RT Journal Article SR Electronic T1 Autoimmune glial fibrillary acidic protein astrocytopathy presented as ataxia, myoclonus and bulbar syndrome: a case report and review of the literature JF BMJ Neurology Open JO BMJ Neurol Open FD BMJ Publishing Group Ltd SP e000142 DO 10.1136/bmjno-2021-000142 VO 3 IS 2 A1 Azael C Novo A1 Begoña Venegas Pérez YR 2021 UL http://neurologyopen.bmj.com/content/3/2/e000142.abstract AB Objective To describe an atypical case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.Results A patient in his 60s presented with 6 months of progressive ataxia, proximal myoclonus and bulbar symptomatology. Cerebrospinal fluid (CSF) analysis showed monocytic pleocytosis, elevated protein level and elevated adenosine deaminase (ADA) level. CSF microbiological studies were negative and brain and cervical MRI showed no significant findings. We tested for nuclear, cytoplasmatic and synaptic neural autoantibodies as well as anti-GFAP antibodies. While awaiting these results, the patient was commenced on methylprednisolone boluses (1 g/day for 5 days), noting rapid neurological improvement. Eventually, CSF tests were positive for anti-GFAP antibodies.Conclusion We report atypical manifestations of GFAP astrocytopathy. Further research is needed to fully understand the spectrum of neurological manifestations of this autoimmune disease and facilitate timely diagnosis.Data sharing not applicable as no data sets generated and/or analysed for this study. Not applicable.