Successful treatment of periodic paralysis (channelopathy) with coenzyme Q10

[pattismith]

Acta Myol. 2016 Oct

Successful treatment of periodic paralysis with coenzyme Q10: two case reports
Yuwei Da,

1 Lin Lei,1 Karin Jurkat-Rott,2 and Frank Lehmann-Horn

3

Abstract
Primary periodic paralyses (PPs) are autosomal dominant ion channel disorders characterized by episodic flaccid weakness associated with variations in serum potassium level. The main prophylactic therapy of choice for PPsis carbonic anhydrase inhibitors that are not always effective. In this report, we described two PP patients who were successfully treated with coenzyme Q10. They remained asymptomatic since initiation of treatment, which may be associated with promotion of energy synthesis, anti-oxidant activity, influence of the fiber type composition and regulation of the expression of gene. To our knowledge, this is the first report of primary periodic paralyses which have been successfully treated with CoQ10. More observations need to substantiate this clinical finding in PPs.

 

[pattismith]

in fact, the first case was rescue by CoQ 10 and the second by Idebenone!


Case 1
A 29-year-old male had a history of several recurrent attacks of limb weakness since the age of 19. The frequency of episodes was at least once a month in the past year and increased to once a week in the last half a year. Severity of weakness varied ranging from mild weakness of lower limbs to tetraplegia. Predisposing factors included poor sleep and exposure to cold conditions. Ictal serum potassium blood level during an attack was normal and potassium supplementation was ineffective. Results of routine hematological and biochemical tests including thyroid function were all normal. Molecular genetic analysis revealed the R675Q mutation in the sodium channel encoded by the SCN4A gene associated with PPs (2). Since prophylactic medicine was unavailable in our hospital, he was only treated ex adiuvantibus with CoQ10 – 10 mg three times a day – to improve the energy metabolism of muscle. Surprisingly, the patient did not develop any episodes of paralysis during three months of treatment, even when exposed to the previously known triggers.

Case 2
The second case is a man of 50 years old who complained of paroxysmal generalized muscle weakness accompanied by myalgia and stiffness since he was 37. Episodes occurred once or twice a month averagely during the past one year and worsened in the latest weeks, up to nearly twice a week. The blood potassium level during episodes was as low as 2.8 mmol/L and potassium intake could alleviate paralysis. There was a positive family history whereby his father and two of his siblings had similar but less severe attacks. Clinical examination between episodes and thyroid function were normal. The result of a long exercise test was positive. He was diagnosed with HypoPP despite gene tests for CACNA1S and SCN4A were negative, which account for approximately 10% of HypoPP cases (3). In light of the possible therapeutic effectiveness of CoQ10 with little side effect, he was administrated tentatively idebenone 30mg three times a day, which is a synthetic quinone similar to CoQ10. Similarly, the patient responded well without any episodes during a one-month treatment.

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Discussion
Periodic paralysis is a channelopathy related to abnormal sarcolemmal excitability with limited drugs. We incidentally observed that CoQ10 significantly reduced frequency of episodes in two PP patients without knowing its exact mechanism of action. However a variety of physiological functions of CoQ10 may explain its effect: 1) CoQ10 serves as an electron carrier between respiratory chain enzymes, facilitating cellular energy production and activating the Na+-K+ exchange pump, which is a central target for regulation of cell membrane excitability (4, 5); 2) acting as an antioxidant, CoQ10 may protect muscle from a Na+ overload in muscle fibers, observed in muscle biopsies from PPs patients, and also increase the mitochondrial production of reactive oxygen species (6, 7); 3) CoQ10 can affect the muscle fibers for a long-term by increasing the percentage of type 2 fibers in vastus lateralis muscle (8). Type 2 fibers contain more Na+-K+ exchange pump and atrophy of type 2 fibers predominated over type 1 in muscles of PPs patients (9, 10). Therefore, CoQ10 may play a protective role from episodes by expanding overall content of Na+-K+ exchange pump in skeletal muscle. Furthermore, CoQ10 functions as a major skeletal muscle gene regulator, affecting the expression of a large number of genes (8). This is the first report of primary periodic paralyses which have been successfully treated with CoQ10. More observations need to substantiate this clinical finding in PPs.

 

[Mick]

he was only treated ex adiuvantibus with CoQ10 – 10 mg three times a day – to improve the energy metabolism of muscle

The dose that worked is ridiculously low. Surely it is for a muscle disease. So what I suspect, what actually seems to have happened there...
The disease exhausted the energy reserves of the muscle tissue (= coenzyme Q10). So even a small quantity stopped the symptoms. I wonder how much strain can this patient really withstand, ie. can he perform strenuous exercises, can he/she push the limit...

I wish I could get better on such a low dose...

 

[Gingergrrl]

@duncan (just in case the info is helpful) and you find the most interesting studies @pattismith!

 

[Mick]

So perhaps I might wanna try idebenone.

Can anyone recommend a reliable and affordable source in the US (where they ship abroad) or in Europe?

I try to google it out but I can't find a source that is reliable (or inexpensive for that matter, and these less expensive seem completely unreliable; I wouldn't also go for Chinese sources).