Results from the eculizumab trial are due to be released in the second half of this year, there is also a trial of anti IL-6 (Tocilizumab) being done in Japan on autoimmune diseases that I thought I had read included NMO patients and/or Japanese OSMS. Bumby/Jim

Nihon Rinsho Meneki Gakkai Kaishi. 2012;35(2):129-35.
Treatment of neuromyelitis optica.
Fujihara K.
Source
Department of Multiple Sclerosis Therapeutics, Tohoku University Graduate School of Medicine.
Abstract
Neuromyelitis optica (NMO) or Devic's disease is an inflammatory neurologic disease characterized by severe optic neuritis and transverse myelitis. Other features of NMO include female preponderance, higher onset age, severe functional disability, longitudinally extensive spinal cord lesions (longer than 3 vertebral segments), and oligoclonal IgG bands negativity. Brain lesions are not uncommon in NMO.
The relation between NMO and multiple sclerosis (MS) has long been a matter of controversy, but since the discovery of anti-aquaporin 4 (AQP4) antibody (NMO-IgG), an NMO-specific autoantibody, the clinical, MRI, and laboratory features that distinguish NMO from MS have been clarified. Anti-AQP4 antibody binds to the extracellular domain of AQP4, which is highly expressed in endfeet of astrocytes.
Recent neuropathological studies, analysis of CSF-GFAP levels during relapse and experimental studies strongly suggest that NMO is an anti-AQP4 antibody-mediated astrocytopathic disease and that T cell-mediated CNS inflammation is necessary to develop NMO. Also, IL-6 is remarkably elevated in the CSF and appears to regulate plasmablasts to produce anti-AQP4 antibody. Therefore, from the therapeutic point of view, depletion of anti-AQP4 antibody, suppression of T cell response to trigger relapse and anti-IL-6 therapy seem to be pivotal.
High-dose intravenous methylprednisolone is the first-line therapy for acute exacerbations of NMO. But plasma exchange should be started soon if corticosteroid is not efficacious. If untreated, AQP4 antibody-positive patients are highly likely to experience relapses within a year. Thus, immunosuppressive therapy (corticosteroids, immunosuppressants, rituximab) should be initiated without delay.
Preliminary results suggest that eculizumab, an anti-C5 monoclonal antibody, can also prevent relapse in NMO, Meanwhile, interferon-beta, a first-line disease modifying drug of MS, is not effective in NMO. Symptomatic therapy for pain, paresthesia, spasticity, dysuria and constipation which commonly occur in the chronic stage of NMO is also important to improve patients' quality of life.
PMID:
22576570
[PubMed - in process]
Free full text
LinkOut - more resources

http://www.ncbi.nlm.nih.gov/pubmed/22576570

Hi Grace and thanks, Sarah is now with Social Security, and there are not a program for Devic and when I asked for preventive medication they said it was very expensive. Now Sarah is at home, she is good as two weeks ago, but Im not sure it was a relapse. The doctors say its a relapse.



Thanks again,





2012/5/20  <

Hi Fredy,





All of us here take regular preventative/maintenance medications to hopefully prevent relapse. �Gabapentin and Tegretol (carbamazepine) are not preventative treatments but are used to treat symptoms only (neuropathic pain, tonic spasms, etc).




Some commonly used drugs to hopefully prevent relapse are the oral medications, Imuran (azathioprine) with or without prednisone, and Cell Cept (mycophenolate mofetil). �Some patients use monthly IV pulse steroid therapy. �Many of us here also use regular infusions. �For me, it's Rituxan (rituximab), for others it might be Cytoxan (cyclophosphamide), or it might be a combination that includes IVIG. �There are also patients here who use regular Plasmapheresis treatments as a means of preventing attacks.




Why hasn't Sarah been put on a preventative/maintenance treatment by her physician? �I can remember how ill she was with the onset of her disease. �Fredy, with very rare exceptions, all of the damage from an NMO attack is done during the acute neuro inflammatory event, and it can result in permanent and severe disability. �It's very important that you discuss a preventative therapy with Sarah's physician.




Please keep me informed.





Grace









-- 
Correo Alterno: 

Sorry it was at draft folder:

Hi,�she only takes gabapentina(1800 ) and carbamazepina (600), nothing�for relapse. How to know if its relapse or no, The Dr. say Devic is Active.

�
Thanks
�


�
2012/5/17  <
Hi Fredy,





It's been such a long time since I've heard from you. �I've thought about your wife often over the years. �Fredy, did her neurologist ever put her on preventative/maintenance medication? �If I am not mistaken, the last time that we spoke she was not using any drug therapy to prevent relapse.




Grace









-- 
Correo Alterno: 

Hello Grace
�
Im Fredy, Sarah's husband, she has devic since 2007. She is at hospital now, on Sunday 13 had a fever (38.1) and sometimes, now she has only a little weakness in legs, after analysis yesterdey her Doctor ordered methilprednisolone, at night she sweat. How to know if she has a relapse or not? Methilprednisona has negative effects?. Here only chek her bood and urine. She always has stomach pain or colic, now the pains were stronger,� can these pains and not a relapse the problem?
�
Thanks

-- 
Correo Alterno: 

-- 

You received this message because you are subscribed to the Google Groups "Devic's Support" group.

To post to this group, send email to [email address removed] />

To unsubscribe from this group, send email to [email address removed] />
For more options, visit this group at http://groups.google.com/group/devics-support?hl=en.

Hello Grace
�
Im Fredy, Sarah's husband, she has devic since 2007. She is at hospital now, on Sunday 13 had a fever (38.1) and sometimes, now she has only a little weakness in legs, after analysis yesterdey her Doctor ordered methilprednisolone, at night she sweat. How to know if she has a relapse or not? Methilprednisona has negative effects?. Here only chek her bood and urine. She always has stomach pain or colic, now the pains were stronger,� can these pains and not a relapse the problem?
�
Thanks

-- 
Correo Alterno: 

Hello Grace, Im Fredy Sarah's husband, she has devic since 2007. She is at hospital now, on�Sunday 13 had a fever (38.1) and sometimes,�now she has only a little weakness in legs, after analysis yesterdey�her Doctor ordered methilprednisolone, at night she sweat. How to know if she has a relapse or not? Methilprednisona has negative effects?. Here only chek her bood and urine. She always has stomach pain or colic, now the pains were stronger, �can these pains and not a relapse the problem?
�
Thanks
�
Fredy
�
�
�

I have visited several doctors in the past few weeks and when I mention NMO I have been rewarded with a blank stare and the question "What is NMO?"  Tonight I was hit by a bolt of lightning which gave me a jolt and an idea.  To help spread the word about NMO I am going to make copies of the attached article and take one or more with me whenever I have a medical appointment of any type.  I plan on distributing them to doctors, nurses, physicians assistants, or any other medical person whom I feel needs some basic knowledge about NMO.  I am sharing my plan in hope that others in the NMO Family might follow my example.  Just think of the results if 1000 of us could spread the word in this painless manner.  If you disagree, throw this away, but if you think it might help spread the word, turn your printer on!

Thanks in advance for your help,
Bob Hunter
I think it would be very exciting!
Sent from my iPad
On May 6, 2012, at 8:45 AM, [email address removed] wrote:
> Neuromyelitis Optica: An Antibody-Mediated Disorder of the

> Central Nervous System

> Jiwon Oh andMichael Levy,

>

> Department of Neurology, Johns Hopkins University School of Medicine, 600 N. Wolfe Street, Pathology 509, Baltimore, MD 21287, USA

>

> Correspondence should be addressed to Michael Levy, [email address removed]

>

> Received 7 July 2011; Revised 4 October 2011; Accepted 13 October 2011

>

> Academic Editor: Philippe Cabre

>

> Copyright © 2012 J. Oh and M. Levy. This is an open access article distributed under the Creative Commons Attribution License,which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

>

> 1. Introduction

>

> Neuromyelitis optica (NMO) is a recurrent inflammatory disease that preferentially targets the optic nerves and spinal cord leading to blindness and paralysis.

>

> In 1870, Allbut was the first to report a case of NMO [1], but it was Devic who described the disorder in detail,and summarized 16 cases in the existing literature in 1894 [2]. Based on this initial description, historically, NMO has been regarded as a severe, generally monophasic disorderof the optic nerves and spinal cord and was thought tobe a variant of multiple sclerosis (MS). A convincing body of evidence in the past decade has established NMO as a

> distinct disease entity from MS. NMO is now recognized as a recurrent disease that largely targets the spinal cord and optic nerves but can also affect the brain as well. NMO occupies a unique position in the spectrum of inflammatory

> central nervous system demyelinating disorders in that it is the only such disorder that has an associated disease-specific antibody, aquaporin-4 antibody (AQP4 Ab), or NMOIgG. Recognition of this antibody has been instrumental in elucidating the underlying pathobiology and in guiding treatment options for NMO.

>

> 2. Clinical Features

>

> The hallmarks of NMO include bilateral optic neuritis and longitudinally extensive transverse myelitis. Woman and African Americans are over represented in the US patient population. NMO is associated with the NMO-IgG biomarker, which targets the aquaporin-4 water channel on astrocytes. The humoral pathology of NMO lesions include IgG and IgM deposits and infiltration by granulocytes suggesting that the NMO-IgG may be involved in the pathogenesis of disease. This review of the recent NMO literature covers the clinical features, epidemiology, radiology and pathology of disease and includes discussion of the important basic science research work in the field.

>

> Clinical features attributable to locations outside of the optic nerves and spinal cord can also occur in patients with NMO. Hypothalamic-pituitary axis dysfunction can manifest as hypersomnolence, hyponatremia, hypothermia, hypothyroidism, and hyperprolactinemia [8]. In addition, confusion, abrupt changes in level of consciousness, cortical blindness, and imaging findings suggestive of posterior reversible encephalopathy syndrome (PRES) have also been reported [9].

>

> The clinical course of NMO historically took one oftwo forms: monophasic or relapsing, with relapsing forms comprising approximately 80–90% of cases. However, after an index event, the distinction between monophasic and relapsing NMO is often difficult to make since relapses can occur many years after an event. In the vast majority of cases (∼80%), a relapse occurs by 2-3 years after the index event[3, 10].  Clinical features that may predict a relapsing course of disease include older age, female gender, less severe motor impairment with the initial myelitis event, and evidence of systemic autoimmunity [3].

>

> Clinical attacks typically progress over days, with varying degrees of recovery seen in the ensuing weeks to months. Recovery is usually incomplete, and most patients sustain residual disability, which increases with subsequent attacks [3]. Factors predictive of mortality in patients with relapsing NMO include the presence of other systemic autoimmune disorders, higher attack frequency in the first two years, and poor motor recovery following the index myelitis event [10, 11]. Longitudinal case series of NMO patents with followup ranging from 5 to 10 years have demonstrated that the majority of patients (47–100%) have significant ambulatory difficulties at follow-up. Residual visual deficits are also common, with >60% of patients reporting significant vision loss in at least one eye. Mortality due to respiratory failure has been reported to take place in up to 32% of patients [12, 13]. Of note, this mortality figure was derived from the original Mayo Clinic study [3], which took place prior to the widespread recognition of NMO and NMOSDs, and the patient population may have been biased with respect to clinical disease severity. Therefore, the prognosis of NMO may not be as grave as was reported in these earlier studies.

>

> In 1999, Wingerchuk et al. proposed diagnostic criteria for NMO which were based on clinical and radiographic features [3]. With the discovery of AQP4-Ab, these criteria were revised in 2006 to include the testing of this disease specific antibody. In addition, the necessary clinical features included were modified and simplified in an attempt to improve the diagnostic properties of the criteria. At present, the 2006 proposed diagnostic criteria for NMO consist of the presence of optic neuritis and transverse myelitis as well as 2 out of 3 of a contiguous spinal cord MRI lesion extending over more than 3 vertebral segments (i.e., longitudinally extensive), brain MRI notmeeting diagnostic criteria for MS, and NMO-IgG seropositive status [14]. These criteria are

> 99% sensitive and 90% specific for the diagnosis of NMO and have been independently validated in different patient populations [15].

>

> The recent literature suggests that in addition to its utility in the diagnosis of NMO, the presence of NMO-IgG may have a role in disease prognosis. In a prospective study of patients with longitudinally extensive transverse myelitis (LETM), 55% of those positive for NMO-IgG relapsed with recurrent LETM or optic neuritis, while none of the seronegative patients relapsed [4]. Similarly, in a series of patients with recurrent optic neuritis, the presence of NMOIgG

> heralded a 50% chance of developing transverse myelitis [16], while only 6.6% of seronegative patients developed transverse myelitis. More recently, Jarius et al. found that in acute monosymptomatic optic neuritis, 50% of NMO-IgG

> seropositive patients progressed to NMO within 12 months, while none of the seronegative patients progressed after a median follow-up of 26 months [17].

>

> In light of the fact that NMO is a disorder that has the potential to cause significant disability, the ability to recognize and differentiate NMO and related disorders from other demyelinating disorders is important from a clinical

> perspective. The term “NMO spectrum disorders” has been coined to reflect a variety of disorders thought to be related to NMO but do not quite meet the clinical diagnostic criteria for definite NMO. Disorders that are typically included in this classification are NMO-IgG seropositive limited forms of NMO (single or recurrent LETM, recurrent or simultaneous bilateral ON), Asian opticospinal MS (OSMS), optic neuritis or LETM associated with systemic autoimmune disease, and optic neuritis or myelitis associated with brain lesions typical of NMO (e.g., hypothalamic or brainstem lesions) [12]. Whether the NMO-IgG seronegative forms of these disorders are a forme fruste of classic NMO or whether they are variants of other autoimmune diseases is, at present, unclear. Until we are able to better identify with certainty that these are distinct disorders, the designation of NMO spectrum disorders is useful, as it has specific prognostic and therapeutic implications for these potentially related

> disorders.

>

> 3. Epidemiology

>

> NMO has a distinct epidemiological profile in comparison to MS. The median age of onset of NMO is typically in the fourth or fifth decade, which is older than the average age of onset ofMS. The age of onset can be quite variable, however, and NMO is occasionally seen in children and the elderly. There is a significant female predominance in both diseases, but it is even more polarized in NMO, with a female-to-male ratio ranging from 5–11 : 1 [3, 10, 13, 18].

>

> From a global perspective, NMO occurs much more commonly in nations with a predominately nonwhite population make-up, where it is a common cause of CNS demyelination. In Japan, up to 15–40% of MS is comprised of the opticospinal variant, which may be a synonymous disorder with NMO [19]. Lau et al. reported that up to 36% of MS cases in Hong Kong had selective involvement of the optic nerves and spinal cord [20], and NMO comprised

> 17% of possible MS cases in French Afro-Caribbeans in Martinique [21]. In a population-based study in Cuba, NMO comprised approximately 10% of demyelinating disorders [22]. In contrast, in countries consisting of a predominately white population, NMO comprised less than 2% of all demyelinating disorders, and the majority of cases occurred in white patients [23]. Similarly, in theMayo series, Wingerchuk et al. found that NMO still tends to occur predominately in white populations [3].

>

> Our experience at the NMO clinic at Johns Hopkins Medical Institution has shown a significant racial predilection for NMO with African American populations comprising approximately 50% of patients, which is clearly

> epidemiologically distinct from MS (unpublished data).

>

> Continued at resource:

> 
_______________________________________________

Spectrum is the new home of Devic's Support
View posts on Spectrum: http://spectrum.guthyjacksonfoundation.org/
To change your Spectrum options or unsubscribe to this email list please visit http://spectrum.guthyjacksonfoundation.org/pg/DevicsSupport/settings
Read Frequently Asked Questions: http://spectrum.guthyjacksonfoundation.org/pg/DevicsSupport/FAQ/
Questions about this email list can be sent to [email address removed]
			

We just wanted to take a minute to thank you all for everything you did for our family during Collin's illness over the past several years. all the information, help, & friendships we have made with you all will never be forgotten. We appreciate all the thoughts & prayers for our family during our time of loss. the last 5 weeks have been very painful for us as we are trying to adapt to life without our sweet Super Hero. Thank you to everyone of you for everything you did.

We hope to stay a part of the NMO community. though Collin's battle is over, we will still be here fighting for our NMO family. This fight is near & dear to our hearts. No one should go through what Collin went through & our new goal in life is to work to make this stop! We love you all!

We also greatly appreciate the Guthy-Jackson Foundation's willingness to honor our Super Hero by starting the "Collin McDaniel Hope Grant" for the purpose of Pediatric NMO research. It is an honor to our family to know our Collin will never be forgotten. Thank you so much to Victoria, Bill, Ali & all the others who have worked so hard.

Much Love,
Carey, Lisa, Angellyn & Mishelle McDaniel

http://www.cnn.com/interactive/2012/05/us/gift.of.charles/?hpt=hp_c1


�
This is an amazing story of a family that adopted a boy�who died from cancer.� CNN covered the last week of their lives and a little more.� I think it's really very touching and I thought maybe it would help stregthen and encourage some of the families that are dealing with NMO.� It was pretty powerful to me.
�
�

You ate so wonderful grace. Can't wait to see pictures if your grand baby 

Hugs

Shannon
Sent from my iPhone
On 2012-05-04, at 12:29 PM, [email address removed] wrote:
> Hello all and thank you for your patience.  I returned home on May 2, and will be addressing the queries that I received during my absence as quickly as possible.  Please bear with me a bit longer as I will be responding to your requests in the order in which they were received.  Most of you have requested scholarly literature addressing very specific issues and it takes a bit of time to get the articles together.  If you have another issue that I can help you with, please either email or leave a message on my machine.

>

> Thanks again for being so patient.

>

> Grace

> 
_______________________________________________

Spectrum is the new home of Devic's Support
View posts on Spectrum: http://spectrum.guthyjacksonfoundation.org/
To change your Spectrum options or unsubscribe to this email list please visit http://spectrum.guthyjacksonfoundation.org/pg/DevicsSupport/settings
Read Frequently Asked Questions: http://spectrum.guthyjacksonfoundation.org/pg/DevicsSupport/FAQ/
Questions about this email list can be sent to [email address removed]