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Mild Encephalitis/Encephalopathy with reversible splenial lesion syndrome: An unusual presentation of anti-GFAP astrocytopathy.

Author information

Affiliations

  • 1. Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Hôpital Bicêtre, Pediatric Neurology Department, National Referral Center for Rare Inflammatory Brain and Spinal Diseases, France.
      Authors
    • Oger V 1
    • Salah L 1
    • Yazbeck E 1
    • Maurey H 1
    • Bellesme C 1
    • Sevin C 1
    (6 authors)
  • 2. Laboratoire D'Immunologie, Institut Fédératif de Biologie, CHU de Toulouse, Toulouse, France.
      Authors
    • Bost C 2
    (1 author)
  • 3. Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Pediatric Radiology, Hôpital Bicêtre, Paris, France, Paris Sud University Hôpital Bicêtre, Le Kremlin Bicêtre APHP, Paris, France.
      Authors
    • Adamsbaum C 3
    (1 author)
  • 4. Assistance Publique Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Hôpital Bicêtre, Immunology Department, Le Kremlin-Bicêtre, France.
      Authors
    • Chrétien P 4
    (1 author)
  • 5. Département de Neurologie, Hôpital Pierre Paul Riquet, CHU de Toulouse, Toulouse, France.
      Authors
    • Benaiteau M 5
    (1 author)
    • Show all (7)

ORCIDs linked to this article

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society, 18 Mar 2020, 26:89-91
https://doi.org/10.1016/j.ejpn.2020.03.002 PMID: 32340854 

Abstract 

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a rare recently defined antibody-mediated encephalitis. Meningo-encephalomyelitis presentation is frequent with lymphocytic pleiocytosis in the cerebro-spinal fluid and brain MRI classically demonstrates in 50% of cases, a linear perivascular enhancement extending radially from the ventricles. Here, we describe 2 cases of pediatric autoimmune GFAP astrocytopathy with limbic encephalitis presentation and peculiar MRI characteristics: one with normal MRI and the second suggestive of Mild Encephalitis/Encephalopathy with reversible splenial lesion syndrome (MERS). These two cases illustrate that anti-GFAP antibodies should be sought in children presenting limbic encephalitis with a normal and/or MERS suggestive MRI, as treatment strategies may differ.

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Read article at publisher's site: https://doi.org/10.1016/j.ejpn.2020.03.002

Read article for free, from open access legal sources, via Unpaywall: http://manuscript.elsevier.com/S1090379820300696/pdf/S1090379820300696.pdfUnpaywall

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