Elevated CSF – What else could it be??

I am assuming that by your CFS level you are referring to your protein level in your spinal fluid after a spinal tap or lumbar puncture? If that is the case, then your protein level is very elevated & you probably do have CIDP. CIDP is usually dx by three things; elevated protein level in the spinal fluid, lack of relexes, & a sural nerve biopsy, which you will get of you go to the Mayo Clinic. They will also give you a myriad of tests to rule out everything under the sun (spinal nerve root biopsy, bone marrow biopsy, lip biopsy, CT Scans, MRIs, x-rays, etc…) Been there, done all that.

But just because IVIG doesn’t work doesn’t mean that you have ALS. For 40% of those with CIDP, IVIG doesn’t work at all, or not for awhile. I hate to suggest it, but I think they should try a few infusions of solumedrol (steroids), as that will probably tell you if you have CIDP. My neuro at the U of MN said that is now the first choice of treament for CIDP. In the beginning, when I was at my worst, & IVIG & PP did nothing to help me, steroids would give me a little something back. Also, there are many other treatments other than IVIG that can be tried. Please keep posting, & let us know how things go for you…
Blessings, Pam

Hi, I am a fellow Ne.resident. We lived in Battle Creek and have now retired to South Yankton Ne. If you find no satisfaction with you Dr., I go to an excellent Neurologist at Avera Sacred Heart Hospital in Yankton SD., and I highly recommend him. My legs were completely paralyzed and with IVIG treatments and Pt and OT I am starting to walk (on my walker) and making progress. If you want more info feel free to contact me. Be well, Regina

Hi Yankeebrad:

I think its really unfortunate and irresponsible that Mayo would throw such a horrible thing as ALS out to you before exhaustively ruling out every other possibility via a barrage of tests.

In many cases/variants of immune-mediated neuropathies, IVIG is not effective, or if it is, it is a very gradual improvement over time. Response to IVIG is not a way to diagnose CIDP.

I also have an asymetric form of CIDP, where weakness is much greater in one limb over the other. I have marked to severe muscle atrophy in areas. I do have sensory symptoms in addition to motor weakness. And, my CSF protein level has never recorded higher than 53. However, I do have oligoclonal bands in my CSF, and none in blood, which is a marker for MS – but, my NCV clearly shows demyelination and axonal damage, and conduction block, clearly indicative of the asymetric variant of CIDP. I’ve been seen by Neurologists at 2 top universities, and Mayo clinic in Arizona, and they’ve all come to same conclusion – asymetric multifocal CIDP.

These things are not that cut and dried…..people present symptoms differently, and respond to different treatments.

IVIG over the long haul is a stabilizing treatment for me, but provides no quick miracle turnarounds. IV Solumedrol was no good for me, and speeded up an exacerbation. I have found relief from plasma exchange as well.

I guess my message to you is to keep the doctor searching for answers – get the tests performed. If you don’t respond to IVIG, there’s steroids, immunosuppresents, chemotherapy, and even targeted proteins such as Rituxan.

I think the Mayo doctor was being lazy. Did they even perform an NCV (nerve conduction velocity?)

Yankeybrad

I don’t understand why the docs headed so quickly to ALS — did they give you an explanation? Does ALS come with elevated CFS proteins? Not responding to one of several known treatments surely can’t be the reason. Everything I’ve read indicates that your response to one treatment is not indicative of response to other treatments.

Helen

Yankeebrad, in answer to your question CSF protein is raised in trauma and infection, and CIDP. ALS used to be called Motor Neurone Disease because there is motor invovement only. The diagnosis of CIDP is a clinical (bedside) diagnosis confirmed by Lumbar Puncture, it has sensory and usually later motor features.
Treatment is initialy IVIG with about 40% success rate, then plasma exchange with another 40% then prednisone and later cytotoxic immunosupressant drugs. DocDavid

You talk a lot about your one leg, is there other involvement? The reason I ask is because CIDP tends to be very symmetrical (not always), but usually what is happening on one side of the body happens on the other side as well. How are your hands? CIDP is an illness that tends to affect the peripheral nerves first, that would be the feet & hands, & then acsending from there up the legs & up the arms. It also tends to have a lot of sensory involvement, or numbness as the motor functions are affected. Also, the EMG would usually show some involvement with the nerves being blocked somewhat.

Did you go to the Mayo in Rochester, MN? That is where I had all of my testing done (3 months inpatient), but I was in terrible shape, not totally paralyzed, but could not walk, use my hands & couldn’t lift up my arms & legs. They were good about getting a dx for me, but then basically sent me home saying there was nothing they could do for me. IVIG, PP, & steroids didn’t help me much. It was at a teaching hospital that I got the protocol that actually saved my life. So don’t give up & not have a correct dx, there are some excellent neuros out there, just need a referral to get in to see one.
Pam

Yankeebrad, what a dilemma. There is no specific test for ALS though muscle biopsy can help. I don’t think CSF protein is affected in ALS as it is a degenerative disease, not inflammatory like CIDP. I understood you had lost all tendon reflexes which means a polyneuropathy, if it is only one leg affected you have a mononeuropathy which is a very different group of diagnoses.
From your description, I would support CIDP as the diagnosis. DocDavid

DocDavid,

I just want you to know that it is great to have you back, it’s not the same around here without you and your help.

Jer