Background <p>This case reports aimed to clarify the characteristic MRI findings in GABA<sub>A</sub> receptor encephalitis.</p> Case presentation <p>We report the cases of two encephalitis patients who had a history of thymoma surgery and in whom anti-GABA<sub>A</sub>R antibodies and characteristic MRI findings were observed. These patients were clinically diagnosed as having thymoma-associated paraneoplastic encephalitis (TAPE) before the antibodies were identified. TAPE is often associated with antibodies to various neuronal surface antigens, including GABA<sub>A</sub>R, AMPAR, CASPR2, LGI1, and GlyR. MRI findings of autoimmune encephalitis are extremely variable, but when multiple homogenous high-signal-intensity lesions are observed on FLAIR images, as in these cases, anti-GABA<sub>A</sub>R antibodies are likely to be involved. We have named here this MRI finding the “cotton-wool-like appearance”.</p> Conclusion <p>In cases of encephalitis with such characteristic radiological findings, neurologists should first investigate the presence or a history of thymoma surgery, and then promptly consider testing for anti-GABA<sub>A</sub>R antibodies.</p>

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Characteristic MRI findings in GABAA receptor encephalitis with thymoma: a report of two cases

  • Ayaka Kakurai,
  • Reiji Koide,
  • Kosuke Matsuzono,
  • Yuhei Anan,
  • Takafumi Mashiko,
  • Ryota Tanaka,
  • Keiko Tanaka,
  • Shigeru Fujimoto

摘要

Background

This case reports aimed to clarify the characteristic MRI findings in GABAA receptor encephalitis.

Case presentation

We report the cases of two encephalitis patients who had a history of thymoma surgery and in whom anti-GABAAR antibodies and characteristic MRI findings were observed. These patients were clinically diagnosed as having thymoma-associated paraneoplastic encephalitis (TAPE) before the antibodies were identified. TAPE is often associated with antibodies to various neuronal surface antigens, including GABAAR, AMPAR, CASPR2, LGI1, and GlyR. MRI findings of autoimmune encephalitis are extremely variable, but when multiple homogenous high-signal-intensity lesions are observed on FLAIR images, as in these cases, anti-GABAAR antibodies are likely to be involved. We have named here this MRI finding the “cotton-wool-like appearance”.

Conclusion

In cases of encephalitis with such characteristic radiological findings, neurologists should first investigate the presence or a history of thymoma surgery, and then promptly consider testing for anti-GABAAR antibodies.