Longitudinally extensive transverse myelitis with AQP4 antibodies revealing ovarian teratoma

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

“…NMOSD-related symptoms markedly improved after treatment with corticosteroids in patients with breast carcinoma [5,6], carcinoid [13,14], ovarian teratoma [15,16], as well as in Case 3. All these patients had a good prognosis after radical treatment, Int J Neurosci Downloaded from informahealthcare.com by Nyu Medical Center on 06/21/15…”

“…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].…”

Aquaporin-4 autoimmunity masquerading as a brainstem tumor

“…However, there is growing evidence for the coexistence of AQP4Ab with cancer, which could be regarded as a paraneoplastic manifestation. 4,14 In this clinical context, both AQP4Ab testing and surgical biopsy were required to arrive at the correct diagnosis and treatment. The risks of respiratory failure are high for NMO, especially when the disease involves the cervical cord.…”