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Management of Hypokalemic Periodic Paralysis in a Primigravid Patient
Abstract Number: F3C-8
Abstract Type: Case Report/Case Series
Introduction: Hypokalemic periodic paralysis (HPP) is a rare, autosomal dominant disease characterized by sudden bouts of muscle weakness. It is caused by a mutation in the gene for the voltage-gated calcium (CACNA1S) or sodium (SCN4A) channel of skeletal myocytes (1, 2). Symptoms occur following stress, meals rich in carbohydrates or sodium, and hypothermia. Management focuses on preventing hypokalemia, which can be difficult in the pregnant patient as physiologic changes include a decrease in serum potassium. Epidural management is complex as patients may be insensitive to the effects of lidocaine and epinephrine must be avoided as it can decrease potassium secondary to β-receptor activation. The following case-report illustrates the management of a patient with HPP at our institution.
Case Report: A 26-year-old G1P0 presented for scheduled induction of labor at 39w1d. Medical history included asthma and HPP, for which she took eplerenone and oral potassium. The patient reported minor episodes every 2 months and major episodes (weakness and dysphagia) every 3-4 months with her symptoms occurring at a potassium below 4 mEq/L. She stated that she did well at a potassium of 4.8 mEq/L and did not have resistance to lidocaine based on prior exposure. Her symptoms continued during pregnancy, including a right arm contracture shortly before admission. Prior to induction, two 18-gauge IV’s were placed, with one dedicated for potassium infusion. Baseline basic metabolic panel (BMP) showed a potassium of 4 mEq/L. An epidural was placed and position was confirmed using a test dose of 2% lidocaine without epinephrine which was negative for dysgeusia or tinnitus. The epidural was started with a 0.125% bupivacaine infusion at a rate of 8 cc/hr without a bolus dose. We followed her potassium with a BMP every 4 hours and repleted to a high-normal level with 20-40 mEq PO. She required a low-transverse c-section for non-reassuring fetal heart tones, which was accomplished using her epidural and 2% lidocaine boluses. Her epidural was removed following delivery. She had daily BMP’s and continued oral potassium until discharge. During our care, her potassium was 4-4.6 mEq/L and she did not develop symptoms.
Discussion: Specific topics to address in the obstetric patient with HPP include resistance to local anesthetics and avoidance of epinephrine during test dosing. The stress of labor puts these patients at high risk for an acute episode and vigilance for symptoms is important. Preventative management strategies include: dedicated IV or central line for potassium infusion, frequent monitoring of serum potassium, scheduled potassium replacement, and avoidance of drugs that cause hypokalemia or may have reduced effect. In the event of an acute episode, management includes IV potassium and supportive care which may include intubation if respiratory muscles are affected.
1. Statland, et al. Neurol Clin. 2014;32:801-815.
2. Fontaine, B. Adv Genet. 2008;63:3-23.