Autoimmune encephalitis misdiagnosis and mimics

Area of Science:

• Neurology
• Immunology

Background:

• Accurate diagnosis of autoimmune encephalitis (AE) is crucial.
• Exclusion of mimic conditions is a key diagnostic requirement.
• Misdiagnosis can lead to delayed or inappropriate treatment.

Purpose of the Study:

• To characterize neurological disorders that mimic autoimmune encephalitis (AE).
• To identify factors contributing to misdiagnosis of AE.
• To improve diagnostic accuracy for AE.

Main Methods:

• An independent PubMed search was conducted for studies on AE mimickers and misdiagnosed neurological disorders.
• Fifty-eight studies involving 66 patients were included in the analysis.
• Categorization of misdiagnosed conditions into neoplastic, infectious, genetic, neurodegenerative, and other neurological or systemic autoimmune disorders.

Main Results:

• A total of 66 patients across 58 studies were identified with conditions misdiagnosed as AE.
• Common misdiagnosed categories included neoplastic (17), infectious (15), and genetic (13) disorders.
• Factors contributing to misdiagnosis included unmet AE diagnostic criteria, atypical imaging, non-inflammatory cerebrospinal fluid, non-specific antibodies, and partial immunotherapy response.

Conclusions:

• Various neurological conditions can mimic autoimmune encephalitis (AE), complicating diagnosis.
• Diagnostic challenges include atypical presentations and non-specific findings.
• Careful evaluation against AE diagnostic criteria and consideration of confounding factors are essential for accurate diagnosis.