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The importance of tissue-based assay in the diagnosis of autoimmune encephalitis

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Abstract

Background

The evaluation of autoimmune encephalitis (AIE) usually includes antibody testing with commercial kits capable of detecting only preselected antibodies. A non-antigen-specific assay may help detect other antibodies. In this study, we evaluate the utility and clinical relevance of an immunofluorescence assay (IFA) in the evaluation of AIE.

Methods

Immunofluorescence assay was performed on 1949 patients' serum/CSF between 2017 and 2020 and clinical relevance was designated to each case based on clinical course, suggested criteria and ancillary testing.

Results

Sixty-one patients (3.1%) had positive serum IFA, positive CSF, or both. Twenty-eight out of 42 patients who were positive only on IFA were designated as clinically relevant (67%), 8 inconclusive (19%), and 6 non-relevant (14%). Pleocytosis was significantly higher in the clinically relevant cases (74% vs. 20% for non-clinically relevant cases). Encephalopathy was the most common presentation (36%), followed by cerebellar syndrome (32%) and seizures (25%). The initial diagnosis changed due to IFA results in 13/28 (46%) cases and IFA result led to the initiation or modification of treatment in all cases (68% and 43%, respectively). Twenty-five patients were treated with 1st line immunotherapy and 12 with 2nd line immunotherapy, with 92% responding to treatment. Twenty-six clinically relevant patients underwent cancer workup: seven (25%) had confirmed malignancy and three had high suspicion of malignancy (total of 37%).

Conclusion

Non-antigen-specific assays, such as IFA, can identify antibodies not detected in commercially available kits and therefore are recommended in the evaluation of autoimmune encephalitis.

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Acknowledgements

We would like to thank Mrs. Bogdanovich Margarita and Mrs. Hadas David for technical support and assay preparation and Dr. Tom Gov for data collection.

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Authors and Affiliations

  1. Department of Neurology, Tel-Aviv Medical Center, Affiliated to the Sackler Faculty of Medicine, Tel Aviv University, 6 Weizmann Street, 6423906, Tel-Aviv, Israel

    Avi Gadoth & Yahel Segal

  2. Encephalitis Center, Tel Aviv Medical Center, Tel Aviv, Israel

    Avi Gadoth, Yael Paran, Orna Aizenstein & Yifat Alcalay

  3. Infectious Disease Unit, Tel Aviv Medical Center, Tel Aviv, Israel

    Yael Paran

  4. Department of Radiology, Tel-Aviv Medical Center, Affiliated to the Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

    Orna Aizenstein

  5. Immunology Laboratory, Tel Aviv Medical Center, Tel Aviv, Israel

    Yifat Alcalay

Authors
  1. Avi Gadoth
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  2. Yahel Segal
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  3. Yael Paran
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  4. Orna Aizenstein
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  5. Yifat Alcalay
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Corresponding author

Correspondence to Avi Gadoth.

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Conflicts of interest

AG has a patent for MAP1B as the antigen for PCA-2-associated paraneoplastic syndrome and as a biomarker for SCLC which is diagnosed by IFA (1 patient in this study). YS, YP, OA, YA have no conflict of interest.

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Gadoth, A., Segal, Y., Paran, Y. et al. The importance of tissue-based assay in the diagnosis of autoimmune encephalitis. J Neurol 269, 3588–3596 (2022). https://doi.org/10.1007/s00415-022-10973-8

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  • DOI: https://doi.org/10.1007/s00415-022-10973-8

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