An Overview of Autoimmune and Paraneoplastic Encephalitides
Michael J Bradshaw1,2,3, Jenny J Linnoila2,3
1Partners Multiple Sclerosis Center, Brigham and Women's Hospital, Boston, Massachusetts.
Seminars in Neurology
|July 17, 2018
Summary
Autoimmune encephalitis, a group of immune-mediated brain disorders, requires prompt recognition and treatment to prevent severe disability. This review details their clinical features, diagnosis, and management, including new insights into immunotherapy and infectious triggers.
Area of Science:
- Neuroimmunology
- Neurology
- Clinical Medicine
Background:
- Autoimmune encephalitis (AE) and immune-mediated encephalitides are increasingly recognized neurological disorders.
- Their incidence and prevalence are comparable to infectious causes of encephalitis, posing significant morbidity risks.
- Delayed diagnosis and treatment exacerbate patient outcomes, necessitating broader clinical awareness.
Purpose of the Study:
- To provide a comprehensive overview of autoimmune and paraneoplastic encephalitides.
- To elucidate the clinical manifestations, diagnostic approaches, and management strategies for AE.
- To highlight recent advancements, including novel immunotherapy-related AE, infectious triggers, and overlap syndromes.
Main Methods:
- Review of current literature on autoimmune encephalitis.
- Integration of neuroanatomical localization with specific antibody-associated syndromes.
- Focus on clinically relevant information for diagnosis and management.
Main Results:
- Detailed review of clinical presentations, diagnostic criteria, and therapeutic interventions for various AE subtypes.
- Discussion of AE associated with intracellular and cell surface/synaptic neuronal autoantibodies.
- Inclusion of emerging topics such as AE secondary to cancer immunotherapies and infectious triggers.
Conclusions:
- Autoimmune encephalitis represents a significant and growing area of neurological disease.
- A systematic, neuroanatomically guided approach combined with antibody identification is crucial for diagnosis.
- Prompt and appropriate management is essential to improve patient outcomes and reduce long-term morbidity.
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