Case ReportBrainstem and limbic encephalitis with paraneoplastic neuromyelitis optica
Introduction
Recently, seropositive neuromyelitis optica spectrum disorders (NMOSD) presenting with transverse myelitis or optic neuritis have been reported in association with various cancer types, raising the possibility of paraneoplastic NMOSD [1]. Here we describe a patient with breast cancer and an anti-neuromyelitis optica (NMO) limbic and brainstem encephalitis syndrome who improved markedly with excision of the tumor. Clinicians should consider investigating for an underlying treatable neoplasm in patients who present with NMOSD.
Section snippets
Case report
A 29-year-old Caucasian woman had 2 days of intractable nausea, vomiting, and hiccups. Abdominal imaging, endoscopy and ultrasound were normal. Her nausea and vomiting were refractory to several medications and hiccups were responsive only to intravenous chlorpromazine. A mammogram revealed a hypo-echoic left breast nodule and CT scan demonstrated extensive axillary and subpectoral adenopathy. The breast lesion was poorly differentiated invasive ductal carcinoma (estrogen receptor negative,
Discussion
NMO-immunoglobulin G (IgG) antibody is highly specific for NMOSD [2]. 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
Conflicts of Interest/Disclosures
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
References (12)
- et al.
Aquaporin 4 and neuromyelitis optica
Lancet Neurol
(2012) - et al.
Paraneoplastic neuromyelitis optica spectrum disorder associated with stomach carcinoid tumor
Hematol Oncol Stem Cell Ther
(2014) - et al.
Longitudinally extensive transverse myelitis with AQP4 antibodies revealing ovarian teratoma
J Neuroimmunol
(2013) - et al.
Aquaporin-4 autoantibodies in a paraneoplastic context
Arch Neurol
(2008) - et al.
Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS)
J Neurol
(2014) - et al.
Neuromyelitis optica unique area postrema lesions Nausea, vomiting, and pathogenic implications
Neurology
(2011)