Devic’s Syndrome: Close to a Cure?

Devic’s Syndrome:  Close to a Cure?
by Wise Young, PhD MD
W. M. Keck Center for Collaborative Neuroscience
Rutgers University, Piscataway, New Jersey 08854-8082
18 December 2008

Devic’s syndrome was first described in 1870 by Sir Thomas Allbutt (what a name to have had as a kid!) who pointed out an association between myelitis and optic nerve disorder.  In 1894, Eugene Devic and his student Fernand Gault described 16 patients who had lost vision in one or both eyes and developed spastic weakness, sensory loss, and incontinence.  For many years, Devic’s syndrome was just one of several variants of multiple sclerosis that presented with relapsing optic neuritis followed by spastic weakness and sensory loss.  The other name of the condition was neuromyelitis optica (NMO).

A major breakthrough came in 2004 when a specific marker NMO-IgG was found for the disorder [1].  IgG stands for immunoglobulin (a kind of antibody).   NMO-IgG is a autoantibody (an antibody against a protein in one’s own body) and turned out to target aquaporin-4 (a protein that is responsible for water channels in cells).  The identification of NMO-IgG as a marker for Devic’s syndrome allows the disease to be distinguished from other autoimmune diseases, i.e. myasthenia gravis [2], systemic lupus erythematosus [3], necrotizing myelopathy [4], paraneoplastic myelopathy [5].  More important, it has allowed the creation of animal models and the study of the mechanisms of the disease [6].

Aquaporin-4 is a water channel that plays an important role in the blood brain barrier and astrocytic function.  Devic’s disease is now classified as an “autoimmune channelopathy” disease [7].  With that recognition, the treatment of the disease has become more rational.  While intravenous high-dose glucocorticoids is recommended for acute relapses of the condition, clinicians are using rescue plasmapheresis for severe, progressive, and steroid-resistant cases.  In between attacks, immunosuppression with azathioprine or mycophenolate mofetil is recommended for mild conditions and rituximab for those with more recent severe attacks [8].

Much progress has been made in immunoablative therapies.  In Hong Kong, Mok, et al. [9] recently described the use of immunoablative cyclophosphamide to treat refractory lupus-related neuromyeltis optica.  This approach uses a chemotherapeutic agent (cyclophosphamide) to kill a significant part of the immune system, in the hopes that it will eliminate those cells in the immune system that are producing the particular antibodies.  When the immune system reconstitutes itself from the remaining cells, the auto-antibodies are sometimes eliminated.

An alternative approach may be immunoablation followed by umbilical cord blood transplants.  This approach will replace the immune system with normal hematopoeitic cells from cord blood.  This would allow more intensive immunoablation and increase the probability of ablating the auto-immune cells.  If autologous cord blood is available, this would be the least risky.  Haller, et al. [10] used autologous umbilical cord blood infusions to treat type 1 diabetes, another autoimmune diseases.  However, heterologous cord blood can also be used.

Use of cord blood or bone marrow transplants have now been successfully to treat many patient with autoimmune diseases.  According to Burt, et al. [11], 26 reports from 1997-2007 indicated that 854 patients treated with immune-ablation and transplantation had less than 1% treatment related mortality (2/220 patients for nonmyeloablative, 3/197 for dose-reduced myeloablative, and 13% for intensive myeloablative regimens).  While no randomized trials have been performed, all the trials indicate potent disease-remitting effects.

In summary, for sufferers of Devic’s syndrome, effective treatments are becoming available.  The identification of a specific marker NMO-IgG has not only eased the diagnosis of the condition but also provide a rational approach to therapy of the condition.  Not only are there therapies that can reduce the impact of relapsing attacks and prevent attacks but a cure may be possible by myeloablation and hematopoietic cell replacement.  For a long time, myeloablation had an unacceptable mortality of >10%.  However, with reduced-dose myeloablative regimens, the mortality is now approaching 1%, less than the mortality of the diseases themselves.  Of course, eliminating the autoimmune disease does not necessarily restore function that has been lost, but regenerative therapies are being worked on.

References

  1. Wingerchuk DM and Weinshenker BG (2005).  Neuromyelitis Optica.  Curr Treat Options Neurol.  7: 173-182.  Link
  2. Furukawa Y, Yoshikawa H, Yachie A and Yamada M (2006).  Neuromyelitis optica associated with myasthenia gravis: characteristic phenotype in Japanese population.  Eur J Neurol.  13: 655-8. Link
  3. Jacobi C, Stingele K, Kretz R, Hartmann M, Storch-Hagenlocher B, Breitbart A and Wildemann B (2006).  Neuromyelitis optica (Devic’s syndrome) as first manifestation of systemic lupus erythematosus.  Lupus.  15: 107-9.  Link
  4. Okai AF, Muppidi S, Bagla R and Leist TP (2006).  Progressive necrotizing myelopathy: part of the spectrum of neuromyelitis optica?  Neurol Res 28: 354-9.  Link
  5. Jacob A, Matiello M, Wingerchuk DM, Lucchinetti CF, Pittock SJ and Weinshenker BG (2007).  Neuromyelitis optica: changing concepts.  J Neuroimmunol 187: 126-38.  Link
  6. Mueller S, Dubal DB and Josephson SA (2008).  A case of paraneoplastic myelopathy associated with the neuromyelitis optica antibody.  Nat Clin Pract Neurol 4: 284-8.  Link
  7. Lalive PH, Perrin L and Chofflon M (2007).  [Neuromyelitis optica/Devic’s syndrome: new perspectives].  Rev Med Suisse 3: 950-5.  Link
  8. Wingerchuk DM and Weinshenker BG (2008).  Neuromyelitis optica.  Curr Treat Options Neurol 10: 55-66. Link
  9. Mok CC, To CH, Mak A and Poon WL (2008).  Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica.  J Rheumatol 35: 172-4.  Link
  10. Haller MJ, Viener HL, Wasserfall C, Brusko T, Atkinson MA and Schatz DA (2008).  Autologous umbilical cord blood infusion for type 1 diabetes.  Exp Hematol 36: 710-5.  Link
  11. Burt RK, Loh Y, Pearce W, Beohar N, Barr WG, Craig R, Wen Y, Rapp JA and Kessler J (2008). Clinical applications of blood-derived and marrow-derived stem cells for nonmalignant diseases. Jama 299: 925-36. Link

Recommended Reading

Devic’s disease – Wikipedia, the free encyclopedia

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41 Responses to “Devic’s Syndrome: Close to a Cure?”

  1. Bonnie Lewarne Says:

    Our Son has recently been diagnosed with Devic’s Disease. Are any of the mentioned Treatments currently available. If so, where and who could we contact.

    thanks for any help you can give.

  2. Wise Young Says:

    Dear Bonnie, I sorry to hear about the diagnosis. I had originally written this in response to a person who had logged onto http://carecure.org asking about Devic’s Syndrome. Recent advances in use of umbilical cord blood to replace bone marrow has advanced considerably in the past few years. Unfortunately, there are not that many patients with Devic’s syndrome and therefore there have not been sufficient experience with the condition. However, over 6000 people worldwide get umbilical cord blood transplants each year. The indications are mostly for hematopoietic disorders and immunodeficiency syndromes. However, the treatment can be used for autoimmune disorders. I don’t know whether anybody is offering the treatment for Devic’s syndrome or MS in general. I will ask around to find out.

    Wise.

  3. jaima jansen hanson Says:

    our son has devic’s. we would like to be contacted if any information becomes available. He was diagnosed December 27, 2008 after a severe attack that has left him very much paralyzed and blind in his left eye. We are in duluth, mn.

    • Wise Young Says:

      I will post on CareCure and here if I come across any new information concerning the treatment of Devic’s and other autoimmune diseases. There should be major advances in the coming year. Several centers are now applying umbilical cord disease to treat autoimmune disease.

  4. maddelin Says:

    Hello,

    My dear cousin (like a brother) was diagnosed about 3 years ago with Multiple Sclerosis in our country Dominican Republic and has been getting treatment and terapy and gointo to doctors in Miami. Today (feburary 4, 2009) while in Miami a new doctor says it is not MS but Devics. We are desperate to know what would be the best treatment? Is there any posible cure?

    Anything would be great to know.

    • Judy Wong Says:

      I have been seeing a Doctor William Sheremata in Miami for the last 21 years. He has been treating me for Devic’s. I am pleased with his treatment. I was on imuran and prednisone and now I am tapering. The new treatment I am doing is called rituxan-b. I go for an infusion every six months, and pleased with the results. I have been paralyzed twice(recovered 100%), one or the other leg gets weak. I now walk with a cane. I was blind for three months. I lost the right vision and the left eye recovered.

      I hope this helps.

      • stephani Says:

        My sister was diagnosed in May 2009. She was totally paralyzed
        from her neck down. She is regaining feelings and can move her
        right arm on her own. Thanks for sharing. Your story supports the
        hope we hold. God bless!

      • melinda Mcclement Says:

        Hi,

        My 31 year old daughter has been diagnosed with Transvere mylitus and that now it will most likely turn into Devics disease. For the past 5 years she has complained about severe pain in her legs, sometimes back and neck. She shakes like a leaf, and has numerous other issues along with some psychological issues. I am trying to get her into Mayo. Her current dr. gives her pain meds, but that can cause other issues.

        So sorry you have. How old were you when you got, how did you know something was wrong, on a scale of 1-10 how bad is pain, what is the worst thing about this disease and how do you keep the faith to continue life as positive as possible.

        Thanks,

        Melinda

      • Suzie in Miami Says:

        I have had Devic’s for about 5 years now. I also am seeing Dr. William Sheremata at the University of Miami’s Miller School of Medicine.
        I was paralyzed also, but never blinded. Dr. S. gave me 4 infusions of Rituxan over the course of a month, since then, I can walk and run (with some pain of course). He also prescribed Imuran and Prednisone (which I hope I will eventually be able to taper off, because I got Diabetes from taking it).
        Dr. S. is good, but he is retired now. He still sees his loyal patients, but will not take new ones. If you call over there, I’m sure they can refer you to one of his colleagues.

  5. Brenda Green Says:

    I have been diagnosed with NMO and would like to know if there are are cures for this disease?

    • melinda Mcclement Says:

      Hi,

      Did you find out anything about cures? How old were you when you got, how did you know something was wrong, how painful is it 1-10, how do you keep a positive attitude?

      I have a 31 year old daughter who has had it for 5 years. She also has alot of other medical problems along with mental illness disorders.

      I don’t know what to do next.

      Thanks for any help or ideas.

      I hope you are feeling better.

      Thank you,

      Melinda

  6. Grace Mitchell Says:

    Hello,

    I have just completed reading your article and wanted to comment, and ask a question. Since the mortality rate for NMO is high in itself (Some estimates put it at roughly 33% within the first five years.) and since the mortality rate for myeloablative regimens is now approaching 1%, it would appear that the benefit of the procedure for NMO patients far outweighs the risk.

    That being said, because NMO is B Cell mediated, and the NMO IgG autoimmune antibody *appears* to be a pathological one, would it not be logical to assume that at some point, the patients body would once again begin to produce the NMO IgG?

    Thank you,
    Grace

  7. Dawn M Says:

    I’m guessing my line of thinking is too simplistic but I’ll ask anyway.

    My best friend has Devic’s. She has an appointment to see Dr. W in PHX in March. My sister-in-law is pregnant. Can my sister-in-laws cord blood be used for my best friend? If I have anymore children in the future, can mine be used?

  8. Cynthia Says:

    i just wanted to post a link to a Devic’s on-line support group for those people here who have questions.

    The people there are very supportive and you’ll find lots of information on projects that you, as a patient, can participate in

    http://groups.google.com/group/Devics-support?hl=en&pli=1

  9. diana williams Says:

    I have a 7yr old grand daugther who has devic’s, niamh lost the sight in the left eye when she developed devic’s at the age of 4 leaving her with avery little vision in the right.
    niamh’s mother as recently given birth to a son and at this time made arrangements to have the stem cells from the cord blood collected and stored with hope that they may one day benfit Niamh and possibly help restore some of her sight, she has been on azaothioprine for the last 18mths and as not sufferd from any relapse within the past year.

  10. MARIA Says:

    MY NEICE HAS BEEN DIAGNOSED WITH DEVICS DISEASE. SHE IS NOW 24 YEARS OLD AND JUST WENT COMPLETLY BLIND AS A RESULT OF HER CONDITION. CAN SOMEONE PLEASE HELP. IF THERE IS A SUPPORT GROUP IN BALTIMORE, MD. PLEASE CALL ME AT 449-559-2509.

  11. lisa Says:

    Hello

    I have devic . Please join the support group it is very helpful, informational and a place where we understanding each other.

    Thank you
    Lisa Lansdowne

  12. Rose Nedved Says:

    I was diagnosed in April 08. How long do I need to take the Imuran
    and Gabapentin? I feel that I am not improving.

    • Judy Wong Says:

      Hi Rose,
      I thought Imuran with prednisone works together. That is what I was taking for 21 years and had awesome results. I am now switching to rituxan-b to taper off the prednisone and no longer take imuran. Have you tried Imuran and prednisone together? To be able to take rituxan-b one must have been taking imuran first.

  13. April Duenas Says:

    I was diagnosed with Devic’s in December 2008,and would like to know if there was a support group available in The Hartford Ct area?

  14. How to Get Six Pack Fast Says:

    The style of writing is very familiar to me. Have you written guest posts for other bloggers?

  15. Mark Garrison Says:

    My wife was diagnosed with MS 7 years and and took Rebif for 6 years and then switched to Tysabri last year with improved results. She just found out today that she has Devic’s and not MS. Are MS drugs a possible treatment?

  16. Melissa Depina Says:

    I was diagnosed in April 1, 2008 with NMO. I’m currently taking Imuran and Prednisone for my conditon. I would like to know if there will ever be a cure for this disease..

  17. Patricia Wynn Says:

    Greetings to all, I am curious on what is being done about Devics disease now. I’m sorry that I can’t give a positive outlook at this point, but I’m interested in getting the word out and seeking to establish a forum in my late daughter’s name. Anyone interested please e-mail me. Perhaps I can at lease explain some syptoms and medication to enhance a production life until a cure is found.

    Thanks
    Patricia Wynn/Chelsea Wynn

    • Melissa C. Says:

      Patricia/Chelsea – do you have any websites that you could recomend or treatments available for Devic’s Disease – my husband was diagnosed with the disorder this week and we are just looking for us much information as possible. Thanks so much.
      – Melissa

  18. anne bukin Says:

    hi, my brother has been diagnosed with Devics. We are devastated. He went to bed one night and woke completely paralised from the chest down and blind in one eye. His sight has now returned, will he ever get back movement in his body?This happened two years ago.

    Thanks
    Anne from Manchester England

  19. paul a Says:

    Dr Young:

    Wife has refractory RRMS and contemplating Revimmune at J Hopkins if we can get in. Copaxone not working. Currently blind, poor gait, cognition issues, swallowing pbms and some cognition deficits along with a sucky immune system (CD4 150ish)

    What do u think?

  20. lindsay andino Says:

    im diagnosed recently 15 days ago with NMO, and treated more than 6 mth with avonex to MS. Im taking Imuran and Prednisone for my condition. how long? how take my body to response and fell me better? actually i cant walk. please anyone give me hope.

  21. karen windon Says:

    My 75 year old brother has been diagnosed with Devics disease, his legs are paralyzed, also has a catherter and has no control of his bowels, he is very depressed, as can be expected. is their no hope that he will ever improve any.

  22. SylviaCarter Says:

    I woke up 6-30-2008 paralyzed below the waist. I was diagnosed with TM and found to be NMO antibody +. Treated with massive doses of steroid and cytoxan, spent 6 weeks in rehab and able to sit pivot,but unable to walk or stand (wheelcaair bound). I am 71 years old and have multiple stable health problems. My rheumatologist says he fears another attack of TM or blindness and wants me on another med.I have tried cytoxan and 500mg bolus(IV) of medrol and had negative reaction to both. I am taperinng off prednisone (just down to 1- mg daily). How close to a cure?
    Under what conditions are people chosen for trials?

  23. Sylvia Carter Says:

    I woke up 6-30-2008 paralyzed below the waist. I was diagnosed with TM and found to be NMO antibody+. Treated with massive doses of steroids and cytoxan, spent 6 weeks in rehab, and able to sit pivot, but unable to walk or stand (W/C bound). I am 71 years old and have multiple stable health problems. My rheumatologist says he fears another attack of TM or blindness and wants me on aniother med. I have tried cytoxan and 500mg IV bolus of medrol and negative reaction to both. I am tapering off prednisone (down to 10 mg daily). How close to a cure? Under what conditions are people chosen for trials>

  24. karen windon Says:

    I would be interested in hearing from anyone that has Devic’s and any new breakthroughs. I have a neighbor that had transverse melitis when she was 20 years old and was paralyzed from the chest down, and after 9 years, she was able to walk again and appears perfectly normal.

  25. Karen Says:

    Hi
    In 1985 I was diagnosed with MS. Back then spinal tap and Baer,Vers were the tests that led my Drs. to their diagnosis. I was treated with Prednesone and ATCH. In 2 years time I was paralized and in a wheelchair and cathed. Fast forward to 2010. In Jan. I started having spasims in my arms & legs which were very painful. I was having black spots especially in my left eye. So I went to my Family Dr. and was refered to a Neurologist who did MRI’S of the brain & neck. They came back as no MS in the brain and 3 fuesed vertabrea’s in my neck. The Neuro. ordered a NMO test to look for markers. Has anyone who has been paralized for 25 yrs ever gotten ANY movement back? I go to see the Neuro again in May and we shall have our conclusive diagnosis. Please let me know ASAP.

  26. Mckenzie Says:

    I was diagnosed with MS 7 yrs ago, then about 2 months ago I lost sight in one eye followed by loss of sight in the other eye a few days later. My neuro did another MRI and determined that with my symptoms and the pattern of the lesions, I have Devic’s and not MS. He says he has never treated a patient with Devic’s and has to do research before he can offer and treatment options or advice from here. So, I am now sitting here “lost” and without treatment. I am in Idaho and don’t know if anyone else has any ideas or suggections?? My sight is now completely gone except for some shadows. I am leaving to attend blind school from July 7, 2010 til teh end of dec 2010 – so anything you can offer, would be appreciated. At this point, I am contemplating getting a new Neuro since mine seems to be at a stand still.

  27. Rose-Marie Coppola Says:

    As a musician I have been devastated as Devic’s has affected by hands and my walking ( prior to Devic;s I was a strolling Accordionist!)

    So far my vision has not been affected.

    I have been seen at the Mayo Clinic twice and at Johns Hopkins, Walter Reed and Bethesa Naval Hospital.

    I am searching for more help.

    Thank You.

  28. m.yousef Says:

    HI i have been diagnosed with Devic’s disease .. 2009 , i have weakness in my legs , bladder , bowels system. I was at hospital for 6 months . Q.E bham . Made a good recovery with an aid of a walking stick. i had an Plasma exchange. Was put on steriods to build me back up. self catherise still same no improvement. Taking aziathioprine to protect from another attack. ########rare condition with no cure….

  29. deborah lesko Says:

    any updates on cordblood helping sufferers with nmo?

  30. Melissa C. Says:

    My husband was just diagnosed with Devic’s this week – any support groups, websites or any info on this would be greatly appreciated.

  31. BeeBee Says:

    Would be nice if someone cared to reply, there are not that many questions here.

  32. Drey Says:

    My daughter was first diagnosed with MS in 1997 and was rediagnosed with Devic’s about 10 years ago – completely blind/other problems as well – there has to be a cure somewhere.

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