A Unique Case of Hypokalemic Periodic Paralysis with Resolution after Successful Surgery for Cervical Stenosis

Introduction: Hypokalemic periodic paralysis is a rare neuromuscular condition that presents with episodic severe muscle weakness, often triggered by strenuous exercise or high carbohydrate diets, that can be further divided into two major types, primary and secondary. The primary type could be genetically inherited or sporadic. The most common mutation causing this condition is CACNA1S gene mutation and the SCN4A gene mutation. The secondary type could be caused by any renal or gastrointestinal pathology causing loss of potassium, along with certain drugs like loop diuretics, thiazide diuretics, laxatives, corticosteroids, beta adrenergic agonists, insulin, and glucose. Here we present a unique case of a 29-year-old white male patient who presented with periodic paralysis attributed to his hypokalemic status. Case Description: This previously healthy patient did not have any of the secondary causes or any other known condition to cause his periodic paralysis. His serum potassium was between 3.6 to 3.8 mmol/L, and genetic analysis proved that he has a mutation in his CACNA1S gene. Moreover, he has a mutation in 11B-HSD gene causing him to have aberrant mineralocorticoid excess. He reported several episodes of loss of consciousness which is atypical of primary hypokalemic periodic paralysis. Later, after he was treated for cervical stenosis as he was investigated for symptoms of cervical radiculopathy, he reported complete resolution of his symptoms and declines any further episodes of loss of consciousness. At this time, potassium supplements were stopped, given no indication to continue them. However, he was advised to continue to take amiloride if his serum potassium levels dropped below 4 mmol/L. Discussion: The patient’s clinical presentation is believed to be attributed to his cervical spine lesion instead of his hypokalemic periodic paralysis. More research is required to determine the level of genetic penetrance in each case to customize the treatment accordingly with precision medicine, improving outcomes and minimizing potential adverse effects.

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Publication Details

Journal
Journal of the American Society of Nephrology
Published
2024-10-01
DOI
https://doi.org/10.1681/asn.2024pztj9hrj
Primary Topic
Cervical and Thoracic Myelopathy
Type
article
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article

A Unique Case of Hypokalemic Periodic Paralysis with Resolution after Successful Surgery for Cervical Stenosis

Matthew Nguyen, Ramy Magdy Hanna, Ahmed Zaher
Journal of the American Society of Nephrology
Cervical and Thoracic Myelopathy
article

A Unique Case of Hypokalemic Periodic Paralysis with Resolution after Successful Surgery for Cervical Stenosis

Matthew Nguyen, Ramy Magdy Hanna, Ahmed Zaher
article en

Abstract

Introduction: Hypokalemic periodic paralysis is a rare neuromuscular condition that presents with episodic severe muscle weakness, often triggered by strenuous exercise or high carbohydrate diets, that can be further divided into two major types, primary and secondary. The primary type could be genetically inherited or sporadic. The most common mutation causing this condition is CACNA1S gene mutation and the SCN4A gene mutation. The secondary type could be caused by any renal or gastrointestinal pathology causing loss of potassium, along with certain drugs like loop diuretics, thiazide diuretics, laxatives, corticosteroids, beta adrenergic agonists, insulin, and glucose. Here we present a unique case of a 29-year-old white male patient who presented with periodic paralysis attributed to his hypokalemic status. Case Description: This previously healthy patient did not have any of the secondary causes or any other known condition to cause his periodic paralysis. His serum potassium was between 3.6 to 3.8 mmol/L, and genetic analysis proved that he has a mutation in his CACNA1S gene. Moreover, he has a mutation in 11B-HSD gene causing him to have aberrant mineralocorticoid excess. He reported several episodes of loss of consciousness which is atypical of primary hypokalemic periodic paralysis. Later, after he was treated for cervical stenosis as he was investigated for symptoms of cervical radiculopathy, he reported complete resolution of his symptoms and declines any further episodes of loss of consciousness. At this time, potassium supplements were stopped, given no indication to continue them. However, he was advised to continue to take amiloride if his serum potassium levels dropped below 4 mmol/L. Discussion: The patient’s clinical presentation is believed to be attributed to his cervical spine lesion instead of his hypokalemic periodic paralysis. More research is required to determine the level of genetic penetrance in each case to customize the treatment accordingly with precision medicine, improving outcomes and minimizing potential adverse effects.

Journal of the American Society of NephrologyVol. 35(10S)
University of California, Irvine (US), University of California, Irvine Medical Center (US)
Good health and well-being
Openalex Percentile: Top 64%
Cervical and Thoracic Myelopathy
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