Si mi nivel de potasio en la sangre es normal, ¿eso prueba que no tengo parálisis periódica hipopotasémica?

No. Aunque tener niveles bajos de potasio en la sangre durante los ataques es típico de la parálisis periódica hipopotasémica, entre ataques, las personas con parálisis periódica hipopotasémica pueden tener un nivel normal de potasio en la sangre (con frecuencia en el rango normal bajo).

Los ataques de parálisis generalmente se desencadenan por la caída del nivel de potasio en la sangre. Tales fluctuaciones de potasio ocurren en todos, pero en personas con parálisis periódica hipopotasémica familiar, estas caídas de potasio pueden producir episodios de parálisis. Por ejemplo, una comida rica en carbohidratos da como resultado la secreción de insulina en la sangre, lo que provoca una caída del nivel de potasio en la sangre a medida que el potasio y la glucosa ingresan a las células. En personas normales, una caída de este tipo en el potasio en sangre no produce síntomas. Sin embargo, en las personas con parálisis periódica hipopotasémica familiar, la caída del potasio en la sangre a menudo desencadena un episodio de parálisis.

Los niveles de potasio en la sangre pueden permanecer bajos mientras el músculo se recupera de un ataque reciente. Durante un ataque, los músculos que se paralizan se hinchan y absorben potasio, lo que provoca una disminución del potasio en la sangre. Pero a medida que se resuelve la hinchazón, el nivel de potasio en la sangre vuelve al rango normal. En consecuencia, un nivel normal de potasio en la sangre después de tal recuperación no debe considerarse evidencia en contra de una persona que tiene parálisis periódica hipopotasémica.

Al evaluar los niveles de potasio en sangre, es importante tener en cuenta los tratamientos recientes. Haber tomado potasio recientemente o estar tomando un medicamento que reduce el potasio en la sangre, como la acetazolamida, tendrá efectos en los niveles de potasio en la sangre.

También es importante considerar otras razones por las que el potasio es bajo. Algunas personas tienen niveles bajos crónicos de potasio en la sangre, por ejemplo, debido a una enfermedad renal (p. ej., síndrome de Bartter). Pueden tener parálisis periódica "secundaria" o "sintomática" a pesar de no tener uno de los trastornos familiares del canal de parálisis periódica "primaria".

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If my blood potassium level is normal, does that prove I don’t have hypokalemic periodic paralysis?

No.  Although having low levels of blood potassium during attacks is typical of hypokalemic periodic paralysis, between attacks, people with hypokalemic periodic paralysis can have a normal blood potassium level (frequently in the low normal range). 

Attacks of paralysis are typically triggered by the level of potassium dropping in the blood.  Such potassium fluctuations occur in everyone, but in people with familial hypokalemic periodic paralysis, these drops in potassium can produce episodes of paralysis.  For example, a large carbohydrate meal results in secretion of insulin into the blood, which results in a drop of the blood potassium level as potassium and glucose enter cells. In normal people, such a drop in blood potassium produces no symptoms. In people with familial hypokalemic periodic paralysis, however, the drop in blood potassium often triggers an episode of paralysis. 

Potassium levels in the blood can remain low as muscle is recovering from a recent attack.  During an attack, muscles that become paralyzed swell and take up potassium, causing a drop in potassium in the blood.  But as the swelling resolves, the level of potassium in the blood returns to the normal range. Consequently, a normal blood potassium after such a recovery should not be considered evidence against a person having hypokalemic periodic paralysis.

When evaluating blood potassium levels it is important to take into account recent treatments.  Having just taken potassium or being on a drug that lowers blood potassium, such as acetazolamide, will have effects on blood potassium levels. 

It is also important to consider other reasons for potassium being low.  Some people have chronic low blood potassium, for example due to kidney disease (e.g., Bartter syndrome).  They can have “secondary” or “symptomatic” periodic paralysis despite not having one of the familial “primary” periodic paralysis channel disorders.

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Structure reveals mechanism behind periodic paralysis

Three states of the voltage-sensing domain (VSD) of a membrane-channel protein. In the normal state, the water-accessible space (magenta) does not extend through the channel, preventing sodium (gray spheres) from passing through. In the disease state, a clear passage allows sodium to leakthrough, resulting in muscle paralysis. In the “rescued” state, the binding of guanidinium (blue and yellow spheres) effectively closes the channel and blocks sodium leakage. The red sphere represents the location of the disease-causing mutation. The side-chain sticks represent the voltage sensors of the sodium channel.

Please continued Here:https://als.lbl.gov/wp-content/uploads/2018/09/381.pdf

Myopathy of Hypokalemic Periodic Paralysis

EREDITARY intermittent flaccid paralysis of skeletal muscle is at present classified into three types on the basis of the changes in the serum potassium during the paralytic attack. The hypokalemic form was the first to be clinically recognized, and is the most common of the three variants, more than 600 cases having been described in the literature by 1959.¹ The clinical manifestations of this disease will not be detailed here as many reviews are available.^2-5 Worthy of emphasis, however, is that permanent proximal muscle weakness may occur. This feature was first noted by Oppenheim⁶ in 1891 and was later thought to be a variant of spinal muscular atrophy occurring in conjunction with periodic paralysis.⁷ However, it has now become apparent that permanent myopathic weakness is not uncommon, and not dependent upon severe or repeated paralytic attacks.^8-10

Please Continued Here:https://jamanetwork.com/journals/jamaneurology/article-abstract/568933

What is hypokalemic periodic paralysis?

What is hypokalemic periodic paralysis?

Hypokalemic periodic paralysis is an inherited condition characterized by transient episodes of extreme muscle weakness and/or paralysis of the muscles in the arms and legs, along with a decreased serum potassium concentration during an attack. These episodes may last from a few hours to one day.

Very interesting, please continue here:https://www.patientslikeme.com/conditions/1540-hypokalemic-periodic-paralysis

Hyper- and Hypokalemic Periodic Paralysis Study

Hyper- and Hypokalemic Periodic Paralysis Study 

Periodic paralysis is a relatively rare, life-long disorder characterized by intermittent bouts of paralysis, progressive weakness, and diminished quality of life. Two drugs, acetazolamide (ACZ) and dichlorphenamide, have been prescribed to treat the disorder, however, dichlorphenamide is no longer available. In this multi-center, parallel, randomized trial researchers will compare the effects of dichlorphenamide vs. placebo in patients with hyperkalemic (HYP) and hypokalemic (HOP) periodic paralysis. The trial consists of two 9-week studies—one study will enroll persons with hyperkalemic periodic paralysis and the other study will enroll persons with hypokalemic periodic paralysis. Participants will be randomly assigned to one of two treatment groups: dichlorphenamide or placebo (an inactive substance). During the studies, participants will be asked to keep a daily diary to record the time, length, and severity of each episode of weakness (attack). The study coordinator will contact participants weekly to review the diary information. The 9-week phase will be followed by a 1-year open-label dichlorphenamide extension without placebo to determine the long-term effects of dichlorphenamide on the course of the disease and on inter-attack weakness. Duration of the trial for participants is approximately 65 weeks, including a screening phase to determine eligibility, the first 9-week treatment phase, and the one-year open-label extension phase. 

Please Continued Here:https://www.ninds.nih.gov/Disorders/Clinical-Trials/Hyper-and-Hypokalemic-Periodic-Paralysis-Study

HYPERKALEMIC PP VS HYPOKALEMIC PERODIC PARALYSIS

1. HYPERKALEMIC PERIODIC PARALYSIS MUTATED GENE SCN4A CHROMOSOME 17q DEFECTIVE CHANNEL SODIUM MODE OF INHERITENCE AUTOSOMAL DOMINANT 
2.  Hyperkalemic Periodic Paralysis • term hyperkalemic is misleading since patients are often normokalemic during attacks. • Onset first decade • M : F 1:1 • Attacks are brief and mild, usually lasting 30 minutes to 4 hours. • Weakness affects proximal muscles, sparing bulbar muscles. • Attacks are precipitated by rest following exercise and fasting. 
3.  In a variant of this disorder, the predominant symptom is myotonia without weakness (potassium-aggravated myotonia). • The symptoms are aggravated by cold, and myotonia makes the muscles stiff and painful. • Clinically apparent myotonia is seen less than 20% of patients, but electrical myotonia may be found in 50-75%. 
4. Pathophysiology  In hyperKPP, Na+ channels fail to inactivate and prolonged openings and depolarization result.  Increased extracellular K+ levels worsen the inactivation of Na+ channels.

Please continued Here: https://www.slideshare.net/laxmikantjoshi2/hypokalemic-periodic-paralysishpp

Hypokalemic Estimated.

hypokalemic periodic paralysis is estimated to affect 1 in 100,000 people. Men tend to experience symptoms of this condition more often than women.

If a genetic disorder runs in my family, what are the chances that my children will have the condition?

When a genetic disorder is diagnosed in a family, family members often want to know the likelihood that they or their children will develop the condition. This can be difficult to predict in some cases because many factors influence a person's chances of developing a genetic condition. One important factor is how the condition is inherited. For example:

• Autosomal dominant inheritance: A person affected by an autosomal dominant disorder has a 50 percent chance of passing the mutated gene to each child. The chance that a child will not inherit the mutated gene is also 50 percent (illustration). However, in some cases an autosomal dominant disorder results from a new (de novo) mutation that occurs during the formation of egg or sperm cells or early in embryonic development. In these cases, the child's parents are unaffected, but the child may pass on the condition to his or her own children (illustration).
• Autosomal recessive inheritance: Two unaffected people who each carry one copy of the mutated gene for an autosomal recessive disorder (carriers) have a 25 percent chance with each pregnancy of having a child affected by the disorder. The chance with each pregnancy of having an unaffected child who is a carrier of the disorder is 50 percent, and the chance that a child will not have the disorder and will not be a carrier is 25 percent (illustration).
  
  
  Continue Here: https://ghr.nlm.nih.gov/primer/inheritance/riskassessment

Información sobre los niveles anormales de potasio que los pacientes tienen con (HypoKpp)

Le doy información sobre los niveles anormales de potasio que los pacientes tienen con (HypoKpp)

La información que estoy poniendo se obtiene de muchos pacientes que tienen (HypoKpp) de todo el mundo que tienen el mismo problema con los niveles de potasio en la sangre.

Cuando obtenemos la sangre para saber el nivel de potasio y potasio sale con niveles normales como 4.0 4.5, pero tenemos ataques de parálisis, con niveles de potasio que son normales para los médicos, y que confunde a los médicos y las personas que están a nuestro alrededor.

Somos conscientes de que no todos los pacientes con (HypoKpp) responden de la misma manera a los tratamientos que realizan los médicos y que a veces los medicamentos no funcionan para todos, y que hay casos en los que los pacientes con (HypoKpp) Medicamentos lo hacen bien.

En pacientes con parálisis periódica no es necesario que el nivel de potasio caiga fuera del rango normal para que se desencadene un ataque: pacientes con HypoKpp si el nivel de potasio es normalmente alrededor de 4, entonces cualquier cambio por debajo podría causar un ataque.

3.5 está en el extremo inferior de la normalidad en todos los pacientes. Lo que es importante tener en cuenta es que, en el tipo de parálisis periódica, el potasio se desplaza fuera de la sangre, este es el nivel más bajo y, a medida que el ataque comienza a mejorar, los niveles aumentarán.

Los médicos están buscando el potasio disponible en el torrente sanguíneo. La parálisis periódica ocurre a nivel celular: puede tener gran cantidad de potasio en la sangre, pero si los canales iónicos no funcionan correctamente, los músculos seguirán débiles. Esa es una gran diferencia en el pensamiento del doctor,
Los doctores asumen que dado que el potasio en la sangre no es bajo, la Parálisis Periódica no puede ser la respuesta

Un  ejemplo (el tanque de gasolina puede estar lleno pero si su bomba de combustible no funciona, su vehículo no arrancará).

Los pacientes que tienen (Hypokpp) necesitan que el número de potasio sea alrededor de 4.5 *.

Los pacientes con niveles de potasio alrededor de 3.0 a 3.5 * están en parálisis total.