Periodic Paralysis and Anesthesia

 Hello All, 

This is an article written and created with a compilation of information, excerpts, references and links related to the problems and issues of the use of anesthesia with individuals with the various forms of Periodic Paralysis. There is quite a bit of technical information, which can be shared with your doctor, anesthesiologist or dentist (though most of them should know this information).

  Periodic Paralysis and Anesthesia

 Article and Compilation

by

The Periodic Paralysis Network (PPN) February 2014

Periodic Paralysis (PP) is a mineral metabolic disorder, also known as an ion channelopathy, which is a dysfunction of the ion channels. There are four types of PP, Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis, Normokalemic Periodic Paralysis and Andersen-Tawil Syndrome. Ion channels transport the electrolytes, such as sodium and potassium through the cells. This transport is faulty in individuals with ion channel dysfunction and extreme care must be used when anesthesia is going to be utilized. This is due to the possibility of developing serious symptoms such as breathing issues or failure, arrhythmia, blood pressure issues, choking, muscle weakness or paralysis, longer recovery after surgery, malignant hyperthermia or death. Managing the use of anesthesia in individuals with Periodic Paralysis is mostly aimed at preventing attacks of paralysis or the other symptoms during or after surgery. The manner in which the situation is handled for the individual depends on which form of Periodic Paralysis is involved. ^{1, 2, 3, 4, 5}

Hypokalemic Periodic Paralysis and Anesthesia

For individuals with Hypokalemic Periodic Paralysis, anesthesia is a known trigger for paralytic episodes. According to research, in order to successfully manage the patient there is need for an evaluation before surgery, avoidance of known triggers, careful monitoring during the surgery and immediate and proper treatment if an issue arises.

 
According to the National Journal of Maxillofacial Surgery, “pre-operative stress along with necessary fasting and administration of dextrose containing fluids” precipitates attacks. “The guidelines for care include control of plasma potassium, avoidance of large glucose and salt loads, maintenance of body temperature, acid-base balance, and careful use of neuromuscular blocking agents. Good pre-medication to allay anxiety, avoidance of stress and adequate analgesia is vital in preventing an attack. Fluctuations in electrolytes, infection, and pain can lead to paralysis in the post-operative period. Hypokalemia manifests earlier than paralysis and so its correction can prevent paralysis. Dextrose containing solutions administered during surgery should be avoided...” ^{6, 7, 8}

This information was found in Miller’s Anesthesia written by Ronald D. Miller:
 
”Management of HypoPP patients should focus on avoiding triggers and medications causing shift of potassium. General anesthesia, postoperative stress, glucose-containing intravenous solutions, and long-acting neuromuscular blockers are associated with postoperative paralytic events.138 Epidural analgesia has been shown to reduce both pain-related hyperventilation and serum catecholamines, thereby minimizing changes in serum potassium levels.138” ⁴

Hyperkalemic Periodic Paralysis and Anesthesia

Nothing was written about the use of anesthesia and Hyperkalemic Periodic Paralysis before 2002. Early research concluded that anesthesia might be used without complications if the potassium levels were with-in normal levels prior to surgery, if the carbohydrate levels were up, if anesthetic drugs, which released potassium, were not used and if normal body temperature levels were maintained.

 This information was found in Miller’s Anesthesia written by Ronald D. Miller:

”Potassium, cholinesterase inhibitors, and depolarizing muscle relaxants will aggravate the myotonia in HyperPP patients.65 Prolonged muscle weaken ss has been reported when succinylcholine is used.141 Although one third of patients had no signs of myotonia,142 masseter spasm and respiratory and skeletal muscles stiffness could still occur during intubation and ventilation.65 Therefore, neostigmine and succinylcholine should be contraindicated in HyperPP patients. Ideally, all patients with HyperPP need to be admitted preoperatively so that proper preoperative fasting can be accompanied by the administration of dextrose-containing potassium-free maintenance fluid.143 Postoperatively, HyperPP patients may remain paralyzed for up to several hours. Preventive measures such as maintaining normal body temperature and low serum potassium levels and avoiding hypoglycemia are helpful in limiting such paralysis.144 Although patients with sodium channel pathology have often been considered to be susceptible to MH, (Malignant Hyperthermia) there is no increased risk for MH in these patients.145 General anesthesia with and without nondepolarizing muscle relaxants has been shown to have satisfactory outcomes.141,143,144,146 Regional techniques may also be appropriate for this patient group.142,146 Abortion of the hyperkalemic attack may be accomplished by administering glucose, insulin, epinephrine, and calcium supplements, or alternatively, glucagon may be used. β-Adrenergic treatment with metaproterenol has also been shown to prevent attacks and facilitate recovery.” ^4,9,10,11

Malignant Hyperthermia (MH)

 

As mentioned previously, individuals with Periodic Paralysis are at risk for developing malignant hyperthermia during or after surgery. All forms of Periodic Paralysis are the result of mutations on Chromosome 17. Malignant hyperthermia is also the result of a mutation on Chromosome 17, thus creating the potential for those with Periodic Paralysis, including, Normokalemic Periodic Paralysis, to develop the serious and life-threatening symptoms involved with the use of anesthesia. ^{12, 13}

 

The following information about MH is an excerpt from an article from CINCH (Consortium for the Clinical Investigation of Neurologic Channelopathies), which may be shared with physicians, dentists and anesthesiologists:

 

Malignant Hyperthermia

“Patients with ion channel diseases are at increased risk of malignant hyperthermia reactions with general anesthesia for surgery. In malignant hyperthermia, muscle cells become overactive (hypermetabolic) in response to the most commonly used drugs for general anesthesia. With inhaled agents (such as halothane, isoflurane, enflurane, desflurane, sevoflurane and even agents such as ether, cyclopropane and methoxyflurane ) the ion channels in the muscle cells open and remain open. This results in excessive calcium release causing muscles to continuously contract, producing massive amounts of heat, rapid rise in temperature and disruption of nearly every organ system in the body. In the past this reaction was fatal in 70% of cases. However, with the introduction of the medication, dantrolene, the mortality has now fallen to 5% of cases. It is essential that anesthetists and surgeons are made aware that a patient has periodic paralysis or even that a parent suffers with the disorder, if a son or daughter is going for surgery. The anesthetists and surgeons should be familiar with neuromuscular disorders and the associated risks. If they are not in a specialist center, the patient may need to be transferred to such a center or at the least the patient's neurologist should be contacted prior to surgery.

Prolonged Paralysis

 

Other medicines used during general surgery are called depolarizing agents (such as Succinylcholine). These medicines act by blocking the signal from the nerve (Acetylcholine) from reaching it's receptor on the muscle. This causes temporary paralysis in patients during surgery, relaxing the muscles to make surgery easier. In patients with ion channel disorders, such as periodic paralysis, this can result in prolonged paralysis.

Normally, when people are given the drug succinylcholine to relax muscles, a little bit of potassium from inside the muscle cells leaks out into the bloodstream. This is because succinylcholine, like acetylcholine, docks on the receptors and opens some gates in the cell, letting certain molecules flow in and out. Normally, this poses no problem. In the periodic paralyses, ion channels that normally open when acetylcholine docks on membrane receptors don't function normally, and people experience temporary paralysis attacks in response to a variety of dietary, exercise, stress-related and other environmental changes.

After surgery, patients with periodic paralysis have found they woke up in the recovery room and couldn't move; they may not get full strength back for hours or days. The exact cause of the prolonged paralysis with anesthesia in some people with periodic paralysis isn't known. It could be the stress of the surgery and/or any of the anesthetic drugs. It is critical for the medical team to distinguish between a periodic paralysis attack and a malignant hyperthermia reaction as the treatments are completely different.”  ^{14, 15, 16}

Important link for more information related to Malignant Hyperthermia"

http://www.mhaus.org/

Andersen-Tawil Syndrome and Anesthesia

Some research indicated that malignant hyperthermia is not usually an issue for individuals with Andersen-Tawil Syndrome. However, it is an issue because individuals with ATS have shifting of potassium into both high and low ranges causing symptoms and paralysis. The other issue with anesthesia use and ATS is a need for special precautions due to the serious issue of the long QT interval heartbeat, a diagnostic marker for the condition and Torsades de Pointes another extremely serious arrhythmia. There are many medications that must be avoided, which are used routinely in preparation for surgery and during surgery including the glucose and sodium IVs, as well as most forms of anesthesia.⁷

General Anesthestic considerations for patients with LQTS Management

From Drexel University:

Avoid triggers of QT prolongation and Torsadesde Pointes (TdP)

Provide Peri-op:

Anxiolysis

β-blockade

Analgesia

Maintain:

Normothermia

Normoxia

Euglycemia

Normocarbia

Avoid hemodynamic extremes:

Bradycardia

Tachycardia

Hypotension

Hypertension

Correct serum electrolytes esp:

Potassium

Magnesium

Prophylaxis beneficial even with normal serum concentrations:

Prevent and treat arrhythmia:

Continue ECG monitoring in more than 1 lead

If ICD/pacemaker, ensure proper functioning

Have defibrillator and temporary pacemaker available

Consult cardiology as needed ¹⁸

More important information available for ATS and Anesthesia at:

http://c.ymcdn.com/sites/www.pana.org/resource/resmgr/Docs/Tekwe,_C_Spring2011.pdf

http://www.ncbi.nlm.nih.gov/books/NBK1264/

http://www.researchgate.net/publication/40446152_Resuscitated_sudden_cardiac_death_in_Andersen-Tawil_syndrome

http://www.docstoc.com/docs/44783177/609085---ANESTHETIC-MANAGEMENT-FOR-CESAREAN-DELIVERY-ANDERSON-TAWIL

http://acibadem.dergisi.org/pdf/pdf_AUD_108.pdf

http://translate.google.com/translate?hl=en&sl=tr&u=http://acibadem.dergisi.org/pdf.php3%3Fid%3D108&prev=/search%3Fq%3DAnesthesia%2Band%2BAndersen-Tawil%2BSyndrome%26start%3D30%26client%3Dfirefox-a%26sa%3DN%26rls%3Dorg.mozilla:en-US:official%26channel%3Dsb%26biw%3D1138%26bih%3D508

Lidocaine
 
Topical, regional and local anesthesia may cause potassium to drop in individuals with Hypokalemic Periodic Paralysis, Normokalemic Periodic Paralysis or Andersen-Tawil Syndrome if it contains epinephrine. The most often discussed and utilized local anesthesia is lidocaine. For some individuals it may work well if the epinephrine is removed. For others it may cause hypokalemia or arrhythmia regardless of the epinephrine being removed. For others still, it may not work at all or the usual amount may be needed during a procedure. Lidocaine and other local types of anesthesia need to be used with extreme caution.⁴

In Summary

“Although neuromuscular diseases are relatively uncommon, patients with these conditions will present to the operating room and to non-operating room procedure areas for diagnostic studies, treatment of complications, or surgical management of related or unrelated disorders. Overall debility, with diminished respiratory muscle strength and increased sensitivity to neuromuscular blockers (NMBs), predisposes these patients to postoperative ventilatory failure and pulmonary aspiration, and may slow their post-procedure recovery because of difficulty with ambulation and increased risk of falling. A basic understanding of the major disorders and their potential interaction with anesthetic agents is necessary to minimize the risk of perioperative morbidity.”¹⁹

”The anesthetic management of patients with periodic paralysis first involves knowing the patient's history and their particular disease characteristics. The concurrent diseases must be ruled out (such as Andersen's disease). The primary goal of the anesthetic is to avoid events (perioperatively) that are known to precipitate muscle weakness. Electrolytes should be normalized, hypothermia should be avoided and frequent monitoring of the serum potassium level is indicated. The ECG should be constantly monitored for signs of arrthymias. These patients can be considered at risk of MH, thus avoidance of MH triggers is indicated. Use of nondepolarizing muscle relaxants is thought to be acceptable, although abnormal sensitivity to these agents may be encountered and adequate muscle strength must be assured prior to extubation.” ²⁰

Anyone with Periodic Paralysis needs to be extremely cautious when planning any surgical procedures, which may use anesthesia.

References and Links

 

Periodic Paralysis general info about care with meds and treatment

1.http://en.wikipedia.org/wiki/Hypokalemic_periodic_paralysis

2.http://en.wikipedia.org/wiki/Ion_channel

3.http://rarediseasesnetwork.epi.usf.edu/cinch/learnmore/faqs.htm

 Pre-operative

4.https://www.inkling.com/read/millers-anesthesia-7th/chapter-37/periodic-paralysis-hyperkalemic

Hypokalemic Periodic Paralysis

5.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3700163/

6.http://www.njms.in/article.asp?issn=0975-5950;year=2012;volume=3;issue=2;spage=220;epage=221;aulast=Abbas

7.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112/

8.https://www.google.com/search?q=Periodic+Paralysis+and+anesthesia&ie=utf-8&oe=utf-8&aq=t&rls=org.mozilla:en-US:official&client=firefox-a&channel=sb#channel=sb&q=Periodic+Paralysis+and+anesthesia&rls=org.mozilla:en-US:official&start=10

Hyperkalemic Periodic Paralysis

9.http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2044.1992.tb02327.x/pdf

10.http://journals.lww.com/anesthesiology/pages/articleviewer.aspx?year=2002&issue=07000&article=00033&type=Fulltext

11.https://www.inkling.com/read/millers-anesthesia-7th/chapter-37/periodic-paralysis-hyperkalemic

Malignent Hyperthermia

12.http://www.mountsinai.org/patient-care/health-library/diseases-and-conditions/periodic-paralysis-syndrome

13.http://www.ncbi.nlm.nih.gov/books/NBK1146/

14.http://en.wikipedia.org/wiki/Malignant_hyperthermia

15.http://www.mhaus.org/

16.http://www.nlm.nih.gov/medlineplus/ency/article/001315.htm

Andersen-Tawil Syndrome

17.http://www.ncbi.nlm.nih.gov/books/NBK1264/

18.http://c.ymcdn.com/sites/www.pana.org/resource/resmgr/Docs/Tekwe,_C_Spring2011.pdf

 

Conclusion
19.http://accessanesthesiology.mhmedical.com/content.aspx?bookid=564&sectionid=42800567&jumpsectionID=42807020

20.http://www.anesthesiaweb.com/protected/archive/ask/archive_ask_specific.shtml

Until later...

More links: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112

Added September 4, 2015:PROLONGED PARALYSIS

Other medicines used during general surgery are called depolarizing agents (such as Succinylcholine). These medicines act by blocking the signal from the nerve (Acetylcholine) from reaching it's receptor on the muscle. This causes temporary paralysis in patients during surgery, relaxing the muscles to make surgery easier. In patients with ion channel disorders, such as periodic paralysis, this can result in prolonged paralysis...

..After surgery, patients with periodic paralysis have found they woke up in the recovery room and couldn't move; they may not get full strength back for hours or days. The exact cause of the prolonged paralysis with anesthesia in some people with periodic paralysis isn't known. It could be the stress of the surgery and/or any of the anesthetic drugs. It is critical for the medical team to distinguish between a periodic paralysis attack and a malignant hyperthermia reaction as the treatments are completely different.
https://www.rarediseasesnetwork.org/cinch/learnmore/faqs.htm
http://www.mhaus.org/.../be-prepared/associated-conditions
http://www.ncbi.nlm.nih.gov/pubmed/11870726
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2900112/


Added February 25, 2017

Advances in Anesthesiology
Volume 2015 (2015), Article ID 562378, 6 pageshttp://dx.doi.org/10.1155/2015/562378

Review Article

 Mutations in Sodium Channel Gene SCN9A and the Pain Perception Disorders

https://www.hindawi.com/archive/2015/562378/