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Cerebral cortex impairment in neuromyelitis optica spectrum disorder: A case report and literature review

      Highlights

      • Cortical impairment with leptomeningeal enhancement can be considered an imaging performance component of NMOSD.
      • The cortex adjacent to the cerebral falx was the most susceptible areas.
      • Aquaporin-4 antibody (AQP4) may be involved in the process of cortical and leptomeningeal damage.

      Abstract

      Background

      Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder of the central nervous system that usually presents with acute myelitis and/or optic neuritis. Cortical lesions used to be regarded as “red flags” of NMOSD, and indicated the possibility of alternative diagnoses. We recently had reported a NMOSD patient with weakness in both lower limbs resulting from cortical damage, which was usually attributed to spinal cord lesions. Surprisingly, we identified another patient bearing an uncanny resemblance of imaging manifestations. Cases of NMOSD with cortical impairment reported in the English language literature were reviewed and the demographic characteristics, clinical features, response to treatment, and imaging findings of these cases were analyzed.

      Results

      Our literature review identified other nine cases excluding our previously reported one. All patients had abnormal magnetic resonance imaging (MRI) signals findings in the cortex and leptomeninges. Eight cases reported symptoms of specific cortical impairments. Seven cases underwent follow-up MRI examination and all displayed normal images. Three cases had a previous history of Sjögren's syndrome.

      Conclusions

      Cortical impairment with leptomeningeal enhancement can be considered an imaging performance component of NMOSD. Serious damage to the blood-brain barrier and Aquaporin-4 antibody (AQP4) may be involved in the process of cortical and leptomeningeal damage. We identified the frontal lobes as being the most susceptible area, especially the cortex adjacent to the cerebral falx. NMOSD cortical lesions may be reversed by methylprednisolone and immunosuppressant treatment.

      Keywords

      Abbreviations:

      NMOSD (neuromyelitis optica spectrum disorder), MRI (magnetic resonance imaging), AQP4 (aquaporin-4 antibody), NMO (neuromyelitis optica), AQP4-IgG (aquaporin 4 immunoglobulin G), MOG-Ab (myelin-oligodendrocyte glycoprotein antibody), ANA (anti-nuclear antibodies), SSA (anti-Sjögren's-syndrome-related antigen A antibodies), RF (rheumatoid factor), TPO-Ab (anti-thyroid peroxidase autoantibodies), TG-Ab (anti-thyroglobulin autoantibodies), OS (oculus sinister), LETM (longitudinally extensive transverse myelitis), ref (references. N.P, number of patients), F/M (Female/Male), APS (area postrema syndrome), EP (epilepsy), LETM (longitudinally extensive transverse myelitis), ON (optic neuritis), NA (not applicable), PLEX (plasma exchange)
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      References

        • Apoorva Jayarangaiah R.S.
        • Epperla. Narendranath
        Sjögren's syndrome and Neuromyelitis Optica spectrum disorders (NMOSD) – a case report and review of literature.
        BMC Neurol. 2014; 14: 200
        • Calabrese M.
        • Oh M.S.
        • Favaretto A.
        • Rinaldi F.
        • Poretto V.
        • Alessio S.
        • . . .
        • Gallo P.
        No MRI evidence of cortical lesions in neuromyelitis optica.
        Neurology. 2012; 79: 1671-1676
        • Dos Passos G.R.
        • Oliveira L.M.
        • da Costa B.K.
        • Apostolos-Pereira S.L.
        • Callegaro D.
        • Fujihara K.
        • Sato D.K.
        MOG-IgG-associated optic neuritis, encephalitis, and myelitis: lessons learned from neuromyelitis optica spectrum disorder.
        Front. Neurol. 2018; 9: 217
        • Guo Y.
        • Weigand S.D.
        • Popescu B.F.
        • Lennon V.A.
        • Parisi J.E.
        • Pittock S.J.
        • . . .
        • Lucchinetti C.F.
        Pathogenic implications of cerebrospinal fluid barrier pathology in neuromyelitis optica.
        Acta Neuropathol. 2017; 133: 597-612
        • Isose S.
        • Mori M.
        • Takahashi N.
        • Ito S.
        • Kuwabara S.
        Clumsy hand syndrome due to a localised cortical lesion in the primary sensory cortex in neuromyelitis optica.
        J. Neurol. Neurosurg. Psychiatry. 2011; 82: 559-560
        • Kim J.E.
        • Kim S.M.
        • Ahn S.W.
        • Lim B.C.
        • Chae J.H.
        • Hong Y.H.
        • . . .
        • Lee K.W.
        Brain abnormalities in neuromyelitis optica.
        J. Neurol. Sci. 2011; 302: 43-48
        • Kim W.
        • Lee J.E.
        • Kim S.H.
        • Huh S.Y.
        • Hyun J.W.
        • Jeong I.H.
        • . . .
        • Kim H.J.
        Cerebral cortex involvement in neuromyelitis optica spectrum disorder.
        J. Clin. Neurol. 2016; 12: 188-193
        • Lalji A.
        • Izbudak I.
        • Birnbaum J.
        Cortical blindness and not optic neuritis as a cause of vision loss in a Sjogren's syndrome (SS) patient with the neuromyelitis optica spectrum disorder (NMOSD): challenges of ascribing demyelinating syndromes to SS: a case report.
        Medicine (Baltimore). 2017; 96: e7454
        • Lucchinetti C.F.
        • Guo Y.
        • Popescu B.F.
        • Fujihara K.
        • Itoyama Y.
        • Misu T.
        The pathology of an autoimmune astrocytopathy: lessons learned from neuromyelitis optica.
        Brain Pathol. 2014; 24: 83-97
        • Luppe S.
        • Robertson N.P.
        MOG-IgG in neuromyelitis optica.
        J. Neurol. 2014; 261: 640-642
        • Nakashima I.
        • Fujihara K.
        • Sato S.
        • Itoyama Y.
        Oligoclonal IgG bands in Japanese patients with multiple sclerosis. A comparative study between isoelectric focusing with IgG immunofixation and high-resolution agarose gel electrophoresis.
        J. Neuroimmunol. 2005; 159: 133-136
        • Pekcevik Y.
        • Orman G.
        • Lee I.H.
        • Mealy M.A.
        • Levy M.
        • Izbudak I.
        What do we know about brain contrast enhancement patterns in neuromyelitis optica.
        Clin. Imaging. 2016; 40: 573-580
        • Pittock S.J.
        • Lennon V.A.
        • de Seze J.
        • Vermersch P.
        • Homburger H.A.
        • Wingerchuk D.M.
        • . . .
        • Weinshenker B.G.
        Neuromyelitis optica and non organ-specific autoimmunity.
        Arch. Neurol. 2008; 65: 78-83
        • Popescu B.F.
        • Parisi J.E.
        • Cabrera-Gomez J.A.
        • Newell K.
        • Mandler R.N.
        • Pittock S.J.
        • . . .
        • Lucchinetti C.F.
        Absence of cortical demyelination in neuromyelitis optica.
        Neurology. 2010; 75: 2103-2109
        • Saji E.
        • Arakawa M.
        • Yanagawa K.
        • Toyoshima Y.
        • Yokoseki A.
        • Okamoto K.
        • . . .
        • Kawachi I.
        Cognitive impairment and cortical degeneration in neuromyelitis optica.
        Ann. Neurol. 2013; 73: 65-76
        • Sun H.
        • Sun X.
        • Li J.
        • Huo Y.
        • Wu L.
        • Huang D.
        • . . .
        • Wu W.
        Gender differences among Chinese patients with neuromyelitis optica spectrum disorders.
        Mult. Scler. Relat. Disord. 2017; 17: 5-8
        • Sun H.
        • Wu L.
        Teaching NeuroImages: cortical damage with leptomeningeal enhancement in neuromyelitis optica spectrum disorder.
        Neurology. 2018; 91: e1087-e1088
        • Tahara M.
        • Ito R.
        • Tanaka K.
        • Tanaka M.
        Cortical and leptomeningeal involvement in three cases of neuromyelitis optica.
        Eur. J. Neurol. 2012; 19: e47-e48
        • Wingerchuk D.M.
        • Banwell B.
        • Bennett J.L.
        • Cabre P.
        • Carroll W.
        • Chitnis T.
        • . . .
        • International Panel for, N. M. O. D.
        International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.
        Neurology. 2015; 85: 177-189
        • 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
        • Wu L.
        • Huang D.
        • Yang Y.
        • Wu W.
        Combined screening for serum anti-nuclear and anti-aquaporin-4 antibodies improves diagnostic accuracy for distinguishing neuromyelitis optica from multiple sclerosis.
        Eur. Neurol. 2014; 72: 103-108
        • Zhong Y.H.
        • Zhong Z.G.
        • Zhou Z.
        • Ma Z.Y.
        • Qiu M.Y.
        • Peng F.H.
        • Zhang W.X.
        Comparisons of presentations and outcomes of neuromyelitis optica patients with and without Sjogren's syndrome.
        Neurol. Sci. 2017; 38: 271-277