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.
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:
- Physician’s Sheet for Thyrotoxic Hypokalemic Periodic Paralysis
- Hospital Management Guidelines for Thyrotoxic Periodic Paralysis Patients
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
- Akut behandling af et hypokalemisk paralytisk anfald (Emergency Care for Hypokalemic Attack in Danish)
- Physician’s Sheet for Hypokalemic Periodic Paralysis
- Anesthesia in Hypokalemic Periodic Paralysis
- Hospital Management Guidelines – HypoKPP
- GUIDELINES ved indlæggelse for Hypokalemisk Periodika Paralyse Patient (in Danish)
- Practical Aspects in the management of Hypokalemic Periodic Paralysis
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
- Physician’s Summary for Hyperkalemic Periodic Paralysis
- Physician’s Sheet for Paramyotonia Congenita
- Hospital Management Guideline Form – HyperKPP
Emergency Care for Attacks in Normokalemic Periodic Paralysis
- Normokalaemic periodic paralysis (normoKPP) is characterized by transient and recurrent muscle weakness, and some patients also show muscle stiffness induced by cold exposure. It is caused by a mutation in the muscle voltage-gated sodium channel alpha subunit (SCN4A) gene. Due to the diversity of the clinical manifestations of patients, it is difficult for clinicians to differentiate some of patients with atypical normoKPP from those who suffer from other forms of periodic paralysis.
- Hospital Management Guidelines for Periodic Paralysis Patients
- Malignant Hyperthermia in Sodium Channel Periodic Paralysis (including Normokalemic PP)
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.