Paraneoplastic neuromyelitis optica spectrum disorder: A case report and review of the literature. (February 2018)

• Record Type: Journal Article
• Authors: Annus, Ádám; Bencsik, Krisztina; Obál, Izabella; Kincses, Zsigmond Tamás; Tiszlavicz, László; Höftberger, Romana; Vécsei, László
• Is Part Of: Journal of clinical neuroscience. Volume 48(2018)
• Page Start: 7
• Page End: 10
• Publication Date: 2018-02
• Subjects: Neuromyelitis optica -- Aquaporin-4 -- Paraneoplastic; Brain -- Surgery -- Periodicals; Neurosciences -- Periodicals; Nervous system -- Surgery -- Periodicals; Brain -- surgery -- Periodicals; Neurosurgical Procedures -- Periodicals; Neurosciences -- Periodicals; Electronic journals; 616.8
• DOI: 10.1016/j.jocn.2017.10.030
• Languages: English
• ISSNs: 0967-5868

Abstract

Highlights: A rare case of paraneoplastic neuromyelitis optica spectrum disorder (NMOSD) is presented. A review of similar cases is given. A possible pathomechanism of NMOSD is proposed. Abstract: Neuromyelitis optica spectrum disorders (NMOSD) are demyelinating, autoimmune diseases affecting the central nervous system. Typically, recurrent optic neuritis and longitudinal extensive transverse myelitis dominates the clinical picture. In most cases NMOSD are associated with autoantibodies targeting the water channel aquaporin-4 (AQP-4). NMOSD usually present in young adults. Clinical findings suggestive of NMOSD in elderly patients should raise the suspicion of a paraneoplastic etiology. To our knowledge, we report the first case of a 66 year-old female patient with paraneoplastic NMOSD that is associated with squamous cell lung carcinoma. Anti-AQP-4 was present in both the serum and cerebrospinal fluid of the patient. However, immunhistological staining of the malignant tissue did not show presence of AQP-4 on the surface of tumour cells.