Q. My 70-year-old aunt, who I am very close with, has had a rapid decline in the last few months. She started losing her memory pretty quickly over the past few months and began slurring her speech. The strangest symptoms are her hallucinations and extreme paranoia. She’s also had one episode that appeared to be a seizure of some sort. She’s had a CT scan, an MRI, and a PET scan which show no evidence of her having had a stroke or having a brain tumor or aneurysm or any other type of brain injury. She is not a heavy drinker. I’ve spent a lot of time with her, and she only drinks one or occasionally two glasses of wine with dinner. She has no history of mental health problems, and her personality was nothing like this before. She’s also been experiencing involuntary movements and problems walking, but the hospital did a DaTscan that they say mostly rules out Parkinson’s disease, and based on all of the brain scans and other tests, and the fact that all of these symptoms came on so quickly, the doctors do not think it is anything related to Parkinson’s nor do they think it is any type of frontotemporal dementia, although there have been very differing opinions among the many specialists she has seen. One diagnosis from one of the neurologists she’s seen sounds feasible to me from what I’ve read. Have you ever heard of autoimmune encephalitis in seniors? From what I’ve read, it could be treatable if caught early. Her doctor is in the process of scheduling a brain biopsy, which sounds scary but is apparently the main way to diagnose autoimmune encephalitis to see if that is what she has.
How can we plan for her future long-term care needs if she doesn’t get better, if it wasn’t caught early enough, or if it actually is a form of dementia or Parkinson’s? Thanks for your help!
A. I’m sorry to hear about your aunt. I hope she gets the diagnosis and treatment she needs and that it is something treatable that they caught early enough for her to make a full recovery.
I have indeed heard about autoimmune encephalitis, and from what little you’ve described, it does sound like a fitting diagnosis, but of course I am not a physician. I have heard about autoimmune encephalitis for the same reason that many more doctors have been aware of it and have been diagnosing it (or misdiagnosing it) in the last several years — because of all of the media attention surrounding the true story of Susannah Cahalan, a New York Post reporter who wrote a 2012 best-selling memoir called Brain on Fire: My Month of Madness, which was made into a 2016 film called Brain on Fire, which you can watch on Netflix. In the book and film, Cahalan describes how she had been healthy and at age 24 suddenly began to experience odd symptoms. She began losing her memory, felt numb, became paranoid, erratic, and sensitive to light. She didn’t know what was happening to her and began to question everything in her life, from her job to her relationship.
Cahalan’s symptoms only got worse, and she felt like she was losing her sanity. She frightened family members and baffled doctors when she began to experience seizures, hallucinations, increasingly psychotic behavior, irrational thoughts, and even catatonia. She slurred her words, drooled, appeared rigid, and could barely walk, similar to someone with advanced Parkinson’s. Cahalan spent a month in the hospital, and blood tests and brain scans revealed no clues to what was going on with her brain, so most of her physicians concluded that she had mental illness, most likely schizophrenia mixed with bipolar disorder.
One doctor who was familiar with autoimmune encephalitis knew exactly what was happening in her brain after he asked Cahalan to perform the simple exercise of drawing a clock, a very common test for elders with suspected dementia but not a test typically given to a 24-year-old suspected of having mental illness. Cahalan drew a clock with the numbers 1 to 12 all on the right-hand side of the clock, and the left-hand side was completely blank. The doctor, Dr. Souhel Najjar, explained to Cahalan and her family that she was experiencing left-side spatial neglect and that it was likely the right side of her brain responsible for the left field of vision was inflamed. This led to a brain biopsy and a correct diagnosis and treatment for anti-NMDA receptor encephalitis, a rare autoimmune disease that attacks the brain. With proper treatment and early intervention, Cahalan was able to make a full recovery!
Diagnosing Autoimmune Encephalitis in Seniors
According to a study in the journal Neurology, autoimmune encephalitis can resemble dementia in seniors and is often mistaken as such. Neurologists at the Mayo Clinic in Rochester, Minnesota, have confirmed that patients whose symptoms mimic those of neurodegenerative diseases such as dementia can actually have an autoimmune cause for their conditions. In fact, 35 percent of senior patients with autoimmune encephalitis were initially misdiagnosed with a neurodegenerative disorder.
Misdiagnosis is unfortunate because, similar to Cahalan, if discovered early, autoimmune encephalitis responds to immunotherapy and other relatively simple treatments. Sadly, however, the disease often goes untreated in seniors because of misdiagnosis as a neurodegenerative or psychiatric condition.
“It can be devastating for patients to be labeled with a neurodegenerative disease but actually have an immune-mediated dementia, because they’re missing out on a treatment that can reverse their symptoms,” says Eoin P. Flanagan, M.B., B.Ch., a consultant in Neurology at Mayo Clinic. “If treatment is delayed, patients tend not to respond as well. It’s important to recognize this condition because you might miss your opportunity for treatment.”
Proper diagnosis is essential because if left untreated, autoimmune encephalitis can quickly become serious. Senior patients can experience a worsening of symptoms over time with alternating flare-ups and periods of recovery. Without proper treatment, autoimmune encephalitis can lead to coma or permanent brain injury. In rare cases, it can be fatal.
When Autoimmune Encephalitis Is the Root Cause of Memory Loss
Autoimmune encephalitis was once considered extremely rare. When Susannah Cahalan was diagnosed, she was reportedly only the 217th person in the world to be diagnosed with the disorder and among the first to receive the combination of steroids, immunoglobulin infusions, and plasmapheresis she credits for her recovery. Ever since Cahalan’s book and subsequent biographical drama, doctors are finding more cases as their ability to diagnose it accurately improves. In fact, a 2018 study found 13.7 cases per 100,000 people.
Autoimmune encephalitis affects women more often than men. It can happen at any age. It is not genetic, as it does not appear to run in families. In many cases, the cause is unknown. But experts say it can be caused by:
- Exposure to certain bacteria and viruses, such as streptococcus and herpes simplex virus.
- A type of tumor called a teratoma, generally in the ovaries, that causes the immune system to produce specific antibodies.
- Some cancers that can trigger an autoimmune response (when the immune system attacks the body’s own tissues).
Typical Symptoms of Autoimmune Encephalitis
Dr. Michael Lane is a neurologist at the Oregon Health and Sciences University who has particular expertise in treating patients with autoimmune encephalitis. He describes how symptoms may develop over a period of days or weeks, but this can vary. The early phase of the disease may include flu-like symptoms, such as headache, fever, nausea, and muscle pain. Psychiatric symptoms may appear, disappear, and reappear. Later symptoms may be more severe, such as a lower level of consciousness and possible coma.
Common symptoms include:
- Impaired memory and understanding;
- Unusual and involuntary movements;
- Involuntary movements of the face (facial dyskinesia);
- Difficulty with balance, speech, or vision;
- Insomnia;
- Weakness or numbness;
- Seizures;
- Severe anxiety or panic attacks;
- Compulsive behaviors;
- Altered sexual behaviors;
- Behavior changes such as agitation, fear, or euphoria;
- Loss of inhibition;
- Hallucinations;
- Paranoid thoughts;
- Loss of consciousness or coma.
Generally, a diagnosis of autoimmune encephalitis requires three conditions:
- Short-term memory loss;
- Psychiatric symptoms or other symptoms of an altered mental state all within three months of one another; and
- At least one of the following:
- Numbness, weakness, or paralysis that affects a specific limb or area of the body;
- Seizures that can’t be explained by other conditions;
- A high white blood cell count in the cerebrospinal fluid;
- An MRI that shows signs of brain inflammation; and/or
- Ruling out other causes.
Dr. Lane describes how autoimmune encephalitis can be difficult to diagnose. Because it has been considered rare, doctors often mistake it for a neurological disorder in seniors, as mentioned above. It is also often misdiagnosed as a mental health disorder. As mentioned, early diagnosis is important to avoid serious complications.
If you show signs of autoimmune encephalitis, your doctor will do a neurologic exam to measure your reflexes, nerve functions, thinking, and other processes. Doctors also perform spinal taps and blood tests to look for antibodies that may indicate autoimmune encephalitis. An MRI (magnetic resonance imaging) scan of the brain is also now used to identify signs of the disease. Doctors also perform other tests, such as a brain biopsy, to the diagnose the condition.
For more details on diagnosis and symptoms of autoimmune encephalitis, please read the article “Autoimmune encephalitis in the elderly: who to test and what to test for” in the National Library of Medicine journal.
When a Loved One Is Diagnosed with Dementia or Parkinson’s
What if it is not autoimmune encephalitis, and your loved one is diagnosed with dementia or Parkinson’s disease? There are new diagnostic tools for both of these neurodegenerative diseases. Please read my article “New Amazing Alzheimer’s Diagnostic Tools and Treatments” for details on diagnosis of Alzheimer’s and “Part 2: New Amazing Parkinson’s Diagnostic Tools and Treatments” for details on diagnosis of Parkinson’s. Despite these amazing diagnostic tools and treatments, neither Parkinson’s, which often occurs along with Lewy body dementia, nor any other type of dementia, have a cure, which makes planning for long-term care imperative.
If you or a loved one has been diagnosed with Parkinson’s disease, Alzheimer’s disease, Lewy body dementia, frontotemporal dementia, or any other type of dementia or neurodegenerative disease, and you have not yet done Incapacity Planning, Estate Planning, or Long-Term Care Planning, please call us at one of the numbers below to make an appointment when you are ready:
Northern Virginia Alzheimer’s Planning: 703-691-1888
Fredericksburg, VA Parkinson’s Planning: 540-479-1435
Rockville, MD Dementia Planning: 301-519-8041
Washington, DC Medicaid Asset Protection Planning: 202-587-2797
About Evan H Farr, CELA, CAP
