TY - JOUR
T1 - Autoimmune Encephalitis in the ICU
T2 - Analysis of Phenotypes, Serologic Findings, and Outcomes
AU - Mittal, Manoj K.
AU - Rabinstein, Alejandro A.
AU - Hocker, Sara E.
AU - Pittock, Sean J.
AU - M Wijdicks, Eelco F.
AU - McKeon, Andrew
N1 - Publisher Copyright:
© 2015, Springer Science+Business Media New York.
PY - 2016/4/1
Y1 - 2016/4/1
N2 - 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.
AB - 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.
KW - Autoimmune
KW - Encephalitis
KW - Encephalopathy
KW - ICU
KW - Paraneoplastic
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U2 - 10.1007/s12028-015-0196-8
DO - 10.1007/s12028-015-0196-8
M3 - Article
C2 - 26319044
AN - SCOPUS:84941348079
SN - 1541-6933
VL - 24
SP - 240
EP - 250
JO - Neurocritical care
JF - Neurocritical care
IS - 2
ER -