Autoimmune Dementia

An autoimmune cause means a potentially reversible course

If autoimmune dementia is misdiagnosed as an irreversible progressive neurodegenerative disorder, it may delay a correct diagnosis beyond the window of reversibility (6–12 months) and result in devastating consequences for the patient and family. An accurate diagnosis and early-initiated immunotherapy give patients the best possible outcome.

35%

Among Mayo Clinic patients diagnosed with and
treated for an autoimmune dementia, 35% were initially
misdiagnosed as having a neurodegenerative disorder.


When to consider testing

Consider autoimmune testing for patients presenting with new-onset dementia or cognitive impairment and
one or more of the following:

  • Rapid onset and progression
  • Fluctuating course
  • Psychiatric accompaniments (psychosis, hallucinations)
  • Movement disorder (myoclonus, tremor, dyskinesia)
  • Headache
  • Autoimmune stigmata (e.g., physical signs or personal/family history of diabetes, thyroid disorder, vitiligo, prematurely gray hair, myasthenia gravis, rheumatoid arthritis, systemic lupus erythematosus)

  • History of cancer
  • Smoking history (20+ pack years) or other cancer risk factors
  • Inflammatory cerebrospinal fluid
  • Neuroimaging atypical for degenerative etiology
  • Prior treatment with immune checkpoint inhibitors
  • Antibody prevalence in epilepsy and encephalopathy APE2) score is ≥41

Which test should I order?


A Test in Focus

Sean Pittock, M.D., gives an overview of the dementia autoimmune evaluations. He discusses when this testing should be ordered, how this testing compares to previous testing approaches, and what clinical action can be taken due to the results of this testing.

Learn more about how to order this evaluation at your institution.

Antibodies included:

Plasma membrane specificities

Nuclear and cytoplasmic specificities

Additional Resources

The diagnosis of autoimmune encephalopathies, dementias, and epilepsies [Communiqué]

Many disorders of the central nervous system (CNS) previously considered neurodegenerative and untreatable are now recognized as having an autoimmune cause.1

Mayo Clinic Minute: What is Lewy Body Dementia?

Dementia with Lewy bodies is a disease that can be tricky to diagnose. That's because symptoms are often similar to Alzheimer’s and Parkinson’s diseases.