Diagnosis Of Hypokalemic Periodic Paralysis Research Articles

The role of nephrologists in management of hypokalemic periodic paralysis: a case report

BackgroundHypokalemic periodic paralysis is a chronic condition characterized by sporadic attacks of weakness associated with acute hypokalemia. Attacks are typically associated with specific triggers, such as prolonged rest following exercise or consumption of a high-carbohydrate meal. Most commonly, this condition is caused by an autosomal dominant calcium channel mutation, and patients typically have an established family medical history of hypokalemic periodic paralysis. Long-term complications include the development of progressive proximal myopathy. Oral potassium chloride may be considered for the treatment of an acute attack, with administration of acetazolamide or dichlorphenamide as long-term prophylaxis. Nephrologists can play an important role in the recognition and treatment of previously undiagnosed hypokalemic periodic paralysis.Case presentationWe summarize the case of a 19-year-old white man who presented to the emergency department with undiagnosed attacks of hypokalemic periodic paralysis, and who reported, at follow-up, improvement in the severity and frequency of attacks with dichlorphenamide.ConclusionsThis case demonstrates the crucial role nephrologists can play, not only in the diagnosis of hypokalemic periodic paralysis, but also in the ongoing management of this condition. Patients should be advised to regularly follow up with their nephrology team for evaluation due to the risk of developing myopathy.

Hypokalemic periodic paralysis as initial manifestation of primary Sjogren’s syndrome: A case report

Sjogren’s syndrome (SS) is common autoimmune diseases manifesting with various glandular and extra glandular features which are difficult to evaluate and manage. Hypokalemic flaccid paralysis may be the rare extra glandular clinical manifestation of SS. We present a case of acute flaccid paralysis which on evaluation turned out to be primary sjogren’s syndrome (pSS). We present a case of 30 year old young woman with no known comorbidities presented with acute flaccid paralysis. Her evaluation reveals severe hypokalemia with K-1.6 meq/l, metabolic acidosis with pH-7.28 and urinary pH ≥6.5. An initial diagnosis of hypokalemic periodic paralysis (HPP) secondary to distal renal tubular acidosis (dRTA) was made. She was later found to have sjogren’s syndrome on the basis of positive ANA & Anti-Ro (SS-A) Antibody and salivary gland biopsy. She was managed with potassium supplements, sodium bicarbonate and immunomodulators. Primary Sjogren’s syndrome usually associated with distal renal tubular acidosis which may cause hypokalemia leading to HPP, rarely which may be the initial presenting symptom in young women as in our case. Early diagnosis and treatment of these patients would lead to less complication and better quality of life.

Transient complete atrioventricular block and ST-segment elevation induced by coronary vasospasm due to iatrogenic hyperkalemia: a case report

BackgroundHyperkalemia and acute coronary syndrome are not only all responsible for syncope related to complete atrioventricular block, but also share parts of electrocardiogram manifestations. Additionally, they influence each other.Case presentationA 32-year-old Chinese man presented with severe hypokalemia (1.63 mmol/l) at midnight in the emergency room. He developed unexpected rebound hyperkalemia (7.76 mmol/l) after 18 hours of oral and intravenous potassium chloride supplementation at a concentration of about 10 g/day and a rate of 10 mmol/hour. Subsequently, the patient complained of chest discomfort and dyspnea, followed by syncope for several minutes, approximately 2 hours after potassium reduction treatment had been started. The instant electrocardiogram showed complete atrioventricular block and elevated ST segment in the inferolateral leads, which resolved 15 minutes later, before hyperkalemia was corrected. Combined with mild coronary stenosis and negative myocardial injury markers, transient complete atrioventricular block induced by coronary vasospasm due to iatrogenic hyperkalemia was diagnosed. Normal urine potassium excretion, acid–base state, and other examinations made the diagnosis of hypokalemic periodic paralysis possible.ConclusionsHyperkalemia may provoke acute coronary syndrome, and early coronary angiography is an effective strategy for identifying the direct cause of acute complete atrioventricular block.

HIPOKALEMIK PERIODIK PARALISIS

A 7-year-old boy of Java origin, visited the doctor with progressive weakness everytime he ate food containing Monosodium Glutamat (MSG). Progressive weakness began from his legs and spreaded to the arms as well (sometimes also to the neck). He could neither walk nor do anything for 5–6 hours then began to resolve spontaneously.. We suspected the diagnosis of hypokalaemic periodic paralysis upon the history of episodes of flacid paralysis and low serum concentration of potassium (< 3,5 mmol/L) during the attacts. The clinical examination showed that this boy is in good general and nutritional conditions; electrocardiogram, laboratory blood count, urinalisis, thyroid, liver, kidney function, and ANA test were normal as well. The family history of flacid paralysis was negative. We have promptly administered potassium orally and his condition was improved progressively including less degree of flacid paralysis (the weakness). A genetic testing, electromyography (EMG), muscle biopsy and another examination has not performed.

Hypokalemic Periodic Paralysis With Reversible Electrophysiological Abnormalities: A Case Report

Hypokalemic periodic paralysis (HPP) is a reasonably rare condition, presenting complains being those associated with acute systemic weakness and hypokalemia. Majority of the cases are characterized as familial or primary, whereas sporadic cases are associated with various other conditions such as barium poisoning, hyperthyroidism, renal disorders, endocrinopathies and gastrointestinal potassium losses. We report a unique case in a 5-year-old female child following gastrointestinal losses (vomiting and diarrhea). According to the patient’s mother, for the past 2 days, she was lethargic, unable to walk and hold the neck. She underwent pyelolithotomy for recurrent kidney stones 5 years back. Her neurologic examination revealed diminished bulk overall, decreased tone, power and reflexes in lower limbs with down-going plantars. Labs (except potassium) and electrocardiography were normal. Nerve conduction studies (NCS) favored the diagnosis of Guillain-Barre syndrome. Intravenous immunoglobulin and potassium replacement was started. After a week, patient’s condition had a remarkable improvement. We did a repeat NCS which came out to be normal. Potassium serum values were normal too. We turned back to the other differential diagnosis of HPP. Our case report highlights several important factors. Firstly, it states that the NCS can be deceptive in patients with flaccid paralysis, especially when we are not suspecting hypokalemia. It further explains the importance of suspecting a diagnosis of HPP in a patient presenting with features of Guillain-Barre syndrome following an NCS. J Med Cases. 2018;9(6):177-178 doi: https://doi.org/10.14740/jmc3058w

P34-14 The utility of clinical exercise test in diagnosis of hypokalaemic periodic paralysis

P34-14 The utility of clinical exercise test in diagnosis of hypokalaemic periodic paralysis

Hypokalemic periodic paralysis: a case series, review of the literature and update of management

The objective of this study was to present a case series of patients with hypokalemic periodic paralysis. We described all patients with diagnosis of hypokalemic periodic paralysis admitted to the Al Ain Hospital (UAE) during the year 2006. Seventeen patients, all males and mostly Asians, were presented to the Al Ain Hospital over a 12-month period. The majority were admitted during the summer months. Four were thyrotoxic. All of the 17 patients received oral potassium supplements and recovered well without any major complications. In conclusion, clinicians should have a high index of suspicion, especially among Asians presenting with flaccid paralysis and hypokalemia. The main steps in the management include exclusion of other causes of hypokalemia, potassium replacement, hydration and close monitoring of the cardiac rhythm and serum potassium levels. When possible, the underlying cause must be adequately addressed to prevent the persistence or recurrence of paralysis.

Correlating phenotype and genotype in the periodic paralyses

Periodic paralyses and paramyotonia congenita are rare disorders causing disabling weakness and myotonia. Mutations in sodium, calcium, and potassium channels have been recognized as causing disease. To analyze the clinical phenotype of patients with and without discernible genotype and to identify other mutations in ion channel genes associated with disease. The authors have reviewed clinical data in patients with a diagnosis of hypokalemic periodic paralysis (56 kindreds, 71 patients), hyperkalemic periodic paralysis (47 kindreds, 99 patients), and paramyotonia congenita (24 kindreds, 56 patients). For those patients without one of the classically known mutations, the authors analyzed the entire coding region of the SCN4A, KCNE3, and KCNJ2 genes and portions of the coding region of the CACNA1S gene in order to identify new mutations. Mutations were identified in approximately two thirds of kindreds with periodic paralysis or paramyotonia congenita. The authors found differences between the disorders and between those with and without identified mutations in terms of age at onset, frequency of attacks, duration of attacks, fixed proximal weakness, precipitants of attacks, myotonia, electrophysiologic studies, serum potassium levels, muscle biopsy, response to potassium administration, and response to treatment with acetazolamide. Hypokalemic periodic paralysis, hyperkalemic periodic paralysis, and paramyotonia congenita may be distinguished based on clinical data. This series of 226 patients (127 kindreds) confirms some clinical features of this disorder with notable exceptions: In this series, patients without mutations had a less typical clinical presentation including an older age at onset, no changes in diet as a precipitant, and absence of vacuolar myopathy on muscle biopsy.

Hypokalaemia and paralysis.

It is not uncommon for patients to present to the emergency room with severe weakness and a markedly low plasma potassium concentration. We attempted to identify useful clues to the diagnosis of hypokalaemic periodic paralysis (HPP), because its acute treatment aims are unique. We retrospectively reviewed charts over a 10-year period: HPP was the initial diagnosis in 97 patients. Mean patient age was 29+/-1.1 and the male:female ratio was 77:20. When the final diagnosis was HPP (n=73), the acid-base state was normal, the urine K(+) concentration was low, and the transtubular K(+) concentration gradient (TTKG) was <3. In patients with thyrotoxic periodic paralysis (TPP) (n=39), hypokalaemia was very commonly accompanied by hypophosphataemia (1.9+/-0.1 mg/dl). A clinical diagnosis of sporadic periodic paralysis (SPP) was made if hyperthyroidism and a family history of HPP were both absent (n=29). One subgroup of patients with HPP had a severe degree of hypernatraemia (167+/-5.0 mmol/l, n=3). There were only two patients with familial periodic paralysis (FPP). In 24 patients, the initial diagnosis was HPP, but subsequent studies failed to confirm this diagnosis. Each of these patients had an acid-base disorder, a high rate of renal K(+) excretion in the presence of hypokalaemia, and a TTKG of close to 7. With respect to therapy, much less K(+) was given to patients with HPP, yet 1:3 subsequently had a plasma K(+) concentration that eventually exceeded 5.0 mmol/l. Using plasma acid-base status, phosphate and K(+) excretion parameters allows a presumptive diagnosis of HPP with more confidence in the emergency room.

Hypokalaemic periodic paralysis (A Case Report)

A case of young (20 years old) mole presenting with dramatic onset of weakness in all the limbs is presented. Patient had three such episodes in the past 2 years lasting for 4-6 hrs. There was no family history of such weakness. A diagnosis of hypokalaemic periodic paralysis was made. Patient was given intravenous potassium, improved considerably and put on acetazolamide prophylaxis. The case is being reported because of its rarity and academic interest.