Type of Myotonia: Becker's

Country: England

Cindy,

Botox works by blocking acetylcholine receptors in the muscles. Some studies in the past have indicated that people with myotonia have lower cholinesterase enzymes, which is the enzyme that breaks down acetylcholine. This could cause that particular neurotransmitter to build up and result in contractures. So I'm assuming that's why the doctors are thinking it might be helpful.

However that's not really the root cause of MC and Botox has some very undesireable side effects. It can damage the muscles and cause permanent weakness where it's injected and the amount that's absorbed into the bloodstream can cause difficulty breathing and swallowing. I know this firsthand from a friend who has to get injections for eyelid spasms.

If you have an injured tendon or ligament (which I think you mentioned in the past) you can get contractures and fasciculations or twitching around that area. In the US they do a lot of prolotherapy to treat the injury and it seems to help the contractures let up, especially around the heel. You might want to do a search on prolotherapy and podiatry. Have you seen any podiatrists or just orthopedists?

Klonopin is an anti-seizure drug commonly used for epilepsy. Carbamazepine or Tegretol is in the same class and is usually the benzo prescribed for MC. Mexitil has pretty much replaced it as the drug of choice. It works a little differently but I don't think it's nearly as addictive. Another medication you might want to check out is Neurontin (Gabapentin). It's not generally prescribed for myotonia but you have some muscle pain and neuropathy that is beyond normal MC.

Jan

Type of Myotonia: Thomsen's MC

Country: US

Thanx for your reply Jan. About the possible undesirable effects of Botox. Permanant weakness would be welcomed in the gastroc area. But the swallowing and breathing bit bothers me. We already get that. Yikes...

Yes I saw and was politely dumped by 2 podiatrists. They said this was a job for my neuro. And the ortho is the one to suggest botox. I will check out
prolotherapy.

Right now, I am on tegretol and my dose was just incresed. My neuro is dead set against mexil. But I feel Klonapin would be more evil that mexil.

Klonapin is my magic drug. It turns every muscle into mush, and I feel like a brick wall has been released from my body. It makes me move like I am 20 years younger. More than likely, my feet will be cured too. (They were last time. Although they are many times worse now.) According to my last podiatrist, the huge and very tight gastroc, pulls on my heels, and whips my feet into equinus. Which then tears my tendons. Which then causes tremendous pain. I can't even stretch anymore.

I don't know if your doctors have ever mentioned gastrocnemius recession, but it might be worth looking into. I know some people with other neuromuscular diseases with contractures who have had good success with this type of surgery:

Gastrocnemius Recession

The degree of contracture and muscle tension you mention is pretty severe for MC. Have you ever had a head injury or brain infection? I had a lot of trouble with contractures and neuropathy after a closed head injury from a car accident and later after getting encephalitis.

Jan

Type of Myotonia: Thomsen's MC

Country: US

The ortho turned me away and was the one to suggest Botox. I was figuring on the recession. But nope. But if I could avoid surgery, I am game.

I am pretty certain, that it is from the MC. I do have hyper reflexia for as long as I can remember.

I thought it was cute that you asked me if I ever had a head injury. One time, my stiff legs took my head into a wall, and I was actually paralyzed, and my world went dark for a few minutes. I slurred when I talked for some time after that. I think head injury will be the demise of me.

Wow - any time you lose consciousness (not to mention getting paralysis along with it) that's a very significant head injury. It may have been minimized by your doctor at the time, but it certainly could be responsible for low level seizures and that's exactly the type of seizure activity that Klonopin helps so much.

It really is a bear to get off of but some people do a pulse dose where they only take it every couple of days. I have a friend with MS who uses the wafers when she needs a good night's sleep. She also has some pretty severe contractures.

Have you noticed any benefit from magnesium? There's a new magnesium spray that seems to help skeletal muscle tension - kind of like bathing in Epsom Salts but you just spray it on. I think you can get it from Swanson vitamins. That might help with the hyperreflexia (main causes of that condition are brain and/or spinal cord injury).

Jan

Type of Myotonia: Thomsen's MC

Country: US

Never told my doc. I was conscious, but couldn't move. And couldn't see anything but black. It was very scary. I screamed the first chance I got afterwards.

I once had a seizure during surgery. But that was the only time.

No never tried magnesium. The spray sounds interesting. I am willing to try anything.

I really think that my first podiatrist trashed my feet. When I went to see him I had just heel pain, and needed new orthotics. He put me into hard, flat orthotics when I was used to 2-3 inches of soft orthotics. OMG the pain!!! Then sent me off to stretch. The pain was so much worse after streching already painful feet. And I have never been the same.

The orthotic guy said that you should never take a person with tight heel cords, that is used to heels, and force them into flat hard orthotics. Major no no.

This worsening also happened over the winter months. Which likely contributed.

Cindy,

Maybe Lois will jump in here with some insights based on her experience, but I strongly suspect your hyperreflexia and foot problems could be related to your head injury to some extent. In fact the temporary paralysis you experienced could have been a spinal cord injury to the cervical portion of the spine which often occurs with head trauma. The myotonia would be worsened as a result because of the guarding response, but it is probably not the root cause.

Since you said you have had hyperreflexia as far back as you can remember, have you asked your parents if you had any significant falls or injuries as a toddler or young child? I was always smashing into things and falling when I was little when my legs would stiffen up - I don't specifically remember hitting my head but I sure went through Band-Aids.

Having been through brain injury therapy myself and also working as a therapist I have seen this type of reaction to head trauma many times. If you want to pursue it at all, you can contact your local or state brain injury association and ask about neurologists or physiatrists in the area who deal with head injuries.

The fact that you responded so well to the Klonopin is really a clue that there is a brain-mediated issue. Low level seizures don't always show up on an EEG but they might want to check that plus do an MRI just to see if there are any small brain or spinal cord lesions. After I had West Nile Virus I had painful contractures mostly at night in my feet and hands. They didn't pick up anything on the EEG until they did an auditory evoked potential where they play a sequence of clicking sounds. It set off a focal seizure both times and I couldn't stop shaking and all my muscles started cramping.

A good neurologist or physiatrist can differentiate the EMG myotonic discharges from other abnormal muscle responses. I wonder why your neurologist is so against prescribing Mexitil...

Jan

Type of Myotonia: Thomsen's MC

Country: US

Well, a lot of tests can be skipped. I likely had em all. You see, I have a c5/c6 radiculopathy that caused me a tremendous amount of pain several years ago. This was caused by degen arthritis. The neurosurgeon said suffer and let it be, and you likely won't need surgery. I wanted to smack him at the time, and missed 2 months work. But actually, he was right. Although I still have mild and fluctuating daily pain and numbness in my arms. Anyways, I had Xrays, Mri's, CAT's and myelograms. The only thing they found extra was severe axial alantal arthritis.

The neurosurgeon said I could have a mild myelopathy due to the hyper reflexia. But now that I think of it, I can remember having it clear back to my twenties. And it could have been longer. Which likely rules out the myelopathy from age related arthritis.

I had a brain MRI done due to some dizziness. The ENT thought I had meneieres. But the dizziness was actually from pansinusitis and eustacian tube dysfunction. The MRI only showed advanced aging or small vessel disease. Which is what it would show for anyone else my age.

Had another EMG of my arms, (for radiculopathy) done by a Physiatrist. And my neuro felt that his work was far inferior to my first EMG done at a neuros office.

Both neuros old and new, disregarded my hyper reflexia. And treated me for myotonia. My present neuro even said, make sure you tell them this Botox is for Myotonia.

The only reason I would get Klonapin, is because my old PCP once gave it to me for annoying leg problems, and it worked great on my whole body. (He thought maybe I had restless legs.) My CPK was elevated at the time too.

I get some nasty tight muscles. And pain to go with it. Every single day of my life, I am in pain from them. The pains get MUCH worse in the cold winter months

Sometimes there are benign cases of idiopathic hyper reflexia. So maybe it means nothing for me.

And Klonapin is a CNS depressant which would tone down muscles no matter the cause. Just like a CNS stimulant such as coffee makes myotonia worse. Klonapin actually took away the majority of my Myotonia. And turned my muscles into soft mush.

So as you can see, by this SMALL set of examples, I have certainly been around the block. All I really want is to feel better.

My feet were not this bad before Mr Podiatrist trashed my feet. Sure the heel cords were short to begin with. But I was used to heel lifts for nearly a decade. And he should have never of put me in flat hard orthotics.

Jan - I have been mulling this over, and I really can't answer it any better than you did. The reason I didn't reply right away is because of my over-riding reaction of anger, frustration and indignation at physicians who are too lazy and stagnant to think, and who are willing to latch onto an existing diagnosis, especially if it is an odd and not well understood one, and attribute all complaints and symptoms to it, even if it makes no sense.

I am trying to reel in my case manager/nurse advocate instincts, because all it makes me want to do is fight the injustice. I don't tolerate arrogance, ignorance or hubris very well, and I don't believe anyone should.

Anyway, Cindy, what you describe really does sound like sequellae of a traumatic brain injury with some cervical spinal trauma, as well. A well written letter of frustration and dissatisfaction with the level of physician commitment to finding the etiology of your problems can go a long way to getting you the tests and the specialists you need. But, to be effective, you need to write the letter to the Manager of Quality Improvement of your medical group, and cc your insurance company's QI/QM Manager, PLUS send a copy to your primary doctor and to the State Insurance Commissioner, if you feel that insurance constraint issues have any role in the neurologist's unwillingness to remove his brain from the formaldehyde it's been soaking in.

If you need any help, just e-mail me. Lois

Type of Myotonia: Myotonia Congenita, Becker's type

Country: USA