Encéphalite auto immune à anticorps anti GFAP : à propos d’une observation et revue de la littérature

Garrigues, P.; Saredi, Simona; Kessali, L.; Saraoui, H.; Broucker, T. de

doi:10.1016/j.revmed.2019.03.313

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“…Thus, 46 patients were included for analysis, involving 6 patients published in prior reports. [9][10][11][12][13] The demographics and clinical characteristics of the 46 patients with CSF GFAP-IgG are summarized in Table 1 and Table 2. Adult and pediatric features are similar.…”

Section: Clinical Characteristics Of Patients With Csf Gfap-iggmentioning

confidence: 99%

“…acute onset, 7 patients (15%) with a progressive setup. Thirtyseven of 45 patients (82%) had prodromal symptoms the month preceding neurologic presentation, including fever of unknown origin for 30 of them(30/43 [70%]).The predominant clinical syndrome was meningoencephalitis (28 patients [61%]), including one patient who did not have clinical and biological meningitis, followed by meningoencephalomyelitis(11 patients [24%]), meningitis (4 patients [9%]), and myelitis (3 patients [6%]). Rhombencephalitis was reported in 30 cases (30/46 [65%]), with cerebellar dysfunction (25/44 [57%]), eye movement disorders (17/46 [37%]), and swallowing problems (5/46 [11%]).…”

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Glial Fibrillary Acidic Protein Autoimmunity

Dumonceau

Améli²,

Rogemond³

et al. 2022

Neurology

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Objectives:To report the clinical, biological, imaging features, and the clinical course of a French cohort of patients with glial fibrillar acidic protein (GFAP) autoantibodies.Methods:We retrospectively included all patients tested positive for GFAP antibodies in the cerebrospinal fluid, by immunohistochemistry and confirmed by cell-based assay using cells expressing human GFAPα, since 2017, from two French referral centers.Results:We identified 46 patients with GFAP antibodies. Median age at onset was 43 years, and 65% were men. Infectious prodromal symptoms were found in 82%. Other auto-immune diseases were found in 22% of patients, and coexisting neural autoantibodies in 11%. Tumors were present in 24%, and T cell dysfunction in 23%. The most frequent presentation was subacute meningoencephalitis (85%) with cerebellar dysfunction in 57% of cases. Other clinical presentation included myelitis (30%), visual (35%) and peripheral nervous system involvement (24%). MRI showed perivascular radial enhancement in 32%, periventricular T2 hyperintensity in 41%, brainstem involvement in 31%, leptomeningeal enhancement in 26%, and reversible splenial lesions in 4 cases. 33/40 patients had a monophasic course, associated to a good outcome at last follow-up (Rankin Score≤2: 89%), despite a severe clinical presentation. Adult and pediatric features are similar. Thirty-two patients were treated with immunotherapy. 11/22 patients showed negative conversion of GFAP antibodies.Interpretation:GFAP auto-immunity is mainly associated with acute/subacute meningoencephalomyelitis with prodromal symptoms, for which tumors and T cell dysfunction are frequent triggers. The majority of patients followed a monophasic course with a good outcome.

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