Transcripts Now Available for All 2021 RNDS Presentations!

The 2021 Rare Neuroimmune Disorders Symposium (RNDS) took place October 8-10, 2021. Throughout the RNDS, we heard from over 40 speakers who presented information, research, updates, and community stories about the rare neuroimmune disorders. The videos were made available on our YouTube channel shortly following the event. We are excited to announce that now, all videos are also available in our Resource Library, along with closed captioning and transcripts for each presentation!

Since the end of 2019, SRNA has made a commitment to make our resources accessible to our community, which includes providing transcripts for all auditory resources and ensuring our visual materials meet accessibility standards for those who are visually impaired. Having a disability should not preclude anyone from accessing the information they need to learn about their disorder, advocate for themselves, and receive the best care possible. We often encourage our members to share information found in our Resource Library with their healthcare provider(s) to aid in their care. With the use of transcripts, it is easier to search for information and provide a printed out copy to providers. We are excited to offer these resources to our community!

If you didn’t get the chance to attend the 2021 RNDS or you would like to revisit a talk you attended, you can find all talks in the 2021 RNDS Resource Center. You can find a recap of the talks given at the 2021 RNDS here. Please help us plan for our 2022 symposium and other events by filling out this survey.

The Patient Advocate Foundation’s Rare Disease CareLine

People with rare neuroimmune disorders often face financial burdens from the high cost of medical care, rehabilitation, assistive equipment, and more. The Patient Advocate Foundation (PAF) is a nonprofit that promotes access to affordable, quality health care for people with chronic, debilitating, or life-threatening illnesses. It provides important thought leadership in developing policies to support its mission, working with other organizations to advocate for health care policies and collaborating with community partners to advance person-centered care and an equitable health care system for all Americans.

PAF has recently launched a new resource called the Rare Disease CareLine. This program is designed to provide free and confidential navigational assistance with financial and practical challenges that impact people with rare disease’s ability to access healthcare. The goal of the CareLine is to help people with rare diseases access care and treatment recommended by their physician. PAF remains committed to offering financial, access to care, and social needs navigation to those affected by a rare disease. Some of the areas in in which the CareLine is designed to help you are: resolving coverage issues such as off-label denials, network limitations, benefit exclusions, prior authorization, finding resources for financial aid, understanding and selecting coverage options, or applying/appealing for disability.

Request assistance online or call 800-532-5274 option 2 to access the CareLine.

New SRNA Resources for Understanding NMOSD Treatment Options

With the FDA approval of three medications (Soliris, Uplizna, and Enspryng) for maintenance in NMOSD over the last couple of years, there are now more treatment options in addition to the already existing off-label therapies. We at SRNA have therefore developed additional online resources to help you learn more about aspects of NMOSD care and therapeutics in relation to these new advancements that can significantly impact quality of life.

We have launched a brand-new NMOSD resource pack that includes:

  • An “NMOSD Therapeutics at a Glance Tool” that gives information through questions and answers on all three FDA-approved drugs and the three off-label medications that are widely used. The information includes side effects, trial results and efficacy, the safety profile, the mechanism of action, and mode of therapeutic intervention for all six drugs and more.
  • An “NMOSD Medications Page” that has this information in another format, as well as specific pages for all six drugs.


Mycophenolate Mofetil





We hope these resources will help you learn more about all NMOSD therapies and, with this knowledge, you will be better able to advocate for your care. Please reach out if you have any questions!

Mental Health Awareness Month

May is Mental Health Awareness Month. It is important for our community members to learn about the effects of rare diseases, including ADEM, AFM, MOGAD, NMOSD, ON, and TM, on mental health. According to a survey conducted by Rare Disease UK, only 1 in 7 respondents felt they received adequate psychological support following a rare disease diagnosis.  A common symptom of rare neuroimmune disorders is depression, and many of our community members experience the effects of depression following diagnosis.

As discussed in our TM information sheet:

“Individuals with ADEM, AFM, NMOSD, MOGAD, NMOSD, ON, and TM should be educated about the effect of these rare neuroimmune disorders 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 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 rare neuroimmune disorders, there is a very robust response to combined aggressive psychopharmacologic and psychotherapeutic interventions. With appropriate recognition and treatment of depression, complete symptom remission is standard.

During the early recovery period following a rare neuroimmune diagnosis, family education is essential to develop a strategic plan for dealing with the challenges to independence following a return to home. 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 and their families. Being saddened or demoralized by the diagnosis 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.”

Below are some resources on mental health for people with rare neuroimmune disorders and their caregivers:

  1. Behavioral and Psychological Changes in Demyelinating Conditions
  2. Behavioral and Psychological Changes in Demyelinating Conditions, Part 2
  3. ABCs of NMOSD – Psychosocial Aspects of NMOSD: Anxiety, Depression, and How to Cope
  4. The Central Nervous System (CNS) Connection: Understanding Depression, Cognitive Functioning, and Fatigue in CNS Demyelinating Disorders
  5. Quality of Life: Mindset and Healthy Habits
  6. How to Manage and Cope with Grief

You can find more resources in the Cognition and Depression section of our Resource Library.

COVID-19 and Rare Neuroimmune Disorders: Resources

The world has surpassed the one-year mark of the COVID-19 pandemic, and we know there are still many questions left to be answered for the rare neuroimmune disorder community. Please read the below information carefully to know and understand the risks associated with this virus in the setting of a rare neuroimmune disorder.

Risk Factors

Risk factors associated with COVID-19 may vary significantly for each individual diagnosed with a rare neuroimmune disorder (ADEM, AFM, MOGAD, NMOSD, ON, and TM). Although many who live with these diagnoses are healthy individuals, there are many with compromised or suppressed immune systems, respiratory concerns, and other factors that make their susceptibility to illness more significant. Each individual and family should know their risks and take appropriate precautions relative to their diagnosis and, if in doubt, consult your treating physician.

SRNA COVID-19 Resource Page

In March 2020, SRNA launched our COVID-19 Resource Page, where we compiled resources and information to aid our community in finding answers to their questions about the virus. Please keep in mind that SRNA cannot provide individualized medical advice. The information we have provided is for informational purposes only and is not intended to take the place of consultation with your physician. If you have concerns about your health or treatment, please contact your physician, pharmacist, or other medical professional.

COVID-19 Vaccines and Rare Neuroimmune Disorders with Dr. Benjamin Greenberg

Dr. Benjamin M. Greenberg, member of SRNA’s Board of Directors and SRNA’s Medical and Scientific Council, discussed COVID-19 vaccinations and the implications for those with rare neuroimmune disorders in a multi- part video series.

COVID-19 Survey

Participate in a research study on the experiences of individuals with rare neuroimmune disorders during the COVID-19 pandemic, including experiences with COVID-19 vaccinations. Participation involves an interview over the phone, which is expected to last approximately 30 minutes.

COVID-19 Q&A Series

We’re committed to sharing what we learn from our medical experts with you and providing answers to your questions. Last April, members of our Medical and Scientific Council, Dr. Benjamin Greenberg, Dr. Michael Levy, and Dr. Carlos Pardo, shared information in a Q&A series.

If you have any questions you would like answered or would like to contact us, please email [email protected] or call +1 (855)380-3330.

The Neuromyelitis Optica Spectrum Disorder Financial Assistance Program at The Assistance Fund

Struggling with out-of-pocket expenses due to neuromyelitis optica spectrum disorder? The Assistance Fund (TAF) helps patients and families facing high medical out-of-pocket costs by providing financial assistance for copayments, coinsurance, deductibles, and other health-related expenses. Since its founding in 2009, TAF has helped nearly 135,000 children and adults in all 50 states, Washington, DC, and Puerto Rico.

Among TAF’s more than 70 disease programs is the Neuromyelitis Optica Spectrum Disorder Financial Assistance Program. The Neuromyelitis Optica Spectrum Disorder Financial Assistance Program provides financial assistance for out-of-pocket costs associated with prescription drug assistance (copays, deductibles, and coinsurance) on FDA-approved treatment, health insurance premiums, therapy administration costs, disease management (such as prescribing physician copayments), treatment-related travel costs, and diagnostic laboratory tests.

To be eligible for assistance, patients must be U.S. citizens or permanent residents, meet certain income requirements, have a diagnosis of the disease named in the disease program, have government or private health insurance, and a prescription for an FDA-approved treatment for the disease. Once a patient is enrolled in a disease program, their coverage lasts the entire calendar year and there is no cap on the amount of assistance in that calendar year.

To learn more, or to apply today, visit:, or call (855) 803-6515.

COVID-19 Updates and Survey Opportunity

Since the beginning of the COVID-19 pandemic, SRNA has kept an updated information page to keep our community informed on the latest updates throughout the pandemic. We compiled resources and information on the status COVID-19 and rare neuroimmune disorders, and we asked members of our Medical and Scientific Council to give the expertise into how COVID-19 may impact people diagnosed with ADEM, AFM, MOGAD, NMOSD, ON, and TM. Some of the resources are:

For a full list of resources, please visit the COVID-19 section of our Resource Library. The information and resources provided are meant to be an educational resource and do not constitute medical advice. For specific information about treatments, symptoms you may be experiencing or questions about personal exposure as someone with a rare neuroimmune disorder or as a caregiver, please contact your physician directly.

Dr.Benjamin Greenberg will be giving a talk on COVID-19 and Rare Neuroimmune Disorders at our upcoming Virtual Rare Neuroimmune Disorders Symposium (RNDS). The RNDS will take place this Saturday, August 29th and is free to attend. You can register for the event here.

SRNA recently launched a research study, Experiences during COVID-19 Pandemic Among Those with Rare Neuroimmune Disorders. The intention of this research project is to describe the experiences of individuals with rare neuroimmune disorders during the COVID-19 pandemic. If you are interested in participating in the study, you can find information and the link to sign up on the study page here.

If you have any questions about COVID-19 and rare neuroimmune disorders, please share them with us using this form.

New Q&A Sessions on COVID-19 with SRNA’s Medical and Scientific Council

Over the past several weeks, SRNA has collected questions from our community on COVID-19 and how it may impact people with rare neuroimmune disorders. Recently, we had the chance to ask several members of our Medical and Scientific Council to shed light on this developing situation and answer the most frequently asked questions we collected. You can view these FAQs here.

Please note that our medical experts are answering questions to the best of their current medical knowledge, and information is changing rapidly. Please also keep in mind that SRNA cannot provide individualized medical advice. The information provided here is for informational purposes only and is not intended to take the place of consultation with your physician. If you have concerns about your health or treatment, please contact your physician, pharmacist, or other medical professional.

Dr. Benjamin Greenberg of University of Texas Southwestern answered general questions regarding COVID-19, including prevention techniques, when you should start wearing a mask, and how to safely bring in groceries from the store. He also answered many questions specific to the rare neuroimmune disorder community, such as “Because I have TM, can I be around other people?” and “Are there special precautions for caring for one at home who is ventilator dependent?” Additionally, Dr. Greenberg answered several questions about medications and supplements such as anti-inflammatory medications and Vitamins C and D.

Dr. Michael Levy of Massachusetts General Hospital and Harvard Medical School also took time to answer some of the frequently asked questions from our community. In an interview with SRNA’S Associate Director of Research and Education GG deFiebre, Dr. Levy answered questions about the relationship between COVID-19 and long-term medications such as Rituxan and azathioprine. He also discussed the changes that hospitals and medical centers are implementing to keep patients safe and the considerations someone should take into account before deciding whether to visit a hospital for non-emergency treatment (such as IVIG infusions) and check-up MRIs. Finally, Dr. Levy discussed ways to properly sanitize mobility devices, and he discussed the possible risks associated with acute treatments for individuals who are newly diagnosed with a rare neuroimmune disorder or who are experiencing a relapse.

Dr. Carlos Pardo of the Johns Hopkins University School of Medicine responded to the remaining questions from our community in an interview with GG. He answered questions about the susceptibility of people with rare neuroimmune disorders and other autoimmune diseases to becoming infected with COVID-19, and he described the reaction of the immune system in those infected with this coronavirus. Dr. Pardo explained the ways in which this coronavirus is similar and different from other viruses, and he discussed what we have learned so far from observing the situations in China and Italy. Finally, Dr. Pardo emphasized that the best precautions people can take are to follow CDC’s guidelines on isolation, quarantine recommendations, and extreme care to protect family members that are exposed to higher risk because they have rare neuroimmune disorders.

The Patient Experience with Transverse Myelitis: a Book Review

By Allen Rucker

Allen Rucker grew up in Bartlesville, Oklahoma and has degrees from Washington University, St Louis, the University of Michigan, and Stanford. In the 1970’s, he co-founded the pioneering video group, TVTV, winning the Columbia-duPont Journalism Award. In the 80’s he wrote sketch comedy with Martin Mull, Harry Shearer, Fred Willard, and others, winning the WGA Annual Award for Comedy and two CableAce Awards. In 1996, he became paralyzed from the waist down from transverse myelitis. Post-paralysis, he has written or co-written eleven books, including three books on “The Sopranos,” one of which was a #1 New York Times bestseller, and a memoir of life after TM, “The Best Seat in The House.” He is chair of the WGA Writers with Disabilities Committee and the annual Media Access Awards and writes regularly for New Mobility magazine and the Christopher Reeve Foundation website.

Sandy Siegel is a force of nature. I have known him for almost twenty-three years and am continually blown away by the energy, commitment, and compassion he’s freely given to people with transverse myelitis and associated disorders. Like many of you, Sandy was the first person I talked to after my own injury who knew more than a few medical-school facts about TM. When you first speak to Sandy, you intuitively grasp the mantra often repeated in this expansive and definitive book on living with this weird and mysterious disorder – you are not alone.

Sandy claims early on in the book that “no one in the world has spoken to more people with TM” than he has. Without a doubt. He then explains why. “This is likely to be the case until the end of time, as it would be difficult to imagine another human being stupid enough to post their home phone number all over the internet.”

Sandy’s “anthropological” overview of TM – he’s not a doctor but knows this specialty cold from a laymen’s point of view – begins and ends with his late wife, Pauline, who first contracted TM in 1994. She taught him, as have many other sufferers, what he knows about living with rare neuroimmune disorders. This book is grounded in the myriad of personal, and often personally told, stories and the myriad of approaches or solutions to the inevitable presence of pain, loss of bodily function, self-doubt, and social anxiety associated with TM, not to mention how to deal with pesky matters like incontinence and airplane travel. Here Sandy, more than in any other book I know of on the subject, went to the source – actual TM’ers and their caregivers and friends.

The book is long, goodness knows, but never tedious or dry. Sandy can tell and retell stories and they are not only full of both the emotional and medical components of TM, they are infused with the main thing that makes this book great – Sandy’s voice. Throughout, Sandy is kind, patient, and non-judgmental in describing one’s adjustment to TM, even for those who push people away and never transcend the bitterness and loss.

Sandy is the rabbi of transverse myelitis — there to help and give comfort and not to profit personally. Answering the phone at 11:30 on a Thursday night to listen to and advise someone newly faced with TM is not a pathway to personal aggrandizement or even a decent night’s sleep.

Not that he hasn’t faced his own dark moments of guilt and inadequacy. Speaking as husband and caregiver stunned by his wife’s misfortune, he asks: “How does one experience an intense tragedy and life-changing event without running headlong into some self-doubt? I have had significant questions about my character throughout this entire experience with Pauline’s illness. I have experienced significant inner struggle between who I am and the person I would like to be.…

As he delineates the reality of living with one of these disorders, Sandy is sympathetic but never less than blunt about the often-ugly consequences. He occasionally writes in sentences so direct and unsentimental that they almost read as aphorisms. Here is a sampling:

  • “Pain, depression, and fatigue can conquer the soul of the strongest of human beings.”
  • “There is nothing more demoralizing for a person with transverse myelitis than to have regular bowel and bladder accidents.”
  • “TM complicates a good marriage. TM is going to obliterate a bad marriage.”
  • “Nerve pain is the game changer with TM… For people who are paralyzed and have nerve pain, paralysis is the least of their burdens.”

Sandy’s role throughout Pauline’s long illness was that of caregiver and despite his doubts, he was a stellar one. Very few caregivers know as much about the most intimate details of life with TM – spasticity, bowel/bladder failure, sexual dysfunction, infections, UTI’s, thoughts of suicide, the awful, often silent presence of fatigue – and what he doesn’t know, he has found some remarkable people to tell their own tales.

Some of these stories are heartbreaking. I will never forget Elisa Holt’s account of discovering that her first-born son, Noah, had contracted, at six months of age, Acute Flaccid Myelitis (AFM). In the blink of an eye, Noah went from healthy to paralyzed from the waist down. In facing what she calls “a parent’s worst nightmare; a nightmare I didn’t even know existed,” Elisa and her husband guide their little boy through what will surely be years of intense physical therapy in their collective effort to “take back AFM.” Her parting advice to any parent in the same situation: “You’ll make it through the wilderness.”

Sandy knows a hundred of such stories of struggle and courage and his empathy seems to know no bounds. One of my favorite passages in the whole book involves those with TM, even if they are physically impaired, who have many ongoing side effects that others can’t easily see. Sandy offers up his sage if acerbic advice: “Perhaps people with TM should think about wearing a sign with print that says, ‘I haven’t peed in eight years. My feet and legs and butt are in so much burning pain that I would scream all day long if I were allowed…my fatigue has me so totally exhausted that I need a nap ten minutes after I wake up. I have transverse myelitis…and because you can’t see the symptoms…doesn’t give you a pass to make judgements about me in any way.’

I could go on picking out sound bites of hard facts and insights into the mindset of someone with TM and still feel I’ve failed to convey the scope, depth, and magnanimous spirit of this definitive “brain dump,” as Sandy calls it. As it began with Pauline, so it ends with Pauline. She is his lodestar on this journey. Without Pauline’s illness and her openness about it, and Sandy’s remarkable tenacity and passion, there probably would never have been a Transverse Myelitis Association -now called Siegel Rare Neuroimmune Association – and hundreds of thousands of sufferers, myself included, would be left not knowing how to deal with the next day, or the day after, how to find expert medical help, or how to discover a community of people who know exactly what you’re talking about. Sandy has helped build a public edifice of knowledge and goodwill that will far outlive him, and he has now written a book full of the wisdom of a lifetime dealing with TM.

We all owe him our supreme gratitude.

You can order a copy of the The Patient Experience with Transverse Myelitis by Sanford J. Siegel, PhD on Amazon here. Both paperback ($18) and Kindle ($2.85) versions are available. The book is self-published. All proceeds from sale of the book are donated to SRNA.

New AFM Information Sheet

The term Acute Flaccid Myelitis (AFM) was coined in 2014, but it is likely that many individuals with initial presentation of flaccid limb weakness and/or paralysis were diagnosed as having transverse myelitis or Guillain Barre Syndrome in previous years. As scientists and clinicians learn more about this disorder, SRNA strives to keep our community updated on their findings and to provide timely and accurate information and resources. As a part of this effort, we have recently updated our AFM disease information sheet so that information on AFM is easily accessible in a concise and helpful manner. The AFM information sheet has sections on Epidemiology, Signs and Symptoms, Diagnosis, Acute Treatments, Prognosis, and Rehabilitation and Symptom Management. You can find the AFM information sheet here.

Acute Flaccid Myelitis

Acute Flaccid Myelitis (AFM) is a type of inflammation in the spinal cord that has specific clinical and MRI features. AFM abnormalities noted on MRI are predominantly found in the gray matter (lower motor neuron) of the spinal cord. In 2012, an outbreak of AFM occurred in California and more cases were reported in the summer and fall of 2014, 2016, and 2018 across the United States. Non-polio enteroviruses have been implicated as potential causal factors in the development of AFM. The enterovirus D68 and enterovirus A71 have been suspect in many of these cases, although others such as coxsackie viruses have been implicated as well. Enterovirus D68 most often causes a respiratory illness and has been circulating in the United States during the summer and fall every two years since 2014, which coincides with the increase of cases of AFM seen every other year. It has not been definitively proven that these particular viruses have directly caused cases of AFM but the temporal onset of neurological symptoms with infections produced by those viruses implicate them as direct or indirect triggers of the neurological problem.


There are no conclusive studies that identify the actual numbers of individuals specifically affected by AFM, but from 2014 to 2018, the CDC confirmed over 500 reports of those affected by AFM. Not all cases are reported to the CDC nor confirmed by the CDC, so this number is likely an underestimation. There have been reports of AFM in both children and adults, but AFM cases primarily affect children under the age of 18.

Until the recent characterization of AFM in 2014, it is likely that many individuals with initial presentation of flaccid limb weakness and/or paralysis have been diagnosed as having transverse myelitis or Guillain Barre Syndrome in previous years.

Signs & Symptoms

Most of those diagnosed with AFM report having a respiratory or gastrointestinal illness before the onset of weakness. The predominant presentation is a rapid onset of weakness that may affect the limbs, face, oropharyngeal muscles, or the muscles that control breathing. Those with AFM may not be able to breathe, swallow, or move their eyes normally. Weakness varies greatly ranging from mild to very severe. AFM may result in weakness, partial paralysis, or total paralysis of just one limb or all limbs. The pattern of paralysis and how individuals present are widely variable. Weakness most often occurs in proximal muscles, meaning the muscles closest to the center of the body. Pain in the neck, back, or limb may be an early symptom. Autonomic instability, such as issues with heart rate, may occur as well. Since it is mainly the gray matter of the spinal cord that is damaged in individuals with AFM, they may not have bladder or bowel dysfunction or issues with sensation. However, some individuals may have inflammation in both the white and gray matter of the spinal cord (upper and lower motor neuron), so some of those with AFM may experience impaired sensation, bladder, and/or bowel dysfunction.


Acute flaccid myelitis is diagnosed based upon clinical exam, magnetic resonance imaging (MRI) of the spinal cord, and analysis of cerebrospinal fluid (CSF) (usually with increased white blood cells or pleocytosis). On MRI of the spinal cord, AFM lesions are longitudinal throughout the grey matter (the anterior horn cells). Sometimes imaging may appear normal early in the disease, but repeat imaging shows the lesions. In some situations, electrophysiological studies of the nerves and muscle (called nerve conduction and electromyogram [NCS/EMG]) may help to determine if there is injury to the lower motor neuron. Testing may also include blood draws, respiratory tract samples or collection of other bodily fluids to determine if a viral or infectious cause is present.

Acute Treatments

Specific treatments and intervention for AFM have not yet been identified, but some of the treatments available for transverse myelitis have been used (high dose intravenous (IV) steroids, intravenous immunoglobulin (IVIG), and plasma exchange (PLEX)). The purpose of the treatments is to attempt to reduce inflammation in the spinal cord and further prevent the individual’s immune system from causing damage. IVIG has antibodies that may have effect on limiting inflammation or neutralization or replication of enteroviruses and is widely used in treating AFM. The data on use of steroids or PLEX are mixed. Fluoxetine was used in several centers in the US in 2016 and was well tolerated but was not associated with improved outcomes among treated children. As is usual with treatment of rare neuroimmune disorders, in which placebo-controlled trials are difficult to perform, treatment must be individualized. Physical and occupational therapy are also believed to be significant for recovery in AFM.


Recovery varies among individuals with AFM. Most do not recover fully, but patients do regain strength and motor function over time to varying degrees. The most affected muscle may be the least likely to recover. Again, physical and occupational therapy are also believed to be critical for recovery in AFM.

Rehabilitation and Symptom Management

After the acute phase, rehabilitative care to improve functional skills and prevent secondary complications of immobility involves both psychological and physical accommodations. Rehabilitation may begin in the intensive care unit with the goal to transition individuals to an inpatient or outpatient rehabilitation program. There is very limited information in the medical literature specifically dealing with rehabilitation after AFM. However, much has been written regarding recovery from spinal cord injury (SCI), in general, and this literature applies. The physical issues include speech and oral motor skills training, bowel and bladder management, maintenance of skin integrity, spasticity, activities of daily living (i.e., dressing), mobility, sexual dysfunction (for adults), and pain.

The long-term management of AFM requires attention to a number of issues. These are the residual effects of any spinal cord injury, including AFM. 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.


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.

Activity-based rehabilitation includes weight-bearing exercise, functional electrical stimulation (FES), locomotor training, task-specific practice, and massed practice. Individuals with AFM may not respond to FES, but therapists can adjust FES parameters to try to get a better muscle contraction. FES even without obvious muscle contraction may have benefits. Weight-bearing exercise has been shown to improve bone mineral density, range of motion, muscle tone, and bowel function. Vibration during weight bearing may also activate denervated muscles. Weight-bearing exercises can progress to locomotor training on the treadmill with appropriate orthotics. Aqua therapy can be helpful for stretching tight muscles. Task specific practice involves relearning functions that were lost due to AFM such as bed mobility and coming to a seated position, feeding, dressing, and personal hygiene. Some children who were younger when AFM onset occurred will have to learn these for the first time. Massed practice involves repetition and increased level of activity. It has been shown that children can tolerate up to 5 hours of therapy a day. It is important to keep in mind that spinal cord injuries in children result in risks of skeletal subluxations and decreased bone mineral density which can result in fractures.

Other rehabilitation factors to consider are pulmonary management for those with ventilator dependence, and speech and language pathologists for those children with difficulty swallowing (dysphagia) and talking (dysphonia).

Individuals with AFM 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 shoe-laces 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.

Nerve Transfers

Some individuals may benefit from nerve transfer procedures, which is when nerves are taken from one area of the body and are transferred to a denervated nerve. The previous experience derived from obstetric brachial plexus injury has guided some of the approaches in patients with AFM. The value in recovery of selected upper extremity muscle groups in AFM patients appears promising although there is still need for a well-documented and validated approach to prove their beneficial outcomes. There are some cases reported in the literature of successful nerve transfers, but additional studies are needed to learn the correct timing for when nerve transfers should occur after onset.

Bladder Function

Bladder dysfunction may not occur in all individuals with AFM. Immediately after the onset of AFM, 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 females, intermittent internal self-catheterization, an indwelling catheter, or electrical stimulation. Surgical options may be appropriate for some people.

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, mini-enemas, 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 diagnosed with AFM should take into account baseline function before the onset of AFM. Until we learn more about this issue in AFM, individuals experiencing sexual dysfunction may want to refer to the strategies used in individuals with other rare neuroimmune disorders or spinal cord injuries.

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 may be an issue in AFM, particularly when a cervical spine lesion involved both the gray matter and the white matter. Damage to the white matter in the neck leads to a spastic weakness in the legs and can be seen in some AFM patients. The goal is to maintain flexibility with a stretching routine using exercises for active stretching and a bracing program with splints for a prolonged stretch. 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. 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.


Pain is common following AFM. 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.


Individuals with AFM should be educated about the effect of AFM 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 AFM is similar to the other neurologic symptoms patients endure, which are mediated by the effects of the immune system on the brain. While the prevalence of depression among individuals with AFM is not known, depression is remarkably prevalent in TM, occurring in up to 25% of those diagnosed at any given time, and is largely independent of the patient’s degree of physical disability. 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 AFM, there is a very robust response to combined aggressive psychopharmacologic and psychotherapeutic interventions.

Autonomic Dysreflexia

Autonomic dysreflexia can occur when a spinal cord is damaged above the T6 level. Symptoms can include nausea, sweating, fast heart rate and/or profound blood pressure changes (up or down). Episodes can be triggered by urinary tract infections, catheterizations, constipation or painful events in the lower extremities. Care should be given to minimize triggers and manage any blood pressure variations during an event.

Respiratory Dysfunction

A subset of patients with aggressive forms of AFM may experience marked respiratory and diaphragmatic dysfunction. It may occur when the neurons that control diaphragm movement innervated by the phrenic nerve, or intercostal muscles that partially control the mechanics of breathing, fail due to damage of motor neurons in the spinal cord. Thus, patients can have difficulty breathing and require long term ventilatory support. Over months or years patients can be weaned from the ventilator as motor control of the diaphragm comes back, but in some patients it has not yet returned. Strategies such as a diaphragm pacer are being used now although there is still need for a validated demonstration of its efficacy. Those with respiratory issues may also experiences issues with heart rate, like tachycardia (high heart rate) and bradycardia (low heart rate).