Diagnosis is uncertain in cases with atypical presentations and nonspecific serum antibodies.
Autoimmune encephalitis is now frequently considered in the differential diagnosis for patients presenting with subacute cognitive dysfunction. To describe the characteristics of autoimmune encephalitis misdiagnosis, 107 misdiagnosed patients were identified from 6 participating centers.
The median age of the cohort was 48 years. Onset was insidious in 48%. False-positive antibodies occurred in 50%, most commonly against glutamic acid decarboxylase 65 (GAD65), voltage-gated potassium channel (VGKC), and N-methyl-D-aspartate receptor (NMDAR). MRI was abnormal in 18%, and cerebrospinal fluid (CSF) pleocytosis was present in 19%. Neural antibodies were present in serum in 48% and in CSF in 8%. Electroencephalography was abnormal in 39%. Alterna…
Reviewing Author
DisclosuresConsultant/Advisory BoardAlexion Pharmaceuticals; Amgen; Astoria; Biogen; Bristol Myers Squibb; Celltrion; Genentech; Hoffmann-La Roche; Genzyme; EMD Serono; Immpact-Bio; Immunic Therapeutics; Kyverna; Lundbeck; Novartis; Sandoz; TG Therapeutics
Grant/Research SupportNational Institutes of Health; National Multiple Sclerosis Society; U.S. Department of Defense
Leadership Positions in Professional SocietiesConsortium of Multiple Sclerosis Centers (Treasurer)
DisclosuresConsultant/Advisory BoardAlexion Pharmaceuticals; Amgen; Astoria; Biogen; Bristol Myers Squibb; Celltrion; Genentech; Hoffmann-La Roche; Genzyme; EMD Serono; Immpact-Bio; Immunic Therapeutics; Kyverna; Lundbeck; Novartis; Sandoz; TG Therapeutics
Grant/Research SupportNational Institutes of Health; National Multiple Sclerosis Society; U.S. Department of Defense
Leadership Positions in Professional SocietiesConsortium of Multiple Sclerosis Centers (Treasurer)