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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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Background: Spinal dural arteriovenous fistula is a rare and underdiagnosed disorder. Because of the nonspecific clinical presentation of the condition, patients are often referred to different specialists, resulting in delayed diagnosis.Case Report: A 76-year-old male presented with a 1-month history of gait trouble. His impairment was asymmetric, distally predominant, sensitive, and motor. Symptoms worsened with standing and walking. The patient also had sphincterial dysfunction. Classic spinal cord magnetic resonance imaging (MRI) showed an extended hypersignal indicating nonspecific myelopathy. Repeat spinal cord MRI that included a T2 spin echo sequence revealed abnormalities suggesting dural arteriovenous fistula. Medullar angiography confirmed the diagnosis, and endovascular treatment was successfully performed. Six months posttreatment, the patient reported resolution of his neurologic disabilities except for some residual paresthesia in his inferior limbs.Conclusion: Physicians should be aware of the specific abnormalities shown on spinal cord MRI that indicate dural arteriovenous fistula, as well as the criteria for performing medullar angiography, so that the condition can be diagnosed and treated in a timely manner. Early therapeutic treatment is the principal prognosis factor.

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