Anti-Ma2–associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report

• Published in: Medicine (Baltimore) Vol. 96; no. 40; p. e8228
• Main Authors: Ju, Weina, Qi, Baochang, Wang, Xu, Yang, Yu
• Format: Journal Article
• Language: English
• Published: United States The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved 01.10.2017; Wolters Kluwer Health
• Subjects: Aged; Antibodies - blood; Antibodies - cerebrospinal fluid; Antigens, Neoplasm - immunology; Clinical Case Report; Humans; Limbic Encephalitis - complications; Limbic Encephalitis - immunology; Lymphoma, Non-Hodgkin - complications; Lymphoma, Non-Hodgkin - immunology; Male; Nerve Tissue Proteins - immunology; Paraneoplastic Syndromes, Nervous System - complications; Paraneoplastic Syndromes, Nervous System - immunology; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - complications; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - immunology
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000008228

We report the rare case of a 74-year-old man with anti-Ma2-associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease. The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations. Brain and spinal cord magnetic resonance imaging (MRI) yielded normal results. Anti-Ma2 antibodies were detected in both serum and cerebrospinal fluid. A 4-hour electroencephalogram showed irregular sharp slow waves and δ waves in the temporal region. Electromyography showed peripheral nerve demyelination. Positron-emission tomography/computed tomography (PET-CT) examination revealed hypermetabolism in the lymph nodes of the whole body. Biopsy of the lymph nodes showed non-Hodgkin lymphoma. A clinical diagnosis of lymphoma and PNS was made. The patient was treated with intravenous dexamethasone (15 mg/day) for 3 days. We have presented a rare case of a PNS involving both the central and peripheral nervous systems. The clinical features of this case indicated anti-Ma2-associated encephalitis and chronic inflammatory demyelinating polyneuropathy. PET-CT played a critical role in enabling early diagnosis and prompt treatment in this case.