of muscles increased in 27 patients during follow-up. Eight of the patients with increased fat replacement had no attacks of paralysis during follow-up, and two of these patients had never had attacks of paralysis. DISCUSSION: The study demonstrates that HypoPP can be a progressive myopathy in both patients with and without attacks of paralysis.

An Instance of Hypokalemic Periodic Paralysis in Adolescent Brothers: A Case Report.

Hypokalemic periodic paralysis (HypoPP) is a rare autosomal dominant disease caused by mutations in either calcium or sodium transmembrane voltage-gated ion channels of skeletal muscle or endoplasmic reticulum. Most cases of HypoPP are associated with a mutation in the gene encoding a calcium channel, the CACNA1S gene. Mutations in the channels create leakage currents that disrupt resting potential and depolarize the muscle fiber resulting in transient flaccid paralysis and low extracellular potassium (K+). Patients experience episodes of muscle paralysis typically provoked by exertion and diet. Treatment focuses on the prevention of such episodes with carbonic-anhydrase inhibitors or potassium-sparing diuretics as well as to treatment of acute episodes with oral K+ supplementation. Due to the rarity of the disease, the literature surrounding the disease and pharmacological management is limited. We present a case of two adolescent brothers who present with a confirmed diagnosis of periodic episodes of paralysis and are seeking treatment. Both brothers experience paralytic episodes provoked by acute changes in diet and exercise. However, the lack of literature and treatment guidelines surrounding the disease emphasizes the importance of documenting cases and the effectiveness of treatment outcomes. Additionally, it reminds providers to keep HypoPP on the differential when faced with a young patient experiencing paralytic episodes.

How is Guillain-Barre syndrome associated with COVID-19 infection differentiated from hypokalemic periodic paralysis? a case report.

Patients with coronavirus disease 2019 (COVID-19)-associated Guillain-Barre syndrome (GBS) exhibit a range of clinical symptoms, such as cranial nerve paralysis and axonal or motor-sensory electrophysiological signals. Case presentation: A 61-year-old retired black African female was brought into the emergency room on 13 May 2022, with a 4-day history of shortness of breath and high-grade fever and a 1-day history of global body weakness (bilateral paralysis of the upper and lower extremities). Motor examination indicated reduced muscular strength in all limbs, with a Medical Research Council score of 2/5 in the right arm of the upper extremities, 1/5 in the right leg of the lower extremities, 1/5 in the left leg of the lower extremities, and 2/5 in the left arm of the upper extremities. Her electrocardiogram revealed ST depression in the anterior-lateral leads and sinus tachycardia. For the COVID-related infection, azithromycin 500 mg per day for 5 days was begun. After cerebrospinal fluid findings supported the diagnosis of GBS, she underwent intravenous immunoglobulin 400 mg/kg every day for 5 days. Clinical discussion: In the majority of COVID-19-related GBS cases, areflexic quadriparesis developed suddenly. A COVID-19 infection related to a GBS case was the only one that had preceding signs, including ageusia and hyposmia. By testing serum potassium levels, this study determined that there is no connection between GBS and hypokalemia, which can lead to diagnostic and therapeutic conundrums by evaluating serum potassium levels, which showed a normal value. Conclusion: One of the neurological symptoms of the COVID-19 infection is GBS. Several weeks after a COVID-19 acute infection, GBS is frequently observed.

A Case of Sudden-Onset Flaccid Paralysis In a Previously Healthy Person.

Flaccid paralysis is a neurological syndrome characterized by weakness and paralysis of the limbs, followed by reduced muscle tone. Common causes of flaccid paralysis include blockage of the anterior spinal artery, trauma to the spinal cord, cancer, arterial disease, or thrombosis. A potential differential diagnosis in a 35-year-old male presenting with sudden-onset flaccid paralysis with no history of trauma is hypokalemic periodic paralysis. Treatment with potassium can alleviate symptoms in affected patients. .

Optical measurement of gating pore currents in hypokalemic periodic paralysis model cells.

Hypokalemic periodic paralysis (HypoPP) is a rare genetic disease associated with mutations in CACNA1S or SCN4A encoding the voltage-gated Ca2+ channel Cav1.1 or the voltage-gated Na+ channel Nav1.4, respectively. Most HypoPP-associated missense changes occur at the arginine residues within the voltage-sensing domain (VSD) of these channels. It is established that such mutations destroy the hydrophobic seal that separates external fluid and the internal cytosolic crevices, resulting in the generation of aberrant leak currents called gating pore currents. Presently, the gating pore currents are thought to underlie HypoPP. Here, based on HEK293T cells and by using the Sleeping Beauty transposon system, we generated HypoPP-model cell lines that co-express the mouse inward-rectifier K+ channel (mKir2.1) and HypoPP2-associated Nav1.4 channel. Whole-cell patch-clamp measurements confirmed that mKir2.1 successfully hyperpolarizes the membrane potential to levels comparable to those of myofibers, and that some Nav1.4 variants induce notable proton-based gating pore currents. Importantly, we succeeded in fluorometrically measuring the gating pore currents in these variants by using a ratiometric pH indicator. Our optical method provides a potential in vitro platform for high-throughput drug screening, not only for HypoPP but also for other channelopathies caused by VSD mutations.

An Interesting Case of Weakness and Atrial Tachycardia in the Emergency Department: Thinking Beyond Hearts and Minds.

Thyrotoxic periodic paralysis is a rare but life-threatening presentation of hyperthyroidism that manifests with sudden, painless episodes of muscle weakness due to hypokalemia. We present the case of a middle-aged Middle Eastern female who attended our Emergency Department with sudden onset weakness to the lower limbs, resulting in her inability to walk. She had a power of 1/5 in the lower limbs, and subsequent investigations showed a low potassium level, and primary hyperthyroidism secondary to Grave's disease was diagnosed. A 12-lead electrocardiogram showed atrial flutter with a variable block, along with U waves. The patient reverted to sinus rhythm following administration of potassium replacement and was also treated with Propanalol and Carbimazole. The patient made a full neurological recovery.  Emergency physicians and all frontline healthcare workers should be aware that electrolyte problems can cause paralysis. Furthermore, hypokalemic periodic paralysis can be caused by an undiagnosed thyrotoxic state. Be aware that if left untreated, hypokalemia can cause serious atrial and ventricular arrhythmias. Achieving a euthyroid state and blunting hyperadrenergic stimulation, in addition to replacing potassium, all help to fully reverse muscle weakness.

Hypokalemic periodic paralysis presenting as asymmetric focal flaccid paralysis: A case report and literature review.

Patients with the most common form of hypokalemic periodic paralysis (HypoKPP) exhibit symmetrical limb weakness. However, few patients present with asymmetric limb weakness. Here, we describe a unique case of HypoKPP presenting as asymmetric focal flaccid paralysis. In addition, a literature review is performed to provide a perspective for clinical management of similar cases. We present a detailed characterization of this rare type of HypoKPP. The initial presentation was right hand weakness, which progressed to bilateral lower limb weakness. Neurological examination showed that the affected muscles were uniquely confined to specific nerve innervation, i.e., right distal median nerve-innervated muscle, right deep peroneal nerve-innervated muscle and left side. The patient's serum level of potassium was lower than normal; the decline of long exercise test (LET) was higher than normal range; neurophysiological assessment revealed low amplitude compound muscle action potential (CMAP) during attack, the CMAP and patient's weakness rapidly returned to normal level after potassium supplementation. Therefore, HypoKPP can be formally diagnosed based on neurological examination, medical history, timely neural electrophysiological examinations and measurement of blood potassium level.

Steroid-induced hypokalemic periodic paralysis: a case report and literature review.

BACKGROUND: Hypokalemic periodic paralysis (HPP) is a rare channelopathy characterized by episodic attacks of acute muscle weakness concomitant with hypokalemia. The etiology of hypokalemia is the shift of potassium into the cells, and the clinical symptoms resolve when potassium starts to leak back to the serum. Most of the time, the underlying ion channel defects are well compensated, and an additional trigger is often required to initiate an attack. Well-known trigger factors include carbohydrate-rich meals, exercise followed by rest, stress, cold weather, and alcohol consumption. CASE PRESENTATION: Here, we present the case of a 26-year-old Asian man who suffered from an acute onset of bilateral lower limb weakness with hypokalemia following dexamethasone injection. He was diagnosed with HPP. CONCLUSIONS: We would like to remind physicians to think of steroids as an unusual precipitating factor while managing patients with HPP, per results of this case study.

Thyrotoxic periodic paralysis associated with lactic metabolic acidosis: Case report of an African man and review of literature.

BACKGROUND: Thyrotoxic periodic paralysis (TPP) is a rare and most often acquired subtype of hypokalemic periodic paralysis. The association of varying degrees of muscle weakness, hyperthyroidism and hypokalemia characterizes it. The treatment requires potassium supplementation, control of hyperthyroidism and prevention measures. It is a frequent disease in Asian men, but much rare in Caucasian or African populations. This is the first report of TPP associated with lactic metabolic acidosis in an African man. CASE PRESENTATION: A 23 year-old African man, native from Morocco, with recurrent episodes of tetraparesis for eleven months, and abdominal pain, was referred for evaluation. Biochemical investigations showed severe hypokalemia associated with hyperthyroidism and lactic metabolic acidosis. His EKG showed signs of hypokalemia such as sinus tachycardia and U waves. After potassium supplementation, neurological recuperation was quick and complete. Thyroid ultrasound identified a hypoechogenic and hypervascularized goiter, associated with high levels of thyroid antibodies, in favor of Grave's disease. With antithyroid drugs and life-style changes, the patient did not have any other attack. REVIEW OF LITERATURE: In addition to the case report, this article presents an extended review of literature, from the first large study reporting the diagnosis and incidence of TPP in 1957 to nowadays. Are reported here the latest information concerning epidemiology, clinical manifestations, complementary examinations, management and genetic finding. The lactic acidosis observed initially is exceptional, never described in TPP. TPP is a diagnostic and therapeutic emergency, requiring careful potassium supplementation, in order to avoid the risk of the onset of rebound hyperkalemia, to be maintained until the etiological treatment is effective. Paraclinical assessment with emergency EKG and electromyogram are essential to assess the impact. DISCUSSION: It is essential in the face of any hypokalaemic periodic paralysis, including in non-Asian subjects, to search hyperthyroidism. CONCLUSIONS: This report demonstrates the importance of thyroid testing in case of acute muscle weakness, even in non-Asian patients in order to diagnose TPP. This is a rare but possible etiology, to be distinguished from the familial form of hypokalemic periodic paralysis. It also questions on the impact of TPP on energetic metabolism, in particular on lactic metabolism.

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.