Abstract
Autoimmune encephalitis (AE) can rarely manifest as a predominantly psychiatric syndrome without overt neurological symptoms. This study’s aim was to characterize psychiatric patients with AE; therefore, anonymized data on patients with suspected AE with predominantly or isolated psychiatric syndromes were retrospectively collected. Patients with readily detectable neurological symptoms suggestive of AE (e.g., epileptic seizures) were excluded. Patients were classified as “probable psychiatric AE (pAE),” if well-characterized neuronal IgG autoantibodies were detected or “possible pAE” (e.g., with detection of nonclassical neuronal autoantibodies or compatible cerebrospinal fluid (CSF) changes). Of the 91 patients included, 21 (23%) fulfilled our criteria for probable (autoantibody-defined) pAE and 70 (77%) those for possible pAE. Among patients with probable pAE, 90% had anti-NMDA receptor (NMDA-R) autoantibodies. Overall, most patients suffered from paranoid-hallucinatory syndromes (53%). Patients with probable pAE suffered more often from disorientation (p < 0.001) and impaired memory (p = 0.001) than patients with possible pAE. Immunotherapies were performed in 69% of all cases, mostly with high-dose corticosteroids. Altogether, 93% of the patients with probable pAE and 80% of patients with possible pAE reportedly benefited from immunotherapies (p = 0.251). In summary, this explorative, cross-sectional evaluation confirms that autoantibody-associated AE syndromes can predominantly manifest as psychiatric syndromes, especially in anti-NMDA-R encephalitis. However, in three out of four patients, diagnosis of possible pAE was based on nonspecific findings (e.g., slight CSF pleocytosis), and well-characterized neuronal autoantibodies were absent. As such, the spectrum of psychiatric syndromes potentially responding to immunotherapies seems not to be limited to currently known autoantibody-associated AE. Further trials are needed.
Highlights
MATERIALS AND METHODSAutoimmune encephalitis (AE) comprises an emerging group of Patients and data acquisition autoinflammatory diseases of the brain
Steroids were used most frequently, RESULTS Sociodemographic and clinical characteristics Altogether, 91 patients were included in the study: 21 (23%) with probable psychiatric AE (pAE) and 70 (77%) with possible pAE (Table 2)
No significant differences between the patients with probable and possible pAE were found in overall Cerebrospinal fluid (CSF), MRI, EEG, and fluorodeoxyglucose positron emission tomography (FDG-PET) alterations
Summary
Autoimmune encephalitis (AE) comprises an emerging group of Patients and data acquisition autoinflammatory diseases of the brain. Probable pAE and 80% of the patients with possible pAE were considered to benefit from immunomodulatory treatment by treating physicians (outcome not documented for five patients), without a significant group difference (p = 0.251). Steroids were used most frequently (in 57% at high dose, in 38% at low dose), RESULTS Sociodemographic and clinical characteristics Altogether, 91 patients were included in the study: 21 (23%) with probable pAE and 70 (77%) with possible pAE (Table 2) In both groups, women were slightly overrepresented (57% in total). With preexisting psychiatric conditions present in 5/19 (26%) of patients with probable AE, these were almost twofold less frequent than in the cohort with possible pAE (34/67; 51%) This difference failed to reach statistical significance (p = 0.059). No significant differences between the patients with probable and possible pAE were found in overall CSF, MRI, EEG, and FDG-PET alterations.
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