Need Help! Neurologist wants to modify my treatment plan
 

Hello All,

I could really use some help in deciding what to do and would welcome any thoughts, suggestions or experience in general or with the drugs my neurologist wants me to begin taking or similar diagnoses. The neurologist states that Aetna, will at some point, want to discontinue me using IVIg. At this particular time I am doing better, but may be still progressing. I am likely in one of my relapse/remit phases. I can not tell if it is my lower back causing some of the issues in my right foot or disease progression. Back to the story, the neurologist wants to start me on Imuran or Cellcept. He was skeptical of whether my disease is caused by an autoimmune issue, he is not the neurologist that gave me my diagnosis, maybe that is why he has been skeptical in the past, but now agrees that what I have appears to be autoimmune in nature. Not sure why he is so skeptical, both tests skin and paraneoplastic I have had point to an autoimmune issue. So he wants to treat it as such and in hopes remove me from IVIg. I think he is foreshadowing getting grief from Aetna.
At this moment, I can only walk about a mile before my feet start giving me so much trouble that I need to sit down, but I can ride a bicycle now. I have been able to lift weights again, very lightly. What do I attribute this to? I am now taking more of my original medication and added an anti inflammatory, which whatever I may have is certainly inflammatory because the anti inflammatory really helps. IVIg is also helping me as well by removing the low energy and flu like symptoms I had before and other things I probably don't realize. Again, I may also be in one of my relapse/remit phases.
My dilemma is whether I should add something that could cause me more issues, or help or try and stay the course I am on. The neurologist says if Aetna pulls funding for the IVIg, and I am not on Imuran or Cellcept then we will be up a creek with no paddle. He says this because this would allow us to come off the IVIg and see how I do on just the Imuran or Cellcept. He also claims there is some chance for reversal?

Clinical Diagnosis

Mr. Nervous1’s diagnosis is small fiber neuropathy (ICD 9: 357.4) with autonomic neuropathy (ICD 9: 337.1) related to an autoimmune disorder (ICD 9: 279.4). He is seropositive for an autoantibody (antibody against the self) which targets neuronal tissue: the Anti-Ganglionic (-3 subunit) Neuronal Acetylcholine Receptor antibody (3-AChR Ab). This antibody is well-established to cause a clinical syndrome of autonomic neuropathy. Mr. Nervous1 has clinical symptoms which support a diagnosis of autonomic neuropathy. Furthermore, he recently underwent skin biopsy procedure, which revealed significantly reduced nerve fiber density within distal sweat glands of the lower extremity, consistent with a small-fiber autonomic neuropathy.

Intravenous immunoglobulin (IVIg) is supported by strong evidence as an effective first-line treatment of several more common immune-mediated neuropathies: chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), Guillain-Barre Syndrome (GBS), and Multifocal Motor Neuropathy (MMN). The efficacy of IVIg has been also proven in some antibody-mediated paraneoplastic neuropathies. 1

Because 3-AChR antibody-mediated neuropathy is a rare condition, there have not been large, randomized controlled treatment trials. There have been multiple published case studies showing efficacy of IVIg in patients with autoimmune neuropathy due to 3-AChR antibody. 2, 3 I feel that the best next step in Mr. Nervous1’s management would be a therapeutic IVIg infusion, at a dose of 2 grams/kg total divided over 5 days.

References:

1. Elovaara, I., Apostolski, S., Van Doorn, P., Gilhus, N. E., Hietaharju, A., Honkaniemi, J., Van Schaik, I. N., Scolding, N., Soelberg Sørensen, P. and Udd, B. (2008), EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases. European Journal of Neurology, 15: 893–908.
2. Iodice V, et al. Immunotherapy for autoimmune autonomic ganglionopathy. Autonomic Neuroscience: Basic and Clinical 146 (2009) 22–25.
3. McKeon A, et al. Ganglionic Acetylcholine Receptor Autoantibody: Oncological, Neurological, and Serological Accompaniments. Arch Neurol. 2009; 66 (6): 735-741.
4. Vernino s, et al. Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies. New England Journal of Medicine 2000; 343: 847-55.

Medications

Current meds – (IVIg – 80grams/2days per month), diclofenac 75mg X 2/day, imipramine 25mg X 2/day, lyrica 25mg/day
Previous meds – (Enbrel, Humira – taken for psoriasis) lexapro, noritryptiline, cymbalta, advil, tramadol, neurontin, flexiril
Supplements – B12, B1, R Lipoic Acid, Magnesium

My History

I am 44 years old and have lifted weights and some form of cardiovascular for 23 years
I am in somewhat good health, 6’0 tall, weight 170, still exercise 3 to 4 days a week.
Autoimmune disease runs in family – Father has Addisons, Sister has Graves, Mother had IBS and liver problems, I have psoriasis and now Autoimmune Small Fiber Peripheral Neuropathy with Autonomic symptoms
Psoriasis first showed up in 1985
Symptoms of Neuropathy showed up possible as early as 2008, but Humira suppressed the symptoms.
Symptoms of the Neuropathy would come and go, almost disappear, but each time duration became longer. The symptoms have not remitted for 1 year now.
Stopped Humira in June of 2012 and symptoms increased dramatically
Started IVIg in November 2013 to current

Symptoms

Hands and feet burn, tingle, pins and needles sensation
Numbness of hands and feet
Tingling feeling in both legs
Tingling feeling in upper body
Lower lumbar Radiculopathy - Stenosis
Heat/Cold Intolerance
Lack of Energy
Constipation
Nervousness
Anxiety
Joint pain
Exercise intolerance
Neck Pain
Scapula Pain
Gait issues/Foot drop
Foot swelling
Muscle cramping causing walking issues along with foot pain
Slight muscle wasting
Weakness in legs

Hi.

I think you should see how the IVIG goes without the other 2 oral meds you mentioned. I don't understand why you have to start the Cellcept and Imuran while you are on IVIG? Isn't this something you can have at a later date? Apologies if I am mistaken as I am not familiar with all the rules of US healthcare plans. But I do prescribe to the idea that sometimes it's best to introduce one thing at a time and see how one goes??

Aussie :)

Ditto what Aussie said!

See how the IVIG goes. If the insurance takes is away, then you can try the Cellcept or other. Personally I don't think they will take it away with your documented Dx. I receive IVIG for CVID and Autonomic/peripheral neuropathy and no problems for the last year of getting it. Of course CVID is a primary immune deficiency so easy to get, but know many people that receive it for neuropathy only without problem. Insurance has settled into the idea that this is a recognized treatment protocol for neuropathy...with success.

But at this point (9 months later) you should know whether the IVIG is helping your symptoms. If so, then push to keep it. Of course not so easy to gauge whether it is slowing or halting progression.

Thanks Aussie,

I agree, how would you know what is helping, plus I am doing better at this time on my current regimen...

N1

Hello en bloc,

I wonder if they will take it away as well? If the doctor does not dink with the diagnosis, it should fly. He said that he has other patients that Aetna is giving problems and it is only a matter of time for me. I know the IVIg helps my symptoms and I do better when on it, of couse I do not what happens when I am off of it at this point. But, it appears that it has not really helped the neuropathy that much. I believe that it would take a alot longer than 9 months for the IVIg to do anything with the neuropathy. If re-myelination is occuring and nerves are re-growing, this would be a slow process.

Do you have noticeable symptom reduction related to neuropathic pain due to IVIg?

Thanks,
N1

The last year I was getting the IVIG primarily for the immune deficiency. We just now started ramping up the dose for neuropathy, so too soon to tell about it helping. It will take me (due to past reactions to IVIG) about 6 months to get up to proper neuropathy...they want to proceed slowly.

Hello en bloc,

What would you say is a dose of IVIg that will help with Neuropathy for a 170lb male?

Thanks
N1

The dosing for neuropathy is 2gm/kg. So 170 lbs is approx 80 kg, so your dose should be in the neighborhood of 160 grams (this is usually split over 2-5 days).

hello en bloc,

I believe this is correct, I receive 80 grams per day over 2 days.
I will double check...

Thanks,
N1

[B]Paraneoplastic Panel Results?[/B]
 

Hello,

I have just received my test results from the paraneoplastic panel and my Anti-Ganglionic Neuronal AChR * 0.07 H result has dropped from 0.09 H
So it would seem that we are on the right track with the IVIg therapy, but my question is this.
Is this a reasonable assumption based on the 10 months of IVIg therapy @ 80gms/ 2days per month?

en bloc? Ms D? anyone?

Thanks,
N1


labs done on 8/7/14
Mayo Referral Test Comment * SEE COMMENT
Acetyl Receptor Binding * 0.00
Acetyl Receptor Striational * Negative
ANNA1 Paraneoplastic Eval * Negative
ANNA2 Paraneoplastic Eval * Negative
ANNA3 Paraneoplastic Eval * Negative
Anti-Glial Nuclear * Negative
Anti-Purkinje Cell Cytoplasm 1 * Negative
Anti-Purkinje Cell Cytoplasm 2 * Negative
Anti-Purkinje Cell Cytoplasm TR * Negative
Anti-Amphiphysin * Negative
Anti-Collapsin Resp Med Prot 5 * Negative
Anti P/Q Type Calcium Channel * 0.00
Anti N-Type Calcium Channel * 0.00
Anti-Ganglionic Neuronal AChR * 0.07 H
Anti-Voltage Gated K Channel * 0.00
last clinical note 8/5/14