title: Spectrum disorder of neuromyelitis optica in a patient presenting with intractable vomiting and hiccups, transverse myelitis and acute encephalopathy.
abstract: Optic neuropathy and transverse myelitis (TM) are common symptoms of multiple sclerosis (MS) but may also be seen in association with the antibody-mediated autoimmune disorder, neuromyelitis optica (NMO). We report a female patient presenting with intractable vomiting and hiccups and TM shortly followed by an acute encephalopathy, most likely due to NMO spectrum disorder. Serum and cerebrospinal fluid NMO antibodies were negative. Serial MRI abnormalities included longitudinally extensive TM of the cervical cord, focal T2-weighted hyperintensity of the area postrema and lesions in both thalami and the hypothalamus. Clinical and MRI involvement of these brain regions, which have high aquaporin expression, in conjunction with a spinal lesion extending over three vertebral segments strongly favoured a diagnosis of NMO. She required several courses of intravenous methylprednisolone and plasmapheresis before receiving intravenous rituximab therapy. NMO spectrum disorder should be considered in the differential diagnosis of atypical central nervous system presentations such as intractable vomiting and hiccups and acute encephalopathy. Recognition of this syndrome has significant implications as its treatment and prognosis differs from MS.
authors.affiliation: Department of Neurophysiology, Liverpool Hospital, Elizabeth Drive, Liverpool 2170, NSW, Australia.
authors.forename: Vishal
authors.initials: V
authors.lastname: Patel
authors.forename: Neil C
authors.initials: NC
authors.lastname: Griffith
authors.forename: Emma
authors.initials: E
authors.lastname: Blackwood
authors.forename: Manu
authors.initials: M
authors.lastname: Dias
authors.forename: Dennis J
authors.initials: DJ
authors.lastname: Cordato
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood / Q000134:cerebrospinal fluid; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000209:etiology*; D005260:Female; D006606:Hiccup / Q000209:etiology*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008775:Methylprednisolone / Q000627:therapeutic use; D009188:Myelitis, Transverse / Q000209:etiology*; D009471:Neuromyelitis Optica / Q000150:complications* / Q000188:drug therapy; D010956:Plasmapheresis; D000069283:Rituximab; D013116:Spinal Cord / Q000473:pathology; D014839:Vomiting / Q000209:etiology*
chemical_list: D000893:Anti-Inflammatory Agents; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D000069283:Rituximab; D008775:Methylprednisolone