Elsevier

Journal of Clinical Neuroscience

Volume 23, January 2016, Pages 159-161
Journal of Clinical Neuroscience

Case Report
Brainstem and limbic encephalitis with paraneoplastic neuromyelitis optica

https://doi.org/10.1016/j.jocn.2015.08.006Get rights and content

Highlights

  • Brainstem and limbic encephalitis can be a manifestation of paraneoplastic neuromyelitis optica (NMO) associated with breast cancer.

  • Prognosis of paraneoplastic NMO is favorable when treated with immunotherapy and cancer resection.

Abstract

The spectrum of disorders associated with anti-neuromyelitis optica (NMO) antibody is being extended to include infrequent instances associated with cancer. We describe a patient with brainstem and limbic encephalitis from NMO-immunoglobulin G in serum and cerebrospinal fluid in the context of newly diagnosed breast cancer. The neurological features markedly improved with excision of her breast cancer and immune suppressive therapy. This case further broadens the NMO spectrum disorders (NMOSD) by an association between NMOSD and cancer and raises the question of coincidental occurrence and the appropriate circumstances to search for a tumor in certain instances of NMO.

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.

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