Mild Encephalitis/Encephalopathy with reversible splenial lesion syndrome: An unusual presentation of anti-GFAP astrocytopathy. (May 2020)

• Record Type: Journal Article
• Authors: Oger, Valentin; Bost, Chloé; Salah, Lucie; Yazbeck, Elise; Maurey, Hélène; Bellesme, Céline; Sevin, Caroline; Adamsbaum, Catherine; Chrétien, Pascale; Benaiteau, Marie; Honnorat, Jérôme; Deiva, Kumaran
• Is Part Of: European journal of paediatric neurology. Volume 26(2020)
• Page Start: 89
• Page End: 91
• Publication Date: 2020-05
• Subjects: Astrocytopathy -- GFAP -- Auto immun -- Encéphalitis -- IRM -- Glial fibrillary acidic protein -- Autoantibody -- Central nervous system; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2020.03.002
• Languages: English
• ISSNs: 1090-3798

Abstract

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. Highlights: Anti-GFAP astrocytopathy is a rare condition in adults and children and specific MRI lesions have been suggested. Clinical outcome is good in most of the cases after immunotherapies. The pathogenicity of anti-GFAP antibodies is far from being demonstrated. Anti-GFAP antibodies should be tested for meningo-encephalitis with normal or MERS suggestive MRI, as treatment may differ.