Autoimmune Dementia: Clinical Course and Predictors of Immunotherapy Response

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.
October 2010
Mayo Clinic Proceedings;Oct2010, Vol. 85 Issue 10, p881
Academic Journal
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.


Related Articles

  • An effective immunotherapy regimen for VGKC antibody-positive limbic encephalitis. S H Wong // Journal of Neurology, Neurosurgery & Psychiatry;Oct2010, Vol. 81 Issue 10, p1167 

    BACKGROUND: Voltage-gated potassium channel antibody-positive limbic encephalitis (VGKC+LE) frequently improves with immunotherapy, although the optimum regimen is unknown. The effectiveness of a combination immunomodulatory regimen was tested in consecutive VGKC+LE patients. METHODS: This was...

  • Therapeutische Apherese bei autoimmuner Enzephalitis. Ehrlich, S.; Fassbender, C.M.; Blaes, C.; Finke, C.; Günther, A.; Harms, L.; Hoffmann, F.; Jahner, K.; Klingel, R.; Kraft, A.; Lempert, T.; Tesch, M.; Thomsen, J.; Topka, H.; Jochim, J.; Veauthier, C.; Köhler, W. // Der Nervenarzt;Apr2013, Vol. 84 Issue 4, p498 

    Background: The correlation between detection of autoantibodies and the pattern and severity of symptoms in patients with encephalitis was the crucial factor for the initiation of immune therapy. The elimination of autoantibodies using therapeutic apheresis by plasma exchange (PE) and...

  • GENERAL CONSIDERATION OF GUILLIAIN BARRE SYNDROME. Gandhi, Zeel A.; Maisuria, Brijal A.; Patel, Rajesh G.; Arora, Bhoomi // International Journal of Pharmaceutical Sciences & Research;Nov2012, Vol. 3 Issue 11, p4135 

    Guillain-Barré syndrome is a rare but important disease that can lead to life threatening respiratory failure. This review summarises the verified consideration of Guillain-Barré syndrome (GBS) which are thought to be caused by direct autoimmune attack on peripheral nerves. Aim- Nerve...

  • Postpartum Acquired Hemophilia Factor VIII Inhibitors and Response to Therapy. Karakuş, Volkan; Çelik, Mustafa; Soysal, Dilek; Payzın, Bahriye // Turkish Journal of Hematology;Jun2012, Vol. 29 Issue 2, p197 

    A letter to the editor is presented which is concerned with postpartum acquired hemophilia factor VIII inhibitors and response to therapy.

  • Successful Treatment of Autoimmune Hemolytic Anemia with Steroid, IVIg, and Plasmapheresis in a Haploidentical Transplant Recipient. Uz, Burak; Özdemir, Evren; Aksu, Salih; Akyol, Tülay Karaağaç; Jones, Roy // Turkish Journal of Hematology;Jun2012, Vol. 29 Issue 2, p199 

    A letter to the editor is presented which is concerned with the successful treatment of autoimmune hemolytic anemia with steroid, IVIg, and plasmapheresis in a haploidentical transplant recipient

  • Pediatric Autoimmune Neuropsychiatric disorder Associated with streptococcus (PANDAS): An update on diagnosis and Treatment.  // Current Medical Literature: Psychiatry;2011, Vol. 22 Issue 3, p81 

    The article discusses important facts related to the diagnosis and treatment of pediatric autoimmune neuropsychiatric disorder associated with streptococcus (PANDAS). The diagnosis is done by observing patients who exhibit symptoms like prepubertal symptom onset, the presence of obsessive...

  • Intravenous immunoglobulin response in treatment-naïve chronic inflammatory demyelinating polyradiculoneuropathy. Kuitwaard, Krista; Hahn, Angelika F.; Vermeulen, Marinus; Venance, Shannon L.; van Doorn, Pieter A. // Journal of Neurology, Neurosurgery & Psychiatry;Dec2015, Vol. 86 Issue 12, p1331 

    Objective: There is no consensus on which treatment should be used preferentially in individual patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Patients unlikely to respond to intravenous immunoglobulin (IVIg) could be prescribed corticosteroids first to...

  • Long-Term Outcome of Anti-Glomerular Basement Membrane Antibody Disease Treated with Immunoadsorption. Biesenbach, Peter; Kain, Renate; Derfler, Kurt; Perkmann, Thomas; Soleiman, Afschin; Benharkou, Alexandra; Druml, Wilfred; Rees, Andrew; Säemann, Marcus D. // PLoS ONE;Jul2014, Vol. 9 Issue 7, p1 

    Background: Anti-glomerular basement membrane (GBM) antibody disease may lead to acute crescentic glomerulonephritis with poor renal prognosis. Current therapy favours plasma exchange (PE) for removal of pathogenic antibodies. Immunoadsorption (IAS) is superior to PE regarding efficiency of...

  • CIDP in Childhood--A Clinical Review. Rubin, Michael // Neurology Alert;Apr2013, Vol. 31 Issue 8, p59 

    HOW DOES CHILDHOOD CHRONIC INFLAMMATORY DEMYELINATING polyradiculoneuropathy (CIDP) compare to the adult form with respect to disease onset, clinical features, long-term outcome, and response to treatment? To address this question, records of all CIDP patients seen at Boston Children's Hospital...


Read the Article


Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics