Autoimmune Encephalitis with Neuronal Surface Autoantibodies and Other Suspected Cases of Autoimmune Etiology: A Single-Center Experience in Poland

Autoimmune encephalitis (AE) is an autoantibody-mediated central nervous system disorder with diverse neuropsychiatric and neurological manifestations, and should be considered in the differential diagnosis of acute and subacute neurological or psychiatric syndromes. In this retrospective study, we analyzed 65 patients: 54 with AE (47 antibody-positive, seven antibody-negative) and 11 antibody-positive without AE. The most frequently detected antibodies targeted N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma-inactivated protein 1 (LGI1), and contactin-associated protein-like 2 (CASPR2)-key synaptic and axonal membrane proteins involved in excitatory neurotransmission, neuronal signaling, and synaptic plasticity. Clinical presentations were heterogeneous, ranging from common neuropsychiatric, cognitive, and seizure manifestations to atypical brainstem or cerebellar features. Symptom distribution analysis further demonstrated distinct patterns among Ab-positive AE, Ab-negative AE, and Ab-positive non-AE groups, with specific symptom-antibody associations providing potential diagnostic clues. Diagnostic complexity was underscored by unusual age at onset, overlap with multiple sclerosis, cases preceded by herpes labialis, and dual-antibody detection. A subset of antibody-positive patients had alternative diagnoses, highlighting the need for careful clinical correlation and cautious interpretation of antibody results. These findings illustrate the diagnostic challenges and broad clinical spectrum of AE, emphasizing the importance of integrating serological, clinical, and imaging data to improve diagnostic accuracy and guide management.

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Journal
International Journal of Molecular Sciences
Published
2025-09-30
DOI
https://doi.org/10.3390/ijms26199541
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
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article

Autoimmune Encephalitis with Neuronal Surface Autoantibodies and Other Suspected Cases of Autoimmune Etiology: A Single-Center Experience in Poland

Iwona Kurkowska‐Jastrzębska, Halina Sienkiewicz‐Jarosz, Katarzyna Polanowska, Katarzyna Kurczych et al.
International Journal of Molecular Sciences
Autoimmune Neurological Disorders and Treatments
article

Autoimmune Encephalitis with Neuronal Surface Autoantibodies and Other Suspected Cases of Autoimmune Etiology: A Single-Center Experience in Poland

Iwona Kurkowska‐Jastrzębska, Halina Sienkiewicz‐Jarosz, Katarzyna Polanowska, Katarzyna Kurczych, Agnieszka Piechal, Agnieszka Cudna
article en

Abstract

Autoimmune encephalitis (AE) is an autoantibody-mediated central nervous system disorder with diverse neuropsychiatric and neurological manifestations, and should be considered in the differential diagnosis of acute and subacute neurological or psychiatric syndromes. In this retrospective study, we analyzed 65 patients: 54 with AE (47 antibody-positive, seven antibody-negative) and 11 antibody-positive without AE. The most frequently detected antibodies targeted N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma-inactivated protein 1 (LGI1), and contactin-associated protein-like 2 (CASPR2)-key synaptic and axonal membrane proteins involved in excitatory neurotransmission, neuronal signaling, and synaptic plasticity. Clinical presentations were heterogeneous, ranging from common neuropsychiatric, cognitive, and seizure manifestations to atypical brainstem or cerebellar features. Symptom distribution analysis further demonstrated distinct patterns among Ab-positive AE, Ab-negative AE, and Ab-positive non-AE groups, with specific symptom-antibody associations providing potential diagnostic clues. Diagnostic complexity was underscored by unusual age at onset, overlap with multiple sclerosis, cases preceded by herpes labialis, and dual-antibody detection. A subset of antibody-positive patients had alternative diagnoses, highlighting the need for careful clinical correlation and cautious interpretation of antibody results. These findings illustrate the diagnostic challenges and broad clinical spectrum of AE, emphasizing the importance of integrating serological, clinical, and imaging data to improve diagnostic accuracy and guide management.

International Journal of Molecular SciencesVol. 26(19)
Maria Curie-Skłodowska University (PL), Medical University of Warsaw (PL), The Maria Sklodowska-Curie National Research Institute of Oncology (PL), Institute of Psychiatry and Neurology (PL)
Openalex Percentile: Top 61%
Autoimmune Neurological Disorders and Treatments
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