Manoj K Mittal1,2, Alejandro A Rabinstein1, Sara E Hocker1, Sean J Pittock1,3, Eelco F M Wijdicks1, Andrew McKeon4,5. 1. Department of Neurology, College of Medicine, Mayo Clinic, 200 First Street S.W., Rochester, MN, 55905, USA. 2. Department of Neurology, University of Kansas Medical Center, Kansas, KS, USA. 3. Department of Laboratory Medicine and Pathology, College of Medicine, Mayo Clinic, Rochester, MN, USA. 4. Department of Neurology, College of Medicine, Mayo Clinic, 200 First Street S.W., Rochester, MN, 55905, USA. mckeon.andrew@mayo.edu. 5. Department of Laboratory Medicine and Pathology, College of Medicine, Mayo Clinic, Rochester, MN, USA. mckeon.andrew@mayo.edu.
Abstract
BACKGROUND: To report the clinical and laboratory characteristics, clinical courses, and outcomes of Mayo Clinic, Rochester, MN, ICU-managed autoimmune encephalitis patients (January 1st 2003-December 31st 2012). METHODS: Based on medical record review, twenty-five patients were assigned to Group 1 (had ≥1 of classic autoimmune encephalitis-specific IgGs, n = 13) or Group 2 (had ≥3 other characteristics supporting autoimmunity, n = 12). RESULTS: Median admission age was 47 years (range 22-88); 17 were women. Initial symptoms included ≥1 of subacute confusion or cognitive decline, 13; seizures, 12; craniocervical pain, 5; and personality change, 4. Thirteen Group 1 patients were seropositive for ≥1 of VGKC-complex-IgG (6; including Lgi1-IgG in 2), NMDA-R-IgG (4), AMPA-R-IgG (1), ANNA-1 (1), Ma1/Ma2 antibody (1), and PCA-1 (1). Twelve Group 2 patients had ≥3 other findings supportive of an autoimmune diagnosis (median 4; range 3-5): ≥1 other antibody type detected, 9; an inflammatory CSF, 8; ≥1 coexisting autoimmune disease, 7; an immunotherapy response, 7; limbic encephalitic MRI changes, 5; a paraneoplastic cause, 4; and diagnostic neuropathological findings, 2. Among 11 patients ICU-managed for ≥4 days, neurological improvements were attributable to corticosteroids (5/7 treated), plasmapheresis (3/7), or rituximab (1/3). At last follow-up, 10 patients had died. Of the remaining 15 patients, 6 (24%) had mild or no disability, 3 (12%) had moderate cognitive problems, and 6 (24%) had dementia (1 was bed bound). Median modified Rankin score at last follow-up was 3 (range 0-6). CONCLUSIONS: Good outcomes may occur in ICU-managed autoimmune encephalitis patients. Clinical and testing characteristics are diverse. Comprehensive diagnostics should be pursued to facilitate timely treatment.
BACKGROUND: To report the clinical and laboratory characteristics, clinical courses, and outcomes of Mayo Clinic, Rochester, MN, ICU-managed autoimmune encephalitispatients (January 1st 2003-December 31st 2012). METHODS: Based on medical record review, twenty-five patients were assigned to Group 1 (had ≥1 of classic autoimmune encephalitis-specific IgGs, n = 13) or Group 2 (had ≥3 other characteristics supporting autoimmunity, n = 12). RESULTS: Median admission age was 47 years (range 22-88); 17 were women. Initial symptoms included ≥1 of subacute confusion or cognitive decline, 13; seizures, 12; craniocervical pain, 5; and personality change, 4. Thirteen Group 1 patients were seropositive for ≥1 of VGKC-complex-IgG (6; including Lgi1-IgG in 2), NMDA-R-IgG (4), AMPA-R-IgG (1), ANNA-1 (1), Ma1/Ma2 antibody (1), and PCA-1 (1). Twelve Group 2 patients had ≥3 other findings supportive of an autoimmune diagnosis (median 4; range 3-5): ≥1 other antibody type detected, 9; an inflammatory CSF, 8; ≥1 coexisting autoimmune disease, 7; an immunotherapy response, 7; limbic encephalitic MRI changes, 5; a paraneoplastic cause, 4; and diagnostic neuropathological findings, 2. Among 11 patients ICU-managed for ≥4 days, neurological improvements were attributable to corticosteroids (5/7 treated), plasmapheresis (3/7), or rituximab (1/3). At last follow-up, 10 patients had died. Of the remaining 15 patients, 6 (24%) had mild or no disability, 3 (12%) had moderate cognitive problems, and 6 (24%) had dementia (1 was bed bound). Median modified Rankin score at last follow-up was 3 (range 0-6). CONCLUSIONS: Good outcomes may occur in ICU-managed autoimmune encephalitispatients. Clinical and testing characteristics are diverse. Comprehensive diagnostics should be pursued to facilitate timely treatment.
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