Hypokalemic Paralysis Research Articles

Sjögren's, Renal Tubular Acidosis and Osteomalacia - An Asian Indian Series.

Objective: To study the profile of Renal Tubular Acidosis (RTA) in Asian Indian patients with Primary Sjogren's Syndrome (pSS). Methods: The Electronic medical records of patients with a diagnosis of pSS seen between 2003 and 2010 at our tertiary care teaching hospital were screened for RTA. Clinical features, immunological profile, acid-base balance and electrolyte status, 25-hydroxyvitamin D (25(OH) D3) levels, histopathological changes in minor salivary gland biopsy samples and radiological findings were retrieved. RTA was diagnosed in cases of hyperchloremic metabolic acidosis with urinary pH values higher than 5.5. Those with known features suggestive of RTA including hypokalemic paralysis, hyperchloremia and nephrocalcinosis without acidosis were defined as incomplete RTA. Results: Of the 380 patients with clinically suspected pSS, 25 had RTA. The median age was 32 (18-60) years. Nineteen patients had complete RTA. Six had incomplete RTA. Only 10 patients (40%) had symptoms related to RTA at presentation. Sixteen patients (64%) had present or past history of hypokalemic paralysis. Pseudofractures were seen in 7 patients and an additional 2 had subclinical radiological osteomalacia. Majority of the patients (61.2%) had a normal 25(OH) D3 level. Those with osteomalacia had significantly lower serum phosphate, blood ph and higher alkaline phosphatase. Serum calcium and 25(OH) D3 levels were not significantly different between patients with osteomalacia and those without. Conclusion: Most patients were asymptomatic for RTA inspite of clinically overt and elicitable features. Skeletal manifestation was a common finding in patients with Sjogren and RTA, despite normal levels of 25 (OH) D3 in a majority.

Isolated Lower motor neuron type facial nerve palsy: An unusual manifestation of Dengue haemorragic fever

Dengue is a febrile illness caused by infection with one of four dengue viruses (DENV). It is highly endemic in many tropical countries. Nervous system involvement is seen with serotypes 2 and 3. Neurological manifestations in dengue fever are rare and include encephalopathy, meningitis, acute pure motor weakness, mononeuropathies, transverse myelitis, stroke (both hemorrhagic and ischemic), acute disseminated encephalomyelitis, Guillain Barre Syndrome, hypokalemic paralysis and neuromyelitis optica. Only a few cases of isolated facial nerve palsy in dengue haemorragic fever has been reported.

PRIMARY ALDOSTERONISM AND PREGNANCY

Objective: Primary aldosteronism (PA) may present in younger age and it may so complicate pregnancy if not diagnosed early. Our aim was to identify female patients in whom PA was diagnosed after pregnancy and to seek for possible complications during pregnancy. Design and method: Retrospective analysis of female patients with PA diagnosed and treated at our Department in the period from 2000 to 2017. Results: We found 12 patients with PA (age at diagnosis 32.4 ± 4.6 years, hypertension duration 5.4 ± 3.1 years) suffering from hypertension 2.4 ± 1.7 years before pregnancy (5 patients had hypertension diagnosed during pregnancy). Three subjects were pregnant twice before the final diagnosis of PA was made. In 8 cases, pregnancy was terminated with caesarean section (3 times due to preterm preeclampsia and twice due to significantly increased blood pressure) and in 7 cases by spontaneous delivery (twice due to term preeclampsia). Preterm delivery occurred in 5 cases – the earliest one in the sixth month of gestation (4 times due to blood pressure related complications). Subsequent diagnosis of PA [(sometimes with a long delay (12 years)] was made on the basis of significantly low potassium values (2.6 ± 0.4 mmol/l; 2 subjects suffered even from hypokalemic paralysis) and hypertension (mostly moderate), elevated plasma/serum aldosterone (57.4 ± 19.4 ng/dl) and suppressed plasma renin activity (0.33 ± 0.1 ng/ml/h) or plasma renin 3.25 ± 1 ng/l). Eleven subjects underwent laparoscopic adrenalectomy [in all cases, diagnosis of a larger cortical adenoma (16.5 ± 5.7 mm) was made] and one subject was classified with bilateral hyperplasia according adrenal venous sampling. Operation has normalized blood pressure in 8 subjects and has improved significantly blood pressure control in remaining 3 subjects. One patient became pregnant after adrenalectomy and her pregnancy went uneventful. Conclusions: Primary aldosteronism is associated with high rate of pregnancy-related complications. The most frequent one is preeclampsia, in some cases leading to preterm delivery. The best prevention of these complications is the early diagnosis of PA, in these particular hypertensive cases the awareness of hypokalemia.

S45. To report abnormal nerve conduction studies in two patients with hypokalemic paralysis

Introduction In hypokalemic paralysis, hypokalemia-induced inactivity of the muscle fibre Na+K+ ATPase pump is supposed to be responsible for motor inexcitability, resulting reduced or non-recordable CMAP amplitudes. Hence, nerve conduction studies (NCS) mainly includes reversible reduction in compound muscle action potential (CMAP) amplitude. Other NCS abnormalities are very uncommon and suggest more widespread channel abnormality. We report two patients with hypokalemic paralysis with reversible NCS abnormalities. Methods Sensory-motor NCS of all four limbs (bilateral median, ulnar, tibial, peroneal and sural nerves) and other relevant blood investigations were performed in two young adult male patients during first episode of reversible quadriparesis. NCS was repeated 24 h after improvement of motor power. Secondary causes of hypokalemia were excluded by relevant investigations. Results Serum potassium (K+) was low ( Conclusion CMAP and F wave may be abnormal during hypokalemic paralysis due to abnormal Na+K+ ATPase at muscle fibres. However,other NCS abnormalities like abnormal SNAPs, prolonged DL, or non recordable proximal CMAP (specially when distal CMAP is recordable) are rarely reported in hypokalemic paralysis. These abnormalities are marker of nerve fibre abnormality and supposed to be normal in muscle disease. These abnormal and reversible NCS in present case series suggests that Na+K+ ATPase abnormalities are not restricted to muscle fibres but probably more widespread and involve nerve fibres also. However, larger study is required to evaluate this aspect of hypokalemic paralysis.

Hypokalemic Quadriparesis Associated with Dengue: A Case Series

Dengue is an important viral cause of febrile illness in tropical and subtropical regions. Manifestations may range from an asymptomatic infection to life threatening hemorrhagic fever and shock syndrome. Neurological presentations of this disease are rare. Here, we are presenting a case series of three confirmed cases of dengue fever with hypokalemic paralysis presenting as acute pure motor reversible quadriparesis. A clinician should keep dengue virus associated hypokalemic paralysis in mind while dealing with a case of fever with quadriparesis.

Etiological Search and Epidemiological Profile in Patients Presenting with Hypokalemic Paresis: An Observational Study.

Introduction:Hypokalemia is associated with increased morbidity and at times mortality. “Hypokalemic paralysis”, particularly if recurrent, has often been considered synonymous with “hypokalemic periodic paralysis (HPP)”; however, diseases such as Gitelman syndrome (GS), Bartter syndrome (BS), and renal tubular acidosis (RTA) can have identical presentation. We have tried to explore the etiological spectrum along with epidemiological and certain clinical, biochemical, and electrophysiological features in patients with hypokalemic paralysis.Materials and Methods:In this observational study, 200 appropriate patients with hypokalemic paralysis (serum K+ <3.5 mmol/L) were evaluated for transcellular shift, extra-renal or renal loss of K+ as the underlying etiology of hypokalemia. We took urinary potassium >25 mmol/day as the cutoff for inappropriate renal loss of potassium in presence of hypokalemia. Serum and urinary osmolality along with arterial blood gas analysis were performed in all patients with renal loss of potassium. Serum and urinary sodium, potassium, calcium, magnesium, chloride, and creatinine were measured in normotensive patients with metabolic alkalosis. Hypertensive patients were evaluated with plasma aldosterone and renin activity.Results:Probable GS topped the list involving 28% individuals of the entire cohort while probable BS, distal RTA, and HPP were diagnosed in 20%, 22%, and 19% cases, respectively. Rural tribal population (61%) and age group of 30–40 years suffered the most (48%) with concentration of cases in hot and humid summer months.Conclusions:We suggest that patients with hypokalemic paresis should be evaluated thoroughly to unmask the underlying etiology that may have a different therapeutic and prognostic connotations and not to use the term “periodic” in cases of recurrent hypokalemic paralysis.

Spectrum of Hypokalemic Paralysis from a Tertiary Care Center in India.

Hypokalemic paralysis is an important and reversible cause of acute flaccid paralysis. The treating physician faces unique diagnostic and therapeutic challenges. We did a prospective study and included all patients with acute flaccid weakness and documented serum potassium of <3.5 mEq/L during the period between January 2009 and August 2015. We studied the incidence, etiology, clinical profile, and acid–base disturbances in patients presenting with hypokalemic paralysis and analyzed the significance of periodic and non-periodic forms of hypokalemic paralysis on patient's outcome. Two hundred and six patients were studied with a mean follow-up of 3.6 ± 1.2 years. Mean age was 37.61 ± 2.2 years (range 18–50 years). Males were predominant (M:F ratio 2.1:1). The nonperiodic form of hypokalemic paralysis was the most common (61%). Eighty-one (39%) patients had metabolic acidosis, 78 (38%) had normal acid–base status, and 47 (23%) patients had metabolic alkalosis. The most common secondary cause was distal renal tubular acidosis (RTA) (n = 75, 36%), followed by Gitelman syndrome (n = 39, 18%), thyrotoxic paralysis (n = 8, 4%), hyperaldosteronism (n = 7, 3%), and proximal RTA (n = 6, 4%). Patients with non-periodic paralysis had more urinary loss (40.1 vs. 12.2 mmol, P = 0.04), more requirement of potassium replacement (120 vs. 48 mmol, P = 0.05), and longer recovery time of weakness (48.1 vs. 16.5 h, P = 0.05) than patients with periodic paralysis. Non-periodic form of hypokalemic paralysis was the most common variant in our study. Patients with periodic paralysis had significant incidence of rebound hyperkalemia.

Complete Hypokalemic Quadriparesis as a First Presentation of Sjögren Syndrome.

Rationale:We hope to increase awareness that hypokalemic paralysis may be the first presentation of Sjögren syndrome, for which potassium-sparing diuretics can be an effective adjunct to potassium replenishment.Presenting concerns:A 73-year-old female presented to a peripheral hospital with quadriparesis and a critically low serum potassium of 1.6 mmol/L with U waves on the electrocardiogram (ECG). The initial arterial blood gas showed a pH of 7.19, bicarbonate of 13 mEq/L, and a CO2 of 35 mm Hg. Over the next 6 days, she was administered a total of 450 mEq of potassium supplements. Despite this, her potassium never increased above 2.9 mmol/L and was thus transferred to the University Hospital for further management. On arrival, her vital signs were within normal limits. Her only other symptoms were fatigue and ocular dryness. Physical exam showed slightly weakened quadriceps muscles bilaterally, graded 4/5. Examination was otherwise unremarkable. Admission investigations included a potassium of 2.8 mmol/L, chloride 118 mmol/L, sodium 136 mmol/L, and eGFR 48 mL/min/1.73 m2. Renin aldosterone ratio was normal.Diagnoses:Distal renal tubular acidosis (RTA) was diagnosed based on a normal anion gap metabolic acidosis, positive urine anion gap, and elevated urine potassium to creatinine ratio. Investigation of underlying causes revealed a positive Antinuclear antibody (ANA), elevated rheumatoid factor, and high anti-Ro/SSA titre which directed us toward a unifying diagnosis of Sjögren syndrome. A renal biopsy was undertaken as an outpatient and demonstrated severe interstitial nephritis with acute and chronic components, parenchymal scarring, atrophy, and small vessel arteriosclerosis.Interventions:In the acute setting, the patient was treated with bicarbonate and amiloride in addition to potassium supplementation.Outcomes:The patient’s hypokalemic paralysis and metabolic acidosis were corrected.Lessons Learned:Severe hypokalemic paralysis in distal RTA associated with Sjögren syndrome can be successfully treated with amiloride in addition to potassium supplementation. We also review the literature on the aberrancies seen in H+ATPase, Band 3, Pendrin, and carbonic anhydrase that may underlie the pathogenesis of distal RTA in Sjögren syndrome.

Renal manifestations of primary Sjogren's syndrome

Background and Objective: Sjogren's syndrome represents a group of diseases characterized by inflammation and destruction of exocrine glands. Renal involvement in Sjogren's syndrome has been reported in 18.4%–67% of patients. Distal renal tubular acidosis (RTA) was reported more common than proximal RTA which can present as acute hypokalemic paralysis. The aim of the present study was to investigate the clinical picture and the outcome in patients with primary Sjogren's syndrome (PSS) with significant renal involvement. Materials and Methods: We conducted a retrospective study of PSS patients with significant renal involvement in our department. Results: A total of 27 patients were included in the study. The mean age was 39.3 ± 4.5 with a mean follow-up of 42 ± 4.8 months. About 18 patients had hypokalemia associated with paralysis, and 4 patients had hypokalemia without paralysis. Female: male ratio was 12.5:1. Number episodes of hypokalemic paralysis range from 1 to 4. The mean time of recovery was 20.4 ± 4 h, and mean potassium requirement was 190 ± 45 mEq intravenously. The mean serum potassium was 2.51 ± 0.55, mean urinary potassium 32 ± 2.3 mEq/day, mean serum bicarbonate 15.03 ± 1.05 mEq/l, and mean blood pH 7.28 ± 0.9. About twenty had distal RTA, and two had proximal RTA. The clinical symptoms were myalgia in 24 (88%), ocular symptoms in 20 (74%), muscular paralysis (66%), oral in 18 (66%), and dental caries in 12 (44%). Five patients underwent renal biopsy of which two showed acute interstitial nephritis, one showed chronic interstitial nephritis, one showed membranoproliferative glomerulonephritis, and one acute tubular injury. Conclusion: The clinical implication of our study is that distal RTA is a common feature of Sjogren's syndrome presenting predominantly as hypokalemic paralysis.

Acute dengue hypokalemic paralysis complicated by hypomagensemia

Endemic to over 100 countries dengue virus infection has now become the most rapidly spreading mosquito-borne disease in the world. Dengue symptoms range from minor flu-like symptoms to major complications like bleeding, shock, and rarely death due to organ failure. Atypical manifestations of neurological, cardiovascular, gastrointestinal origin are collectively grouped into the expanded dengue syndrome. Acute motor quadriparesis have been reported in several cases of dengue infection. We report a rare and non-fatal case of motor quadriparesis due to hypokalemia complicated by associated hypomagnesemia in a dengue positive patient.

Single-incision laparoscopic adrenalectomy in a patient with acute hypokalemic paralysis due to primary hyperaldosteronism.

Acute hypokalemic paralysis is a relatively rare cause of acute weakness. It may resolve spontaneously; however, it may be a potential life-threatening condition. Hypertension may be considered the most important finding in combination with hypokalemic paralysis for raising the suspicion of primary hyperaldosteronism (PHA). A 55-year-old hypertensive Mexican woman was admitted to the Emergency Unit with a sudden onset of generalized paralysis. An endocrinological workup and an abdominal magnetic resonance imaging revealed PHA with a 1.5 cm left adrenal tumor. After preoperative medication, left adrenalectomy was performed with single-incision laparoscopic surgery (SILS). The duration of the surgery was 45 min, and no postoperative complication was encountered. The patient was discharged after 24 h. Hypokalemic paralysis may be due to different conditions, but it may raise the suspicion of PHA in combination with a history of generally mild hypertension. Laparoscopic adrenalectomy is the preferred operation for unilateral adrenal adenomas that cause PHA. Single-incision laparoscopic surgery is a step-forward technique that improves the cosmesis, decreases access-related morbidity, and increases the postoperative recovery. We report a case with acute hypokalemic paralysis due to PHA and treated with SILS.

Review of the Diagnosis and Treatment of Periodic Paralysis.

ABSTRACTPeriodic paralyses (PPs) are rare neuromuscular disorders caused by mutations in skeletal muscle sodium, calcium, and potassium channel genes. PPs include hypokalemic paralysis, hyperkalemic paralysis, and Andersen‐Tawil syndrome. Common features of PP include autosomal dominant inheritance, onset typically in the first or second decades, episodic attacks of flaccid weakness, which are often triggered by diet or rest after exercise. Diagnosis is based on the characteristic clinic presentation then confirmed by genetic testing. In the absence of an identified genetic mutation, documented low or high potassium levels during attacks or a decrement on long exercise testing support diagnosis. The treatment approach should include both management of acute attacks and prevention of attacks. Treatments include behavioral interventions directed at avoidance of triggers, modification of potassium levels, diuretics, and carbonic anhydrase inhibitors. Muscle Nerve 57: 522–530, 2018

A child with distal (type 1) renal tubular acidosis presenting with progressive gross motor developmental regression and acute paralysis

BackgroundDistal (Type 1) renal tubular acidosis (dRTA) is characterized by inability to secrete hydrogen irons from the distal tubule. The aetiology of dRTA is diverse and can be either inherited or acquired. Common clinical presentations of dRTA in the paediatric age group include polyuria, nocturia, failure to thrive, constipation, abnormal breathing and nephrolithiasis. Though persistent hypokalemia is frequently seen in dRTA, hypokalemic muscular paralysis is uncommon and rarely described in children.Case presentationThree and a half years old girl was referred for evaluation of progressive loss of gross motor milestones over 6 months and acute episode of paralysis. Her other developmental domains were age appropriate. Notably, there was no history of polyuria, polydipsia, nocturia and abnormal breathing. Physical examination revealed proximal myopathy (waddling gait and positive Gower’s sign), diminished lower limb reflexes and muscle tone. Her serum potassium was low (2.1 meq/l) and she was subsequently investigated for hypokalemic paralysis. Diagnosis of distal renal tubular acidosis was made, based on hypokalemic hyperchloremic metabolic acidosis with normal anion gap, high urine pH, borderline hypercalciuria, medullary nephrocalcinosis and exclusion of other differential diagnosis. The child showed complete symptomatic recovery upon commencement of standard treatment for distal renal tubular acidosis.ConclusionsThis case report highlights the importance of considering hypokalemia and renal tubular acidosis in the differential diagnosis of acute flaccid paralysis and proximal myopathy. Early diagnosis will prevent costly investigations and enable rapid clinical recovery in the affected child.

Accidental intravenous bolus infusion of potassium chloride in a young man with hypokalemic periodic paralysis

Hypokalemic periodic paralysis is anautosomal dominantdisease characterized by muscle weakness or paralysis with a matching fall in blood potassium levels. Paralysis attacks often occur in adolescence and are induced by strenuous exercise followed by rest, high carbohydrateor high sodiummeal content, sudden changes in temperature, and even excitement, noise, flashing lights and cold temperatures. Despite global daily use of intravenous and oral potassium in the treatment of patients, data about intravenous bolus potassium is rare. Here we report a 29-year-old man complaining of weakness in the upper and lower limbs. By initial diagnosis of periodic hypokalemic paralysis, potassium chloride was ordered by oral route. After a few minutes, we heard patient screaming in pain. He complained of severe acute pain in peripheral intravenous line, palpitation and dyspnea. We noticed that potassium chloride vial was infused directly via cubital vein in few minutes erroneously. Physical exam revealed sinus tachycardia and hypertension. We began hyperkalemia treatment immediately. With appropriate treatment,the patient was discharged with good condition after 12 hours of admission.

Ozone therapy in familial hypokalemic myopathy

We report the case of a 56-year-old male with a familial hypokalemic paralysis suffering from progressive myopathy of the lower limbs. While episodes of paralysis were well controlled with oral potassium supplementation, muscle weakness in the lower limbs gave little response to rehabilitative therapies. The patient underwent a series of treatments of ozone therapy alternated with large autohemoinfusion (GAEI) and rectal insufflation twice a week; GAEI was then maintained once a month. The muscle strength of the various muscle segments was evaluated during physiotherapy. A significant improvement in musculoskeletal strength and therefore in the response to rehabilitation treatments has been observed. Oxygen-ozone therapy appears to be effective in increasing muscle strength in patients with genetic alterations of the potassium and calcium channels, thus making physiotherapeutic rehabilitation more effective.

Clinical, biochemical and electrophysiological evaluation of hypokalemic paralysis – A retrospective analysis

Clinical, biochemical and electrophysiological evaluation of hypokalemic paralysis – A retrospective analysis

Severe hypokalaemia as a cause of acute transient quadriparesis

Hypokalaemic paralysis covers a heterogeneous group of disorders caused either by an enhanced shift of potassium into the cells or following a significant renal or gastrointestinal loss of potassium. We...

Association of hypokalemic periodic paralysis with cystic Kidney disease- A case report

Hypokalemic periodic paralysis (HPP) is a rare heterogenous neuromuscular disorder presenting with acute weakness. Though most cases are known to be familial or primary there are other underlying secondary causes which require evaluation and if not corrected could lead to recurrent episodes.Distal renal tubular acidosis (RTA) is one such association which has been described. Type 1/Distal RTA is known to cause renal potassium wasting thereby leading to hypokalemia. Patients with Distal RT A are unable to acidify their urine. The causes of Distal RTA can be hereditary, congenital, acquired or idiopathic. Severe hypokalemia with RTA has been described along with Medullary sponge Kidney and other cystic lesions in the kidney. There have been previous reports of hypokalemic paralysis occurring secondary to RT A precipitated by cystic kidney disease. We hereby report one such of hypokalemic periodic paralysis secondary to distal RTA which was associated with cystic kidney disease.Asian Journal of Medical Sciences Vol.8(4) 2017 57-59

Abstract #520: Primary Hyperparathyroidism Presenting with Hypokalemic Paralysis

Abstract #520: Primary Hyperparathyroidism Presenting with Hypokalemic Paralysis

A rare cause of thyrotoxic periodic paralysis: liquorice consumption

Thyrotoxic periodic paralysis (TPP) is the most common form of acquired hypokalaemic periodic paralysis. The most common precipitating factors of the disease are a high-carbohydrate diet and exercise.1 2 Some...