Carbonic Anhydrase InhibitorsDichlorphenamide is an FDA approved carbonic anhydrase inhibitor used for primary hypo and hyperforms of periodic paralysis. The exact mechanism of action for these conditions is unknown. It isavailable as the brand name Keveyis from Xeris pharmaceutical. It has also recently been made availablein a generic form. For the brand name Keveyis, Xeris…
Physician’s Summary Clinical Signs Hyperkalemic Periodic Paralysis is a genetically determined disorder which causes episodes of weakness and flaccid paralysis of the limbs. The muscles of the eyes, throat, and trunk may also be affected. Episodes last from minutes to hours, very rarely up to 1-2 days. Some patients experience only a few episodes over…
Patients with Hyperkalemic PP (HyperKPP) develop paralysis or weakness (and sometimes muscle stiffness) when they eat potassium-rich food. This is also referred to as ‘potassium sensitive’ periodic paralysis. This is usually called Hyperkalemic Periodic Paralysis because most patients’ potassium levels rise during attacks. HyperKPP is due to a mutation in the sodium channel. Hyperkalemic Periodic…
Emergency Treatment of the HyperKalemic Periodic Paralysis and/or Paramyotonia Congenita Attack First Actions: Hyperkalemic Periodic Paralysis and Paramyotonia Congenita can occur singly or in combination. While most attacks of Hyperkalemic Periodic Paralysis are brief and do not require emergency intervention, occasionally the serum potassium level will be high enough to cause cardiac distress, or muscle stiffness may…
Submitted by deb on Tue, 06/28/2011 – 02:56 The Importance to Physicians of Support Groups for Rare Conditions The Genetic Drift Vol. 8: Winter, 1992 Consumer Issues in Genetics Services by Jack Dolcourt, M.D. Neonatologist Support groups have traditionally helped fill the void between the medical system and the daily grind of the everyday world. Support groups…
Submitted by deb on Wed, 07/20/2011 – 19:51 Communication is key: Stephen Wilkins, MPH, writes of improving practice management through improved patient/physician communication and its impact on care, in Mind the Gap. Practical aspects in the management of hypokalemic periodic paralysis. Author: Jacob O Levitt MD; Management considerations in hypokalemic periodic paralysis include accurate diagnosis, potassium dosage for acute attacks,…
Submitted by deb on Fri, 01/16/2015 – 00:37 Ding Z, Liu M, Cui L. Reference value of long-time exercise test in the diagnosis of primary periodic paralysis. Chin Med J (Engl). 2014;127(18):3219-23. Abstract BACKGROUND:The long-time exercise test (ET) is used to diagnose the primary periodic paralyses (PPs). However the reference values of ET are many and various….
Submitted by deb on Sun, 11/09/2014 – 21:46 Dichlorphenamide: A Review in Primary Periodic Paralyses. Greig SL; Drugs. 2016 Mar;76(4):501-7. doi: 10.1007/s40265-016-0559-2. Abstract Oral dichlorphenamide (Keveyis™) is a carbonic anhydrase inhibitor that is approved in the USA for the treatment of primary hyperkalaemic and hypokalaemic periodic paralyses and related variants. The efficacy and safety of dichlorphenamide in…
Submitted by deb on Mon, 06/27/2011 – 00:04 Part Three Case Studies – HyperKPP and PMC Patient #11 Two Pregnancies: Familial HyperKPP and PMC Von Eulenburg Symptoms began at age of 11 years Are your symptoms worse with your menses? No Pregnancy No 1: Age 27 Diagnosed at time of this pregnancy: No Episodes consisted of moderate…
Submitted by deb on Mon, 06/27/2011 – 20:16 Patients with Sodium channel HyperKPP and PMC mutations are at increased risk of Malignant Hyperthermia; It has long been recognized that patients with hyperkalemic periodic paralysis are at increased risk for Malignant Hyperthermia (MH) during surgery, showing as fulminant muscle rigidity as seen in the jaw muscles, rhabdomyolysis, hypercarbia, and…