Anti-Tr/DNER Antibody-associated Paraneoplastic Neurological Syndrome Presenting Limbic Encephalitis with Anaplastic Large Cell Lymphoma
An 82-year-old man presented with acute progressive disturbance of consciousness. We suspected autoimmune limbic encephalitis because of abnormal magnetic resonance imaging findings in the bilateral temporal lobes and cerebrospinal fluid pleocytosis. The patient tested positive for anti-Tr/Delta/Notch-like epidermal growth factor-related receptor (DNER) antibodies, and a tissue biopsy revealed complications of anaplastic large cell lymphoma. Typically, anti-Tr/DNER antibody-associated paraneoplastic neurological syndrome presents with cerebellar ataxia and is complicated by Hodgkin lymphoma (HL). However, there are rare cases that present only with limbic encephalitis (LE) and are complicated by tumors other than HL; therefore, aggressive antibody measurement and search for tumors are important.
Authors
- Ichiro Yabe (ORCID: https://orcid.org/0000-0002-1734-7997)
- Monami Tarisawa
- Takashi Inoue (ORCID: https://orcid.org/0000-0003-4691-7255)
- Masato Takahashi (ORCID: https://orcid.org/0000-0002-6641-9598)
- Koji Taniguchi (ORCID: https://orcid.org/0000-0002-8091-6005)
- Masaaki Matsushima (ORCID: https://orcid.org/0000-0001-6585-3686)
- Shintaro Fujii (ORCID: https://orcid.org/0000-0003-2869-7674)
- Akihiko Kudo (ORCID: https://orcid.org/0000-0002-2630-5991)
- Hisashi Uwatoko (ORCID: https://orcid.org/0000-0002-0353-8215)
- Hiroaki Yaguchi (ORCID: https://orcid.org/0000-0001-6643-8740)
- Shinya Tanaka (ORCID: https://orcid.org/0000-0001-6470-3301)
- Noriyuki Otsuka (ORCID: https://orcid.org/0000-0003-3024-6776)
- Ikuko Takahashi‐Iwata (ORCID: https://orcid.org/0000-0002-0919-8071)
- Shinichi Shirai (ORCID: https://orcid.org/0000-0001-9992-5254)
- Taichi Nomura
- Riku Miyaishi
- Keiko Tanaka
Institutions
- Hokkaido University (JP)
- Hokkaido University Hospital (JP)
- Niigata University (JP)
Publication Details
- Journal
- Internal Medicine
- Published
- 2024-12-11
- DOI
- https://doi.org/10.2169/internalmedicine.4530-24
- Citations
- 1
- Primary Topic
- Autoimmune Neurological Disorders and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.37
Funders
- Japan Epilepsy Research Foundation
- Epilepsiatutkimussäätiö
- Japan Society for the Promotion of Science