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First Actions:

Respiratory and cardiac arrest are possible.

• Paralyzed patients may appear to be unconscious, but are awake and aware. Do not assume they cannot feel pain or hear conversations

• Communication: If the patient is unable to speak and can blink ask yes or no questions, say, “Blink once for yes, twice for no.”

• Call for Cardiac Monitoring/EKG

• Draw electrolytes to determine serum K+ (Do not use tourniquet if possible, this can cause falsely high K+ results)

• Check vitals – cardiac dilatation may cause hypotension and low pulse pressure

• Provide o2 as thimble may not reflect o2 saturation due to cardiac dilatation 

• Place the patient in coma position to avoid aspiration during weakness/paralytic attack. 

• Monitor Closely. Weakness can rapidly without warning progress to paralysis, torsades de pointes, cardiac arrest and respiratory failure. 

• IV fluids must be warmed 

Insurer: Cigna open access plus + Medicaid (Molina)

Date of birth: 03/21/2003                       

Address: 13023 Reiner Road Monroe, WA 98272

Phone:  (425) 318-9116

Emergency Contact: Aleshia Prosser

ICE Phone:  (360) 913-6535

Height: 5’9   /   Weight: 170 

Smoke: No   /   Vape: No

Drink: No   /   Drugs: No

Normal Heart Rate:  78   

Normal BP:  110/65     

Reproductive History: 

Age at Menarche: 13

# of pregnancies: 0

If sexually active method of birth control: None

Primary Physician: Susana Escobar (WWMG)

Phone: (360) 659-1231

Neurologist: Casey Carter Nazor (Neurological Associates of WA)

Phone: (425) 455-5440

Patient’s “Safe” Serum k+ levels:

Giusiana is known to become symptomatic at any serum K+ level at or below 3.8 

The hypokalemia in hypokalemic periodic paralysis is due to potassium shifts rather than a deficit. Therefore it is not necessary for potassium to shift out of range. The serum K+ level is not always an indication of the severity of the patient’s condition. Some patients experience severe weakness and/or cardiac instability with only mild hypokalemia or potassium levels still within range. 

Presentation

Patients may present to the ER with an attack of muscle weakness or paralysis and/or cardiac rhythm disturbances or intermittent prolonged QT interval (over 0.39 sec for men and 0.44 sec for women).

• Less commonly patients experience syncope, cardiac arrest, or sudden death. While the ECG may reveal a long QTc interval (LQT), characteristic T-U patterns including enlarged U waves, a wide T-U junction, and prolonged terminal T-wave downslope distinguish ATS from other LQT syndromes. The patient’s serum K+ can vary from attack to attack. Some patients are always normokalemic during attacks, some are hyperkalemic, most seem to be hypokalemic or have symptoms consistent with hypokalemia

Monitoring Notes

• Do not leave the patient unattended! The condition can change for better or worse rapidly. Risk of aspiration.

• Measure serum K+ immediately and put electro-cardiographic monitoring in place. The serum K+ level is not always an indication of the severity of the patient’s condition. Some patients experience severe weakness and/or cardiac instability with only mild hypokalemia or potassium levels which remain in range.

• Respiratory function may deteriorate rapidly and without warning. The patient must not be left unattended. When the patient is paralyzed swallowing may be compromised and the gag reflex lost. Place the paralyzed patient in the coma position due to the possibility of aspiration. Most deaths in HypoKPP come from aspiration.

• In patients with HypoKPP cardiac function can be affected far more seriously during attacks than would be expected from the serum potassium level.

•  Patients should be monitored for several hours after strength is restored, as attacks may reinstate if inadequately treated.

Management of Potassium:

• For most patients, Oral K preferred over IV K. Aqueous K favored over pill form. Giusiana responds better to IV K than oral, but if not available, aqueous K is tolerated provided she is able to swallow. 

• Dosing: 40-60mEq p.o. of potassium, in the absence of underlying deficit (as is the case in hypokalemic periodic paralysis), should cause a rise of 1.0-1.5 mEq in serum potassium. 135-160mEq p.o. of potassium should cause a rise of 2.5 to 3.5 mEq in serum potassium.

• IV fluids must be warmed, room temp fluids may drop body temperature and trigger an episode.

• No D5 in i.v. fluids. If possible, no saline in fluids – may try mannitol 5% to give a bolus of potassium.

• No more than 10-20mEq/hr [i.e. 250-500cc of 40mEq/L solution/hr]. Concentrations >= 60mEq/L causes pain and phlebitis in peripheral veins. So, expect pain with 10-20 mEq K+ in 100cc of 5% mannitol.

• If the patient has not taken K+ before coming to ER, consider dosing a bolus of 60mEq p.o. by aqueous solution. Wait 1 hour. Then re-dose.

• Be careful not to overcorrect. Wait 20-60 min. before re-dosing. Patience is key.

• EKG for rhythm, especially QT interval.

• Monitor serum potassium q30min to q120min, depending on the condition of the patient and medications being administered.

• There is probably no role for acetazolamide or potassium-sparing diuretics in the management of an acute paralysis attack; however, no evidence-based study has proven this hypothesis.

• If associated with myoclonus (jerks), consider a small dose of benzodiazepine as jerk represents exercise, and the trigger of paralysis is rest after exercise.

• For myotonia, keep muscles warm (temperature).

• Fluids to deliver potassium can be Lactated Ringers as well as Mannitol as many hospitals do not stock Mannitol.

Potassium replacement must be done in accordance with clinical findings (muscle weakness and cardiac rhythm abnormalities) taking precedence over the numerical value of serum potassium. 

In normal people:
A decrease from 4.0 to 3.0 mEq can correspond to a 200-400mEq potassium deficit.
A decrease from 3.0 to 2.0 mEq can correspond to a 200-400mEq potassium deficit.

Decreases below 2.0mEq can represent a deficit larger than 800mEq as intracellular potassium begins to compensate.

In periodic paralysis, these deficits may or may not hold true. The hypokalemia in hypokalemic periodic paralysis is due to POTASSIUM SHIFTS rather than a deficit. Therefore, more conservative (i.e., less aggressive) potassium replacement is needed. Doses above 200mEq over a period of two hours are rarely ever required.

PROBLEM LIST: 

• Hypokalemic Periodic Paralysis (Andersen Tawil Syndrome)

• Ehlers Danlos Syndrome III

• Postural Orthostatic Tachycardia Syndrome

• Brainstem Compression

• Mast Cell Activation Syndrome

• Occult Tethered Cord Syndrome

• Bilateral IJV Compression

• Cranio-Cervical and Atlanto-Axial Instability/rotational dislocation 

• Autoimmune Encephalitis

Drug Allergies/DRUGS to AVOID: 

• Gadoteridol (MRI contrast)

• TC-99m

• Zofran (Odansetron)

• Epinephrine

• Benadryl (Diphenhydramine)

• Dexamethasone

• Prednisone

• Other Steroids

• Albuterol

• Fluoroquinolones 

• All drugs which prolong the QT interval are contra-indicated 

• Antibiotics which reduce neuromuscular transmission or prolong QT interval: aminoglycosides: Strepto-, Neo-, Kana-, and Clindamycin Macrolides: Erythromycin, Telithromycin, Azithromycin; Fluoroquinolones: Ciprofloxacin and others

Current Medications: 

• Valproic Acid 500mg morning,  750mg bedtime

• Midodrine 10mg 3x daily

• Propranolol 30mg 4x daily

• Ketamine Nasal Spray 100mg/ml 1-2 sprays in 1 nostril every 4 hrs PRN for pain. Not to exceed 10 sprays in 24 hours. (1 spray = 0.1 ml)

• Zolpidem 5mg PRN 

• IV saline 1 liter 1x daily

• IV potassium 2-5x weekly PRN

• Oral potassium PRN

• Heparin 5ml Intravenously in each lumen of central line 1x daily

• Low Dose Naltrexone 1.5mg nightly

Surgical HX: 

• 2015 Meningocele removal

• 2020 Bilateral rectus abdominis reattachment and  right adductor compartment syndrome release

• 2024 Muscle biopsy (right leg)

• 2024 Tunneled central catheter placement

• 2024 Tunneled central catheter re-placement

• Attacks may last from minutes to days, and occur sporadically.

• Weakness can be local or generalized.

• The deep tendon reflexes can become depressed, diminished, or lost in the course of the attacks.

• The muscle fibers become unresponsive to either direct or indirect electrical stimulation during attacks.

• The generalized attacks usually begin in proximal muscles and then spread to distal ones.

• Respiratory and cranial muscles tend to be spared but eventually may also be paralyzed.

• Muscle exertion and Rest after exercise tends to provoke weakness of the muscles that had been exercised.

• Exercise restricted to a single muscle or a small group of muscles can induce weakness of the exercised muscles without a detectable change of the potassium level in the systemic circulation.

• Exposure to cold may provoke weakness in the primary forms of the disease.

• Complete recovery usually occurs after initial attacks.

• Permanent weakness and irreversible pathological changes in muscle can develop after repeated attacks.

• Patients may present with mental confusion with attacks

• Patients may present with an off-mood/bad mood/irritability during attacks

• Epinephrine

• Benadryl (Diphenhydramine)

• Dexamethasone

• Prednisone

• Other Steroids

• Albuterol

• Insulin

• Fluoroquinolones 

• All drugs which prolong the QTinterval are contra-indicated;

• salbutamol inhalers

• thiazide and other potassium wasting diuretics

• Antibiotics which reduce neuromuscular transmission or prolong QT interval: aminoglycosides: Strepto-, Neo-, Kana-, and Clindamycin Macrolides: Erythromycin, Telithromycin, Azithromycin,

•  Fluoroquinolones: Ciprofloxacin and others

• All drugs which prolong the QT interval are contra-indicated, whether or not the patient has a permanently prolonged QTc interval

• Cold (eg air conditioning, cold food, cold IV fluids, ice packs)

• Muscle exertion

• Rest after exercise

• Medications (eg steroids, benadryl)

• Stress (due to the release of adrenaline)

• High carb meals

• Dehydration

COMMON SYMPTOMS DURING ATTACKS:

• Muscle Weakness

• Muscle Paralysis (flaccid or rigid)

• Myoclonus

• Mental Confusion

• Syncope

• Muscle convulsions 

• Hypoxia

• Hypotension

• Bradycardia or Tachycardia

• Heart Arrhythmias 

• Slurring of words

• Acting “drunk”

OTHER SYMPTOMS DUE TO CO-DIAGNOSES:

• Drop Attacks

• Seizures

• Non-epileptic seizures (muscle convulsions due to periodic paralysis)

• Syncope

• Aphasia

• Joint Dislocations (especially cervical spine)

• Anaphylaxis

• Mental Confusion

• Convulsive Syncope

• Hallucinations (due to encephalitis)

For further information refer to the links below:

Comprehensive Information: 

Anaesthesia and peri-operative care in the primary periodic paralyses: 

PART 1: A REVIEW OF THE LITERATURE

https://drive.google.com/file/d/0B-savOlQA6QCSFQwQUQ5RWhRWVE/view

PART 2: PRACTICAL GUIDELINES

https://drive.google.com/file/d/0B-savOlQA6QCOWdjYVBoTFJWMDA/view

Quick look:

For Hyperkalemic forms: PMID: 20301669

For Myotonic forms: PMID: 23802937

For Hypokalemic forms: PMID: 23833504

For All forms: PMID: 32622512

Periodic Paralysis International

https://hkpp.org

Periodic Paralysis Association

https://periodicparalysis.org