SOP: antibody-associated autoimmune encephalitis

Author:

Rössling Rosa,Prüss HaraldORCID

Abstract

Abstract Background Antibody-mediated and paraneoplastic autoimmune encephalitides (AE) present with a broad spectrum of clinical symptoms. They often lead to progressing inflammatory changes of the central nervous system with subacute onset and can cause persistent brain damage. Thus, to promptly start the appropriate and AE-specific therapy, recognition of symptoms, initiation of relevant antibody diagnostics and confirmation of the clinical diagnosis are crucial, in particular as the diseases are relatively rare. Aim This standard operating procedure (SOP) should draw attention to the clinical presentation of AE, support the diagnostic approach to patients with suspected AE and guide through the necessary steps including therapeutic decisions, tumour screening and exclusion of differential diagnoses. Method Based on existing diagnostic algorithms, treatment recommendations and personal experiences, this SOP gives an overview of clinical presentation, diagnostic procedures and therapy in AE. Additional information is provided within an accompanying text and a table describing the most important autoantibodies and their characteristics. Results The initial steps of the AE flow chart are based on clinical symptoms and the patient’s history. Assignment to paraneoplastic or antibody-mediated AE is sometimes clinically possible. Diagnostics should include MRI, EEG and CSF analysis with antibody panel diagnostic. Definite AE can be diagnosed if the underlying antibody is compatible with the clinical presentation. Classification of probable AE may be possible even with negative anti-neuronal autoantibodies if the clinical presentation and laboratory abnormalities are highly suggestive of AE. The confirmed AE diagnosis requires immediate initiation of immunotherapy. Conclusion The SOP facilitates the recognition of patients with AE and presents the necessary diagnostic and therapeutic steps.

Funder

Deutsche Forschungsgemeinschaft

Publisher

Springer Science and Business Media LLC

Subject

Automotive Engineering

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1. Autoimmune Enzephalitiden – Erkennen und Behandeln;DGNeurologie;2024-06-11

2. Canadian Consensus Guidelines for the Diagnosis and Treatment of Autoimmune Encephalitis in Adults;Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques;2024-02-05

3. Autoimmune Encephalitis: Insights Into Immune-Mediated Central Nervous System Injury;Korean Journal of Radiology;2024

4. Pathophysiological Effects of Autoantibodies in Autoimmune Encephalitides;Cells;2023-12-20

5. SOP: acute hyperkinetic movement disorders;Neurological Research and Practice;2023-07-27

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