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.
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.
Authors: J A Lucas; R J Ivnik; G E Smith; D L Bohac; E G Tangalos; N R Graff-Radford; R C Petersen Journal: J Clin Exp Neuropsychol Date: 1998-04 Impact factor: 2.475
Authors: Michael D Geschwind; K Meng Tan; Vanda A Lennon; Ramon F Barajas; Aissa Haman; Christopher J Klein; S Andrew Josephson; Sean J Pittock Journal: Arch Neurol Date: 2008-10
Authors: Angela Vincent; Camilla Buckley; Jonathan M Schott; Ian Baker; Bonnie-Kate Dewar; Niels Detert; Linda Clover; Abigail Parkinson; Christian G Bien; Salah Omer; Bethan Lang; Martin N Rossor; Jackie Palace Journal: Brain Date: 2004-02-11 Impact factor: 13.501
Authors: Graham Mackay; Kate Ahmad; Jon Stone; Cathie Sudlow; David Summers; Richard Knight; Robert Will; Sarosh R Irani; Angela Vincent; Paul Maddison Journal: J Neurol Date: 2012-04-18 Impact factor: 4.849
Authors: Manoj K Mittal; Alejandro A Rabinstein; Sara E Hocker; Sean J Pittock; Eelco F M Wijdicks; Andrew McKeon Journal: Neurocrit Care Date: 2016-04 Impact factor: 3.210
Authors: H Prüss; M Höltje; N Maier; A Gomez; R Buchert; L Harms; G Ahnert-Hilger; D Schmitz; C Terborg; U Kopp; C Klingbeil; C Probst; S Kohler; J M Schwab; W Stoecker; J Dalmau; K P Wandinger Journal: Neurology Date: 2012-04-25 Impact factor: 9.910
Authors: Katherine E Brick; Chad H Weaver; Rodolfo Savica; Christine M Lohse; Mark R Pittelkow; Bradley F Boeve; Lawrence E Gibson; Michael J Camilleri; Carilyn N Wieland Journal: J Am Acad Dermatol Date: 2014-08-29 Impact factor: 11.527