This site uses cookies to measure how you use the website so it can be updated and improved based on your needs and also uses cookies to help remember the notifications you’ve seen, like this one, so that we don’t show them to you again. If you could also tell us a little bit about yourself, this information will help us understand how we can support you better and make this site even easier for you to use and navigate.

Autoimmune dementia: clinical course and predictors of immunotherapy response


Flanagan, Eoin P., McKeon, Andrew, Lennon, Vanda A., Boeve, Bradley F., Trenerry, Max R., Tan, K. Meng, Drubach, Daniel A., Josephs, Keith A., Britton, Jeffrey W., Mandrekar, Jayawant N., Lowe, Val, Parisi, Joseph E., Pittock, Sean J.


Mayo Clinic Proceedings, Volume: 85, No.: 10, Pages.: 881-897

Year of Publication



Objective: To define the diagnostic characteristics and predictors of treatment response in patients with suspected autoimmune dementia.; Patients and Methods: Between January 1, 2002, and January 1, 2009, 72 consecutive patients received immunotherapy for suspected autoimmune dementia. Their baseline clinical, radiologic, and serologic characteristics were reviewed and compared between patients who were responsive to immunotherapy and those who were not. Patients were classified as responders if the treating physician had reported improvement after immunotherapy (documented in 80% by the Kokmen Short Test of Mental Status, neuropsychological testing, or both).; Results: Initial immunotherapeutic regimens included methylprednisolone in 56 patients (78%), prednisone in 12 patients (17%), dexamethasone in 2 patients (3%), intravenous immune globulin in 1 patient (1%), and plasma exchange in 1 patient (1%). Forty-six patients (64%) improved, most in the first week of treatment. Thirty-five percent of these immunotherapy responders were initially diagnosed as having a neurodegenerative or prion disorder. Pretreatment and posttreatment neuropsychological score comparisons revealed improvement in almost all cognitive domains, most notably learning and memory. Radiologic or electroencephalographic improvements were reported in 22 (56%) of 39 patients. Immunotherapy responsiveness was predicted by a subacute onset (P<.001), fluctuating course (P<.001), tremor (P=.007), shorter delay to treatment (P=.005), seropositivity for a cation channel complex autoantibody (P=.01; neuronal voltage-gated potassium channel more than calcium channel or neuronal acetylcholine receptor), and elevated cerebrospinal fluid protein (>100 mg/dL) or pleocytosis (P=.02). Of 26 immunotherapy-responsive patients followed up for more than 1 year, 20 (77%) relapsed after discontinuing immunotherapy.; Conclusion: Identification of clinical and serologic clues to an autoimmune dementia allows early initiation of immunotherapy, and maintenance if needed, thus favoring an optimal outcome.;

Bibtex Citation

@article{Flanagan_2010, doi = {10.4065/mcp.2010.0326}, url = {}, year = 2010, month = {oct}, publisher = {Elsevier {BV}}, volume = {85}, number = {10}, pages = {881--897}, author = {Eoin P. Flanagan and Andrew McKeon and Vanda A. Lennon and Bradley F. Boeve and Max R. Trenerry and K. Meng Tan and Daniel A. Drubach and Keith A. Josephs and Jeffrey W. Britton and Jayawant N. Mandrekar and Val Lowe and Joseph E. Parisi and Sean J. Pittock}, title = {Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response}, journal = {Mayo Clinic Proceedings} }


autoimmune, autoimmunity, dementia, dexamethasone, diagnosis, electroencephalography, female, glucocorticoids, humans, immunoglobulins intravenous, immunologic factors, immunology, immunotherapy, magnetic resonance imaging, male, methods, methylprednisolone, middle aged, plasma exchange, prednisone, retrospective studies, therapeutic use, therapy, treatment outcome

Countries of Study


Types of Study

Cohort Study

Type of Outcomes


Type of Interventions

Pharmaceutical Interventions

Pharmaceutical Interventions