Hypokalemic periodic paralysis: Difference between revisions

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<embedvideo service="youtube" description="''Hypokalemic periodic paralysis attack (inherited type)'' by Dr Jacob Levitt. This video shows a severe episode of hypokalemic periodic paralysis." dimensions="400" alignment="right" container="frame">https://www.youtube-nocookie.com/watch?v=tbP9VhaREO0&autoplay=0</embedvideo>
<embedvideo service="youtube" description="''Hypokalemic periodic paralysis attack (inherited type)'' by Dr Jacob Levitt. This video shows a severe episode of hypokalemic periodic paralysis." dimensions="400" alignment="right" container="frame">https://www.youtube-nocookie.com/watch?v=tbP9VhaREO0&autoplay=0</embedvideo>
'''Hypokalemic periodic paralysis''' (hypoKPP) is a rare [[channelopathy]] characterized by repeated, temporary attacks of [[Paresis|muscle weakness]] or [[paralysis]] that are the result of a fall in [[potassium]] levels in the blood.<ref name="June-Bum">{{Cite journal|last=Kim|first=June-Bum|author-link=|author-link2=|author-link3=|author-link4=|author-link5=|date=Jan 2014|title=Channelopathies|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3935107/|journal=Korean Journal of Pediatrics|volume=57|issue=1|pages=1–18|doi=10.3345/kjp.2014.57.1.1|issn=1738-1061|pmc=3935107|pmid=24578711|quote=|via=}}</ref>
'''Hypokalemic periodic paralysis''' (hypoKPP) is a rare [[channelopathy]] characterized by repeated, temporary attacks of [[Paresis|muscle weakness]] or [[paralysis]] that are the result of a fall in [[potassium]] levels in the blood.<ref name="June-Bum">{{Cite journal | last = Kim | first = June-Bum | authorlink = | date = Jan 2014 | title = Channelopathies | url = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3935107/|journal=Korean Journal of Pediatrics|volume=57|issue=1|pages=1–18|doi=10.3345/kjp.2014.57.1.1|issn=1738-1061|pmc=3935107|pmid=24578711|quote=|via=}}</ref>


Hypokalemic periodic paralysis can be either genetic (familial) or acquired (not life long), and is classed as a form of acute [[flaccid]] [[paralysis]], and has been described in people with severe [[myalgic encephalomyelitis]].<ref>{{Cite journal|last=Padmaperuma|first=Pacd|last2=Dissanayake|first2=H. A.|date=2018-07-18|title=Periodic paralysis: what clinician needs to know?|url=http://medcraveonline.com/EMIJ/EMIJ-06-00189|journal=Endocrinology & Metabolism International Journal|volume=6|issue=4|pages=1–0|doi=10.15406/emij.2018.6.00189|issn=2473-0815}}</ref><ref name=":0">{{Cite web|url=http://carersfight.blogspot.com/2013/08/paralysis-qualitative-study-of-people.html|title=Paralysis, a qualitative study of people with Severe Myalgic Encephalomyelitis|last=Crowhurst|first=Greg|authorlink=Greg Crowhurst|last2=Crowhurst|first2=Linda|authorlink2=Linda Crowhurst|date=Aug 8, 2013|website=[[Stonebird]]|archive-url=|archive-date=|dead-url=|access-date=2018-12-28}}</ref>  
Hypokalemic periodic paralysis can be either genetic (familial) or acquired (not life long), and is classed as a form of [[acute flaccid paralysis]],<ref name="Padmaperuma2018">{{Cite journal | last = Padmaperuma | first = Pacd | last2 = Dissanayake | first2 = H.A. | date = 2018-07-18 | title = Periodic paralysis: what clinician needs to know? | url = http://medcraveonline.com/EMIJ/EMIJ-06-00189|journal=Endocrinology & Metabolism International Journal|volume=6|issue=4|pages=1–0|doi=10.15406/emij.2018.6.00189|issn=2473-0815}}</ref><ref name="NBK559178">{{Cite book | url = https://www.ncbi.nlm.nih.gov/books/NBK559178/ | title = Hypokalemic periodic paralysis|vauthors=Phuyal P, Nagalli S | date = Nov 25, 2021| publisher = StatPearls Publishing LLC|location=Treasure Island (FL)}}</ref> and has been described in people with severe [[myalgic encephalomyelitis]].<ref name="Crowhurst2013"/>


==Types of periodic paralysis==
==Types of periodic paralysis==
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==Muscle weakness ==
==Muscle weakness ==


Weakness may be mild and limited to certain muscle groups, resulting in [[partial paralysis]] or [[paresis]], or more severe full-body [[paralysis]]. During an attack reflexes may be decreased or absent. Hypokalemic periodic paralysis attacks may last for a few hours, or persist for several days. Recovery is usually sudden, and occurs when the swollen muscles release [[potassium]] as they recover.  
Muscle weakness may be mild and limited to certain muscle groups, resulting in [[partial paralysis]] or [[paresis]], or more severe full-body [[paralysis]]. During an attack reflexes may be decreased or absent. Hypokalemic periodic paralysis attacks may last for a few hours, or persist for several days. Recovery is usually sudden, and occurs when the swollen muscles release [[potassium]] as they recover.<ref name="rarediseases"/><ref name="manual"/>


==Prevalence==
==Prevalence==


Hypokalemic periodic paralysis is to be rare in the general population, but has been found in people with ME/CFS, especially in people with [[severe and very severe ME]].
Hypokalemic periodic paralysis is said to be rare in the general population,<ref name="rarediseases">{{Cite web | url=https://rarediseases.info.nih.gov/diseases/6729/hypokalemic-periodic-paralysis | title = Hypokalemic periodic paralysis|website=Genetic and Rare Diseases Information Center|access-date=2022-03-26}}</ref> but has been found in people with [[ME/CFS]], especially in people with [[severe and very severe ME]].<ref name="Crowhurst2013"/>


==Symptom Recognition==
De Becker et al. (2001) conducted a large study of [[chronic fatigue syndrome]] patients, and found that 27.2% of patients meeting the [[Fukuda criteria]] and 33.2% of patients meeting the [[Holmes criteria]] for CFS experienced episodes of [[paralysis]].<ref name="DeBecker2001">{{Cite journal | last = De Becker | first = Pascale | authorlink = Pascale De Becker | last2 = McGregor | first2 = Neil | authorlink2 = Neil McGregor | last3 = De Meirleir | first3 = Kenny | authorlink3 = Kenny De Meirleir | date = Dec 2001 | title = A definition‐based analysis of symptoms in a large cohort of patients with chronic fatigue syndrome | url = https://onlinelibrary.wiley.com/doi/full/10.1046/j.1365-2796.2001.00890.x?sid=nlm%3Apubmed|journal=Journal of Internal Medicine|volume=250|issue=3 | pages = 234–240|doi=10.1046/j.1365-2796.2001.00890.x|quote=|via=}}</ref>


Hypokalemic periodic paralysis was only discovered fairly recently.
==Symptom recognition==


The [[International Consensus Criteria]] and the International Consensus Primer refer to channelopathies and ion transportation problems, but do not mention hypokalemic periodic paralysis directly.<ref name="ICP2011primer">{{citation
Hypokalemic periodic paralysis was only discovered fairly recently.<ref name="June-Bum" /> It is commonly misdiagnosed as [[conversion disorder]] or "functional" paralysis, and can be missed since potassium levels may be normal between attacks.<ref name="manual"/>
| last1 = Carruthers | first1 = BM | authorlink1 = Bruce Carruthers
 
| last2 = van de Sande | first2 = MI | authorlink2 = Marjorie van de Sande
The [[International Consensus Criteria]] and the International Consensus Primer refer to channelopathies and [[ion transportation]] problems, but do not mention hypokalemic periodic paralysis directly.<ref name="ICP2011primer">{{citation | last1 = Carruthers | first1 = BM | authorlink1 = Bruce Carruthers | last2 = van de Sande | first2 = MI | authorlink2 = Marjorie van de Sande | last3 = De Meirleir | first3 = KL | authorlink3 = Kenny de Meirleir | last4 = Klimas | first4 = NG | authorlink4 = Nancy Klimas | last5 = Broderick | first5 = G | authorlink5 = Gordon Broderick | last6 = Mitchell | first6 = T | authorlink6 = Terry Mitchell | last7 = Staines | first7 = D | authorlink7 = Donald Staines | last8 = Powles | first8 = ACP | authorlink8 = A C Peter Powles | last9 = Speight | first9 = N | author-link9 = Nigel Speight | last10 = Vallings | first10 = R | authorlink10 = Rosamund Vallings | last11 = Bateman | first11 = L | authorlink11 = Lucinda Bateman | last12 = Bell | first12 = DS | authorlink12 = David Bell | last13 = Carlo-Stella | first13 = N | authorlink13 = Nicoletta Carlo-Stella | last14 = Chia | first14 = J | authorlink14 = John Chia | last15 = Darragh | first15 = A | authorlink15 = Austin Darragh | last16 = Gerken | first16 = A | authorlink16 = Anne Gerken | last17 = Jo | first17 = D | authorlink17 = Daehyun Jo | last18 = Lewis | first18 = DP | authorlink18 = Donald Lewis | last19 = Light | first19 = AR | authorlink19 = Alan Light | last20 = Light | first20 = KC | authorlink20 = Kathleen Light | last21 = Marshall-Gradisnik | first21 = S | authorlink21 = Sonya Marshall-Gradisnik | last22 = McLaren-Howard | first22 = J | authorlink22 = John McLaren-Howard | last23 = Mena | first23 = I | authorlink23 = Ismael Mena | last24 = Miwa | first24 = K | authorlink24 = Kunihisa Miwa | last25 = Murovska | first25 = M | authorlink25= Modra Murovska | last26 = Stevens | first26 = SR | authorlink26 = Staci Stevens | title = Myalgic encephalomyelitis: Adult & Paediatric: International Consensus Primer for Medical Practitioners | date = 2012
| last3 = De Meirleir | first3 = KL | authorlink3 = Kenny de Meirleir
| isbn = 978-0-9739335-3-6 | url = http://www.investinme.org/Documents/Guidelines/Myalgic%20Encephalomyelitis%20International%20Consensus%20Primer%20-2012-11-26.pdf }}</ref>
| last4 = Klimas | first4 = NG | authorlink4 = Nancy Klimas
| last5 = Broderick | first5 = G | authorlink5 = Gordon Broderick
| last6 = Mitchell | first6 = T | authorlink6 = Terry Mitchell
| last7 = Staines | first7 = D | authorlink7 = Donald Staines
| last8 = Powles | first8 = ACP | authorlink8 = A C Peter Powles
| last9 = Speight | first9 = N | authorlink9 = Nigel Speight
| last10 = Vallings | first10= R | authorlink10= Rosamund Vallings
| last11 = Bateman | first11= L | authorlink11= Lucinda Bateman
| last12 = Bell | first12= DS | authorlink12= David Bell
| last13 = Carlo-Stella | first13= N | authorlink13= Nicoletta Carlo-Stella
| last14 = Chia | first14= J | authorlink14= John Chia
| last15 = Darragh | first15= A | authorlink15= Austin Darragh
| last16 = Gerken | first16= A | authorlink16= Anne Gerken
| last17 = Jo | first17= D | authorlink17= Daehyun Jo
| last18 = Lewis | first18= DP | authorlink18= Donald Lewis
| last19 = Light | first19= AR | authorlink19= Alan Light
| last20 = Light | first20= KC | authorlink20= Kathleen Light
| last21 = Marshall-Gradisnik | first21= S | authorlink21= Sonya Marshall-Gradisnik
| last22 = McLaren-Howard | first22= J | authorlink22= John McLaren-Howard
| last23 = Mena | first23= I | authorlink23= Ismael Mena
| last24 = Miwa | first24= K | authorlink24= Kunihisa Miwa
| last25 = Murovska | first25= M | authorlink25= Modra Murovska
| last26 = Stevens | first26= SR | authorlink26= Staci Stevens
| title = Myalgic encephalomyelitis: Adult & Paediatric: International Consensus Primer for Medical Practitioners  
| date = 2012
| isbn = 978-0-9739335-3-6
| url = http://www.investinme.org/Documents/Guidelines/Myalgic%20Encephalomyelitis%20International%20Consensus%20Primer%20-2012-11-26.pdf
}}</ref>


==Notable studies==
==Notable studies==
 
*2003, Paralysis: A qualitative study of the people with Severe Myalgic Encephalomyelitis<ref name="Crowhurst2013">{{Cite web | url=http://carersfight.blogspot.com/2013/08/paralysis-qualitative-study-of-people.html | title = Paralysis, a qualitative study of people with Severe Myalgic Encephalomyelitis | last = Crowhurst | first = Greg | authorlink=Greg Crowhurst | last2 = Crowhurst | first2 = Linda | authorlink2 = Linda Crowhurst | date = Aug 8, 2013 | website = [[Stonebird]]|archive-url=|archive-date=|url-status=|access-date=2018-12-28}}</ref> - [[http://carersfight.blogspot.com/2013/08/paralysis-qualitative-study-of-people.html (Full text), not peer reviewed]
2003, [http://carersfight.blogspot.com/2013/08/paralysis-qualitative-study-of-people.html Paralysis: A qualitative study of the people with Severe Myalgic Encephalomyelitis]<ref name=":0" />
*2001, A definition‐based analysis of symptoms in a large cohort of patients with chronic fatigue syndrome<ref name="DeBecker2001">{{Cite journal | last = De Becker | first = Pascale | authorlink = Pascale De Becker | last2 = McGregor | first2 = Neil | authorlink2 = Neil McGregor | last3 = De Meirleir | first3 = Kenny | authorlink3 = Kenny De Meirleir | date = Dec 2001 | title = A definition‐based analysis of symptoms in a large cohort of patients with chronic fatigue syndrome | url = https://onlinelibrary.wiley.com/doi/full/10.1046/j.1365-2796.2001.00890.x?sid=nlm%3Apubmed|journal=Journal of Internal Medicine|volume=250|issue=3 | pages = 234–240|doi=10.1046/j.1365-2796.2001.00890.x|quote=|via=}}</ref> - [https://onlinelibrary.wiley.com/doi/full/10.1046/j.1365-2796.2001.00890.x (Full text)]


== Causes of temporary paralysis==
== Causes of temporary paralysis==
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* after meals with high [[sodium]] content (high salt content)  
* after meals with high [[sodium]] content (high salt content)  
* as a result of sudden changes in [[temperature]], or cold temperatures  
* as a result of sudden changes in [[temperature]], or cold temperatures  
* [[sensory input]], such as noise, or flashing lights cause trigger paralysis   
* [[sensory overload|sensory input]], such as noise, or flashing lights may trigger paralysis   
* and even excitement.
* and even excitement.<ref name="manual">{{Cite web | title = Hypokalemic Periodic Paralysis Owner's Manual | first = Michael M. | last = Segal | first2 = Karin | last2 = Jurkat-Rott | first3 = Jacob | last3 = Levitt | first4 = Frank | last4 = Lehmann | url =https://periodicparalysis.org/hypokalemic-periodic-paralysis-owners-manual/|access-date=2022-03-26}}</ref>


== Potential Treatments ==
== Potential treatments ==


[[Electrolytes]] are one of the suggestions for treating [[Portal:Energy metabolism|energy metabolism]] and [[ion transportation]] problems in [[Myalgic encephalomyelitis|ME]]. [[Potassium]] would seem the obvious [[Electrolytes|electrolyte]].
[[Electrolytes]] are one of the suggestions for treating [[Portal:Energy metabolism|energy metabolism]] and [[ion transportation]] problems in [[Myalgic encephalomyelitis|ME]]. [[Potassium]] would seem the obvious [[Electrolytes|electrolyte]], and is used to treat and reduce attacks of hypokalemic periodic paralysis in people without comorbid ME/CFS.<ref name="rarediseases"/><ref name="manual"/>


== See Also ==
== See also ==
* [[Periodic paralysis]]
* [[Periodic paralysis]]
* [[Channelopathy]]
* [[Channelopathy]]
Line 83: Line 55:


== Learn more ==
== Learn more ==
 
*[https://rarediseases.info.nih.gov/diseases/6729/hypokalemic-periodic-paralysis Hypokalemic periodic paralysis] - Genetic and Rare Diseases Information Center
[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3935107/ Channelopathies (review)] - Kim June-Bum.
*[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3935107/ Channelopathies (review)] - Kim June-Bum.
 
*[https://medcraveonline.com/EMIJ/EMIJ-06-00189.php Periodic Paralysis - What clinicians should know]
[https://medcraveonline.com/EMIJ/EMIJ-06-00189.php Periodic Paralysis - What clinicians should know]


==References==
==References==
{{reflist}}
{{reflist}}
[[Category:Channelopathy]]
[[Category:Signs and symptoms]]
[[Category:Signs and symptoms]]
[[Category:Diagnoses]]
[[Category:Diagnoses]]
[[Category:Neurology]]
[[Category:Neurology]]
[[Category:Musculoskeletal diseases and disorders]]
[[Category:Musculoskeletal diseases and disorders]]
[[Category:Potential comorbidities]]

Latest revision as of 14:13, April 2, 2023

Hypokalemic periodic paralysis attack (inherited type) by Dr Jacob Levitt. This video shows a severe episode of hypokalemic periodic paralysis.

Hypokalemic periodic paralysis (hypoKPP) is a rare channelopathy characterized by repeated, temporary attacks of muscle weakness or paralysis that are the result of a fall in potassium levels in the blood.[1]

Hypokalemic periodic paralysis can be either genetic (familial) or acquired (not life long), and is classed as a form of acute flaccid paralysis,[2][3] and has been described in people with severe myalgic encephalomyelitis.[4]

Types of periodic paralysis[edit | edit source]

Diagram of muscle channelopathies showing hypokalemic periodic paralysis on a spectrum of channelopathy diseases.
Hypokalemic periodic paralysis is a type of muscle channelopathy.
Image: K June-Bum, 2014[1] CC-BY-NC-3.0

Hypokalemic periodic paralysis is one of several types of periodic paralysis.

Other types include hyperkalemic periodic paralysis, which is linked to high potassium levels, thyrotoxic periodic paralysis, linked to thyroid hormone levels.[1]

Muscle weakness[edit | edit source]

Muscle weakness may be mild and limited to certain muscle groups, resulting in partial paralysis or paresis, or more severe full-body paralysis. During an attack reflexes may be decreased or absent. Hypokalemic periodic paralysis attacks may last for a few hours, or persist for several days. Recovery is usually sudden, and occurs when the swollen muscles release potassium as they recover.[5][6]

Prevalence[edit | edit source]

Hypokalemic periodic paralysis is said to be rare in the general population,[5] but has been found in people with ME/CFS, especially in people with severe and very severe ME.[4]

De Becker et al. (2001) conducted a large study of chronic fatigue syndrome patients, and found that 27.2% of patients meeting the Fukuda criteria and 33.2% of patients meeting the Holmes criteria for CFS experienced episodes of paralysis.[7]

Symptom recognition[edit | edit source]

Hypokalemic periodic paralysis was only discovered fairly recently.[1] It is commonly misdiagnosed as conversion disorder or "functional" paralysis, and can be missed since potassium levels may be normal between attacks.[6]

The International Consensus Criteria and the International Consensus Primer refer to channelopathies and ion transportation problems, but do not mention hypokalemic periodic paralysis directly.[8]

Notable studies[edit | edit source]

  • 2003, Paralysis: A qualitative study of the people with Severe Myalgic Encephalomyelitis[4] - [(Full text), not peer reviewed
  • 2001, A definition‐based analysis of symptoms in a large cohort of patients with chronic fatigue syndrome[7] - (Full text)

Causes of temporary paralysis[edit | edit source]

In individuals with the inherited genetic mutation, attacks often begin in adolescence. Attacks of paralysis are most commonly occur:

  • on awakening or after sleep
  • after rest following strenuous exercise (attacks during exercise are rare)
  • after high carbohydrate meals
  • after meals with high sodium content (high salt content)
  • as a result of sudden changes in temperature, or cold temperatures
  • sensory input, such as noise, or flashing lights may trigger paralysis
  • and even excitement.[6]

Potential treatments[edit | edit source]

Electrolytes are one of the suggestions for treating energy metabolism and ion transportation problems in ME. Potassium would seem the obvious electrolyte, and is used to treat and reduce attacks of hypokalemic periodic paralysis in people without comorbid ME/CFS.[5][6]

See also[edit | edit source]

Learn more[edit | edit source]

References[edit | edit source]

  1. 1.0 1.1 1.2 1.3 Kim, June-Bum (January 2014). "Channelopathies". Korean Journal of Pediatrics. 57 (1): 1–18. doi:10.3345/kjp.2014.57.1.1. ISSN 1738-1061. PMC 3935107. PMID 24578711.
  2. Padmaperuma, Pacd; Dissanayake, H.A. (July 18, 2018). "Periodic paralysis: what clinician needs to know?". Endocrinology & Metabolism International Journal. 6 (4): 1–0. doi:10.15406/emij.2018.6.00189. ISSN 2473-0815.
  3. Phuyal P, Nagalli S (November 25, 2021). Hypokalemic periodic paralysis. Treasure Island (FL): StatPearls Publishing LLC.
  4. 4.0 4.1 4.2 Crowhurst, Greg; Crowhurst, Linda (August 8, 2013). "Paralysis, a qualitative study of people with Severe Myalgic Encephalomyelitis". Stonebird. Retrieved December 28, 2018.
  5. 5.0 5.1 5.2 "Hypokalemic periodic paralysis". Genetic and Rare Diseases Information Center. Retrieved March 26, 2022.
  6. 6.0 6.1 6.2 6.3 Segal, Michael M.; Jurkat-Rott, Karin; Levitt, Jacob; Lehmann, Frank. "Hypokalemic Periodic Paralysis Owner's Manual". Retrieved March 26, 2022.
  7. 7.0 7.1 De Becker, Pascale; McGregor, Neil; De Meirleir, Kenny (December 2001). "A definition‐based analysis of symptoms in a large cohort of patients with chronic fatigue syndrome". Journal of Internal Medicine. 250 (3): 234–240. doi:10.1046/j.1365-2796.2001.00890.x.
  8. Carruthers, BM; van de Sande, MI; De Meirleir, KL; Klimas, NG; Broderick, G; Mitchell, T; Staines, D; Powles, ACP; Speight, N; Vallings, R; Bateman, L; Bell, DS; Carlo-Stella, N; Chia, J; Darragh, A; Gerken, A; Jo, D; Lewis, DP; Light, AR; Light, KC; Marshall-Gradisnik, S; McLaren-Howard, J; Mena, I; Miwa, K; Murovska, M; Stevens, SR (2012), Myalgic encephalomyelitis: Adult & Paediatric: International Consensus Primer for Medical Practitioners (PDF), ISBN 978-0-9739335-3-6