Emergency Care

Anesthesia in Hypokalemic Periodic Paralysis

Provided by Frank Lehmann-Horn MD, PhD

From
Lehmann-Horn F, Rüdel R, Jurkat-Rott K. Chapter 46: Nondystrophic myotonias and periodic paralyses. In: Myology, edited by AG Engel, C Franzini-Armstrong. McGraw-Hill, New York, 3rd edition, 2004, pp. 1257-1300.
and
Klingler W, Lehmann-Horn, Jurkat-Rott K. Complications of anesthesia in neuromuscular disorders. Neuromuscular Disord, 15:195-206, 2005.

Emergency Treatment of Thyrotoxic Hypokalemic Periodic Paralysis

 

A True Endocrine Emergency

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. The lower limbs are more frequently and severely involved than the upper. Weakness may be asymmetrical. Proximal strength is more severely impaired than distal strength. Respiratory and cardiac arrest are possible. Lie patient in coma position to avoid aspiration. 

Cardiac Signs

Emergency Treatment of the HyperKPP and/or PMC Attack

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 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 be life-threatening under some circumstances. 

Talk to your physician 

Emergency Treatment of Thyrotoxic Hypokalemic Periodic Paralysis

First Actions:

  • Respiratory and cardiac arrest are possible. 
  • Call for Cardiac Monitoring/EKG
  • Draw electrolytes to determine serum K+
  • Thyroid studies
  • Provide 02 as thimble may not reflect 02 saturation due to cardiac dilitation
  • Lie patient in coma position to avoid aspiration. 

Emergency Treatment of the HyperKPP and/or PMC Attack

First Actions:

 

  • Respiratory and cardiac arrest are possible.
  • Call for Cardiac Monitoring/EKG
  • Draw electrolytes to determine serum K+
  • Provide 02 as thimble may not reflect 02 saturation 
  • Lie patient in coma position to avoid aspiration.

Hyperkalemic Periodic Paralysis and Paramyotonia Congenita can occur singly or in combination. 

Hospital Management Guidelines - ATS

Hospital Management Guidelines for Patients with Andersen-Tawil Syndrome

Patient's Name:

Birthdate: ________Height: _______Weight: _______

Address:

Primary Physician:

Emergency contact:  Name:                                             Relationship:

home phone#                                         cell #                            FAX:

Hospital Management Guideline Form - HyperKPP

Hospital Management Guidelines for Hyperkalemic Periodic Paralysis Patients

Patient's Name:

Birthdate: ________Height: _______Weight: _______

Address:

Primary Physician:

Emergency contact:  Your emergency contact should be someone who can speak for you when you cannot advocate for yourself.
Name:                                                                               Relationship:
home phone#                                         cell #                            FAX:

Hospital Management Guidelines - HypoKPP

Hospital Management Guidelines for Hypokalemic Periodic Paralysis Patients

Patient's Name:

Birthdate: ________Height: _______Weight: _______

Address:

Primary Physician:

Emergency contact:  Your emergency contact should be someone who can speak for you when you cannot advocate for yourself.
Name:                                                                               Relationship:
home phone#                                         cell #                            FAX:

Malignant Hyperthermia and HyperKPP/PMC

Patients with Sodium channel HyperKPP and PMC mutations are at increased risk of Malignant Hyperthermia;

Malignant Hyperthermia

Periodic Paralysis Patients at Increased Risk of MH

Patients with Hyperkalemic periodic paralysis and Paramyotonia Congenita are at increased risk for Malignant Hyperthermia (MH) during surgery. 

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