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Inhibits synthesis of guanine⁵

Typically used for organ donors and other autoimmune diseases

Inhibits antibody synthesis

1. makes patients particularly susceptible to infections

Side effects

1. Headache
2. Constipation
3. Bruising
4. Anxiety
5. hair loss
6. Diarrhea
7. loss of white blood cells
8. prolonged use can lead to tumors

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This article discusses a less severe derivative of NMO called NMO Spectrum disorder. While the article focuses on the struggles of a young athlete, NMOSD is actually characterized by the lack of the autoantibody NMO-IgG. It doesn't always result in blindness, but as the article explains, loss of muscle function, numbness, and pain are all associated with NMOSD

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When she was lifting weights a year and a half ago, she noticed her fingers going numb. "I thought it was just a pinched nerve," she remembered. But then it slowly started going up her arm, and more. "My leg was going limp, my body was going numb. For three weeks, it was like what is going on with me, what's wrong?"

After many trips in and out of the hospital, she got her diagnosis at the Mayo Clinic: Neuromyelitis Optica Spectrum Disorder, NMO for short. It's a rare autoimmune disorder that affects the spinal cord, brain, and optic nerves.

"Some people wake up and are blind, so I am grateful for every day," Paisley told us. She's healing, but still has chronic pain, fatigues easily, and even a cold can be dangerous.

There's no cure, but Paisley has been on medication and gets infusion treatments. The medication left her with a painful side effect in her knees, Avascular Necrosis. "It was sharp pains. Now they feel inflamed all the time." She's had two double knee surgeries, and could have to have more.

Thanks to therapy and hard work, she has regained the use of her right hand again. But she still qualifies for having a handicap tag for her car. She wishes people wouldn't judge her, when they see her parking in those spots. "When you see me walking, unless it's a bad day and I'm limping, I look fine. I am a young girl, pulling into a handicap spot, and people eye me down, like what the heck?"

Paisley and her family are hoping for more awareness and understanding about the disease, which is similar to Multiple Sclerosis. They say it's not easily recognized in the health care field.

Still, she doesn't let this keep her down. "There's no point in having a bad attitude about it. We find things to laugh about. They key is being happy with your life."

There is a benefit for Paisley on Saturday, at The Other Place in Woodland. Bean bags start at 4pm, pasta bar at 6pm, and live music at 9pm.

You can also donate to her GoFundMe page:
https://www.gofundme.com/show-your-love-for-paisley-forsell

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Paisley Powers Through Life-Changing Diagnosis of NMO

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When she was lifting weights a year and a half ago, she noticed her fingers going numb. "I thought it was just a pinched nerve," she remembered. But then it slowly started going up her arm, and more. "My leg was going limp, my body was going numb.

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There is difficulty in diagnosing children with NMO compared to adults

Adults are confirmed with NMO-IgG specificity

Less damaging to perform MRI scans and other invasive detection measures

Literature suggests that children don't exhibit the same severity of symptoms, making it harder to diagnose

This study took a sample of children with various stages of NMOSD among other, similar, demyelinating autoimmune diseases to test for methods to identify NMO and NMOSD for future diagnosis of patients

Confirms that NMO-IgG occurs the same way in children

Also, it does not occur in other, similar autoimmune diseases

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Banwell et al. - 2008 - Neuromyelitis optica-IgG in childhood inflammatory

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ranges from 1-8 people out of every 2 million people, with Asian and African-American populations being more affected¹

More likely to occur in women than men (9:1 women:men)

Median (not average) onset age is 39

more likely to occur in either children or elderly

Not known to be caused genetically

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Intercalates DNA, which is a way of saying it takes the place of nucleotide bases.⁹

Used to treat malignant tumors, specifically acute myeloid leukemia

chemotherapy drug

Relapse in patients over the course of 2 years

Side Effects

1. Nausea
2. Diarrhea
3. Stomach pain
4. Hair loss
5. Flu-like symptoms
6. Discolored urine

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Anti-inflammatory treatment⁸

Intravenous

Not typically used for long-term treatment, but low dosages are being used as therapy treatments

Showed a decrease in relapse and attacks, but only in dosages higher than 10 mg/day

Side effects

1. Hyperglycemia
2. Insomnia
3. Mood Swings
4. Weight Gain
5. Glaucoma
6. Impaired growth in children

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Intended for rheumatoid arthritis patients⁷

Decreases swelling and long term damage

inhibits enzymes needed for purine synthesis (blocks DNA synthesis)

Taken with prednisone

Very few reported cases

Stabilization of patients

Side effects

1. Infections
2. Mouth Sores
3. Rash
4. Leukopenia

Taking folic acid can help with some of the more adverse side effects, such as hair loss

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An antibody that targets the CD20 antigen⁶

1. CD20 is a protein that is found on B-cells and, when functioning normally, aids the cell in immune responses.

Shows a decrease in attacks

Many case studies showing high success rates

Side effects

1. Infections
2. multifocal leukoencephalopathy (a viral infection that targets the myelin in the brain)

3. Decreased blood pressure

4. Flu-like symptoms
5. Nausea
6. Diarrhea

Sometimes taken with steroids to prevent the most common side effects

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Immunosuppressive⁴

Typically used for organ donors and other autoimmune diseases

Inhibition of DNA synthesis

Taken with steroids (prednisone)

Case studies show little to no relapses⁴

Side effects

1. nausea
2. transaminases (liver damage)
3. leukopenia (decrease in white blood cells)
4. diarrhea

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- There are six main medications currently used to treat NMO.2
- Most treatments are immunosuppressive as opposed to immunomodulatory. IMS is more serious and completely shuts down the immune system
- Most of the target drugs are those used to treat Rheumatoid Arthritis, and treat the symptoms, not the cause

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Old Method similar to diagnosing MS

Limits of the old method:

Doesn't include patients whose symptoms include regions of the CNS outside of the optic nerves

some patients have loss of bladder function, muscle weakness, and other non-vision related losses.

Doesn't include patients who meet some of the criteria for MS

This is important because many of the symptoms are the same, but the treatment approaches are very different.

Some MS patients actually show more signs of NMO than MS and thus get diagnosed incorrectly as well

New Method:

Definite criteria:

1. Optic neuritis

- Demyelination of the optic nerve
- Symptoms include pain and partial to total vision loss

1. Myelitis

- Inflammation of the spinal cord

At least ⅔ of the supportive criteria:

1. MRI evidence of a contiguous spinal cord lesion, 3 or more segments in length

- Abnormalities in at least 3 spinal cord segments in a row

1. Onset brain MRI nondiagnostic for multiple sclerosis

2. NMO-IgG seropositivity

- Positive results for the NMO-IgG autoantibody

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https://commons.wikimedia.org/w/index.php?curid=54500574

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https://microbiologyinfo.com/differences-between-antigen-and-antibody/

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Immunoglobulin G, or IgG, is the most common antibody in the body. Recently, NMO-IgG, an autoantibody derivative of IgG was discovered that is highly specific to NMO. It's antigen, or receptor, is aquaporin 4, which is a channel protein that lets water into the cell, and is extremely important for passage through the blood brain barrier. The discovery of NMO-IgG has allowed for better, more accurate diagnosis of NMO.

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Pittock and Lucchinetti - 2016 - Neuromyelitis optica and the evolving spectrum of 

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Neuromyelitis Optica (NMO) is a demyelinating autoimmune disease, which means that the antibodies in the immune system attack the myelin sheath surrounding neurons, preventing them from communicating signals in the brain properly.¹⁻³

NMO is very similar to multiple sclerosis (MS), in that it results in inflammation around the central nervous system (CNS) that causes irreversible damage.¹⁻³

The difference between NMO and MS is that NMO is a direct result of an autoantibody (an antibody that has gone haywire and doesn’t function properly) called NMO-IgG (immunoglobulin G).1,3

NMO-IgG attacks aquaporin 4 (AQP4), one of the most common proteins in the brain, and which is responsible for the uptake of water into the cell, along with other properties.1,3

NMO can result in blindness, caused by the attack on optic nerves, as well as loss of muscle function and inflammation of the spinal cord. It currently has no cure.1,2

Patients with NMO typically have a first attack that alerts them to this disease and they suffer from recurring attacks throughout their life in which they gradually lose eyesight until they are completely blind. During these attacks, patients can also lose muscle and bladder control.¹

Image Source: Cylinder, M. U. M. Neuromyelitis Optica Information and Description. http://www.msunites.com/neuromyelitis-optica/

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Foundation For NMO Research - Guthy-Jackson Foundation

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Foundation For NMO Research - Guthy-Jackson Foundation

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In 2008 Victoria Jackson, the founder of a cosmetic line, found out that her daughter was diagnosed with NMO. As a result Victoria and her husband founded the Guthy-Jackson Charitable Foundation to raise awareness and research treatment for NMO.

The foundation earned over $5.7 million in 2015, $5.58 million of which came from Mr. Guthy himself. Of the money earned, $4.1 million was spent on research or grants. Additionally, the foundation consists of a board of medical doctors who participate in the research, and the foundation releases annual reports detailing the research projects being worked on, as well as all donors from the year.¹⁴

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The Guthy-Jackson Charitable Foundation | Funding NMO Research

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The Guthy-Jackson Charitable Foundation is dedicated to funding basic and clinical research needed to treat and cure Neuromyelitis Optica (NMO).

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It may not have any exciting animations or images, but he does a fairly good job summarizing what doctors currently know about NMO.¹¹

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Neuromyelitis Optica (NMO) Overview -- Mayo Clinic

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1. Pittock, S. J.; Lucchinetti, C. F. Neuromyelitis Optica and the Evolving Spectrum of Autoimmune Aquaporin-4 Channelopathies: A Decade Later. Ann N Y Acad Sci 2016, 1366(1), 20–39. https://doi.org/10.1111/nyas.12794.

2. Wingerchuk, D. M.; Lennon, V. A.; Pittock, S. J.; Lucchinetti, C. F.; Weinshenker, B. G. Revised Diagnostic Criteria for Neuromyelitis Optica. Neurology 2006, 66(10), 1485–1489. https://doi.org/10.1212/01.wnl.0000216139.44259.74.

3. Kimbrough, D. J.; Fujihara, K.; Jacob, A.; Lana-Peixoto, M. A.; Leite, M. I.; Levy, M.; Marignier, R.; Nakashima, I.; Palace, J.; de Seze, J.; et al. Treatment of Neuromyelitis Optica: Review and Recommendations. Mult Scler Relat Disord 2012, 1(4), 180–187. https://doi.org/10.1016/j.msard.2012.06.002.

4. Mandler, R. N.; Ahmed, W.; Dencoff, J. E. Devic’s Neuromyelitis Optica: A Prospective Study of Seven Patients Treated with Prednisone and Azathioprine. Neurology 1998, 51(4), 1219–1220.

5. Jacob, A.; Matiello, M.; Weinshenker, B. G.; Wingerchuk, D. M.; Lucchinetti, C.; Shuster, E.; Carter, J.; Keegan, B. M.; Kantarci, O. H.; Pittock, S. J. Treatment of Neuromyelitis Optica With Mycophenolate Mofetil: Retrospective Analysis of 24 Patients. Archives of Neurology 2009, 66(9). https://doi.org/10.1001/archneurol.2009.175.

6. Jarius, S.; Aboul-Enein, F.; Waters, P.; Kuenz, B.; Hauser, A.; Berger, T.; Lang, W.; Reindl, M.; Vincent, A.; Kristoferitsch, W. Antibody to Aquaporin-4 in the Long-Term Course of Neuromyelitis Optica. Brain 2008, 131(11), 3072–3080. https://doi.org/10.1093/brain/awn240.

7. Minagar, A.; Sheremata, W. A. Treatment of Devic’s Disease with Methotrexate and Prednisone. International Journal of MS Care 2000, 2(4), 43–49. https://doi.org/10.7224/1537-2073-2.4.43.

8. Watanabe, S.; Misu, T.; Miyazawa, I.; Nakashima, I.; Shiga, Y.; Fujihara, K.; Itoyama, Y. Low-Dose Corticosteroids Reduce Relapses in Neuromyelitis Optica: A Retrospective Analysis. Mult. Scler. 2007, 13(8), 968–974. https://doi.org/10.1177/1352458507077189.

9. Weinstock-Guttman, B.; Ramanathan, M.; Lincoff, N.; Napoli, S. Q.; Sharma, J.; Feichter, J.; Bakshi, R. Study of Mitoxantrone for the Treatment of Recurrent Neuromyelitis Optica (Devic Disease). Arch. Neurol. 2006, 63(7), 957–963. https://doi.org/10.1001/archneur.63.7.957.

10. Banwell, B.; Tenembaum, S.; Lennon, V. A.; Ursell, E.; Kennedy, J.; Bar-Or, A.; Weinshenker, B. G.; Lucchinetti, C. F.; Pittock, S. J. Neuromyelitis Optica-IgG in Childhood Inflammatory Demyelinating CNS Disorders. Neurology 2008, 70(5), 344–352. https://doi.org/10.1212/01.wnl.0000284600.80782.d5.

11. Pittock, S. (45) Neuromyelitis Optica (NMO) Overview -- Mayo Clinic - YouTube; Mayo Clinic, 2010.

12. Interview with Christine Ha, MasterChef Winner, Writer, and Chef - American Foundation for the Blind http://www.afb.org/info/living-with-vision-loss/for-job-seekers/our-stories/culinary-arts-and-food-service/chef-and-writer-interview/12345 (accessed Mar 26, 2019).

13. Berman, M. Christine Ha Becomes First Blind “MasterChef” Contestant https://www.medpagetoday.com/blogs/celebritydiagnosis/33102(accessed Mar 26, 2019).

14. The Guthy-Jackson Charitable Foundation | Funding NMO Research https://guthyjacksonfoundation.org/(accessed Mar 26, 2019).

15. Paisley Powers Through Life-Changing Diagnosis of NMO https://www.wdio.com/news/paisley-forsell-nmo-treatment-benefit/5069315/(accessed Mar 26, 2019).

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Winner of MasterChef Season 3, Christine Ha is a blind chef who was diagnosed with NMO at the age of 19.12,13 She is very open about her initial struggle with NMO, how she gradually lost vision in both eyes while in college, and how she has incorporated this struggle into her love of cooking and writing.¹² Christine became the first blind contestant to compete on season 3 of Masterchef and eventually went on to win the show.

"Everyone in this world is dealt a different hand—some better, some worse than others—but what's more important is how you play that hand.¹²"