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From College Soccer Player to Survivor of Autoimmune Encephalitis

From College Soccer Player to Survivor of Autoimmune Encephalitis

September 2, 2020 | By Matt Martin

Article first appeared on the UC Health Media Room

Laura Martin is a 20-year-old college student from Winchester, Kentucky, right outside of Lexington. She was a Kentucky Governor’s Scholar and student at Transylvania University, as well as a standout goalie for the university’s women’s soccer program.

In August 2019, Laura’s future was on the rise. But in a matter of a few days, she and her family were soon facing their lowest moment. Her health suddenly declined. And no one knew if this promising student and gifted athlete would make it past 20 years old.

As she was getting ready for her sophomore year and second season with the women’s soccer team, Laura started behaving differently. Her friends began to notice she wasn’t herself. They told her family, who also noticed a drastic change in Laura’s behavior.

Laura wasn’t sleeping and started having intense paranoid delusions, acting in a way that was completely unrecognizable to her friends and family.

“I didn’t even know who this girl was,” Keri Martin, Laura’s mother, said.

Laura’s condition continued to decline. On Aug. 28, 2019, her family decided to take her to the Emergency Department at a Lexington hospital close to where they are from. When the family arrived, they were told that Laura was suffering from mental illness. Doctors took Laura into the behavioral health unit to be placed on a 72-hour hold.

With no family history of mental illness, Keri requested neurological testing for her daughter. But Laura’s doctors were dismissive of her request, and released her from the hospital after seven days without any solution to her symptoms.

“I thought this was how life was going to be for me now,” Laura said. “Coming back from the hospital was hard. Not being in school and missing my friends was difficult.”

Although Laura seemed better when she returned home, she immediately reverted back to behaviors that frightened her family. After having a poor experience at another healthcare system, Keri and James, Laura’s father, didn’t know what to do. They had many sleepless nights as they tried to take care of their daughter. With help from other family members, they watched Laura around the clock as she got worse day by day.

Laura wasn’t able to sleep and couldn’t walk, write or remember who her family members were. She was losing her cognitive ability at an alarming rate and she could no longer take care of herself for basic tasks.

“As a family, we were broken and at the bottom. There was nothing that could be worse than this,” Keri said.

Keri fought to get Laura in to see a neurologist in Lexington, only to be once again rejected by another physician. The family was told that Laura should be sent to a behavioral health unit. Keri felt that if they sent Laura back to a psychiatric facility, she would die there.

“We didn’t know what to do or where to go,” Keri said.

Running out of time and answers, Laura’s parents decided to bring her to Cincinnati to UC Health, home to the region’s No. 1 preferred provider for neuroscience care.

Immediately upon arrival at University of Cincinnati Medical Center on Oct. 9, 2019, the Martin family knew this experience would be different. Clinicians compassionately spoke to the family and quickly arranged for Laura to have a private room in the neurological unit.

Laura’s treatment when she arrived at UC Medical Center was led by Jordan Bonomo, MD, UC Health neurologist, associate professor in the Department of Emergency Medicine and director for Neurocritical Care Fellowship at the UC College of Medicine.

An experienced group of residents and nurses made Laura’s family feel at ease, leaving them with a glimmer of hope for the first time in months. One resident involved with Laura’s care even told her family that she would advocate for her.

Another resident, Laura DiDomenico, MD, remained in the unit with Laura even after her rotation ended so she could see her treatment through. Laura’s entire care team was united and committed to finding out what was wrong and how to save her life.

“It was an entirely different experience from the moment we walked through the doors of the Emergency Department,” Keri said.

Laura’s Recovery after having “Brain on Fire”

Laura had an EEG that revealed she was suffering from many small seizures, leading to her unusual behavior. The seizures were part of a neurological disease called Autoimmune Encephalitis, which refers to a group of conditions that occur when the body’s immune system mistakenly attacks healthy brain cells, leading to inflammation of the brain. This condition is often referred to as “brain on fire.”

Her family suspected this was the reason for Laura’s rapid health decline, but it wasn’t until they came to UC Health when this was confirmed. No other healthcare system in Lexington would even consider offering Laura neurological testing.

Over the next 12 days at UC Medical Center, Laura’s care team worked tirelessly to find a way to improve her condition. Joseph Broderick, MD, director of the UC Gardner Neuroscience Institute and professor in the Department of Neurology and Rehabilitation Medicine at the UC College of Medicine, took over Laura’s care on her third day in the hospital.

“Inflammatory disorders of the brain can be devastating and challenging to diagnose,” Dr. Broderick said. “But patients can also respond dramatically to the correct treatment.”

In order to improve her condition, Laura started Intravenous Immunoglobin (IVIG) treatment, which is used to treat various autoimmune diseases. The first couple days were difficult for Laura and her family, but as time wore on, her condition gradually improved.

By Oct. 21, 2019, Laura had completed her IVIG treatment and was well enough to return home. Laura’s treatment included IV steroids, and to this day, she continues using oral steroids. Both of these have contributed to her improved condition.

After discharge, she continued to improve further and subsequently went back to work while waiting to restart college. “Laura is a walking miracle,” Keri said.

“It’s gratifying to see such a positive response from Laura. We are very proud of our treatment team who made the diagnosis and started her on the appropriate therapy,” Dr. Broderick said.

Post-hospital treatment, Laura is seen by Aram Zabeti, MD, director of the Waddell Center for Multiple Sclerosis at the UC Gardner Neuroscience Institute and associate professor in the Department of Neurology and Rehabilitation Medicine at the UC College of Medicine.

“As the region’s academic healthcare system, we are proud of our ability to diagnose and treat rare diseases such as Autoimmune Encephalitis,” Dr. Zabeti said. “Early intervention in Laura’s devastating disease saved her education, productivity, family and even her life.”

Handling the COVID-19 Pandemic

When the COVID-19 global pandemic began, Laura’s family made sure to keep a close eye on her to protect her from possible infection due to her compromised immune system. Laura continued her post-hospital steroid therapy during the pandemic. Unfortunately, she wasn’t able to continue her part-time job she recently started, as the store she worked at temporarily closed to prevent the spread of COVID-19.

With the help of Dr. Zabeti, Laura successfully completed her steroid therapy and is no longer considered immunocompromised. She will be able to return to her part-time job when the store reopens.

Going from a healthy, successful college student-athlete to a neurological patient was something Laura could never have expected. She and her family went through adversity, frustration and fear along the way. But now, Laura is able to return to her life she had to give up prior to her diagnosis.

She’s able to sleep, walk, drive and work. Her next goal is to return to college in August 2020 and complete her bachelor’s degree in English with a minor in Secondary Education.

“It’s amazing to know that I have a condition that I can live with, that’s also treatable,” Laura said.

After being written off by other healthcare systems, Laura and her family found hope at UC Health. They know where to go in the future if anything happens.

“Everyone went out of their way to help Laura and all of us,” Keri said. “UC Health gave us our daughter back.”

 

Your generous Donations allow IAES to continue our important work and save lives! 

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 Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

 

 

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE. 

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 For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store!  This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.  

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I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

June-10-2020 | Gary Walters

December 2019

Gary Walters 2 281x500 - I’m Looking Forward to Living the Rest of My Life after Autoimmune EncephalitisThe last two years of my life seem to have disappeared from my memory. Gone. Holidays, weddings, a funeral, birthday parties, Christmas and New Year. Friends and family keep telling me things I’ve done and the way I’ve behaved, some of which are very embarrassing to hear. It’s just not me.

This is the result of the disease Autoimmune Encephalitis (AE), the symptoms of which I was clearly exhibiting without my even realizing. Hallucinations, involuntary movements (which developed to 20-30 movements over four or five minutes), insomnia, loss of inhibition, memory loss and finally seizures, none of which I can remember. Apparently, I had two massive seizures at work (a Secondary School where I teach Physical Education), one of which resulted in the police being called due to my resisting all help. I’m told this was the last of a number of smaller seizures as the disease continued to develop and take hold.

The last seizure resulted in hospitalization for more than two months and being seen by a number of doctors and specialists who put me through a whole host of MRI scans, EEG’s, blood plasma analysis and other tests I don’t recall. I was eventually transferred to another hospital, where a specialist identified AE straight away, where I stayed and was treated for more than three weeks.

Lots of my friends and family traveled to see me, none of which I can remember and feel very guilty about admitting to. I do have a memory of very small flashes of shouting inappropriate comments at some female staff and having to apologize the next day and some very small flashes of walking around on other wards in my underwear looking for my ward. Again, it’s very embarrassing and most of it I can’t fully remember.

I’ve been told of other embarrassing events I performed, which eventually resulted in my being placed in a room by myself and given sedatives to help me “relax.” I was given all sorts of medicines, went through a whole host of tests and discussions with specialists on how to treat and control this very rare disease, and no one knew the right answer. It was a case of controlling the disease from worsening and monitoring my condition very closely.

 

Gary Walters 3 300x300 - I’m Looking Forward to Living the Rest of My Life after Autoimmune EncephalitisEventually, I was transferred back to my local hospital and continued to have a wide range of tests, blood samples were taken every morning to be analyzed and, after three more weeks, I was released to go home with a cannular inserted into a vein in my wrist with 24 tablets to be taken during the morning and 12 at night. I also received IVIG treatment to boost my immune system every morning at the local hospital.

I am now awaiting a decision from my consultant regarding the next steps. He has to speak with specialists in London as he is also unsure about what to do. I’ve been signed off work until after Christmas, which I also find frustrating as I feel back to 100%, but deep down I know that is the right decision.

 

I hope this article gives other sufferers of this very rare disease assurance there are other people out there experiencing similar symptoms of this confusing and inexplicable disease for which hopefully leads to further research and ultimately a successful pathway to complete recovery.

 

Gary Walters 1 300x300 - I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

May 2020

Since I wrote the above piece back in late December 2019, I’m feeling back to 100%. The doctors, specialists, occupational health therapists are all very surprised how quickly I seem to have fully recovered, and it’s all down to the support and treatment I’ve had.

It’s now early May, I’m still taking a large dose of meds including Lamotrigine, Levetiracetam, Prednisone, Adcal etc, which to be honest it’s now a case of a gulp of water and down the hatch. It’s now about 10 tablets in the morning and eight at night — massively down from 24 and 12.

 

I went back to work in early January on a carefully monitored and phased return, building back to full-time before the dreaded COVID-19 hit. I’m not going to lie, going back to work has been tough. The school I work at has moved on, which has is expected in the 12-18 months I’ve been in and out (mostly out). Different students. Different staff. Different expectations. It’s been hard for me, but harder and more frustrating for the brilliant people I’m lucky enough to work with.

When I got back into work I tried to pick up where I left off. It didn’t work, because my expectations of myself are so high. I wanted to know why, how, what had happened to this, that, and the other. I tried to rush back too quickly, and it didn’t work. I wasn’t listening or asking for help enough.

I thought I could pick up where I left off, but I couldn’t. My brain needed time to click back into action. Those billions of neurons needed time. Time, I thought I didn’t have. Fortunately, my employers have been incredibly understanding. They’ve supported me so much, something for which I am eternally grateful.

This is where I know I’m so lucky. I’ve read so many stories of people that are struck with different forms of encephalitis and other life-changing illnesses where they haven’t recovered enough to be able to return to work, or their employers haven’t been as understanding. At times, I feel guilty reading their stories.

As I write this I now only see my specialist twice a year and my meds are being reduced every 10 days. I’m back into full-time work in my original role and physically fit again, running at least three times a week, with lots of sport and the gym. I seem to have a “small” gap in my memory of about 12-18 months, but it could have been a lot worse.

So, it is very possible to make a completely successful and long-lasting recovery from AE and other forms of the disease. I read so many stories of people who have suffered from this terrible illness and are still suffering, so I wanted to write this to let people know there are success stories and you can — with the correct treatment and support network around you — return to full health and look forward to living the rest of your life.

 

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Become an Advocate by sharing your story. It may result in someone receiving an accurate diagnosis who is suffering right now and is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

 

 

 

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International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

 

Be a part of the solution by supporting IAES with a donation today.

 

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My continued story about having Autoimmune Encephalitis

My continued story about having Autoimmune Encephalitis

May-13-2020 | Shadazah Brown (known as Daisy)

Shadazah Brown 2 258x500 - My continued story about having Autoimmune EncephalitisA year ago we introduced you to Shadazah Brown in her piece, A Diagnosis for Daisy. Today she shares an update to her continuing journey. 

December 31, 2019 at 10 p.m., I had a seizure at home. I woke up the following year, January 1, 2020, in the hospital. I didn’t even get to see the ball drop and celebrate with my family.

I woke up crying in so much pain, hooked up to the plasmapheresis catheters in my neck. Some of my hair had been cut for the EEG machine. My stomach hurt because of dry heaves. I felt like nothing.

Since my diagnosis with AE in 2017 — which began with a seizure in a store that caused me to split my forehead open — my seizure medications have been changed regularly to manage my epilepsy. I am also diabetic, meaning the steroid treatments have resulted in additional trips to the hospital to manage high- and low blood sugars. In that same time, the doctors have decided to give me plasmapheresis treatments every three months.

My depression has kicked in much more and I have lost all communication with my friends and family Shadazah Brown 2 1 243x500 - My continued story about having Autoimmune Encephalitismembers. I think they understand what it’s like to be great living a normal 24-year-old life. Working, driving, had a boyfriend, friends, having fun. Then you go to worrying about yourself 24/7 because you the diagnosis requires it. I didn’t ask for this.

Being told you’re totally disabled at the age of 24 hurts a lot. I couldn’t celebrate my 25th birthday because I had plasmapheresis surgery since I needed the treatment right away. I still want to be able to work, drive, drink, party, and live a healthy young person’s life.

I miss having fun so much. I watch so many videos and look at pictures and I cry. People I thought were friends and truly cared, who would be there compassionately, haven’t been. I’ve spent the last two years alone. I have a couple of friends that are still there but remain somewhat distant.

Now that I have to go to hemoglobin infusion once a week for five hours at a time, I have made new friends who are actually older than me and they are really good people to talk to. (Sometimes, I really miss them and can’t wait until infusion… LOL.)

 

 

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Become an Advocate by sharing your story. It may result in someone receiving an accurate diagnosis who is suffering right now and is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

 

 

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

 

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Be a part of the solution by supporting IAES with a donation today.

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The Unique Nature of Seizures in Autoimmune Encephalitis

The Unique Nature of Seizures in Autoimmune Encephalitis

seizure PNK - The Unique Nature of Seizures in Autoimmune Encephalitis

April-29-2020 | Claudia Lopez Lloreda, PennNeuroKnow 

IAES PNK Partnership logo 300x251 - The Unique Nature of Seizures in Autoimmune EncephalitisWhat are seizures?

Seizures can be scary events both for people who suffer from them and for their loved ones. Symptoms of a seizure typically include muscle spasms; loss of consciousness; sudden, rapid eye movements; or sudden mood changes; among other symptoms, and these can last from seconds to minutes1. These are the most severe seizures, but mild seizures, with more moderate physical and behavioral symptoms — such as stiffness of the muscles, feelings of déjà vu, anxiety, temporary confusion, or nausea — can also happen and may negatively affect health. During seizures, the body parallels what is happening in the brain: uncontrolled movements of the body can result from uncontrolled bursts of electrical activity in the brain.

Seizures are a response to hyperexcitability, meaning increased activity, of neurons in the brain, and hypersynchrony, meaning more neurons fire at the same time than normal. Seizures are very different across and within conditions. They can be generalized, affecting the entire brain from the beginning of the seizure, or focal, affecting one specific area although it may later spread. Frequent, unprovoked seizures called recurrent seizures may indicate that the person has a condition called epilepsy. Epilepsy is a chronic neurological disorder in which seizures can cause periods of unusual behavior, sensations, and negative effects on cognition such as a loss of awareness. However, because abnormal electrical activity can happen in response to other alterations in the brain such as brain injury and in response to medications, seizures can also be seen in other conditions.

One of these conditions is autoimmune encephalitis (AE). In AE, the body attacks the brain by creating antibodies against important neuronal proteins. Because these proteins help neurons communicate, the antibodies alter neuronal activity. Altering neuronal activation can lead to the changes that are seen in seizures (hyperexcitability and hypersynchrony). In fact, research shows that seizures in some patients can be a common symptom during the acute phase (early on in disease) of AE2. It is believed that antibodies against the neuronal proteins contribute directly to the disease processes and the development of seizures. It’s also possible that the process of neuroinflammation associated with AE, which increases the amount of toxic inflammatory molecules in the brain, can also contribute to the development of seizures2. Even once the inflammation has been resolved, the brain can still be predisposed to seizures or developing epilepsy, especially if the inflammation resulted in neuronal death3. However, whether epilepsy, a chronic disease, is developed in response to AE is not entirely clear. Some studies suggest that the risk of developing chronic epilepsy is low, from 10-15%4.

In different types of AE, seizures appear differently. Apart from the well-known tonic-clonic seizures (associated with jerking muscle movements), seizures in AE can also show up as faciobrachial dystonic seizures. These are characterized by abrupt involuntary movements, typically on one half of the face and arm of the same side. The frequency, response to therapies, and symptoms of the seizures themselves can all vary. However, the AE that most frequently manifest with seizures and chronic epilepsy are those mediated by antibodies against the LGI1, GABABR, and GABAAR; all-important proteins involved in neuronal communication5.

 

Are seizures associated with AE treated the same way as in epilepsy?

 

Antiepileptic drugs are the standard of care for people with epilepsy. Since seizures are a result of uncontrolled electrical activity and an imbalance of excitation and inhibition in the brain, antiepileptic drugs work by trying to restore that balance. For example, the drug clonazepam prevents seizures by increasing the effectiveness of a molecule in the brain called GABA, which helps the brain dampen the uncontrolled brain activity.

Now, although the normal path for people with epilepsy is treatment with antiepileptic drugs, it may not be particularly effective for people with seizures associated with AE. A study looking at a population of AE patients found that resolution of seizures happened even after discontinued antiepileptic drugs therapy6. In these young patients with AE who experienced unprovoked seizures at the onset of the disease there was a remission rate of 94%, meaning they stopped suffering from seizures, after they stopped taking antiepileptic drugs. Rather, immunotherapy seemed to be the important factor in controlling seizures. The researchers suggested that “long-term use of antiepileptic drugs appears not to be necessary to control seizures in AE”6.

Other studies support the idea that immunotherapy is more effective in attacking seizures in AE. One study looked at three different types of autoimmune encephalitis (anti-LGI1, anti-NMDAR, and anti-GABABR) and their response to immunotherapy and antiepileptic drugs7. They found that seizure freedom was achieved faster and more frequently after the use of immunotherapy than after the use of antiepileptic drugs. However, there may be a specific window in which immunotherapy is effective at controlling seizures.

Importantly, the researchers do mention that differences in seizures characteristics and therefore response to treatment may be due to the specific type of encephalitis. For example, patients with anti-GABABR encephalitis had an increased risk of developing seizures, meaning that the development of seizures may depend on the type of encephalitis7.

 

What do these findings mean for people with AE?

 

These differences in treatment response between AE and epilepsy point to an important trait that needs to be considered: the cause of seizures. In AE, antibodies generated against important neuronal proteins make the brain go awry. Therefore, one of the most effective ways to treat seizures may be attacking the root of the problem with immunotherapy. However, due to the variable nature of AE and the seizures associated with the condition, proper treatment with immunotherapy and/or antiepileptic medication will change from patient to patient.

 

What to do if someone is having a seizure?

 

During the most severe seizures, the person may not be able to control their body movements. For this reason, you may help them clear the area around them to prevent possible injury. If possible, place them on their side and provide cushioning for their head. There are additional indications suggested by the Center for Disease Control (become familiar with these here).

Seizures in AE Handout 

 

Your generous Donations allow IAES to continue our important work and save lives!

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Become an Advocate by sharing your story. It may result in an accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

 

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References

  1. Epilepsy. (2019, August 10). Retrieved March 6, 2020, from https://www.mayoclinic.org/diseases-conditions/epilepsy/symptoms-causes/syc-20350093
  2. Rana, A., & Musto, A. E. (2018). The role of inflammation in the development of epilepsy. Journal of Neuroinflammation, 15(1). doi: 10.1186/s12974-018-1192-7
  3. Vezzani, A., Fujinami, R. S., White, H. S., Preux, P.-M., Blümcke, I., Sander, J. W., & Löscher, W. (2015). Infections, inflammation and epilepsy. Acta Neuropathologica, 131(2), 211–234. doi: 10.1007/s00401-015-1481-5
  4. Steriade, C., Moosa, A. N., Hantus, S., Prayson, R. A., Alexopoulos, A., & Rae-Grant, A. (2018). Electroclinical features of seizures associated with autoimmune encephalitis. Seizure, 60, 198–204. doi: 10.1016/j.seizure.2018.06.021
  5. Spatola, M., & Dalmau, J. (2017). Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis. Current Opinion in Neurology, 30(3), 345–353. doi: 10.1097/wco.0000000000000449
  6.  Huang, Q., Ma, M., Wei, X., Liao, Y., Qi, H., Wu, Y., & Wu, Y. (2019). Characteristics of Seizure and Antiepileptic Drug Utilization in Outpatients with Autoimmune Encephalitis. Frontiers in Neurology, 9. doi: 10.3389/fneur.2018.01136
  7. Bruijn, M. A. D., Sonderen, A. V., Coevorden-Hameete, M. H. V., Bastiaansen, A. E., Schreurs, M. W., Rouhl, R. P., … Titulaer, M. J. (2019). Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABR encephalitis. Neurology, 92(19). doi: 10.1212/wnl.0000000000007475

 

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When Your Brain is on Fire

When Your Brain is on Fire

brain on fire

January-22-2020 | Carolyn Keating, PennNeuroKnow

Imagine you’re a bright twenty-something with a new job and a new relationship.  Everything seems to be going your way until you start becoming paranoid and acting erratically.  Then come the hallucinations and seizures.  You’re admitted to a hospital where you’re (incorrectly) diagnosed with a psychiatric disorder.  You swing from violence into a state of immobility and stupor.  And perhaps even scarier?  You don’t remember any of it.  Sound like a nightmare?  Well, it actually happened to Susannah Calahan, who details her terrifying story first-hand in her 2012 book Brain on Fire: My Month of Madness.

What caused these frightening symptoms?  The answer was a disease that had only been discovered a few years earlier (right here at Penn!): NMDAR encephalitis.  There are four main phases of the disorder.  In the prodromal phase, many but not all patients experience a flu-like illness for up to 3 weeks.  The psychotic phase is accompanied by delusions, auditory and visual hallucinations, depression, paranoia, agitation, and insomnia.  At this stage, most patients are taken to the hospital, where around 40% are misdiagnosed as having a psychiatric disorder like schizophrenia.  As this phase progresses, seizures are very common (although they can occur at any time throughout the illness), as well as involuntary muscle movements like lip-smacking or grimacing, catatonia (muscular rigidity and mental stupor), impaired attention, and memory loss.  The next phase is unresponsiveness, which includes symptoms like the inability to speak, loss of voluntary movement, and sometimes abnormal muscle contractions that cause involuntary writhing movements.  The last phase is the hyperkinetic phase and is characterized by instability of involuntary bodily functions such as breathing, blood pressure, heartbeat, and temperature.  Many patients who breathe too slowly often need to be placed on a ventilator at this stage. The decline to ventilator support can progress very rapidly after several weeks in the psychotic stage, and ultimately patients can be hospitalized for several months with the disease1–3.

What does NMDAR encephalitis actually mean?  This disease is an autoimmune disorder, meaning the body’s immune system mistakenly attacks its own healthy cells.  Normally the body identifies foreign substances by making something called an antibody that recognizes a unique part of the invader, thus targeting it for attack and destruction.  In NMDA encephalitis though, the immune system attacks the brain (that’s where to term encephalitis comes from), specifically a type of neurotransmitter receptor called an NMDA receptor (NMDAR).  These receptors bind the neurotransmitter glutamate, and play an important role in learning, memory, cognition, and behavior.  In fact, the symptoms of NMDAR encephalitis resemble those caused by drugs such as ketamine or PCP that prevent the activation of NMDARs.  For instance, at low doses ketamine and PCP cause paranoia, false perceptions, and impaired attention (like the early stages of NMDAR encephalitis), and at higher doses these drugs cause psychosis, agitation, memory and motor disturbances, and eventually unresponsiveness, catatonia, and coma2.  Several mechanisms have been proposed to explain the symptoms caused by antibodies targeting the NMDAR, but most of the evidence seems to support the idea that the receptors get removed from the cell surface and internalized.  For instance, experiments in the laboratory demonstrate that when animal neurons grown in a dish are exposed to patients’ anti-NMDAR antibodies, the number of NMDARs on the cell surface decreases as the amount of antibodies increase.  When the antibodies are removed, the number of NMDAR receptors on the cell surface returns to baseline within 4 days1.

It’s easy to remove antibodies in a dish, but how do doctors get the body to stop producing antibodies against itself?  Step one is identifying what triggers antibody production in the first case.  Interestingly, NMDAR encephalitis predominantly affects women, and ovarian teratomas (a type of tumor made up of multiple types of tissues, which can include nervous system tissue) are responsible for 50% of cases in young women2.  In patients who have some sort of tumor, removal improves symptoms in 75% of cases.  Interestingly, herpes simplex virus can also cause encephalitis (inflammation of the brain), and about 20% of these patients also develop antibodies against NMDAR2.  Treatment consists of immunotherapy: corticosteroids, IV infusion of immunoglobulins, and/or plasma exchange1, however patients with a viral trigger tend to be less responsive to treatment than those with a teratoma trigger or the 50% of patients with an unknown trigger2.  Once treatments begin improvements in symptoms start within a few weeks, though return to baseline functioning can take up to three years.  Rehabilitation is required for many patients after they leave the hospital.  Deficits in attention, memory, and executive function may linger for years, but luckily over 75% of patients with the disease recover to at or near baseline neurological functioning1.

Doctors and scientists hope to develop new treatments involving immunotherapy combined with small molecules that are able to access the brain to directly combat the effects of anti-NMDAR antibodies, ideally leading to faster control of symptoms and shorter recovery time2.  A brand new animal model of the disease was just described last week that will hopefully lead to more discoveries about how the disease is triggered and potential new therapies4.  And with increased awareness of autoimmune disorders against the brain, doctors will be able to more quickly correctly diagnose patients with this illness and get them the treatment they need.

References:

  1. Venkatesan, A. & Adatia, K. Anti-NMDA-Receptor Encephalitis: From Bench to Clinic. ACS Chem. Neurosci. 8, 2586–2595 (2017).
  2. Dalmau, J. NMDA receptor encephalitis and other antibody-mediated disorders of the synapse: The 2016 Cotzias Lecture. Neurology 87, 2471–2482 (2016).
  3. Dalmau, J. et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 7, 1091–1098 (2008).
  4. Jones, B. E. et al. Anti-NMDA receptor encephalitis in mice induced by active immunization with conformationally-stabilized holoreceptors. bioRxiv 467902 (2018). doi:10.1101/467902

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The Story of an Anti-GAD Autoimmune Limbic Encephalitis Survivor

The Story of an Anti-GAD Autoimmune Limbic Encephalitis Survivor

September 4th, 2019 | Leigh Ann Broyles

Leigh Ann Broyles 2 500x500 - The Story of an Anti-GAD Autoimmune Limbic Encephalitis SurvivorMy struggles with Anti-GAD Autoimmune Limbic Encephalitis started when I fell off the roof of my house. Well, technically my symptoms began way before the so-called “Roof Incident”. For months I had been behaving erratically, feeling physically and mentally exhausted but suffering from insomnia, having terrible headaches, a stiff neck, and vision changes. By the time everyone realized that something was wrong, it was too late for early intervention.

Then came “Roof-apocalypse 2017”. I woke up for work with a large bruise on my hip and leg, and somehow my brain convinced me that it was no big deal. I had fallen off the roof the day before when I was attempting to trim the large tree in our front yard. It was encroaching on our roof, and I had taken matters in to my own hands. I went into work that morning and complained about how much it hurt. The thing is, I have never been up on any roof, let alone my own. I suffer from a fear of heights. Getting up on a foot stool typically has me shaking in my boots. We don’t even have a ladder to climb up on. I was one hundred percent sure that I had been up there, though. My brain remembered falling into the bushes, remembered the impact, and remembered an elderly neighbor coming outside to check on me. Except we don’t have an elderly neighbor, and the tree looked the same. It was still sitting on the roof where it had been all along. I had never been on the roof, and as it turns out, my brain had been lying to me for a while.

A few weeks later, I was late to work, and my boss became worried. I can count on one hand how many times I’ve been late to work in the past 16 years. Being late is exceptionally unlike me, and is one of my pet-peeves. I work as a vet tech for an emergency and critical care department in a very busy specialty veterinary hospital and being late impacts patient care.

My husband had been working overnights at the time, and my boss decided to call him since I wasn’t answering my phone. My husband rushed home in the early hours of the morning and found me in our backyard. I was laying under a bicycle having a grand mal seizure. We still don’t know how long I had been outside, or how long the seizure activity had been happening, but we knew that it was long enough for all of the skin to be worn off of my knuckles, knees, and ankles. I had road rash on one side of my face as well. The date was May 25th, 2017.

I was rushed by ambulance to the closest hospital, and the police were called. My husband (who is the sweetest, most gentle man) was interrogated and accused of beating me. I was accused of being a drug addict and alcoholic. The neurologist on staff refused to acknowledge that my issues were primarily neurological and didn’t even believe that I had suffered from a seizure. He sent out toxicology panels, all which came back negative. At this point, my family was aggravated, and demanded answers. Multiple EEG’s, CT Scans, and MRI’s later, nothing had changed. A false diagnosis of psychological issues vs. potential viral/bacterial meningitis was doled out to them. I was placed on anticonvulsants, IV fluids, and antibiotics (as my white blood cell count was elevated, and my kidneys had taken a hit from prolonged seizure activity).

I seemed to improve, and luckily, was transferred to a physical therapy center. By no means, was I normal at this point, but the neurologist “saw improvement” so he discharged me as quickly as possible. I assume it was like a horrific game of hot potato. I spent an unknown amount of time at the physical therapy hospital. I ended up developing nystagmus on top of my ataxia and aphasia. I had my second seizure, but this time it was witnessed by medical professionals. Honestly, I still don’t remember the first hospital, the physical therapy unit, or the majority of what was to come.

Leigh Ann Broyles 1 500x500 - The Story of an Anti-GAD Autoimmune Limbic Encephalitis SurvivorI was taken to my saving grace, Medical City Plano. I entered through the emergency room and was admitted into their neuro ICU. Because my seizure activity was resistant to multiple anti-convulsant medications at that point, I was intubated and placed in a medically induced coma. For the first time since my initial seizure, my family and friends found comfort in a doctor. Dr. Lei Wang (a neuro-hospitalist) took over my case, and oversaw all things relating to my care. She read through all my records and suspected that I may have Autoimmune Limbic Encephalitis. She immediately repeated all scans and started me on methylprednisolone and IVIG. She was, and continues to be, my knight in shining, sparkly high heels. I know that without her knowledge, insights, and willingness to have an open mind, I would likely be dead. Once she initiated treatment, she sent a plethora of antibody testing out. Some took longer than others, but in the end, I tested positive for GAD 65, and I finally had an answer and a diagnosis. I continually improved, ended up extubating myself (whoops!), and started to feel like a human again. When I “woke up” I had no idea why I was in a hospital and had lost all memory of what had happened over the past 4-6 months. I don’t remember moving into our new house. I don’t remember the Christmas before we moved. I don’t remember work, or illness, or anything in between. It’s a blank, blackness in my mind. I existed, but my memories do not.

In total, I received 9 IVIG transfusions, had over 50 IV catheters, two PICC lines, one central line, was on tapering steroids for a year, and two different anti-convulsant medications for a little longer than that. I spent a month hospitalized between the three facilities. I have completely recovered with no signs of relapse and no major lasting effects two years later. I continue to suffer from depression and anxiety and have been started on medications to help. There was a short time where driving to a doctor’s office would throw me into a full-blown panic attack, but since starting on medications, I can confidently walk in with a smile and steady hands. Better living through chemistry, I suppose!

To all of the patients, the families, and the friends who deal with this disease: It can be ugly, and it can be scary, but in the end please do not give up hope. Remission and recovery are possible. Living a normal life is possible.

Always keep fighting, and don’t ever give up. You are not defined by a disease.

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Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY  provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society  cannot provide medical advice.


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