TITLE

Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response

AUTHOR(S)
Flanagan, E0in P.; McKeon, Andrew; Lennon, Vanda A.; Boeve, Bradley F.; Trenerry, Max R.; Tan, K. Meng; Drubach, Daniel A.; Joseph, Keith A.; Britton, Jeffrey W.; Mandrekar, Jayawant N.; Lowe, Val; Parisi, Joseph E.; Pittock, Sean J.
PUB. DATE
October 2010
SOURCE
Mayo Clinic Proceedings;Oct2010, Vol. 85 Issue 10, p881
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
OBJECTIVE: To define the diagnostic characteristics and predictors of treatment response in patients with suspected autoimmuno 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, neuropsychoiogical testing, or both). RESULTS: initial immunotherapeutic regimens included methylprednisoione in 56 patients (78%), prednlsone in 12 patients (17%), dexamethasone in 2 patients (3%), intravenous immune giobuiin in 1 patient (1%), and plasma exchange in I patient (1%). Forty-six patients (64%) improved, most in the first week of treatment. Thirty-five percent of these immunotherapy responders were initiality diagnosed as having a neurodegenerative or prion disorder. Pretreatment and posttreatment neuropsychological score comparisons revealed improvement in almost au cognitive domains, most notabiy learning and memory. Radiologic or eiectroencephaiographic 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 compiex autoantibody (P=.01; neuronal voitage-gated potassium channel more than calcium channei or neuronal acetyichoilne 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 seroiogic ciues to an autoimmune dementia allows early initiation of immunotherapy, and maintenance it needed, thus favoring an optimal outcome.
ACCESSION #
54592670

 

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