*Emergency & Hospital Management Info

Emergency and Hospital Management Care Information for patients and physicians. Below are links to resources which can be read and downloaded. A few apply to all the forms of periodic paralysis, such as anesthesia and surgical considerations, most are organized by diagnosis. A couple are available in Danish. 

Emergency Care for Attacks of Thyrotoxic Hypokalemic Periodic Paralysis

Patients with Thyrotoxic Hypokalemic Periodic Paralysis typically present at the ER with an acute episode of paralysis involving the muscles of the extremities and limb girdles caused by hyperthyroidism. This is a true endocrine emergency. Respiratory and cardiac arrest are possible.  READ MORE: 

Emergency Care for Attacks of Hypokalemic Periodic Paralysis

Paralytic attacks of Hypokalemic Periodic Paralysis can cause cardiac and/or respiratory distress or arrest.  Patients may become unable to cough and may choke on their own secretions or on vomit. Attacks of profound paralysis or ones in which there is cardiac or respiratory distress require immediate emergency medical attention. READ MORE

Emergency Care for Attacks in Andersen-Tawil Syndrome

Andersen-Tawil syndrome (ATS) is characterized by the triad of periodic paralysis, distinctive dysmorphic features, ventricular arrhythmias and prolonged QT interval. Patients with ATS may present to the ER with an attack of weakness/paralysis and/or cardiac rhythm disturbances or prolonged QT interval. Uncommonly they may come to the ER after an injury due to syncope, or very rarely, due to cardiac arrest. READ MORE

Emergency Care for Attacks in Hyperkalemic Periodic Paralysis and Paramyotonia Congenita

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 arrhythmia, or myotonia may interfere with respiration.  Attacks of weakness in Paramytonia Congenita are usually mild to moderate in severity, but myotonia of chest, diaphragm and throat muscles can become life-threatening. READ MORE

Anesthesia in Hyperkalemic Periodic Paralysis and Paramyotonia Congenita

Emergency Care for Attacks in Normokalemic Periodic Paralysis

Malignant Hyperthermia during surgery is a danger in the Periodic Paralyses, especially to those with Sodium Channel mutations. Check out a video about Malignant Hyperthermia here: What I wish Everyone Knew about Malignant Hyperthermia

Anaesthesia and peri-operative care in the primary Periodic Paralysis Disorders

Adverse reactions to anaesthesia, including life-threatening events, are a serious concern for patients with Periodic Paralysis Disorders. Many of our patients report serious events associated with anaesthesia and side effects which may linger for days to months. There is rarely time for a surgical team to comb through journals and texts for the specialized knowledge needed to care for the patient with periodic paralysis. Malignant Hyperthermia is also a risk, especially for patients with sodium channel mutations. 

In order to make this information easier to find retired PPI Medical Board Member Haemato-oncologist and ATS patient Dr. Annabelle Baughan, PPI Director Deb Cavel-Greant, HKPP Listserv member Jan Magalo, and Neurologist Dr. Frank Weber have prepared both a Literature Review and a set of Practical Guidelines physicians may consult when caring for a patient with one of the Periodic Paralyses.

Part 1: A Review of the Literature

Part 2: Practical Guidelines