The first medical professionals who are likely to come in contact with the patient when this dramatic onset and presentation initially appears is either an ER Attending or a Psychiatrist as they are on the ‘front lines’ in treating these types of symptoms.
It is the JOB of the Emergency Room attending physician to evaluate the patient and determine if they can:
1. STABILIZE the patient and send them home usually with a referral to their General Practitioner, a Psychiatrist or Neurologist, whomever they deem appropriate based on the outcome of their evaluation.
Or
2. Determine that they have an urgent need of additional medical care and admit them to the hospital for further evaluations and treatment. During the ER Attending’s evaluation, it is also up to them to determine if a Neurologist On-call should consult on the case or any other specialist should be called in to consult.
Strong advocacy of family members and friends who bring their loved one to an Emergency Room and insist that this is new behavior that has never been seen before and that a Neurological consult occur have often, but not always, made the difference in critical neurological evaluations occurring that would have otherwise not been realized.
The most common type of Autoimmune Encephalitis, anti-NMDA receptor Autoimmune Encephalitis (ANMDARE) which accounts for 20% of cases, was identified in 2005. Even though The California Encephalitis Project 2007-2011 showed this variant of autoimmune encephalitis occurred more commonly than other etiologies of encephalitis such as enterovirus encephalitis, HSV-1, West Nile Virus or Varicella zoster virus (VZV), many Emergency Department physicians are not familiar with it. Given that it is such a newly recognized disorder; it is not yet taught in medical schools. Emergency Departments do evaluate for encephalitis and meningitis but autoimmune encephalitis often goes unrecognized.
Given the potential for Autoimmune Encephalitis to be even more common than once thought, Emergency Department Staff, would be well advised to keep autoimmune encephalitis as a differential diagnosis for patients presenting with acute psychosis or encephalitis. This may be particularly important in patients with new onset psychosis being referred to a psychiatric facility, especially if they presented with any neurological symptoms!
Another compelling reason the diagnosis should be considered in the Emergency Department (ED) is that autoimmune encephalitis is a treatable disease, and seems to respond better to early treatment. There are numerous co-hort studies and case reports of patients who demonstrated improvement immediately following immunosuppressant therapy or tumor removal. Studies have found that good outcome is associated with early treatment.
Podcast: Dr David Carr Presents Anti-NMDA receptor Encephalitis saying it is a must know diagnois for all emergency medicine practitioners!
If you go to the ER during Phase 1 - of Autoimmune Encephalitis:
The first phase of the disease is a nonspecific prodromal phase with flu like symptoms which include headache, fever, nausea, vomiting, diarrhea, or upper respiratory tract symptoms, low fever, muscle pain, and fatigue.
You may be treated with IV fluids for dehydration, medication for nausea and possibly medication for headache. It’s your basic “Take two aspirin and call me in the morning.” treatment. The patient is stabilized and discharged for home.
If you go to the ER during Phase 2 - of Autoimmune Encephalitis:
The second phase is characterized by neurologic and psychiatric symptoms. Adult patients often show overt first time psychosis with different combinations of psychomotor agitation, delusions, catatonia, violence and aggression. Involuntary muscle movements that are most often facial (dyskinesia), is almost universal in the most common Autoimmune Encephalitis, ANMDARE, and may be present at this phase or may first appear during phase three. It seems very likely that these patients will pass through an Emergency Department, and unfortunately many case reports of Autoimmune Encephalitis describe patients who presented to a physician in phase two and were diagnosed with either organic or drug-induced psychotic disorders, bipolar disorder or possible schizophrenia if the age of the patient coincides with the onset of those mental illnesses. They are referred to psychiatric institutions.
The Emergency Department will usually Admit to psych during Phase 2 and ask the question: “Who gave this patient ketamine?”
This is due to the overt psychosis dissociative state that is “similar to that elicited by ketamine”. The physician has been trained to look for the most common cause and fails to evaluate for encephalitis and possible inflammation occurring in the central nervous system.
The signs of ketamine use are so pronounced that they are very hard to miss. For this reason, a person abusing ketamine who does not want family members to know will probably abuse the drug out of sight of family. It is very often used as a party drug, or it may be used in isolation.
Ketamine causes distortions to perceptions, and at lower dosages, a sort of mellowness. The user will feel out of control of himself, and to a greater or lesser degree dissociated from their body. The user may also experience hallucinations similar to those of a person using LSD. Because the symptoms are not long-lasting, the person who uses the drug at a party may have recovered by the time he gets home. For the patient with Autoimmune Encephalitis, symptoms may fluctuate rapidly. Family and friends may report a ‘waxing and waning’ of symptoms which may indicate to the Emergency Room Attending that the episode was drug induced.
One of the challenges of the physicians who first see the patient when onset occurs is that autoimmune encephalitis mimics these common mental illnesses or the appearance of a person who has misused drugs. It is for these reasons and the fact that autoimmune encephalitis is not well recognized within the medical community or still thought to be rare rather than under recognized, that patients suffering are frequently sent home without a neurological evaluation or neurologist called in to consult. Usually they are discharged from the ER and told to follow up with a psychiatrist or they are admitted as a psychiatric patient in a psychiatric institution. All too often patients with Autoimmune Encephalitis are put on a psychiatric hold which now prevents further neurological evaluations. Misdiagnosed as bipolar or schizophrenic with overt first time psychosis, they face becoming legally committed where they remain untreated and the disease is allowed to progress. Catatonia, low levels of consciousness and coma may occur leading to irreversible brain injury and eventual death if an accurate medical diagnosis and treatment does not occur.
If you go to the ER during Phase 3 - of Autoimmune Encephalitis:
During phase 3 patients often present with abnormal or fluctuating blood pressure (hemodynamic instability) and hypoventilation/hypoxia. Again, patients are likely to present to an ED in this stage, from either home or a psychiatric institution.
The Emergency Department declares: “Medically Cleared No More.”
Unfortunately, the diagnosis is not an easy one to make and it requires a skilled, Emergency Attending, Neurologist or Psychiatrist to tease out this diagnosis and separate it from any underlying pre-existing psychiatric condition. As such, patients with this disease may progress past the earlier psychiatric presentation into a more neurologic presentation.
When the disease progresses to this stage, almost half of these patients will require prolonged ICU stays and mechanical ventilation. Many develop autonomic dysregulation (disturbances, abnormalities) such as combinations of tachycardia, bradycardia, hypertension, hypotension, fever or hypothermia, or hyperthermia. Dyskinesia, hyper salivation that may interfere with airway management, no memory of events, also common is the development of seizure like motor activity, although EEG typically does not show changes consistent with epileptic activity. Mild neurologic symptoms such as facial twitching, as well as catatonia, seizures, mutism, or development of extrapyramidal symptoms when placed on an antipsychotic agent.
