We know that ADEM usually follows an infection of some kind. In 50-75% of cases the beginning of the disease is preceded by a viral or bacterial infection, usually a sore throat or cough (upper respiratory tract infection). Many different bacteria, viruses and other infections have been related to ADEM, but it doesn't appear to be caused by any one infectious agent. Most cases of ADEM begin about 7-14 days after the infection.
On occasion ADEM occurs after a vaccination. This is rare but most commonly occurs after measles, mumps and rubella vaccination. ADEM occurring after vaccination may be delayed, occurring up to 3 months after the vaccination.
ADEM appears to be an immune reaction to the infection. In this reaction, the immune system, instead of fighting off the infection, causes inflammation in the central nervous system and also causes demyelination.
More than half of patients have an illness, usually an infection, 2-4 weeks before developing ADEM. Most of these illnesses are viral or bacterial, often no more than an upper respiratory tract infection.
In children with ADEM, prolonged and severe headaches occur. In addition, the patient develops fevers during the ADEM course. Along with this the patients usually get neurological symptoms which may include confusion, drowsiness, and even coma; unsteadiness and falling, and occasionally visual blurring, double vision, trouble swallowing, and weakness of the arms or legs.
In adults with ADEM, motor (movement) and sensory (tingling, numbness) symptoms tend to be more common. Overall what triggers a diagnosis of ADEM is a rapidly developing illness with neurological symptoms, often with fever and headache, usually following an upper respiratory tract infection, and which has significant MRI and spinal fluid findings consistent with ADEM.
The diagnosis if ADEM needs to be considered whenever there is a close relationship between an infection and the development of neurological symptoms, usually more than one symptoms, often accompanied by headache, fever, and an altered mental state. The symptoms once they begin tend to worsen over a few days, making it clear that the problem is a serious one.
MRI scanning: MRI scanning is an important part of the diagnosis. In ADEM there are usually widespread, multiple changes deep in the brain in areas known as the white matter. There are also sometimes lesions in the grey matter deep in the brain as well. Often the areas affected can be more than half of the total volume of the white matter.
While these changes are characteristic, they are not specific for ADEM and the physicians caring for such patients have to consider other diagnoses which may include multiple sclerosis, direct brain infections, and sometimes tumors.
Over months these changes on MRI should gradually improve and even completely disappear.
Spinal fluid testing: A lumbar puncture is typically needed in patients with ADEM. This is partially to rule out direct infections or other processes that can look like ADEM. The lumbar puncture allows the neurological team to test the cerebrospinal fluid for many different things that assist in the diagnostic process.
In ADEM, the spinal fluid often shows an increase in white cells. The type of which cell increased is usually lymphocytes, which are an active part of the immune system. Occasionally doctors can culture or measure a reaction to a specific virus or bacteria in the spinal fluid that may have triggered ADEM. In ADEM, there are often no oligoclonal bands. Oligoclonal bands are commonly found in MS, and this difference may help to distinguish the two diseases from each other.
ADEM is a rare disease and so there are no well-designed clinical trials comparing one treatment with placebo, or one treatment with another. Everything we know about treatment in ADEM comes from small published series of cases, and there are no guidelines for treatment of ADEM yet.
At this time, intravenous methylprednisolone or other steroid medications are the front line treatment of ADEM. Usually this is given over a 5-7 day course with a tapering dose of steroids orally after that. 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.
If a patient does not respond to IV methylprednisolone the next line 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 ADEM. Treatment is usually given for a few hours daily over 5 days for ADEM. 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 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. It 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 very severe cases, chemotherapy may be necessary. Either cyclophosphamide or mitoxantrone can be used, but only if less toxic therapies are not effective.
Acute disseminated encephalomyelitis (ADEM) - 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 ADEM. 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 ADEM. The neurologists at the Mellen Center specialize in the diagnosis and treatment of multiple sclerosis and related disorders, including ADEM.
- Diagnose ADEM
- 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 ADEM 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.
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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 ADEM 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.
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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 ADEM provide many health services including:
- Patient and family education about ADEM and related problems
- Ongoing assessment and management of ADEM 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 ADEM and related topics
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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 ADEM, we believe that having neuroradiologists on site allow us to do the best work we can to care for our patients.
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Measuring and evaluating the cognitive and emotional state of people with ADEM is often an important part of ADEM 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.
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Social workers provide personal support to people with ADEM 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 ADEM 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
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Psychologists are available to help people with ADEM 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 ADEM is affecting cognitive functions such as attention or memory
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Occupational therapists analyze how ADEM 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
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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
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Vocational Rehabilitation Specialists
A satisfying work life is as important to the person with ADEM as to anyone. For people with ADEM, 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.
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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 ADEM
- 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
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Speech-language pathologists can help people with ADEM 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
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