Long extensive transverse myelitis associated with aquaporin-4 antibody and breast cancer: Favorable response to cancer treatment

Abstract

Context: Long extensive transverse myelitis (LETM) seldom develops in patients with breast cancer who are aquaporin-4 antibody (Aqp-4 Ab)-positive. Whether this association is coincidental is not well understood. Findings: A 62-year-old woman presented with treatment-resistant LETM and Aqp-4 Ab. Two months later, a stage 3 invasive ductal carcinoma was detected in her right breast. Following tumor resection and chemotherapy, her neurologic symptoms and magnetic resonance imaging findings significantly improved and serum Aqp-4 Ab disappeared. The breast tumor samples of this patient and neurologically normal patients showed inflammatory infiltrates and Aqp-4 expressing cells. Conclusion/Clinical Relevance: The temporal association between tumor treatment, amelioration of clinical findings, and seroreversion suggest that coexistence of cancer and LETM is not coincidental. Cancer screening should be considered at least in treatment-resistant LETM cases.

Longitudinally extensive transverse myelitis with AQP4 antibodies revealing ovarian teratoma

“…Alternatively AQP4 antibodies might be produced peripherally and enter the CNS secondarily (Papadopoulos and Verkman, 2012). In a recent report of a patient with paraneoplastic myelitis, breast cancer and AQP4 antibodies, the tumor was also found to express the AQP4 antigen (Armağan et al, 2012). Because AQP4 is an antigen whose expression is not restricted to the CNS, mechanisms leading to paraneoplastic immunity in patients with AQP4 antibodies probably differ from the classic model proposed for PNS.…”

“…Anti-aquaporin-4 (AQP4) antibodies are detected in patients with neuromyelitis optica (NMO), a non-paraneoplastic relapsing inflammatory disorder affecting the spinal cord, optic nerves and sometimes the brain (Lennon et al, 2005). Of interest, a few patients with NMO-spectrum disorder with anti-AQP4 antibodies and systemic cancer have recently been reported (Mueller et al, 2008;Pittock and Lennon, 2008;Armağan et al, 2012). Here we report a patient with AQP4 antibody positive longitudinally extensive transverse myelitis (LETM) revealing ovarian teratoma.…”

Paraneoplastic neuromyelitis optica spectrum disorders: three new cases and a review of the literature

“…Twenty-one patients had carcinoma (11 breast (32%) (present case 3) [4][5][6][7], 3 lung (9%) [4,8,9], 2 thymic (6%) [4,10], 2 thyroid (6%) (present case 1) [4] and 2 uterine cervical (6%) [4,7] and 1 prostate (3%) [11]), 4 had lymphoma (12%) [ 4,7,12], 3 had carcinoid tumor (9%) [4,13,14], 2 had monoclonal gammopathy (6%) [4], 2 had ovarian teratoma (6%) [15,16], 1 had pituitary somatotropinoma (3%) [4], and 1 had acute myeloid leukemia (3%) (present case 2).…”

Brainstem and limbic encephalitis with paraneoplastic neuromyelitis optica

“…Several NMO-IgG related syndromes are now well known, including brainstem encephalitis with intractable nausea, vomiting, and hiccups as in our patient, presumably due to antibody interference with aquaporin-4 channels in the area postrema [3][4][5]. Recently, the existence of a paraneoplastic form of NMO has been suggested by case reports and series [1,[6][7][8][9][10][11][12], most commonly related to breast cancer, but these have been exclusively presentations of optic neuritis or transverse myelitis. In a retrospective review of 180,000 patients screened for paraneoplastic syndromes, 26 out of 28 patients with positive NMO-IgG were diagnosed with NMOSD (93%).…”

“…The patient we report had simultaneous appearance of these entities and suggests a causal relationship between the cancer and the antibody syndrome. Several groups have demonstrated aquaporin-4 expression (target protein of NMO-IgG) and inflammatory cells in tumor tissue [9][10][11] but this is still only supportive of the presence of a paraneoplastic neurologic process, as aquaporin-4 can be expressed outside the central nervous system [2] and in tumor tissue of patients without NMOSD [11].…”