NMO tends to affect young adults, a similar age group to those affected by MS. It affects women more than men. It's unclear how common this is, but in the past it has been thought of as a rare disease. It is likely that some cases considered to be multiple sclerosis in the past had NMO.
NMO does appear to be more common in African-Americans, as well as people of Asian and Pacific rim origin. It can be associated with other disorders of the immune system such as rheumatoid arthritis, Hashimoto's thyroiditis, systemic lupus erythematosis, and Sjögren's syndrome. People with NMO who don't have symptoms of these other disorders may still have bloodwork seen commonly in these other disorders.
Recent studies have shown that in NMO there are deposits of antibodies around blood vessel walls in the spinal cord and optic nerves of affected patients. These findings suggest an immune attack upon these tissues. There are areas of demyelination, inflammation, and occasionally more destructive changes in these affected areas.
The role of genetic factors is still unknown in NMO.
In NMO, symptoms of optic neuritis and transverse myelitis may occur once (monophasic illness) or recur multiple times (relapsing). When they are monophasic, symptoms of optic neuritis and myelitis tend to occur close to each other or even at the same time. When relapsing they can occur at intervals over years.
Other less common symptoms of NMO include vertigo (a spinning sensation), facial numbness, headache, and tremors.
Neuromyelitis Optica (NMO)
The diagnosis if NMO should be considered when there is optic neuritis of one or both eyes closely related in time to the development of spinal cord symptoms and signs. Often the symptoms are more severe than is usual for MS, but the symptoms can overlap.
MRI scanning: MRI scanning is an important part of the diagnosis of NMO. In NMO the brain MRI is usually relatively unaffected. This is different from most patients with MS where the brain is commonly affected with multiple white matter lesions. In NMO the optic nerves may show changes consistent with demyelination. The spinal cord often has a distinct finding of long areas of abnormality extending multiple segments of the spinal cord. During an attack of myelitis the spinal cord may be swollen. These findings are different from multiple sclerosis in which the demyelination is usually over a single segment.
Spinal fluid testing: A lumbar puncture is typically needed in patients with NMO. This is partially to rule out direct infections or other processes that can look like NMO. The lumbar puncture allows the neurological team to test the cerebrospinal fluid for many different things that assist in the diagnostic process.
In NMO, the spinal fluid frequently shows an increase in white blood cells. This may be more of an increase than is usually seen in multiple sclerosis, and there may be cells called neutrophils which are unusual in MS. In addition, patients with NMO usually do not have oligoclonal banding, which is relatively common in MS.
Patients with NMO may show an abnormal blood test, the NMO-IGG test. This seems to be specific for NMO and is an antibody test. It is an antibody that seems to be targeted at the same blood vessel areas that are affected in NMO, and the finding of a positive NMO-IGG blood test strongly supports the diagnosis of NMO. Patients with MS do not seem to show NMO-IGG positivity. Not all patients with NMO have a positive NMO-IGG however.
Like all of the disorders of the immune system which affect the brain, the diagnosis of NMO is a combination of clinical history, examination, MRI scanning, CSF results, and laboratory work.
NMO is a relatively rare disease, and so there are no well defined randomized trials of treatment in this disease. Most of what we know about treatment for NMO comes from case studies or groups of treated patients.
At this time, intravenous methylprednisolone is the front line treatment for an attack of NMO. Usually this is given over a 5-7 day course. In NMO it appears that patients require a very slow taper of oral steroids (usually prednisone) after this course, to avoid relapses. This is different from the typical treatment for MS where steroids are usually tapered over a few days. The aim is to reduce inflammation and speed recovery from the disease. Patients on steroids need to be monitored for increased blood glucose, low potassium, and sleep disturbance. There may be mood changes (irritability, crying, anxiety) when people are on steroid therapy. Other short term complications of steroid therapy include weight gain, flushed cheeks, facial swelling, a metallic taste (when using IV solumedrol), and disturbed sleep. Long term complications of steroids include susceptibility to infection, osteoporosis, development of cataracts, personality change, obesity, skin changes, and rarely injury to the shoulder or hip joint (aseptic necrosis). If the attack of NMO is severe enough hospitalization and therapy may be necessary.
If a patient does not respond to IV methylprednisolone another approach to treatment may be Intravenous Immune Globulin. This is an intravenous treatment using a blood product which has been shown to reduce the activity in certain immune diseases including NMO. Treatment is usually given for a few hours daily over 5 days for NMO. IVIG has the risks of any blood product (allergic reaction, infection) as well as sometimes causing shortness of breath due to fluid overload. Rarely patients lack an antibody important to the system and may react more strongly to IVIG.
Another approach to treatment is a process called plasmapheresis. This may be particularly beneficial in NMO since it seems that it effectively removes antibodies from the circulation. This is a treatment in which the blood is circulated through a machine that withdraws components of the immune system from the circulation, reducing immune activity. It is usually a process which takes a few hours and is done every other day for 10-14 days, often as part of a hospital stay. In NMO a seven treatment course of plasmapheresis is usually needed. Plasmapheresis may required the placement of a central venous catheter to allow for blood to be removed from the system rapidly. Risks of plasmapheresis include discomfort from taking blood, sometimes a tendency to bleed due to a reduction in platelets, and infections.
In the monophasic form of NMO further treatment may not be needed. However in the relapsing forms, further therapy may be necessary to try to head off new attacks. Anecdotally, the standard treatment for multiple sclerosis (glatiramer acetate or interferons) do not seem to be effective in NMO.
Published treatments for relapsing NMO have included a combination of prednisone orally as well as azathioprine orally. Azathioprine partially suppresses the immune system and can have side effects of nausea, fever, inflammation of the pancreas, susceptibility to infections, inflammation of the liver, and fatigue. Both require close monitoring of blood studies.
Other medicines which have been used for the relapsing form, particularly when it does not respond to the prednisone/azathioprine combination, include rituximab or mitoxantrone. These are both powerful medications which need to be administered by physicians who are experienced in their use and monitoring. At the Mellen Center all of these medication have been used and are part of our standard treatments for various diseases.
Transverse Myelitis - Team
The Mellen Center team of caregivers generally consists of:
A neurologist is a medical specialist trained to evaluate problems of the nervous system, including the symptoms of Neuromyelitis optica (NMO, also known as Devic’s disease). Neurologists have an in-depth understanding of the central nervous system and how it is affected by disease. Ideally, it is helpful if the neurologist is affiliated with a research facility, or a teaching hospital, particularly when it comes to rare diseases such as NMO. The neurologists at the Mellen Center specialize in the diagnosis and treatment of multiple sclerosis and related disorders, including NMO.
- Diagnose NMO
- Make recommendations for treatment, testing, and symptom management
- Are available to answer your medical questions, sign forms, fill prescriptions, and provide help with disability-related issues
- May initiate courses of care for NMO including disease modifying therapies, symptom management, and referral to other members of the team for treatment and counseling
A consulting neurologist makes recommendations to your local doctor or neurologist for treatment, testing, and symptom management.
Rehabilitation Specialists (Physiatrists)
At the Mellen Center rehabilitation specialists work closely with the neurologists and other team members to provide high quality rehabilitation services to people with NMO and other disorders as well as to other patients with disorders causing spasticity, balance disorders, and leg weakness. The physiatrists evaluate patients for treatment needs, develop plans of care, and monitor their progress. They are involved in treating patients using Baclofen pumps and Botox injections. They are involved in research using new rehabilitative devices to improve movement. They oversee the provision of physical therapy and occupational therapy at the Mellen Center. At the Mellen Center rehabilitation is fully integrated into the care of the patient.
Advanced Practice Clinicians
Advanced practice nurses (APNs) are clinical nurse specialists, nurse practitioners, and registered nurses with additional education (certification or master's degree) and expertise in a specialty area or clinical practice.
Physician assistants are specialists that undergo a specific training program to enable them to work with physicians in an area of clinical practice. Together, APNs and physician assistants can be considered advanced practice clinicians and are an integral part of the Mellen Center’s health care team.
Working independently and in collaboration with a doctor, advanced practice clinicians are able to provide a wide variety of services.
APCs who treat people with NMO provide many health services including:
- Patient and family education about NMO and related problems
- Ongoing assessment and management of NMO symptoms
- Counseling on general health maintenance and wellness
- Information about medicines and monitoring side effects
- Education in the management of bowel, bladder, or other personal care issues
- Guidance in determining when change might be needed in the treatment plan
- Administration and monitoring of medicines according to treatment and research protocol
- Coordination of outpatient care with home care services
- Consultation to health team members as well as outside providers
- Speaking at community programs about NMO and related topics
At the Mellen Center we have integrated neuroradiology into the care fabric of our center. Our neuroradiologists provide us with state of the art imaging, using the latest in MRI technology to allow us the best evaluation of the brain and spinal cord. Our neuroradiologists are at the forefront of their field in terms of research and imaging technology. They are based in the Mellen Center so that there is a constant interplay between the clinicians and the radiologists at the center. Because MRI imaging is so key to the diagnosis and management of NMO, we believe that having neuroradiologists on site allow us to do the best work we can to care for our patients.
Measuring and evaluating the cognitive and emotional state of people with NMO is often an important part of NMO care. At the Mellen Center we use neuropsychologists to do a detailed evaluation of cognitive function when it becomes clear that problems in this area are interfering with the patients life or the relationship with family or employers. Such information helps guide out counseling, therapy, and sometimes our medical treatment. It also helps us guide patients and families better in their care decision making.
Social workers provide personal support to people with NMO and their families by offering:
- Short-term counseling and referrals for ongoing counseling
- Information about home care assistance services and assistive devices
- Recommendations of community resources, and local and national agencies that offer both information and support for people with NMO and their families
- Financial resource information such as Social Security disability, supplemental security income, Medicaid, Waiver 4, and PASSPORT financial assistance programs
- Information about quality of life issues including living wills and durable power of attorney for health care
Psychologists are available to help people with NMO cope with the cognitive, emotional, and interpersonal aspects of the disease. They offer:
- Psychological evaluation of emotional and interpersonal problems
- Individual psychological counseling sessions to reduce emotional distress and improve stress management skills
- Group psychological counseling to develop strategies for coping with the illness and the resulting life changes
- Neuropsychological testing to determine if NMO is affecting cognitive functions such as attention or memory
Occupational therapists analyze how NMO affects the way people perform their daily tasks, help them learn new ways to do familiar activities, and prescribe proper seating as needed. Occupational therapists assist individuals in maximizing their level of functional independence. They offer:
- Individualized treatment through appropriate exercise and adaptive equipment, following an accurate assessment of each patient’s current level of functional performance
- Ongoing evaluation and appropriate treatment strategies to optimize the range of motion and muscle strength of patients’ upper extremities (arms and hands) to help them successfully complete activities of daily living such as dressing, eating, toileting, and bathing
Physical therapists are available to assess muscle strength, flexibility, coordination, balance, endurance, walking ability, and mobility. They specialize in:
- Improving function and providing instruction on managing physical disabilities
- Recommending appropriate exercises to maintain flexibility, while preventing and reducing pain
- Providing instruction regarding the use of assistive devices, braces, or other mobility aids to maximize independence
Vocational Rehabilitation Specialists
A satisfying work life is as important to the person with NMO as to anyone. For people with NMO, finding work that matches their skills, interests, and abilities can be especially challenging. Vocational rehabilitation specialists assist individuals with:
- Recognizing their skills and abilities
- Exploring new careers
- Locating jobs
- Preparing for interviews
- Developing safe work sites
- Coping with work-related issues
- Learning the many resources available for making career choices
Assessing an individual’s needs is the first step in vocational rehabilitation. Information from the assessment helps the patient and the counselor make the best use of vocational services. During the initial interview, a counselor reviews the person’s educational, work, and medical histories and assesses any factors that might affect his or her ability to work. A vocational evaluation also measures the person’s general abilities, and specific needs and interests. After the consultation, the individual might be referred to other services or community resources.
Registered dietitians provide nutritional counseling through diet management to promote good nutrition while preventing malnutrition. They are available to:
- Assess each patient’s nutritional needs based upon the progression of the disease
- Recommend changes in each patient’s diet to assist in the treatment of NMO
- Develop individual care plans for each patient to promote a good nutritional status
- If necessary, provide calorie and protein supplements to increase daily caloric and nutrient consumption
- Adapt the consistency of foods and liquids if swallowing becomes difficult
Speech-language pathologists can help people with NMO maintain as many verbal communication skills as possible. They also teach techniques that conserve energy, including non-verbal communication. The speech-language pathologists are available to:
- Evaluate and treat speech disorders and communication problems
- Assess swallowing problems to provide assistance with eating and drinking
- Recommend appropriate communication technologies to provide treatment that will aid in the success of daily activities