What is Primary Periodic Paralysis?

Woman prepare a healthy dish of fish, vegetables and salad.

NOT ACTUAL PATIENT

Primary Periodic Paralysis is a rare disease that leads to intense bouts of muscle weakness or temporary paralysis because of changes in blood potassium levels.

This disease is the result of a genetic mutation that is usually passed down from a parent. However, not everyone displays symptoms, so you might not be aware if one of your parents also has it.

There are four main types of Primary Periodic Paralysis:

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Hypokalemic Periodic Paralysis – This is the most common type of Primary Periodic Paralysis. Patients with Hypokalemic Periodic Paralysis have low potassium levels in their blood. It is estimated to affect 1 in 100,000 people.1

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Hyperkalemic Periodic Paralysis – Patients with Hyperkalemic Periodic Paralysis have high potassium levels in their blood. It occurs in around 1 in 200,000 people.1

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Andersen-Tawil Syndrome - Also known as cardiodysrhythmic periodic paralysis or potassium‐sensitive periodic paralysis. Alongside paralytic attacks, your heart may beat too fast or too slow. With Andersen-Tawil Syndrome, potassium doesn’t move in and out of the cells properly. Andersen-Tawil Syndrome only affects 1 in 1,000,000 people worldwide.2

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Paramyotonia Congenita – Also known as sodium channel myotonia, is estimated to affect 1 in 100,000 people worldwide.3 Paramyotonia Congenita causes mutations in the muscle sodium channel gene to prolong the channel’s opening, causing higher-than-normal muscle excitation. The overexcitation of muscles during brief exercise can trigger stiffness in the muscles and prolonged overexcitation can lead to fatigue-like weakness.4 The most commonly affected muscles are located in the face, neck and upper extremities, although it can affect the muscles used for breathing and swallowing, as well as muscles in the lower back.5

Symptoms typically begin in the first or second decade of life. Attacks of flaccid paralysis (weakness) usually occur on awakening in the night or in the early morning. Weakness may be focal or generalized, usually sparing facial and respiratory muscles, and lasting for hours (occasionally days) with gradual resolution. Frequency of individual attacks can vary from daily to a few episodes in a lifetime; attacks often decrease in frequency after age 40.6

Happy couple looking at house with arms around each other

NOT ACTUAL PATIENTS

What causes a Primary Periodic Paralysis episode?

Nutrition has been widely described as a trigger for Primary Periodic Paralysis, and by understanding your triggers and avoiding them, the likelihood of an attack is lowered. Dietary triggers vary based on the type of Primary Periodic Paralysis you have and your individual needs.7 No two patients are the same and your symptoms and triggers may be different.

Common triggers of
Hypokalemic Periodic Paralysis:

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Low potassium levels in your blood

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Low temperatures

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Anxiety

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Eating excessive salt

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Lack of exercise

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Consumption of glucosteroids or alcohol

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Anesthetic methods

To help manage Hypokalemic Periodic Paralysis it might help to:

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Eat a low-sodium diet

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Eat a low-carbohydrate diet8

Common triggers of
Hyperkalemic Periodic Paralysis:

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Potassium-rich food

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Post–exercise rest

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Low temperatures

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Emotional stress

To help manage Hyperkalemic Periodic Paralysis it might help to:

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Eat frequent meals rich in carbohydrates

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Avoid potassium-rich foods8

Common triggers of
Andersen-Tawil Syndrome:

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Both low and high potassium levels in your blood

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Low temperatures

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Exercise

Managing Andersen-Tawil Syndrome can be different for different patients. You might find these help:

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Mild exercise

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A low-carbohydrate diet9

Common triggers of
Paramyotonia Congenita:

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Physical activity10

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Repetitive movements10

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High potassium levels

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Sudden exposure to very cold weather

To help manage Paramyotonia Congenita it might help to:

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Eat a low-potassium diet

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Avoid skipping meals

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Eat carbohydrate-rich snacks between meals11


Do you think you might have Primary Periodic Paralysis?

Do these symptoms sound familiar?

Find out more about getting diagnosed

Have you already been diagnosed with Primary Periodic Paralysis?

Having a rare disease can make you feel isolated, stressed, and anxious. With ORMALVI™ (dichlorphenamide) tablets, you don’t have to face it alone.

Our product support program, Cycle Vita, is available to eligible* patients with Primary Periodic Paralysis.

Find out more about Cycle Vita

References

  1. Finsterer, J. (2008). Primary periodic paralyses. Acta Neurologica Scandinavica, [online] 117(3), pp.145–158. Available at: https://doi.org/10.1111/j.1600-0404.2007.00963 [Accessed 1 Nov. 2023].
  2. Medlineplus.gov. (2017). Andersen-Tawil syndrome: MedlinePlus Genetics. [online] Available at: https://medlineplus.gov/genetics/condition/andersen-tawil-syndrome [Accessed 1 Nov. 2023]
  3. GOSH Hospital site. (2020). Sodium channel myotonia (paramyotonia congenita). [online] Available at: https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/sodium-channel-myotonia-paramyotonia-congenita/ [Accessed 2 Jan. 2024]
  4. Muscular Dystrophy Association. (2017). Paramyotonia Congenita (Eulenberg Disease) – Diseases | Muscular Dystrophy Association. [online] Available at: https://www.mda.org/disease/paramyotonia-congenita [Accessed 2 Jan. 2024].
  5. National Organization for Rare Disorders. (2023). Paramyotonia Congenita. [online] Available at: https://rarediseases.org/rare-diseases/paramyotonia-congenita/#symptoms [Accessed 2 Jan. 2024].
  6. Venance, S.L., et al. (2005). The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain, [online] 129(1), pp.8–17. Available at: https://doi.org/10.1093/brain/awh639 [Accessed 1 Nov. 2023].
  7. youtube.com. (2023). 2023 PPA Conference: Anita Dharwadkar: Periodic Paralysis and Nutrition 005. [online] Available at: https://youtu.be/X6RWMp9sTJY [Accessed 30 Oct. 2023].
  8. Statland, J., et al (2017). Review of the Diagnosis and Treatment of Periodic Paralysis. Muscle & Nerve, [online] 57(4), pp.522–530. Available at: https://doi.org/10.1002/mus.26009 [Accessed 1 Nov. 2023].
  9. Venance, S.L., et al. (2005). The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain, [online] 129(1), pp.8–17. Available at: https://doi.org/10.1093/brain/awh639 [Accessed 1 Nov. 2023].
  10. National Organization for Rare Disorders. (2023). Paramyotonia Congenita. [online] Available at: https://rarediseases.org/rare-diseases/paramyotoniacongenita/#symptoms [Accessed 2 Jan. 2024].
  11. National Organization for RareDisorders. (2023). Paramyotonia Congenita. [online] Available at: https://rarediseases.org/rare-diseases/paramyotonia-congenita/#therapies [Accessed 2 Jan. 2024].

*Some areas of support may not be accessible to all patients. Eligibility criteria may apply to ensure compliance with all applicable federal and state requirements, and benefits may be limited to commercially insured patients only. For more detailed information about eligibility, terms and conditions, please contact the Cycle Vita team at +1 (888) 360-8482.