The Physicians Library contains scientific works as well as some selected commentary materials from physicians and experts in the Periodic Paralysis community. Links to journal articles will also be provided but may require membership to those associations. Please note, we have just begun the gathering process for materials to be included in this library and look forward to its expansion. This will take time however, but the PPI feels this valuable resource will be most helpful as the “go-to” for all the current information available. If you have an article or material you would like to recommend, please feel free to contact us.
Anesthesia for the PEDIATRIC Patient with Hypokalemic Periodic Paralysis
Anesthesia for the ADULT Patient with Hypokalemic Periodic Paralysis
Anesthesia and Peri-Operative Care in the primary Periodic Paralysis Disorders. Part 1: A Review of the Literature
Anesthesia and Peri-Operative Care in the primary Periodic Paralysis Disorders. PART 2: Practical Guidelines
Practical aspects in the
management of hypokalemic periodic paralysis
Management considerations in hypokalemic periodic paralysis include accurate diagnosis, potassium dosage for acute attacks, choice of diuretic for prophylaxis, identification of triggers, creating a safe physical environment, peri-operative measures, and issues in pregnancy. A positive genetic test in the context of symptoms is the gold standard for diagnosis. Potassium chloride is the favored potassium salt given at 0.5-1.0 mEq/kg for acute attacks. The oral route is favored, but if necessary, a mannitol solvent can be used for intravenous administration. Avoidance of or potassium prophylaxis for common triggers, such as rest after exercise, high carbohydrate meals, and sodium, can prevent attacks. Chronically, acetazolamide, dichlorphenamide, or potassium-sparing diuretics decrease attack frequency and severity but are of little value acutely. Potassium, water, and a telephone should always be at a patient’s bedside, regardless of the presence of weakness. Perioperatively, the patient’s clinical status should be checked frequently. Firm data on the management of periodic paralysis during pregnancy is lacking. Patient support can be found at http://www.periodicparalysis.org.
Characterization of hyperkalemic periodic paralysis: a survey of genetically diagnosed individuals
This exploratory study aims to create an evidence-based comprehensive characterization of hyperkalemic periodic paralysis (hyperPP). HyperPP is a rare genetic disorder that causes episodes of flaccid paralysis. Disease descriptions in the literature are based upon isolated clinical encounters and case reports. We describe the experience of a large cohort of genetically diagnosed individuals with hyperPP. We surveyed genetically characterized individuals age 18 and over to assess disease comorbidities, diagnostic testing, management, and quality of life issues relevant to hyperPP. Myotonia was reported by 55.8 % of subjects and paramyotonia by 45.3 %. There is a relative risk of 3.6 (p < 0.0001) for thyroid dysfunction compared to the general population. Twenty-five percent of subjects experienced their sentinel attack in the second decade of life. It took an average of 19.4 years and visits to four physicians to arrive at the diagnosis of hyperPP. In addition to limbs and hands being affected during attacks, 26.1 % of subjects reported their breathing musculature was affected and 62.0 % reported their facial muscles were affected. There was a lifelong trend of increasing attack frequency, which was particularly common during childhood and adolescence. Approximately one-third of individuals experienced progressive myopathy. Permanent muscle weakness was evident and worsened during childhood and after age 40. Those with no chronic treatment regimen have a RR of 2.3 for inadequate disease control compared to those taking long-term medications. This study revealed a multitude of heretofore unidentified characteristics of hyperPP, in addition to providing a different perspective on some previously held notions regarding the condition.