Due to my full body muscle dystrophy, my neuro doc has done extensive bloodwork. The only thing to come back abnormal was ga65 which is an indicator for stiff man (I am stiff but not sure if this is correct) so the tx he wants to do is an iv of retuxemb..we already tried ivig. Just not sure what to do...


Thoughts?

Debbie

The best thing you can do is go with the proposed treatment. There isn't a cure for Stiff Person Syndrome or the variant Stiff Limb Syndrome and there is no clarity about the cause. It looks as though they may have multiple causes. The treatment they have suggested has shown some good results for long term symptom control/management. So have long term, large doses of muscle relaxants ,IVIG which you have had and some of the usual drugs like gabapentin and anti anxiety drugs. Specialist physio can make a massive difference to some people. To some extent it depends which stage of the disease you are in. Ultimately everything they do is about slowing progression to try and minimise the severity of the symptoms as long as possible. For many people the drugs give them good long term symptom control and halt any progression for a long time.

My neurologist believed I had SPS but then changed his mind. Oddly enough, I have an acquaintance who was diagnosed with it at the same time as I was told they didn't think I had it. It's a crappy diagnosis to get but if its what you have then at least you know and can start to move forward.

Good luck with the treatment.

Thanks for your response. Guess I am. Unsure about the diagnosis, my biggest problem is muscle atrophy, but my back and neck r very stiff, at
This point. I have to try something to stay in the game..my body is wasting away ..I truly believe it is all just spread of the rsd (atypical)

Deb

Debbie

Why are you so doubtful about the diagnosis of SPS? Is is because you don't want to have yet another crappy diagnosis? Is it that you don't think much of the doctor who diagnosed you? Is it perhaps fear? Is it that you don't think you meet the profile for SPS?

Any of these would be more than understandable. I just wonder why you are not inclined to believe them in this new diagnosis. How sure have they said that they are about the diagnosis? Could you perhaps ask them to sit with you and explain slowly why they have come to the diagnosis and exactly how sure they are. If you thing they are just lost and this is clutching at straws then I could see why you are reluctant to take more treatments.

There is some decent information out there on the web about SPS although its very rare and poorly understood so you can imagine what that means! If there is anything else I can do to help then please just ask me. I had a clueless neurologist so I always am concerned that my SPS rule-out could have been incorrect. I share a lot of the SPS symptoms hence why they suspected it but they weren't able to do a lumbar puncture due to my movement problems so that meant they could never do all of the necessary tests.

the only reason he said stiff man is because of an elevated GAD 65 and of course some of my symptoms. I guess I am unclear if an elevated gad 65 definitely confirms stiff man because I had that in previous blood work and he said i may have stiff man....i hope it is sms as then there is a possible tx.

Thanks for your support

Debbie

It all seems a bit unclear as to whether there is certainty about the diagnosis or not. Maybe you could try and get them to establish once and for all how certain they are or whether there is anything else they think it could be.

I don't think I would be hoping it was SPS though - I know it feels good to have answers rather than having no clarity about what is wrong but SPS is a pretty nightmarish diagnosis and the treatment options are very limited. In the later stages, there is no effective treatment, care is palliative and it can progress to cause death. You just have to hope you don't progress and the symptom controlling medication is effective for you.

I'm not trying to be the merchant of doom, just honest. Having researched SPS because I was thought to have it, I know how bad it can be. They never confirmed what tests they did on me and the neurologist was clueless so there is a slim chance I could be living with it and not even know! I'm not into giving out sugar coated platitudes but I do sincerely hope that you get an improvement from the latest treatment they are offering. There is some decent info on the web about SPS and you might find it is helpful to do some reading so that you know what other potential medications could help.

First off, wanted to tell you that the proper name for this disease is stiff person syndrome, not stiff man disease. I am female as are many ppl who have this disease. It was once called stiff man but it was changed bc of this.
If your doc is calling it stiff man disease, that suggests to me he may not be very knowledgeable of it. I was diagnosed by a spinal tap. If I were you, I would kindly ask your doctor if he has ever treated this disease this before bc it is very rare, very serious and you need an experienced doctor. I had the blood test and it came back a weak positive, not to mention the symptoms I'd been having. But I'd never heard of this disease before. I also got a second opinion but it wasn't for lack of not believing the first doctor who diagnosed me with it.
Before I went on a med like the one you mentioned, I would want to be 100% sure I had it and the best way to do this is to have a spinal tap. But SPS doesn't generally start with full body muscle symptoms, unless you've had it for years and I would think it would have been diagnosed before it reached that level of severity. . There are a lot of diseases that that could be but then again if you have the GAD65 antibodies, that is usually an indicator of SPS.
I'm not sure what you mean when you say you tried IVIG and it "didn't work". IVIG is used mainly to slow the progression down and it is highly unlikely that you would (or a doctor) be able to tell if it is working or not for a very long time.
There are also several diseases that might show up in a spinal tap that may not in labs. But if this doctor is experienced treating this, then you need to get busy treating it! It will progress and believe me, you do NOT want it to. I know from experience that it is very painful and takes so much from your life that you want to find a treatment that works and asap. I'm not your doctor or a doctor so if he seems to know what he is doing then do what he thinks you should. I wouldn't stick with him if he has never treated it before. The doctor that found mine had to refer me to a doctor that had more experience with it. He told me that and I know he was doing what was best for me.
Good luck. I can't tell how old this post is so if you've already resolved all of this, sorry and the best to you.

If you are still connected to this post, I feel SO MUCH for you with this diagnosis. I am writing because I tested a low positive for the GAD65 antibody, am already on IVIG for Myasthenia Gravis, and supposedly because of this have a crazy IGG ratio. I was referred to the SPS Clinic at Hopkins, because I have been having terrible, increasing spasms for over a year now. Of course Hopkin knows their stuff, but your case here perked my interest. Were you diagnosed by spinal tap? Everything else other than GAD in blood and IGG, and IGG ratio in spinal fluid looks great, yet I have maxed out my baclofen dosage, and the later I get in my IVIG cycle, the worse the spasms are. This IGG stuff has been blamed on Myasthenia Gravis. Does any of this sound alarming to you? All my best wishes that you are able to get some relief.