Burgers, Fries, and Weak Thighs - A Case of Familial Hypokalemic Periodic Paralysis
Sep 28, 2025
A 13-year-old boy experiences sudden muscle weakness after a meal, leading to a diagnosis of familial hypokalemic periodic paralysis. The hosts delve into the genetic mutations involved and discuss crucial management strategies like potassium supplementation. They explore common triggers for episodes, such as carb-heavy meals and exercise, and highlight the importance of genetic testing. Key considerations for anesthesia and cardiac monitoring in these patients are also covered, offering practical insights for clinicians.
21:17
forum Ask episode
web_stories AI Snips
view_agenda Chapters
auto_awesome Transcript
info_circle Episode notes
question_answer ANECDOTE
Presentation After Carb-Rich Meal
A 13-year-old boy ate a high-carb meal (burger, fries, milkshake) then developed profound weakness and K+ 1.7 mmol/L.
He required several days of IV and oral potassium before regaining motor function.
insights INSIGHT
Gating Pore Currents Explain Paralysis
Mutations in CACNA1S or SCN4A create abnormal inward leak currents that make muscle fibers depolarize and become inexcitable during low extracellular potassium.
This 'gating pore' current explains paradoxical depolarization and episodic flaccid paralysis.
insights INSIGHT
Typical Triggers And Age Pattern
Attacks commonly start in the first or second decade and are frequently triggered by carbohydrate-rich meals, rest after exercise, alcohol, or stress.
Frequency often declines after the third decade but attacks can severely impact quality of life.
Get the Snipd Podcast app to discover more snips from this episode
Welcome to PICU Doc on Call, the podcast where we break down real-life cases from the pediatric intensive care unit and share practical insights for clinicians everywhere! I’m Dr. Monica Gray, and I’m joined by my co-host, Dr. Pradip Kamat.
Today, we’re diving into a fascinating case: a 13-year-old boy who suddenly developed muscle weakness and was found to have severe hypokalemia. After some detective work, he was diagnosed with familial hypokalemic periodic paralysis, a rare but important condition to recognize in the PICU.
We’ll discuss the genetic underpinnings, classic clinical features, and common triggers associated with this disorder. Additionally, we’ll guide you through the differential diagnosis, key management strategies, such as potassium supplementation, and explain why genetic testing is so crucial. We’ll also cover essential considerations for anesthesia and cardiac monitoring in these patients.
Whether you’re a pediatric intensivist or just interested in acute neuromuscular care, stick around for some practical pearls you can use on your next shift!
Show Highlights:
Clinical case discussion of a 13-year-old male patient with muscle weakness and hypokalemia
Diagnosis and management of familial hypokalemic periodic paralysis
Genetic basis and mutations associated with hypokalemic periodic paralysis (CACNA1S and SCN4A)
PICU Doc On Call