Paraneoplastic neuromyelitis optica spectrum disorder associated with stomach carcinoid tumor

Abstract

Neuromyelitis optica (NMO), or Devic's syndrome, is an autoimmune central nervous system demyelinating disorder primarily affecting the spinal cord and the optic nerves. It is characterized by the presence of NMO antibodies, alongside clinical and radiological findings. NMO and NMO-spectrum disorders (NMO-SD) have been reported in autoimmune disorders, and are infrequently described as a paraneoplastic syndrome with cancers of lung, breast, and carcinoid tumors of the thyroid. We report a patient who presented with severe vomiting, blurring of vision, vertigo, diplopia, left hemiparesis and hemisensory loss and ataxia. She was found to have a longitudinally-extensive demyelinating lesion extending from the medulla to the upper cervical spinal cord on MRI. Her gastric endoscopy revealed carcinoid tumor of the stomach, and classic paraneoplastic antibodies in the serum were negative. She had extremely high serum gastrin level and high titer of NMO IgG autoantibody. The patient made an excellent recovery with tumor resection and immunotherapy, with both clinical and radiological improvement. On rare instances, NMO or NMO-SD may present as a paraneoplastic neurological syndrome associated with carcinoid tumor of the stomach.

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…”

“…NMOSD-related symptoms reoccurred after removal of tumor in one earlier case [16] and also in Case 1. Three previous cases [13][14][15], along with Case 3, got relapse-free after radical cure of tumors. Serum AQP4-IgG level normalized [6] or reduced [12] after immunotherapies and anti-tumor therapies.…”

“…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).…”

“…AQP4 autoantibodies from serum and CSF of NMO patients bound to AQP4 expressed on cell membrane of thymoma [23] and lung adenocarcinoma [9]. In addition, there are some other evidence to suggest an association of AQP4-IgG with tumors, such as occurrence or reoccurrence of NMOSD accompanying the recurrence or metastasis of tumors [5,8,10,13], freedom from relapse after radical cure of tumors [13][14][15], and reduction in serum AQP4-IgG after immunotherapies and anti-tumor therapies [6,12].…”

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%).…”