By Victoria Freeman
It all started in the winter of 2011. Ben was a normal 16-year-old boy facing the typical routine of any teenager in high school. It was February and he was healthy and optimistic. Then came the first episode. Everything changed in an instant. Little did he know, Ben would soon be in a fight for his life.
The seizures were the first clue that something was wrong. His head would begin to pound, and his body would start to stiffen and jerk uncontrollably. It was as if he were a prisoner inside of his own body, operated by someone else. The right side of his face would start to twitch and he would start chewing on nothing as drool fell from his mouth. Ben couldn’t remember anything, only darkness.
Ben was admitted into the University of Colorado School of Medicine. His mother was by his side as the doctors and nurses began their questioning. Ben’s right arm was completely numb and he struggled with his speech when he attempted to answer their questions. He had difficulty speaking and pronouncing his words. His answers came out slurred. He became frustrated with himself; probably thinking of how he struggled at such a basic skill that he had no problems with only days before.
Doctors performed an ECG, EEG, head CT and a basic metabolic panel. An electrocardiography (ECG) is the process of recording the electrical activity of the heart over a period of time using electrodes placed on a patient’s body. An electroencephalogram (EEG) is a test that detects electrical activity in your brain using small, flat metal discs (electrodes) attached to your scalp. A head CT is a special X-ray test that produces cross-sectional images of the head using X-rays and a computer. These produced normal results. The drug test was negative and his past medical and psychiatric history wasn’t noteworthy. His examination revealed that he had speech difficulties, asymmetric facial grimace, hyperactive deep tendon reflexes and normal sensation.
Ben was scheduled for a spinal tap on his second day at the hospital. The outcome of the procedure resulted in prescribed medication that was discontinued with the return of further negative results.
DAYS 7 – 11
Over the next 5 days, Ben’s condition worsened. He became severely agitated, which led to physical restraint. At night he would wander around the hospital in confusion. There were times that he was able to speak but had trouble finding his words; other times he was completely mute. He also had trouble sensing taste, smell and touch. His mood constantly altered. One minute he was laughing uncontrollably and the next he was angry, withdrawn and sullen. It seemed like he was hallucinating bugs on his body while picking at his skin to remove insects.
His odd behavior and seizures warranted an exhaustive medical evaluation. The broad diagnosis included infectious, inflammatory, autoimmune and endocrine etiologies. The results of a complete metabolic panel were normal. Screening tests for several autoimmune disorders were negative. The doctors were no closer to a diagnosis.
Ben would undergo another spinal tap as the doctors tried their best to discover what could be causing his symptoms. Additional CSF studies eliminated several viral causes. Oligoclonal bands, a marker of inflammation, were not present in the sample. His CSF samples were sent to laboratories to be screened for paraneoplastic antigens and cell membrane antigens in limbic encephalitis, specifically those targeting the NMDA receptor.
Ben’s diagnosis remained unclear. He continued to cycle through different stages of confusion and agitation. He still had rigid, repetitive body movements and abnormal postures. In an attempt to stifle behavioral symptoms, doctors put him on aripiprazole (an antipsychotic), as well as lorazepam (used to treat anxiety), diphenhydramine (an antihistamine), and benztropine (normally used to treat Parkinson’s disease). However these drugs only caused his rigidity to get worse. At the end of week three, Ben was transferred to a psychiatric unit to decrease the need for physical restraint.
Shortly after Ben’s transfer the doctors had their breakthrough. The doctors obtained a diagnosis of anti-NMDA receptor encephalitis. This is a potentially deadly acute form of encephalitis. It is caused by an autoimmune reaction against the NR1 subunit of the NMDA receptors located in the brain. With this diagnosis, Ben underwent several tests for a tumor mass, which is common in patients with anti-NMDA encephalitis. Testicular and renal tests showed no masses, and full-body PET showed no evidence to suggest neoplasm. The doctors wanted to start him on immunotherapy.
Ben had spent his days lying in bed in a stiff position with his arms and legs outstretched. He wouldn’t open his eyes in response to verbal instruction. At times he refused to walk or swallow the liquids the nurses fed him. At other times he was visibly aggressive. He couldn’t sleep. He had severe insomnia and his clothes began to feel heavy and uncomfortable on his body. He developed hypersensitivity to clothing.
He was administered immune globulin (IVIG) for immunotherapy. The IVIG treatment was the answer the doctors were searching for because Ben finally began to improve! IVIG is a plasma exchange. For Ben’s case, the treatment exchanged the plasma in his body, which possessed harmful autoantibodies that were attacking the NMDA receptors in his brain, with protein-filled plasma.
Over the next few weeks the miracle treatment showed signs of improvement. His responsiveness, attentiveness and verbal communication increased. Lorazepam decreased his level of agitation and improved his sleep. Ben was finally able to go home with his parents. He still had problems with short-term memory and language, but his mood swings, irritability and psychotic symptoms gradually diminished. He was regaining pieces of the old him back. There was finally a light at the end of the tunnel. He continued to return for several IVIG treatments and the hope his friends and family had for his recovery never faltered.
3 MONTHS, 16 WEEKS
Three months after discharge Ben had returned to baseline levels of academic, social and family functioning. He even returned to school. He had limited memory of the past three months, which is common in patients who recover from anti-NMDA receptor encephalitis.
Anti-NMDA encephalitis is one of more than one hundred different kinds of autoimmune diseases that afflict around 50 million people in the United States. Around 75% of autoimmune diseases occur in women and are likely the number one cause of disability in women of all ages. The symptoms show as psychiatric, which may confuse the differential diagnosis.
Made up name for the purposes of this article, in reference to an anonymous patient in a published case report from (2011): (http://ajp.psychiatryonline.org/doi/pdf/10.1176/appi.ajp.2010.10020181). Some aspects of this article are narrative extensions of the case report.
Cahalan, S. (2012). Brain on fire: My month of madness. New York: Free Press.
Chapman, M., & Vause, H. (2011). Anti-NMDA Receptor Encephalitis: Diagnosis, Psychiatric Presentation, and Treatment. American Journal of Psychiatry AJP, 245-251.
Update 9/18/2015 – Edited by Paige Jarreau