Kisho is an information platform, not a medical provider. Nothing on this site constitutes medical advice, diagnosis, or treatment recommendations. All content is aggregated from publicly available sources (including ClinicalTrials.gov, PubMed, FDA.gov, and Orphanet) and is provided for informational purposes only. Clinical trial eligibility, treatment decisions, and any health-related actions should always be discussed with a qualified healthcare professional. Kisho does not endorse any specific therapy, organization, or clinical trial. Terms of use · Privacy policy
Hypokalemic periodic paralysis (hypoPP) is characterized by episodes of muscle paralysis lasting from a few to 24-48 hours and associated with a fall in blood potassium levels.
No HPO annotations are available for this condition.
Large-scale studies of the natural history of primary hypokalemic periodic paralysis (hypoPP) have not been performed. Thus, knowledge of the natural history relies largely on personal observations and on individual cases that have been published with a retrospective description of the individual disease history. Pattern of attacks. The natural history and expressivity vary greatly over time. Frequency ranges from a single occurrence of a paralytic attack that may be triggered by exceptional circumstances (extreme physical effort, specific medical intervention) to spontaneous recurrent attacks of variable frequency (ranging from multiple attacks daily to less frequent attacks).
Hypokalemic periodic paralysis (hypoPP) can be a primary condition or a symptom of an overarching syndrome or disease . This GeneReview focuses on primary hypoPP resulting from a genetic ion channel abnormality.
HypoPP should be suspected in individuals who describe episodic paralytic attacks with the following symptoms and signs (Note: Strictly speaking, "periodic" is a misnomer, as the attacks do not occur at regular intervals; "episodic" is a better descriptor.):
Source: GeneReviews — "Hypokalemic Periodic Paralysis"
No approved treatments are currently available for hypokalemic periodic paralysis. The disease remains an area of unmet medical need.
To establish the extent of disease and needs in an individual diagnosed with hypokalemic periodic paralysis (hypoPP), the evaluations summarized in this section (if not performed as part of the evaluation that led to the diagnosis) are recommended.
During an acute paralytic attack
The frequency of consultations needs to be adapted to the individual's signs and symptoms and response to preventive treatment. Neurologic examination with attention to muscle strength in the legs should be performed, in order to detect long-lasting interictal weakness associated with myopathy. For those individuals who take acetazolamide the following parameters should be evaluated every three months: complete blood count, electrolytes, glucose, uric acid, and liver enzyme levels. Renal ultrasound should be performed annually.
Source:
2 clinical trials registered, 1 recruiting. Interventions under study include other interventions. Pipeline includes 1 NA. Research is primarily sponsored by academic and government institutions.
98 publications have been identified in PubMed for hypokalemic periodic paralysis. Research spans Case Report / Case Series (69%), Review / Meta-Analysis (10%), and Basic Science / Preclinical (6%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 68 |
Data assembled from 5 of 12 sources · Last updated Sep 18, 2026, 1:54 PM UTC
Program availability and eligibility requirements are set by each foundation. Contact them directly to learn more about your options.
Claim this page and your organization will be listed here for patients and families to find.
European rare disease database
Genetic and Rare Diseases Info Center
Source: GeneReviews — "Hypokalemic Periodic Paralysis"
The following signs and symptoms suggest a diagnosis other than hypokalemic periodic paralysis (hypoPP): • Associated sensory symptoms, including pain or tenderness • Sensory loss could suggest polyneuropathy such as Guillain-Barr syndrome. • Pain could suggest myositis; however, some individuals with hypoPP report paralytic episodes as painful. • Urinary retention or constipation, which may be observed in other causes of acute or subacute paralysis, but can occur rarely in hypoPP • Associated symptoms that suggest myasthenia or involvement of the neuromuscular junction, including: • Ptosis • Diplopia • Dysphagia • Dysarthria • Alteration or loss of consciousness • Abnormal movement • History of fever days before an attack, which could suggest poliomyelitis or other virus-caused paralysis • History of back pain days before an attack, which could suggest acute transverse myelitis • History of tick bite, which could suggest tick paralysis HypoPP is the most common cause of periodic paralysis. The four major differential diagnoses are normokalemic potassium-sensitive periodic paralysis (normoPP), hyperkalemic periodic paralysis (hyperPP), thyrotoxic periodic paralysis (TPP), and Andersen-Tawil syndrome (ATS) . Table 3. The Different Categories of Periodic Paralyses (PP) with Membrane Excitability Disorder and Associated Findings
HypoPP | NormoPP | HyperPP | TPP1 |
|---|---|---|---|
Main clinical features | Weakness episodes lasting hrs to days w/concomitant hypokalemia | Weakness episodes lasting hrs to days w/concomitant normokalemia | Weakness episodes lasting mins to hrs w/concomitant normo- or hyperkalemia |
Age at first attacks | Late in 1st decade or in 2nd decade | Late in 1st decade or in 2nd decade | 1st years of life |
Main triggers | Rest after exercise, carbohydrate rich meal, salt intake, stress, cold | Rest after exercise, carbohydrate rich meal, salt intake, stress, cold | Cold, rest after exercise, stress, fatigue, alcohol, hunger, changes in activity level, potassium in food, specific foods |
EMG: myotonic discharges | No | Some | Some |
EMG tests | Late decrement w/LET(Pattern IV, V) | Late decrement w/LET(Pattern IV, V) | Pattern IV, V |
Extramuscular expression | None | None | None |
Prevention of paralysis attacks | ACZ, DCP | ACZ, DCP | ACZ, DCP |
Source: GeneReviews — "Hypokalemic Periodic Paralysis"
Biomarker and diagnostic research for hypokalemic periodic paralysis has been reported in the published literature.
Assessment of respiratory status to detect those individuals who may have early respiratory failure
Measurement of serum potassium concentration
Cardiac electrophysiologic testing (EKG) to assess for life-threatening cardiac consequences of hypokalemia
Assessment for swallowing difficulty
Between attacks or in a currently asymptomatic individual
Neurologic examination to assess muscle strength in the legs
Measurement of the following thyroid functions (see Note):
Plasma thyroid-stimulating hormone (TSH)
Free thyroxine (FT4)
Free triiodothyronine (FT3)
In those with long-lasting interictal weakness, consideration of muscle sonography or MRI scan of muscles (e.g. thigh) to evaluate the extent of myopathy
Consultation with a clinical geneticist and/or genetic counselor
Note: (1) Hyperthyroidism may be a trigger for a hypokalemic episode in individuals with hypoPP; (2) thyroid function tests may help to distinguish between hypoPP and TPP in those who have a pathogenic variant in KCNJ18.
For a comprehensive summary of the management of hypokalemic periodic paralysis, see (full text). The principles of treatment are summarized in [, .]
Table 5.
Source: GeneReviews — "Hypokalemic Periodic Paralysis"
Avoid anything that can trigger paralytic attacks in the individual case, including the following:
Unusually strenuous effort
Excess of carbohydrate-rich meals or sweets
Cold
Stress/excitement/fear
High salt intake
Prolonged immobility
Oral or intravenous glucosteroids
Use of cooling, glucose and/or mannitol infusion, excessive sodium- containing fluids and certain anesthetics such as succinylcholine during anesthesia
Use of alcohol
Source: GeneReviews — "Hypokalemic Periodic Paralysis"
Recent studies in mouse models of hypoPP with both SCN4A pathogenic variants and CACNA1S pathogenic variants show that maneuvers to reduce the activity of the Na-K-2Cl (NKCC) co-transporter can reverse an acute attack of hypoPP and protect against an attack triggered by low K+ exposure . The beneficial effect is the result of biasing intracellular chloride to be low, which promotes hyperpolarization of the resting potential. The NKCC co-transporter is activated by hyperosmolarity (hence the importance of avoiding high sodium diet, dehydration, hyperglycemia and mannitol) and is inhibited by loop diuretics such as bumetanide . Search ClinicalTrials.
Source: GeneReviews — "Hypokalemic Periodic Paralysis"
2 trials found
Estimated prevalence: 1-9 in 100,000 (Uncommon).
Research summaries | 10 | 10% |
Laboratory research | 6 | 6% |
Disease patterns and progression | 6 | 6% |
New treatment approaches | 3 | 3% |
Testing and diagnosis research | 2 | 2% |
Clinical study results | 2 | 2% |
Other research | 1 | 1% |
Chanchykov V (2026). [PMID: 41562289](https://pubmed.ncbi.nlm.nih.gov/41562289/). *Muscle & nerve*. [Basic Science / Preclinical]
Kubota T (2026). [PMID: 41485931](https://pubmed.ncbi.nlm.nih.gov/41485931/). *Nihon yakurigaku zasshi. Folia pharmacologica Japonica*. [Review / Meta-Analysis]
Haider S (2026). [PMID: 41371188](https://pubmed.ncbi.nlm.nih.gov/41371188/). *Neuromuscular disorders : NMD*. [Review / Meta-Analysis]
Bossola M (2026). [PMID: 41954147](https://pubmed.ncbi.nlm.nih.gov/41954147/). *Acta Myol*. [Review / Meta-Analysis]
Balagopal A (2026). [PMID: 41798238](https://pubmed.ncbi.nlm.nih.gov/41798238/). *Indian journal of psychiatry*. [Case Report / Case Series]
Liu J (2026). [PMID: 41564807](https://pubmed.ncbi.nlm.nih.gov/41564807/). *Medicina clinica*. [Case Report / Case Series]
Ottu Para NK (2026). [PMID: 42099363](https://pubmed.ncbi.nlm.nih.gov/42099363/). *Cureus*. [Case Report / Case Series]
Yapijakis C (2026). [PMID: 41273566](https://pubmed.ncbi.nlm.nih.gov/41273566/). *Advances in experimental medicine and biology*. [Epidemiology / Natural History]
Gadalla K (2026). [PMID: 32644604](https://pubmed.ncbi.nlm.nih.gov/32644604/). *Unknown Journal*. [Review / Meta-Analysis]
Michel M L (2026). [PMID: 41869153](https://pubmed.ncbi.nlm.nih.gov/41869153/). *Cureus*. [Case Report / Case Series]