Anti-Amphiphysin-associated limbic encephalitis in a 72-year-old patient with aortic angiosarcoma

• Published in: BMJ case reports Vol. 12; no. 3; p. e226798
• Main Authors: Wagner-Altendorf, Tobias A, Wandinger, Klaus-Peter, Frydrychowicz, Alex, Merseburger, Axel S, Münte, Thomas F
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01.03.2019; BMJ Publishing Group
• Series: Case Report
• Subjects: Aged; Aortic Diseases - complications; Aortic Diseases - pathology; Autoimmune diseases; Bone Neoplasms - complications; Bone Neoplasms - pathology; Bone Neoplasms - secondary; Brain damage; Breast cancer; Cancer therapies; Case reports; Coronary vessels; Drug Therapy - methods; Encephalitis; Fatal Outcome; Hemangiosarcoma; Humans; Immunoglobulins; Immunosuppressive Agents - therapeutic use; Kidney cancer; Kidney Neoplasms - complications; Kidney Neoplasms - pathology; Kidney Neoplasms - secondary; Kidney Neoplasms - surgery; Limbic Encephalitis - complications; Limbic Encephalitis - diagnosis; Limbic Encephalitis - drug therapy; Limbic Encephalitis - immunology; Lung cancer; Male; Metastasis; Muscle Neoplasms - complications; Muscle Neoplasms - pathology; Muscle Neoplasms - secondary; Neoplasm Metastasis - pathology; Nephrectomy - methods; Nerve Tissue Proteins - antagonists & inhibitors; Nerve Tissue Proteins - metabolism; Stroke; Unusual Association of Diseases/Symptoms; neurology; oncology
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2018-226798

Paraneoplastic autoimmune encephalopathic syndromes have been described most often in association with small cell lung cancer or breast cancer, tumours of the ovaries, testes, lymphoma and thymoma. Antibodies associated with paraneoplastic encephalopathies are, among others, anti-Hu, anti-Ma2 and, in part, anti-N-methyl-D-aspartate(NMDA)-receptor antibodies. Here, we present the case of a 72-year-old patient hospitalised due to progressive cognitive decline and disorientation. Diagnostic workup revealed paraneoplastic anti-amphiphysin associated limbic encephalitis on the basis of an aortic angiosarcoma with metastases to kidney, muscle and bones. Highly aggressive chemotherapy as well as immunosuppressive therapy and cytoreductive laparoscopic nephrectomy were initiated. However, follow-up revealed further tumour progress and a worsening of neurological symptoms.