Kidney Week

Abstract: TH-PO368

Semaglutide (S) Completely Reverses the Severe Chronic Myopathy of Hyperkalemic Periodic Paralysis

Session Information

• Sodium, Potassium, and Volume Disorders: Clinical
  October 24, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Fluid, Electrolytes, and Acid-Base Disorders

• 1102 Fluid, Electrolyte, and Acid-Base Disorders: Clinical

Authors

• Brand, Kenneth, Baystate Medical Center, Springfield, Massachusetts, United States

Kenneth Brand, MD

• Landry, Daniel L., Baystate Medical Center, Springfield, Massachusetts, United States

Daniel L. Landry, DO, FASN

• Mulhern, Jeffrey, Baystate Medical Center, Springfield, Massachusetts, United States

Jeffrey Mulhern, MD

• Braden, Gregory Lee, Baystate Medical Center, Springfield, Massachusetts, United States

Gregory Lee Braden, MD

Group or Team Name

• Kidney Care and Transplant Services of New England.

Introduction

Hyperkalemic Periodic Paralysis (HPP) is caused by a gene mutation in skeletal muscle Na channel, SCN4a, causing sustained muscle depolarization, hyperkalemia & muscle weakness. After 3 decades most patients develop severe muscle weakness leading to life in a wheelchair. We describe a patient with HPP with severe chronic myopathy & after starting S for weight loss he regained normal strength.

Case Description

A 48 year-male with HPP at age 4 had a Na channel point mutation in the SCN4A gene at 704 with methionine replacing threonine. His father, uncle, sister, & 3 nephews have the same mutation.He was treated with acetazolamide, albuterol, & daranide without success.S was given due to a body mass index greater than 40. We studied the Short Phyicsal Performance Battery ( SPPB) pre S & at 4, 7 & 12 months of S. After the third weekly dose sub q of 0.25 mg he noted less weakness. Before the S he could not rise out of a chair without help & his gait was very slow. The table shows his dramatic results & he was normal at 7 months. He stopped S after having GI side effects on 2.4 mg/week & his weakness returned. He is now on 1.7 mg/week with a normal SPPB and muscle strength at 1 year. .

Discussion

S is a glucagon like peptide (GLP-1) agonist that inhibits glucagon release, enhances the growth of pancreatic beta cells, and increases their production of insulin. Meals on S increase insulin levels 5 fold which shift K intracellularly. As in animal studies, GLP-1 stimulation of its receptors on skeletal muscle can reverse myopathy by promoting muscle cell growth and inhibiting muscle atrophy. By altering insulin signaling and skeletal muscle physiology, S increases intracellular potassium storage and decreases myopathy in HPP. S is an excellent & novel once weekly option that treats not just the hyperkalemic periodic paralysis but also the skeletal muscle weakness in a multimodal way.

SPPB