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Devic`s disease

Medically reviewed by Ramin Ansari

Devic’s disease is a disorder of the central nervous system that strikes the nerves of the eye and spinal chord.

This disease occurs when the body’s immune system mistakenly attacks the cells of the nervous system, and although it mainly affects the optic nerve and spinal chord, it can also affect other parts of the body such as the brain.

In the past, neuromyelitis Optica was considered a type of multiple sclerosis, but with a different pattern of symptoms.

However, medical research has revealed the presence of certain antibodies in the body of patients with Devic’s disease, which are the key to obtaining a differential diagnosis of sclerosis.

Neuromyelitis Optica is a rare disease, more common in non-Caucasian populations, and is nine times more common in women than in men.

Between 80% and 90% of patients with this disease have flare-ups that leave sequels and can cause disability depending on the deterioration of the patient’s condition.

50% of patients lose sight in one or both eyes and may have the following symptoms:

  • Weakness when walking;
  • Painful cramps in the arms and legs;
  • Paralysis of the legs. Patients will need assistance with walking during the first 5 years of illness;
  • Hiccups and vomiting are uncontrollable;
  • Loss of sensitivity;
  • Disruption of the functioning of the bladder and intestines;
  • Children with Devic disease may have seizures and sometimes coma.

Causes of Devic’s disease

Causes of Devic’s disease

Although the pathogenesis of Neuromyelitis Optica is not well understood, experts attribute this pathology to autoimmune diseases. NMO-IgG antibodies specific for this disease were identified in 2004. They are found in about 73% of patients and are not detected in patients with other autoimmune disorders, such as the typical form of multiple sclerosis, SEM, or other inflammatory lesions of the central nervous system. In 2005, the aquaporin-4 protein channel was discovered: 

  1. It is a target for NMO-IgG antibodies. 
  2. Aquaporin-4 is found mainly in the tissues of the spinal cord, in the brain – periventricular, and in the hypothalamus. 
  3. Aquaporin-4 is localized in the processes of astrocytes and the walls of blood vessels that form the blood-brain barrier. 

Damage to protein channels by NMO-IgG antibodies is accompanied by an increase in the permeability of the blood-brain barrier and free penetration of other immune complexes through it with the development of autoimmune inflammation.

  • Morphologically, opticomyelitis is characterized by the development of necrosis of the white and gray medulla and the formation of demyelination zones;
  • Pathological changes can be found in the spinal chord, optic nerves, hypothalamus, and chiasm;
  • In the areas of demyelination and perivascular with opticomyelitis, IgG deposits are found;
  • Chronic spinal inflammatory foci are accompanied by the development of cystic degeneration, atrophy, and gliosis;
  • Sometimes the formation of cavities characteristic of syringomyelia is found;
  • One of the components of morphological changes in opticomyelitis in many cases is autoimmune vasculitis.

Clinical manifestations of Devic’s disease 

Clinical manifestations of Devic's disease

The main manifestations of OM are optic neuritis and myelitis. Lesions of the optic nerves and the spinal chord in some cases can occur simultaneously, but more often – with a time interval that can be months, years, or even decades.

Retrobulbar neuritis is often the first manifestation of the disease, preceding myelitis. The damage to the optic nerves is usually severe, can be unilateral or bilateral. With ophthalmoscopy, a normal picture of the fundus is found, or a slight blurring of the optic nerve discs and edema, in chronic cases – atrophy and pallor of the discs.

Myelitis in Devic’s disease has a severe course, with an acute development of symmetrical, gross motor, sensory and pelvic disorders. In 77-88% of patients after myelitis attack, partial restoration of motor functions occurs, however, complete regression is not guaranteed. In recurrent myelitis, the typical symptoms are para- or tetraparesis, paroxysmal muscle spasms, and radicular pain.

Among paraclinical diagnostic methods, MRI of the spinal cord is of the greatest importance in opticomyelitis. In most patients, MRI performed in the acute phase of myelitis reveals an extensive continuous lesion of the spinal chord that extends to more than 3 vertebral segments, it is swollen and edematous, and the lesion can accumulate a contrast agent, sometimes for several months. On MRI of the brain with Neuromyelitis Optica, either no pathological changes are detected, or nonspecific: asymptomatic and foci of demyelination are found.

The lesions in the brainstem and hypothalamus can also be considered characteristic and specific. Cerebral foci by their localization have a predisposition to those parts of the brain where there is a high level of immunoreactivity to AQP4 (aquaporin 4).

With an attack of myelitis in the analysis of cerebrospinal fluid, one-third of patients have pleocytosis (more than 50 leukocytes per 1 mm3) with the presence of neutrophils, an increase in protein levels.

In the blood serum of patients with OM, in almost half of the cases, various autoantibodies (antinuclear, extractable nuclear antigens, to double-stranded DNA, antithyroid) and their combinations are found, which indicates a predisposition of these patients to the development of autoimmune diseases.

A new step towards the diagnosis of OM was the discovery in 2004 in the plasma of patients with opticomyelitis of autoantibodies – NMO-IgG. which are specific biological markers of Devic’s disease. Since the sensitivity and specificity of this method are very high, the detection of autoantibodies in patients with symptoms of demyelinating disease allows a differential diagnosis between OM and multiple sclerosis, idiopathic transverse myelitis, recurrent or bilateral optic neuritis.

Devic’s disease diagnostics, diagnosis and treatment

Devic disease diagnostics, diagnosis and treatment

Diagnostics

The clinical combination of optic neuritis and myelitis can be observed in typical multiple sclerosis, in systemic autoimmune diseases (systemic lupus erythematosus, Sjogren’s syndrome), in association with infectious processes (pulmonary tuberculosis, various viral diseases). It is impossible to reliably judge the presence or absence of OM based on clinical data alone.

In 2008, the Diagnostic Criteria for Neuromyelitis Optica (by: D. N. Miller) were adopted, which include:

  1. Large criteria (all basic criteria are required, but they can be separated by an indefinite time interval):
  • optic neuritis with damage to one or both eyes;
  • transverse myelitis with MRI-confirmed spinal cord lesion that extends to more than 3 vertebral segments on T2-weighted MRI images and is hypointense on T1-weighted images;
  • lack of data for sarcoidosis, vasculitis, systemic lupus erythematosus, Sjogren’s syndrome, infectious process.
  1. Small criteria:
  • a recently performed MRI of the brain should be free of pathology or detect nonspecific changes (foci in the dorsal regions of the medulla oblongata, foci in the hypothalamus and/or brain stem, “linear” foci located periventricular or in the corpus callosum, but not ovoid and not spreading into the parenchyma of the hemispheres of the brain);
  • a positive test of blood serum or cerebrospinal fluid to NMO-lgG / antibodies to aquaporin 4.

Differential diagnosis

The differential diagnosis depends on the manifestation. With the classic picture, the diagnosis is quite reliable.

If the spinal cord is affected, it is necessary to carry out a differential diagnosis with lesions causing extensive transverse myelitis.

The defeat of the white matter of the cerebral hemispheres and the corpus callosum has a wide differential range, depending on the nature of the distribution of the lesions, but the most important in it is multiple sclerosis. The characteristic features suggesting opitcomyelitis and excluding MS are:

  • periventricular distribution of foci in the area of ​​the aqueduct;
  • absence of perpendicularly oriented (along with the venules) periventricular foci (there are no foci like Dawson’s fingers);
  • more extensive lesion of the corpus callosum;
  • large, often confluent lesions;
  • no contrast enhancement in the form of a half-ring;
  • no damage to the gray matter of the cerebral cortex.

Treatment

Treatment

There are conflicting data on the results of treatment with immunomodulatory and immunosuppressive drugs. To date, only 6 pharmaceuticals have been registered in the world that can be used in the pathogenetic therapy of opticomyelitis, 3 of which are interferons. However, their clinical efficacy has not been proven. 

  1. To stop attacks of myelitis and neuritis, high doses of corticosteroids and plasmapheresis are used. 
  2. With an intractable attack, it is possible to use rituximab or mitoxantrone. 
  3. To prevent exacerbations, prednisone is used.
  4. Symptomatic therapy of Neuromyelitis Optica is based on the use of muscle relaxants, intrathecal infusion of baclofen, antidepressants, central analgesics, physiotherapeutic treatment. 
  5. To restore the motor and sensory function of the limbs, improve coordination, reduce spasticity, the patient is shown physical therapy.
  6. For the treatment of an attack of opticomyelitis, high doses of corticosteroids (methylprednisolone 1000 mg per day, intravenously, for five consecutive days) are used.
  7. Supportive therapy with prednisolone 1 mg/kg per day is then recommended as part of initial immunosuppressive therapy to prevent recurrent attacks. 
  8. Sometimes myelitis does not respond well to corticosteroid therapy; in such cases, plasmapheresis, immunosuppressive therapy, including oral prednisolone and azathioprine, is indicated. Over time, the dose of prednisolone is gradually reduced to the minimum maintenance dose or is canceled altogether, leaving only monotherapy with azathioprine.

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Lone Star Neurology
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Edward Medina
Edward Medina
15:34 30 Jun 22
Just such an amazing staff that makes you feel like part of their family. I’ve been going there for over 5 years now... and each visit I get the very best care and treatments that I have ever received in the 20+ years that I’ve been dealing with severe debilitating migraines. Since i started seeing them the number of my migraines has dropped from 15-20 a month to 2-3 every 3 month. I highly recommend them …they will change your life!read more
Daneisha Johnson
Daneisha Johnson
22:20 19 May 22
Dr. Askari was very kind and explained everything so I could understand. The other staff were nice as well. I would... have gave 5 stars but I was a little taken aback when I checked in and had to pay 600.00 upfront. I think that should have been discussed in a appointment confirmation call or email just so I could have been prepared.read more
Jean Cooper
Jean Cooper
16:54 29 Apr 22
I love the office staff they are friendly and very helpful. Dr. JODIE is very caring and understanding to your needs... and wants to help you. I will go back. would recommend Dr. Dr. Jodie to other Patients in a heart beat. The team works well together.read more
Linda M
Linda M
19:40 02 Apr 22
I was obviously stressed, needing to see a neurologist. The staff was so patient and Dr. Ansari was so kind. At one... point he told me to relax, we have time, when I was relaying my history of my condition. That helped ease my stress. I have seen 3 other neurologists and he was the only one who performed any assessment tests on my cognitive and physical skills. At one point I couldn't complete two assessments and got upset and cried. I was told, it's OK. That's why you're here. I was truly impressed, and super pleased with the whole experience!read more
Leslie Durham
Leslie Durham
15:05 01 Apr 22
I've been coming here for about 5 years. The staff are ALWAYS friendly and knowledgeable. The Doctors are the absolute... best!! Jodie Moore is always in such a great mood which is a plus when you are already stressed. Highly recommendedread more
Monica Del Bosque
Monica Del Bosque
14:13 25 Mar 22
Since my first post my thoughts have changed here. It's unfortunate. My doctor and PA were great, but the office staff... is horrible. They never call you back when they say they will, they misinform you, they cause you too much stress wondering what's going on, they don't keep you posted. They never answer the phone. At this point I've left four messages in the last week, and I have sent three messages. Twice from their portal and one direct email. No response. My appointment is on Monday morning at 8:30am, no confirmation on my insurance and what's going on. What the heck is going on, this is ridiculous!I've given up... the stress her office staff has put me through is just not worth it. You can do so much better, please clean house, either change out your office staff, or find a way for them to be more efficient please. You have to do something. This is not how you want to run your practice. It leaves a very bad impression on your business.read more
Ron Buckholz
Ron Buckholz
23:32 23 Mar 22
I was actually pleasantly surprised with this visit! It took me a long time to get the appointment scheduled because no... one answers your phones EVER! After a month, I finally got in, and your staff was warm, friendly, and I was totally impressed! I feel like you will take care of my needs!read more
Steve Nabavi
Steve Nabavi
16:28 16 Mar 22
It was a nice visit. Happy staff doing all they can do to comfort the patients in a very calming environment. You ask... me they are earned a big gold star on the fridge. My only complaint they didn't give me any cookies.read more
Katie Lewis
Katie Lewis
16:10 10 Feb 22
Had very positive appointments with Jodie and Dr. Sheth for my migraine care. Jodie was so fast with the injections and... has so much valuable info. I started to feel light headed during checkout and the staff was SO helpful—giving me a chair, water, and taking me into a private room until I felt better. Highly recommend this practice for migraine patients, they know what they’re doing!!read more
Joshua Martinez
Joshua Martinez
16:02 10 Dec 21
I was scheduled to be checked and just want to say that the staff was fantastic. They were kind and helpful. I was... asked many questions related to what was going on and not once did I feel as though I was being brushed off. The front desk staff was especially great in assisting me. I'm scheduled to go back for a mri and am glad that I'll be going there.read more
Isabel Ivy
Isabel Ivy
21:42 03 Nov 21
I had such a good experience with Lone Star Neurology, Brent my MRI Tech was so awesome and made sure I was very... comfortable during the appointment. He gave me ear plugs, a pillow, leg support and blanket, easiest MRI ever lol 🤣 My 72 hour EEG nurse Amanda was also so awesome. She made sure I was take care of over the 3 days and took her time with the electrodes to make sure it was comfortable for me! Paige was also a huge help in answering all my questions when it came to my test results, and letting me know her honest opinions about how I should go forth with my treatment.read more
Leslie Luce
Leslie Luce
17:37 20 Oct 21
The professionalism and want to help attitude of this office was present from the moment I contacted them. The follow... up and follow through as well as their willingness to find a way to schedule my dad was above and beyond. We visited two offices in the same day with the same experience. I am appreciative of this—we spend a lot of time with doctors and this was top notch start to finish.read more
robert Parker
robert Parker
16:38 16 Apr 21
I love going to this office. The staff is friendly and helpful. The doctor is great. I am getting the best... neurological tests and treatment I have ever had. The only reason I did not give them a 5 star rating is because it is impossible to reach a live person at the office to reschedule appointments. Every time I have tried to get through to the office it says all people are busy and I am sent to a voicemail. If they could get their phone answering fixed, I would give them a strong 5 stars.read more
MaryAnn Hornbaker
MaryAnn Hornbaker
00:26 25 Feb 21
Dr. Harney is an excellent Dr. I found him friendly , personable and thorough. I evidently am an unusual case. ... Therefore he spent a Hugh amount of time educating me. He even gave me literature to further explain my condition and how to follow up. This is something you rarely get from your doctors. So I am more than please with my doctor and his staff.read more
Roger Arguello
Roger Arguello
03:05 29 Jan 21
Always courteous, professional. The staff is very friendly and always work with you to find the best appointment time.... The care team has been great. Always taking the time to listen to your concerns and to find the best treatment.read more
Margaret Rowland
Margaret Rowland
01:12 27 Jan 21
I have been a patient at Lone Star Neurology for several years. Now both my adult daughters also are patients there. I... love Jodie. She is always so prompt whether it is a teleamed call are a visit in the office. She takes the time to explain everything to me and answers all my questions. I am so blessed to have Jodie as my doctor.read more
Susan Miller
Susan Miller
03:01 13 Jan 21
My husband had an accident 5 years ago and Lone Star Neurology has been such a blessing to us with my husbands care.... Jodie Moore is his provider and she is amazing! Jodie is very knowledgeable, caring, and thorough. She takes her time with you, making sure your needs are met and she is happy to answer any questions you may have. Lone Star Neurology’s patients are very lucky to have Jodie providing their care. Thank you Lone Star Neurology and especially Jodie for everything you have done for us. Jodie, you are the best!read more
Windalyn C
Windalyn C
01:32 09 Jan 21
Jodie is wonderful. She is very caring and knowledgeable. I have been to over a dozen neurologists, and none were able... to help me as much as they have here. Thanks!read more
Katie Kordel
Katie Kordel
00:40 09 Jan 21
Jodi Moore, nurse practitioner, is amazing. I have suffered from frequent, debilitating headaches for almost 20 years.... She has provided the best proactive and responsive care I have ever received. My quality of life has been greatly improved by her caring approach and tenacity in finding solutions.read more
Ellie Natsis
Ellie Natsis
15:41 07 Jan 21
I have had the best experience at this neurologist's office! For over a year I have been receiving iv treatments here... each month and my nurse, Bobbie is beyond wonderful!! She's so attentive, knowledgeable, caring, and detail oriented. She makes an otherwise uncomfortable experience much more pleasant and definitely puts me at ease! She also helps me with my insurance,ordering this specialty medication and dealing with the ordering process which is no easy feat.Needless to say, she goes above a beyond in every way and I'm so grateful to this office and to Bobbie for all they do for me!read more
Matt Morris
Matt Morris
15:39 07 Jan 21
Let me start by saying that I have been coming here for years. Due to my autoimmune disease, I am in this office... once every three weeks for multiple hours at a time. The office is very clean and the staff very friendly. My only complaint would be there communication via phone. They aren't the best at responding if you leave a voicemail and expect a call back. I understand that this is prob just due to the sheer number of alls they receive daily. What I can say I like the best about the office are the people. Bobby who handles my infusions is great. I never have any issues with her setting up my infusions. She is very quick to reply to messages sent via text and if she were to leave then my whole opinion of the office may change. I also enjoy people like Matt, Lauren, and Jodi. I appreciate all that they do for me and without this team I'm not sure I would be as happy as I am to visit the office as frequently as I have to. Please ensure that these folks are recognized as they are what makes my visit to this office so tolerable :).read more
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