Re: dystonia secondary to cp - what more can be done

Posted By CCF MD mdf on December 06, 1998 at 23:30:42:

In Reply to: dystonia secondary to cp - what more can be done posted by Anne on December 06, 1998 at 18:08:29:






I am 18 yrs old and have mild cp due to post term delivery.  When I was around 9 yo both of my feet began to out toe.  It progressed to the point that I had 45 degrees of torsion due to bone malalignment and another 45 due to continuous muscle spasms.   At 13 I underwent bilat. tibia derotational osteotomies with internal fixation.  Besides the fact that I had many complications from the surgery, my feet now had internal rotation.  This was due to the fact that the orthopedic surgeon set my tibias with approx. 15-20 degrees of internal rotation.  He was not aware that I had dystonia because the neuro who did the presurgical consult did not recognize that dystonia played a role (though he actually specialized in movement disorders.  At this time my hands had also gotten much worse and I could barely write my own name.  There were tight cramps followed by periods of weakness in both hands.  Two years (and several neurologists later)a dr finally suspected dystonia.  By this time, thankfully, my hands were a good deal better (though still crampy with very slow handwriting).  I took various medicines (baclofen at 5mg made me very nauseated and dizzy, artane had the same effect, Zanaflex worked well but didn't last that long)before I found Dantrium (which I now take 200mg - 250mg day).  Initially it worked very well at 100mg but as my dystonia has gotten worse and I have built up a tolerance to it the effects have become much less pronounced.  I have also been taking Neurontin (2700mg/day)for about a year.  Last February I was involved in a small fender bender which caused the dystonia to spread to my neck.  The pain is severe in that I barely sleep at night.  I have received botox in my neck (the SCM along with 2 of the other muscles involved). The first month 150mg, the second 100mg (due to poss. swallowing problems, and the third 150mg.  Though it has had no effect on my neck, the Dr. says he will try it on my legs later this month.
Though I am grateful to be able to walk (& not have a life-threatening condition), I keep hoping something more can be done.  The dystonia has gotten much worse in my legs in the past year.  14 months ago I was able to walk with low heeled shoes, now I can only wear athletic shoes and have a very difficult time walking bare-footed.  The pain in my neck makes it difficult, if not impossible, to do many of the things I want to do.  I am in college and have worked very hard to complete prerequisites for nursing school.  I have been accepted to my first choice school and may even receive a scholarship.  Right now I need to know if there is ANYTHING else that can be done, as I do not want to have to give up my major due to a physical problem.  Any advice you can offer would be much appreciated.
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It sounds like you've had enough contact with neurologists that the distinction between spasticity and dystonia has been made clear. From your post, I can't really separate the two enough to know for sure about the dystonia, but in my experience many people with CP do have dystonic spasms and postures, so I'll take your word for it.
You didn't mention treatment with anticholinergics such as Artane (trihexiphenidyl) or benzodiazepines such as Klonopin (clonazepam). These are our first-line medical treatments. Baclofen, Zanaflex, and Dantrium are all useful for spasticity but we've found them not particularly helpful in dystonia.
You've had BoTox, which is the most effective (albeit temporary) treatment for dystonia in a limited distribution. The tricky thing is to be sure that the BoTox is injected into the correct group of muscles. THe only way to know that is to have a BoTox expert examine you and devise a plan for which muscles to inject. For the neck, EMG guidance is less important than an understanding of the pattern of dystonia and therefore which muscles are activated abnormally. You may have had a suboptimal pattern of injection, or you may have not received an adequate dose (less likely, if the SCM received 150 units).
BoTox is used for spasticity, too. Any situation in which reducing force of muscle contraction could help, you can think of using BoTox. It's really pretty good stuff. I think you should be sure you see an experienced BoTox practitioner and get a second opinion regarding the pattern of injections.
You are welcome to call 800 223-2273 and ask for neurology appointments at 4-5559. Tell them you want a movement disorders specialist. They'll set you up. I hope this helps. CCF MD mdf.