Clinical, diagnostic and therapeutic challenges and pitfalls in AIE

Clinical challenges Diagnostic pitfalls Therapeutic challenges
1. Lack of comprehensive clinical data; Insufficient data can hinder accurate diagnosis.
2. Diagnostic challenges for atypical cases; Unusual presentations may delay or complicate diagnosis.
3. Overlapping symptoms with other disorders; Symptoms may resemble other neurological conditions.
4. Variability in disease progression; The disease can manifest differently in different individuals.
1. Inaccessible antibody testing; Results may take several weeks to obtain in many institutions.
2. Lack of autoantibodies does not exclude immune-mediated disorders; The absence of autoantibodies does not rule out an immune cause.
3. Positive autoantibody test does not guarantee accurate diagnosis; A positive test does not always mean the disorder is present.
4. Lack of specific biomarkers; Current biomarkers may not be definitive for all cases.
1. Difficulty in early diagnosis; The initial symptoms can be nonspecific and may mimic other neurological or psychiatric diseases.
2. First-like vs. second-like therapies: While first-line therapies (steroids, IVIG, and plasmapheresis) can be effective for many, more aggressive second-line therapies, such as rituximab or cyclophosphamide is required in some patients.
3. Treatment resistance; some patients may not respond adequately to standard therapies.
4. Relapse and side effect; Even after successful initial treatment, AIE can relapse and potential side effects ranging from infections to organ toxicities.

IVIG, intravascular immunoglobin treatment.

Exp Neurobiol 2024;33:1~17 https://doi.org/10.5607/en23036
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