Original article| Volume 45, 102348, October 2020

Multiple sclerosis with intractable vomiting and atypical area postrema lesion


      • Distinguishing seronegative NMOSDs from multiple sclerosis is a clinical challenge with important treatment implications.
      • Challenging cases with overlapping features of MS and NMOSDs may demand longitudinal follow-up to make a definite diagnosis.
      • The area postrema expresses aquaporin-4 abundantly and area postrema syndrome is one of the typical features of NMOSDs.
      • Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgGs) and autoantibodies against aquaporin-1 (AQP1-Abs) are the potential new markers for seronegative NMOSDs.



      Area postrema syndrome is considered as one of the most typical presentations of neuromyelitis optica spectrum disorders (NMOSDs) (
      • Wingerchuk D.M.
      • Banwell B.
      • Bennett J.L.
      • Cabre P.
      • Carroll W.
      • Chitnis T.
      • et al.
      International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.
      ). The involvement of area postrema is rarely seen in multiple sclerosis (MS). We are here report a case of a young woman with multiple sclerosis, presented with intractable vomiting, and her MRI brain showed acute T2 hyperintense signal over the area postrema.

      Case presentation

      A 36-year-old Asian woman who is known to have schizophrenia and multiple sclerosis since 2012. She was noted to have spastic gait, and the MRI brain in 2012 showed multiple perpendicular periventricular T2 lesions suggestive of multiple sclerosis (MS). However, she defaulted her neurologist follow-up and was not on any treatment for MS. She was admitted in 2016 with intractable vomiting, and her MRI brain showed T2 hyperintense signal over area postrema with focal contrast enhancement. Her MRI cervical spine was normal. The visual evoked potential study showed bilateral prolonged P100 latencies. Oligoclonal bands were detected in her CSF analysis. Both the serum aquaporin-4 IgG (AQP4 IgGs) antibody and myelin oligodendrocyte glycoprotein (MOG-IgGs) were negative. Her intractable vomiting resolved after a short course of intravenous methylprednisolone. She was treated as MS with interferon-beta 1a. She has been in remission since 2016, and her functional status also improved from the expanded disability status scale (EDSS) of 2.0 (in 2016) to 1.0 (in 2020).


      We proposed that although area postrema lesion is typically seen in NMOSDs, it may also be seen in MS. Current MRI criteria for MS and NMOSDs are not sufficiently specific, and the diagnostic criteria should only be used in the appropriate clinical context.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Multiple Sclerosis and Related Disorders
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Wingerchuk D.M.
        • Banwell B.
        • Bennett J.L.
        • Cabre P.
        • Carroll W.
        • Chitnis T.
        • et al.
        International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.
        Neurology. 2015; 85: 177-189
        • Reich D.S.
        • Lucchinetti C.F.
        • Calabresi P.A
        Multiple sclerosis.
        N. Engl. J. Med. 2018; 378: 169-180
        • Wingerchuk D.M.
        • Lennon V.A.
        • Lucchinetti C.F.
        • Pittock S.J.
        • Weinshenker B.G
        The spectrum of neuromyelitis optica.
        Lancet Neurol. 2007; 6: 805-815
        • Lennon V.A.
        • Wingerchuk D.M.
        • Kryzer T.J.
        • Pittock S.J.
        • Lucchinetti C.F.
        • Fujihara K.
        • Nakashima I.
        • Weinshenker B.G.
        A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis.
        Lancet. 2004; 364: 2106-2112
        • Lennon V.A.
        • Kryzer T.J.
        • Pittock S.J.
        • Verkman A.S.
        • Hinson S.R.
        IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel.
        J. Exp. Med. 2005; 202: 473-477
        • Thompson A.J.
        • Banwell B.L.
        • Barkhof F.
        • Carroll W.M.
        • Coetzee T.
        • Comi G.
        • et al.
        Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria.
        Lancet Neurol. 2018; 17: 162-173
        • McNicholas N.
        • Hutchinson M.
        • McGuigan C.
        • Chataway J
        2017 McDonald diagnostic criteria: a review of the evidence.
        Mult. Scler. Relat. Disord. 2018; 24: 48‐54
        • Duvernoy H.M.
        • Risold P.Y.
        The circumventricular organs: an atlas of comparative anatomy and vascularization.
        Brain Res. Rev. 2007; 56: 119-147
        • Price C.J.
        • Hoyda T.D.
        • Ferguson A.V
        The area postrema: a brain monitor and integrator of systemic autonomic state.
        Neuroscientist. 2008; 14: 182-194
        • Pittock S.J.
        • Lennon V.A.
        • Krecke K.
        • Wingerchuk D.M.
        • Lucchinetti C.F.
        • Weinshenker B.G
        Brain abnormalities in neuromyelitis optica.
        Arch. Neurol. 2006; 63: 390-396
        • Pittock S.J.
        • Weinshenker B.G.
        • Lucchinetti C.F.
        • Wingerchuk D.M.
        • Corboy J.R.
        • Lennon V.A
        Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression.
        Arch. Neurol. 2006; 63: 964-968
        • Birkhead R.
        • Friedman J.
        Hiccups and vomiting as initial manifestations of multiple sclerosis.
        J. Neurol. Neurosurg. Psychiatry. 1987; 50: 232-233
        • Filippi M.
        • Rocca M.A.
        • Ciccarelli O.
        • De Stefano N.
        • Evangelou N.
        • Kappos L.
        • et al.
        MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines.
        Lancet Neurol. 2016; 15: 292-303
        • Cacciaguerra L.
        • Meani A.
        • Mesaros S.
        • Radaelli M.
        • Palace J.
        • Dujmovic- Basuroski I.
        • et al.
        Brain and cord imaging features in neuromyelitis optica spectrum disorders.
        Ann. Neurol. 2019; 85: 371-384
        • Andersson M.
        • Alvarez-Cermeno J.
        • Bernardi G.
        • Cogato I.
        • Fredman P.
        • Frederiksen J.
        • et al.
        Cerebrospinal fluid in the diagnosis of multiple sclerosis: a consensus report.
        J. Neurol. Neurosurg. Psychiatry. 1994; 57: 897-902
        • Freedman M.S.
        • Thompson E.J.
        • Deisenhammer F.
        • Giovannoni G.
        • Grimsley G.
        • Keir G.
        • et al.
        Recommended standard of cerebrospinal fluid analysis in the diagnosis of multiple sclerosis: a consensus statement.
        Arch. Neurol. 2005; 62: 865-870
        • Awad A.
        • Hemmer B.
        • Hartung H.P.
        • Kieseier B.
        • Bennett J.L.
        • Stuve O
        Analyses of cerebrospinal fluid in the diagnosis and monitoring of multiple sclerosis.
        J. Neuroimmunol. 2010; 219: 1-7
        • Karussis D.
        The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
        J. Autoimmun. 2014; 48-49: 134-142
        • Schwenkenbecher P.
        • Wurster U.
        • Konen F.F.
        • Gingele S.
        • Sühs K.-.W.
        • Wattjes M.P.
        • Stangel M.
        • Skripuletz T.
        Impact of the McDonald criteria 2017 on early diagnosis of relapsing-remitting multiple sclerosis.
        Front. Neurol. 2019; 10: 188
        • Tzartos J.S.
        • Stergiou C.
        • Kilidireas K.
        • Zisimopoulou P.
        • Thomaidis T.
        • Tzartos S.J.
        Anti-aquaporin-1 autoantibodies in patients with neuromyelitis optica spectrum disorders.
        PLoS ONE. 2013; 8: e74773
        • Saadoun S.
        • Waters P.
        • Owens G.P.
        • Bennett J.L.
        • Vincent A.
        • Papadopoulos M.C.
        Neuromyelitis optica MOG-IgG causes reversible lesions in mouse brain.
        Acta Neuropathol. Commun. 2014; 2
        • Melamed E.
        • Levy M.
        • Waters P.J
        • Sato D.K.
        • Bennett J.L.
        • John G.R.
        • Hooper D.C.
        • Saiz A.
        • Bar-Or A.
        • Kim H.J.
        • et al.
        Update on biomarkers in neuromyelitis optica.
        Neurol. Neuroimmunol. Neuroinflamm. 2015; 2: e134
        • Saadoun S.
        • Waters P.
        • Owens G.P.
        • Bennett J.L.
        • Vincent A.
        • Papadopoulos M.C.
        Neuromyelitis optica MOG-IgG causes reversible lesions in mouse brain.
        Acta Neuropathol. Commun. 2014; 2
        • Palace J.
        • Leite M.I.
        • Nairne A.
        • Vincent A
        Interferon Beta treatment in neuromyelitis optica: increase in relapses and aquaporin 4 antibody titers.
        Arch. Neurol. 2010; 67: 1016-1017
        • Juryńczyk M.
        • Zaleski K.
        • Selmaj K
        Natalizumab and the development of extensive brain lesions in neuromyelitis optica.
        J. Neurol. 2013; 260: 1919-1921