Paraneoplastic brainstem encephalitis in a patient with exceptionally long course of a metastasized neuroendocrine rectum neoplasm

• Published in: BMC cancer 2014-09, Vol.14 (1), p.691-691, Article 691
• Main Authors: Boch, Michael, Rinke, Anja, Rexin, Peter, Seipelt, Maria, Brödje, Dörte, Schober, Marvin, Gress, Thomas M, Michl, Patrick, Krug, Sebastian
• Format: Article
• Language: English
• Subjects: Adult; Anti-Inflammatory Agents - therapeutic use; Antigens; Biopsy; Brain Stem - pathology; Case Report; Colleges & universities; Consent; Disease; Encephalitis - complications; Encephalitis - diagnosis; Encephalitis - drug therapy; Female; Gastroenterology; Humans; Laboratories; Liver; Liver Neoplasms - diagnosis; Liver Neoplasms - secondary; Medical imaging; Metastasis; Methylprednisolone - therapeutic use; Neuroendocrine Tumors - complications; Neuroendocrine Tumors - diagnosis; Neuroendocrine Tumors - pathology; Paraneoplastic Syndromes, Nervous System - complications; Paraneoplastic Syndromes, Nervous System - diagnosis; Paraneoplastic Syndromes, Nervous System - drug therapy; Patients; Rectal Neoplasms - complications; Rectal Neoplasms - diagnosis; Rectal Neoplasms - pathology; Treatment Outcome; Tumors
• ISSN: 1471-2407; 1471-2407
• DOI: 10.1186/1471-2407-14-691

Paraneoplastic neurological syndromes (PNS) have frequently been described in patients with lung or breast cancer. However, some reports also described a correlation to carcinoid tumors, probably triggered via the excessive release of hormones. We report the case of a 40-year-old woman that was diagnosed with a neuroendocrine neoplasm (NEN) of the rectum and multiple synchronous liver metastases ten years ago. She initially responded well to transarterial chemoembolization (TACE), resulting in prolonged disease stabilization. However, ten years after initial diagnosis the patient developed unspecific neurological symptoms that could not be classified by standard neurological diagnostic work-up. Special laboratory analysis revealed a high titer of anti-Ri (ANNA-2), a well-characterized antibody that is associated with paraneoplastic neurologic syndromes. The patient's symptoms improved markedly after a 5-day-course of high-dose glucocorticoid therapy. To our knowledge, this is the first report of a Ri-positive PNS in a patient with hormone-negative rectal NEN. PNS can complicate the patient's clinical course, response to treatment, impact prognosis and even be interpreted as metastatic spread. However, owing to their rarity, the knowledge of these syndromes is very helpful in order to be able to provide evidence-based diagnostic and therapeutic approaches.