Title: Potassium Peril: A Rare Case with Review of Literature of Hyperkalemic Paralysis as a presentation of Obstructive Uropathy and CKDAuthor: Dr. Sunidhi Rohatgi 1, Abulkalam A. Sirajwala2, M.D., Patel Rahenuma Y.3,Affiliations: 1 MBBS, Medical College Baroda, India, email id: sunidhirohatgi@gmail.com2 M.D., Medical College Baroda, India, email id: asirajwala263@gmail.com3 MBBS , Government Hospital, Bharuch, India, email id: renupatel2603@gmail.comFunding : Self funded.There is no conflict of interest .This case report has passed through the ethics approval committee of the medical college.Written patient consent was taken.We give our consent to reproduce material from other sources.Correspondence address: 233 Rajeev Gandhi Special, Jhalawar Road, Kota, Rajasthan-324005, India.E Mail id: sunidhirohatgi@gmail.comPhone: +91-8824696633.KEY CLINICAL MESSAGE:Hyperkalemic periodic paralysis is a rare disease, It is divided into two types Primary and secondary. Primary is autosomal dominant caused by mutation in the SCN4A gene that codes for voltage-gated sodium channels Na1.4, which alters the usual structure and function of sodium channels. Secondary hyperkalemic periodic paralysis is caused secondary to chronic renal disease, Addison’s disease, rhabdomyosis, or excessive ingestion of potassium supplements or potassium rich foods such as potatoes or bananas. In the present case, a 55 year old male patient with chronic renal disease and hydronephrosis, hypertension presented with both upper and lower limb weakness mimicking GBS like symptoms, having high potassium levels in blood and high Serum Creatinine and Urea levels with no cardiac arrythmias. HYPP is a rare presentation of hyperkalemia in Chronic Kidney Disease.Keywords:Hyperkalemia, Periodic paralysis, Chronic renal disease, Hydronephrosis.INTRODUCTION-Hyperkalemic Periodic paralysis is an autosomal dominant muscle channelopathy with nearly complete penetrance. It is characterized by recurrent episodes of muscle weakness associated with elevated levels of blood potassium (1,2). It was first discovered by Tyler et al in 1951, in a study of seven generations of individuals with typical periodic paralysis in the absence of hypokalemia(3).HYPP is a rare condition begins in childhood and can continue until middle adulthood or may even last into late adulthood. It presents as muscle weakness, ranging from mild weakness to paralysis. During these episodes, it is common to have higher than normal blood levels in potassium. (4). It is divided into hereditary (Primary) and acquired (secondary) forms.The disease affects approximately 1 in every 2,00,000 individuals. (5).In the present case a male patient 55 years age presented in medicine emergency with bilateral upper and lower limb hemiparesis due to hyperkalemia manifesting as a rare presentation of Chronic Kidney Disease with hydronephrosis and recent history of hypertensionBlood investigation showed raised serum potassium level with raised serum creatinine and urea levels.CASE HISTORYA elderly male patient aged 55 years presented to us in medicine emergency with high grade fever associated with burning micturition since 8 days, easy fatigability, weakness in bilateral lower limbs since 4 days, which increased over course leading to fall at home and which was bilaterally symmetrical and involved proximal muscles. There were also complaints of decrease in appetite, nausea, and vomiting for 4 days. The patient noticed a drop in urine output since last 2 days.Blood pressure level was 160/100 mm Hg at the time of admission.Patient did not have any complaints of breathlessness, altered sleep pattern, hiccups, chest pain, pedal edema, fever, abdominal pain, altered behaviour. Reflexes in Lower extremities were absent. Both upper limb reflexes were decreased grade +1.CNS examination-Higher Functions- normalCranial Nerves- NormalMotor- Power: upper limb- grade 2 ; lower limb- grade 0Involuntary movements- noneSensory: NormalReflexes- upper limb +1: lower limb grade 0 (absent)Autonomic function- NormalCerebellar Function- NormalMeningeal irritation- NoneBack and Spine- NormalPeripheral nerves and vessels- NormalHe was diagnosed with hypertension 7 days before his presentation. Ultrasonography showed size of Right kidney 155 x 80 mm in size, bulky showing gross hydronephrosis with severe thinning of renal parenchyma, thickness being 2.2 mm. Multiple calculi at renal pelvis of average size 10-12 mm were present. Left kidney measured 88 x 45 mm in size, corticomedullary junction being attenuated, tiny calculi at poles of average size 3-4 mm.DIFFERENTIAL DIAGNOSIS:1) Hypokalemic Periodic paralysis, 2) Thyrotoxicosis Periodic paralysis, 3) Andersen-Tawil syndrome. The differentiating features of these are :Hypokalemic Periodic Paralysis: Thers is a defect of the alpha-1 subunit of dihydropyridine-sensitive calcium channels and sodium channel protein type 4 subunit (SCN4A) encoded sodium channels in skeletal muscle. (6). It is the most common presentation of Periodic paralysis. Incidence is 1 in 1,00,000 people. It begins in late childhood with frequency of attacks ranging from weeks to months. Each episode may last several hours to days. Between the attacks Potassium levels are normal, but if it persists between attacks, then it may be due to secondary to renal potassium loss. Electrocardiogram shows increase in amplitude of U waves, decrease in amplitude of T-wave and depression of ST segment. (4,7).Thyrotoxicosis Periodic Paralysis: It may be associated with potassium ion channels defect, mutation in gene encoding Kir 2.6 which is regulated by Thyroid hormones. T3 and T4 increases sensitivity of beta adrenergic stimulation resulting in increased acitivity of Na-K ATPase in the skeletal muscle membrane leading to influx of potassium into the cells and decreased level in serum. (8). Onset is between 20-39 years of age, commonly seen at night or early morning or summer. Symptoms may last for several hours to days, can be triggered by excessive physical activity , stress, high carbohydrate intake, cold exposure, infection, alcohol intake, corticosteroid, bronchodilator, menstrual cycle. (9). Characteristic is hypokalemia, generalized muscle weakness, proximal muscles, lower extremities, myalgia ( 10).Andersen-Tawil syndrome: It is caused by mutation on the Kir 2.1 encoding gene. (11). Patient have a triad of periodic paralysis, Ventricular arrythmias and dysmorphic features (short stature, clinodactyly, hypertelorism, micrognathia). ECG shows prolonged QT interval . Potassium may be low, normal or even high. It is 10 times rarer than hypokalemic periodic paralysis. (11).