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Autoimmune glial fibillary acidic protein astrocytopathy associated meningoencephalomyelitis and bilateral sensorineuronal deafness

  • Author Footnotes
    1 Ip and Lam contributed equally as co-first authors of the article.
    Bonaventure Ip
    Footnotes
    1 Ip and Lam contributed equally as co-first authors of the article.
    Affiliations
    Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong SAR, China
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  • Author Footnotes
    1 Ip and Lam contributed equally as co-first authors of the article.
    Crystal Lam
    Footnotes
    1 Ip and Lam contributed equally as co-first authors of the article.
    Affiliations
    Division of Clinical Immunology, Department of Pathology, Queen Mary Hospital, China
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  • Vincent Ip
    Affiliations
    Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong SAR, China
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  • Anne Chan
    Affiliations
    Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong SAR, China
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  • Vincent Mok
    Affiliations
    Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong SAR, China
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  • Elaine Au
    Affiliations
    Division of Clinical Immunology, Department of Pathology, Queen Mary Hospital, China
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  • Eric Chan
    Affiliations
    Division of Clinical Immunology, Department of Pathology, Queen Mary Hospital, China
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  • Alexander Lau
    Correspondence
    Corresponding author.
    Affiliations
    Division of Neurology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, Prince of Wales Hospital, Shatin, Hong Kong SAR, China
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  • Author Footnotes
    1 Ip and Lam contributed equally as co-first authors of the article.
Published:December 31, 2019DOI:https://doi.org/10.1016/j.msard.2019.101922

      Highlights

      • A woman developed meningo-encephalomyelitis associated GFAP astrocytopathy.
      • Patient had acute hearing loss, tetraplegia, bulbar palsy and respiratory failure.
      • Partial recovery was noted with immunosuppressants, plasma exchange and rituximab.

      Abstract

      Autoimmune encephalitis is an important group of disease that can mimic infectious encephalitis, with one of the most severe forms being meningoencephalomyelitis. One of the recently identified biomarkers, glial fibillary acidic protein (GFAP), targets the cytosolic intermediate filament protein of astrocytes and causes a variety of clinical symptoms. Here, we report an adult Chinese woman presented with acute onset of confusion, CSF lymphocytosis, markedly elevated total protein mimicking tuberculosis meningitis with rapid deterioration resulted in coma and respiratory failure. She was diagnosed with anti-GFAP meningoencephalomyelitis, which later developed tetraplegia, sensorineural hearing loss, brainstem, bulbar and respiratory dysfunction. Intravenous immunoglobulin and methylprednisolone resulted in partial improvement. Further immunotherapy with plasma exchange and rituximab resulted in marked recovery.

      Keywords

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