Long-term clinical follow-up and sequential imaging by MRI are helpful to increase confidence of a diagnosis of ADEM. Should there be clinical relapses with evidence of new MRI lesions, it is not compatible with a diagnosis of monophasic ADEM, and depending on the clinical and imaging features, the diagnosis may be modified to either multiphasic ADEM or MS. Although consensus is lacking, some physicians recommend that those with ADEM receive follow-up MRIs for a period of up to five years to ensure that there is no new inflammatory activity after the initial ADEM attack (i.e., to exclude the diagnosis of MS.)

After the acute phase, rehabilitative care to improve functional skills and prevent secondary complications of immobility involves both psychological and physical accommodations. There is very little written in the medical literature specifically dealing with rehabilitation after ADEM. However, much has been written regarding recovery from spinal cord injury (SCI) in general, and this literature applies. The physical issues include bowel and bladder management, sexual dysfunction, maintenance of skin integrity, spasticity, activities of daily living (i.e., dressing), mobility, and pain.

It is important to begin occupational and physical therapies early during the course of recovery to prevent the inactivity-related problems of skin breakdown and soft tissue contractures that lead to a decreased range of motion. Assessment and fitting for splints designed to passively maintain an optimal position for limbs that cannot be actively moved is an important part of the management at this stage.

The long-term management of ADEM requires attention to a number of issues. These are the residual effects of any spinal cord or brain injury, including ADEM. In addition to chronic medical problems, there are the ongoing issues of ordering the appropriate equipment, reentry into school, re-socialization into the community, and coping with the psychological effects of this condition by the patients and their families. During the early recovery period, family education is essential to develop a strategic plan for dealing with the challenges to independence following return to the community.

Neurocognitive Issues

Individuals who have had ADEM may have difficulties with attention, short-term memory, decision making and processing speed that impacts their ability to return to their prior level of functioning in school and their occupation. Neuropsychological evaluation may be helpful in quantifying these cognitive deficits and providing recommendations for work and school accommodations such as extended time for assignments and rest breaks.

Visual Issues

For patients who have had optic nerve inflammation, residual vision loss can be experienced. Patients can possibly note blurred vision, loss of color vision, difficulty with depth perception and glares or halos around lights at night. Furthermore, patients who fully recover vision after optic neuritis may experience transient returns of blurred vision during times of stress, exertion, or heat exposure.

Bladder Function

Bladder function is almost always at least transiently impaired in patients with inflammation in the spinal cord (TM). Immediately after the onset of TM, there is frequently a period of transient loss or depression of neural activity below the involved spinal cord lesion, referred to as “spinal shock,” which lasts about 3 weeks. Following this period, two general problems can affect the bladder. The bladder can become overly sensitive and empty after only a small amount of urine has collected, or relatively insensitive, causing the bladder to become over extended and overflow. An overly distended bladder increases the likelihood of urinary tract infections and, in time, may threaten the health of the kidneys. Depending on the dysfunction, treatment options include timed voiding, medicines, external catheters for males (a catheter connected to a condom), padding for women, intermittent internal self-catheterization, an indwelling catheter, or electrical stimulation. Surgical options may be appropriate for some people. Common bladder problems include incontinence, frequency, nocturia (frequent urination at night), hesitancy, and retention. Treating incontinence, frequency, and nocturia is often easier than treating hesitancy and retention, where clean intermittent urinary catheterizations are the basic component to success. Working with a good urologist is imperative to prevent potential serious complications, particularly one who understands spinal cord disease. Urodynamic testing is necessary to determine urine retention to check risk for urinary tract infections, particularly if there is a history of UTIs to guide the urologist in terms of the best management.

Bowel Function

Another major area of concern is effective management of bowel function. A common problem in spinal cord injury is difficulty with evacuation of stool, although fecal incontinence can also occur. The neurologic pathways for defecation are similar to those of the bladder. Many lacking voluntary control of the bowel may still be able to achieve continence by diet, strategic use of stool softeners and fiber, and the technique of rectal stimulation. Other aids include suppositories, anal irrigation, and oral medications. A high-fiber diet, adequate and timely fluid intake, and medications to regulate bowel evacuations are the basic components of success. Regular evaluations by medical specialists for adjustment of the bowel program are recommended to prevent potentially serious complications. There are some surgical options, although this is rarely necessary.

Sexual Dysfunction

Sexual dysfunction involves similar innervation and analogous syndromes as those found in bladder dysfunction. Treatment of sexual dysfunction in adults with ADEM should take into account baseline function before the onset of ADEM. Of the utmost importance is adequate education and counseling about the known physical and neurologic changes that ADEM has on sexual functioning. Because of the similarities in innervation between sexual and bladder function, patients with sexual dysfunction should be encouraged to empty their bladders before sexual stimulation to prevent inopportune incontinence. The mainstays of treatment of erectile dysfunction in men are inhibitors of cGMP phosphodiesterase, type 5, which will allow most men with TM to achieve adequate erections for success in intercourse through a combination of reflex and/or psychogenic mechanisms. Although less effective in women, these same types of medications have been shown capable of enhancing a woman’s sexual functioning. The most commonly used oral erectile dysfunction drugs are Viagra (sildenafil), Levitra (vardenafil), and Cialis (tadalafil). Although sexual experience is impacted by spinal cord injury, sensual experience and even orgasm are still possible. Lubricants and aids to erection and ejaculation (for fertility) are available. Adjustment to altered sexuality is aided by an attitude of permissive experimentation, as the previous methods and habits may no longer serve.

Skin Breakdown

Skin breakdown occurs if the skin is exposed to pressure for a significant amount of time, without sensation or the strength to shift position as necessary. Sitting position should be changed at least every 15 minutes. This can be accomplished by standing, by lifting the body up while pushing down on armrests, or by just leaning and weight shifting. Wheelchairs can be supplied with either power mechanisms of recline or tilt-in-space to redistribute weight bearing. A variety of wheelchair cushions are available to minimize sitting pressure. Redness that does not blanch when finger pressure is applied may signal the beginning of a pressure ulcer. Good nutrition, vitamin C, and avoidance of moisture all contribute to healthy skin. Pressure ulcers are much easier to prevent than to heal.

Spasticity

Spasticity means stiffness or muscle spasms and is often a very difficult problem to manage. Some stiffness in our muscles is necessary in order to control our movement, but when they become too tight, the result can range from slightly bothersome stiffness (particularly upon wakening) to uncontrollably painful spasms. When the latter occurs, small triggers such as changes in position, temperature, humidity, or presence of infections can cause this painful spasticity. The key goal is to remain flexible with exercise, a daily stretching routine, and a bracing program with splints, as needed. These splints are commonly used at the ankles, wrists or elbows. Also recommended are appropriate strengthening programs for the weaker of the spastic muscles acting on a joint and an aerobic conditioning regimen. These interventions are supported by adjunctive measures that include antispasticity drugs (e.g., diazepam, baclofen, dantrolene, tizanidine), therapeutic botulinum toxin injections, and serial casting. In cases where spasticity is severe, a baclofen pump, which provides the medication directly to the spinal cord, may be considered. The therapeutic goal is to improve the function of the individual in performing specific activities of daily living (i.e., feeding, dressing, bathing, hygiene, mobility) by improving the available joint range of motion, teaching effective compensatory strategies, and relieving pain. Left untreated, severe spasticity can lead to shortening of the affected muscle or joint called contractures, further impacting mobility, rehabilitation, and independence.

Pain

Changes in sensation often occur and can manifest as lack of sensation, or numbness, as well as painful sensations called neuropathic pain. This pain is described in many different ways, including burning, squeezing, stabbing, or tingling. Having the sensation of pain means the nerve signal is getting through, but in an inappropriate way. While this can get better over time, there is a long list of medications to treat these symptoms. The same medication doesn’t work for everyone, so the trial and error of finding the right medication can be frustrating. Alternative therapies such as acupuncture and meditation have also been utilized, with varying success.

While the body is constantly working toward repair, once damage is done to the central nervous system, there will always be evidence of this damage, usually evidenced on an MRI. Clinical fluctuations of old symptoms, particularly in the setting of infection, stress, heat (Uhthoff’s phenomenon), menstrual cycle, or anything that increases core body temperature or throws the body off of its normal course are also possible. It is important to note that this is not inflammatory driven and therefore in no way represents worsening of the condition.

The first step in treating pain effectively is obtaining an accurate diagnosis. Unfortunately, this can be very difficult. Causes of pain include muscle strain from using the body in an unaccustomed manner, nerve compression (i.e., compression of the ulnar nerve at the elbow due to excessive pressure from resting the elbow on an armrest continuously) or dysfunction of the spinal cord from the damage caused by the inflammatory attack. Muscle pain might be treated with analgesics, such as acetaminophen (Tylenol), non-steroidal, anti-inflammatory drugs such as naproxen or ibuprofen (Naprosyn, Aleve, Motrin), or modalities such as heat or cold. Nerve compression might be treated with repositioning and padding (i.e., an elbow pad for an ulnar nerve compression).

Nerve pain can be a significant challenge to find effective treatment. Nerve messages traveling through the damaged portion of the spinal cord may become scrambled and misinterpreted by the brain as pain. Besides the treatments listed above, certain antidepressants such as amitriptyline (Elavil), or anticonvulsants, such as carbamazepine, phenytoin, or gabapentin (Tegretol, Dilantin, Neurontin) may be helpful. Stress and depression should also be addressed, since these conditions make pain harder to tolerate.

Depression

Individuals with ADEM should be educated about the effect of ADEM on mood regulation and routinely screened for the development of symptoms consistent with clinical depression. Warning signs that should prompt a complete evaluation for depression include failure to progress with rehabilitation and self-care, worsening fixed low mood, pervasive decreased interest, and/or social and professional withdrawal. A preoccupation with death or suicidal thoughts constitutes a true psychiatric emergency and should lead to prompt evaluation and treatment. Depression in ADEM is similar to the other neurologic symptoms patients endure, which are mediated by the effects of the immune system on the brain. Depression is not due to personal weakness or the inability to “cope.” It can have devastating consequences; not only can depression worsen physical disability (such as fatigue, pain, and decreased concentration) but it can have lethal consequences. Despite the severity of the clinical presentation of depression in ADEM, there is a very robust response to combined aggressive psychopharmacologic and psychotherapeutic interventions. With appropriate recognition and treatment of ADEM depression, complete symptom remission is standard.

Ongoing problems typically include ordering the appropriate equipment, dealing with re-entry into school, work, and community, and coping with the psychological effects of this condition on both those diagnosed with ADEM and their families. Being saddened or demoralized by the diagnosis of ADEM is appropriate. The inability to move past this grief in a reasonable period of time such that it interferes with relationships and functional living needs to be addressed and treated. Many fear that depression reflects on oneself as an inadequate ability to cope with their diagnosis and feel weak. But it is not a personal strength issue, and depression is very much a physiological manifestation and treatable. Both talking to a psychiatrist/psychologist and medication management can be beneficial, and some studies indicate a synergistic effect of combining the two. Depression can rebound and can at times become more resistant to treatment.

Fatigue

Fatigue is the lack of mental and/or physical energy. Fatigue can be a direct result of a disease process (primary fatigue) or an indirect result (secondary fatigue). In ADEM, fatigue is more often thought to be a result of secondary fatigue. Examples of secondary fatigue include fatigue from medications, depression, stress, poor sleep patterns, infections, or changes in walking, which increase energy requirements. The key is to try to identify the underlying cause of the fatigue – for example, if one is not sleeping well because of pain, bladder dysfunction, or depression, this needs to be identified and addressed; not getting consistent sleep will worsen every other aspect of ADEM! If too much energy is exerted due to changes in walking, physical therapy can help identify better body mechanics that will help conserve energy. When nothing else can be identified as contributing to fatigue, REST is recommended! Conserving energy such that activities are planned and paced can allow for these activities to be more enjoyable rather than stressful. Also, reorganizing home and office can help to reduce the amount of wasted energy exerted so that energy can be saved up for activities that are enjoyable. Also, exercise routines incorporated in the day can actually help build stamina and reduce fatigue in the long-run – it’s also a great stress reducer! Pilates, yoga, and swimming are great, but the key is to find something enjoyable and not overdo it.

Rehabilitation and Activities of Daily Living

An appropriate strengthening program and an aerobic conditioning regimen are recommended. The effects on mobility as a result of ADEM can vary widely, however, from paralysis to mild weakness. Either way, physical therapy is instrumental in returning function. Because physical therapists deal with many different types of injuries and diseases, it is ideal to work with one who has a particular interest in spinal cord rehabilitation when possible. Assistive devices may be necessary for weakness – it can be difficult and oftentimes humbling to take the necessary step of using an assistive device, but when faced with the alternative of broken hips, heads, and the downstream effects of lost wages or jobs, it is an important and sometimes indispensable step in maintaining independence. It is also always very important to remember to exercise, as tolerated, in order to maintain physical health and stamina.

Individuals with ADEM may find ordinary tasks such as dressing, bathing, grooming, and eating very difficult. Many of these obstacles can be mastered with training and specialized equipment. For example, long handled sponges can make bathing easier, as can grab bars, portable bath seats, and hand-held shower heads. For dressing, elastic shoelaces can eliminate the need to tie shoes, while other devices can aid in donning socks. Occupational therapists are specialists in assessing equipment needs and helping people with limited function perform activities of daily living. A home assessment by an experienced professional is often helpful.

Physical therapists assist with mobility. Besides teaching people to walk and transfer more easily, they can recommend mobility aids. This includes everything from canes (single point vs. small quad cane vs. large quad cane) to walkers (static vs. rolling vs. rollator) and braces. For a custom-fabricated orthotic (brace), an orthotist is necessary. Careful thought should go into deciding whether the brace should be an ankle-foot orthosis, whether it should be flexible or stiff, and what angle the foot portion should be in relationship to the calf portion. Some will benefit by a knee-ankle foot orthosis. Each person should be evaluated individually. The best results occur when a physician coordinates the team so that the therapists and orthotists are united on what is to be achieved. The physician best trained to take this role is the physiatrist.