抗Tr/DNER抗体相关副肿瘤性神经系统综合征伴间变性大细胞淋巴瘤并表现为边缘叶脑炎
Anti-Tr/DNER Antibody-associated Paraneoplastic Neurological Syndrome Presenting Limbic Encephalitis with Anaplastic Large Cell Lymphoma.
作者信息
Fujii Shintaro, Yaguchi Hiroaki, Takahashi-Iwata Ikuko, Inoue Takashi, Tarisawa Monami, Nomura Taichi, Kudo Akihiko, Uwatoko Hisashi, Shirai Shinichi, Matsushima Masaaki, Miyaishi Riku, Otsuka Noriyuki, Tanaka Keiko, Taniguchi Koji, Takahashi Masato, Tanaka Shinya, Yabe Ichiro
机构信息
Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Japan.
Department of Surgical Pathology, Hokkaido University Hospital, Japan.
出版信息
Intern Med. 2025 Jul 1;64(13):2057-2061. doi: 10.2169/internalmedicine.4530-24. Epub 2024 Dec 12.
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.
一名82岁男性出现急性进行性意识障碍。由于双侧颞叶磁共振成像结果异常及脑脊液淋巴细胞增多，我们怀疑为自身免疫性边缘叶脑炎。患者抗Tr/Delta/Notch样表皮生长因子相关受体（DNER）抗体检测呈阳性，组织活检显示为间变性大细胞淋巴瘤并发症。通常，抗Tr/DNER抗体相关副肿瘤性神经系统综合征表现为小脑共济失调，并伴有霍奇金淋巴瘤（HL）。然而，也有罕见病例仅表现为边缘叶脑炎（LE），且伴有HL以外的肿瘤；因此，积极进行抗体检测和寻找肿瘤很重要。
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