A 46-year-old woman presented with urinary retention, headache, tremor, ataxia, mental slowness, and tetra-hyperreflexia following urinary infection. MRI showed signs of encephalomyelitis with diffuse postcontrast 3D fluid-attenuated inversion recovery leptomeningeal enhancement, a common finding in several neuroinflammatory conditions1 (Figures 1 and 2). An extensive diagnostic workup (serum and CSF neurotropic viruses, Borrelia burgdorferi, Treponema pallidum, serum anti–myelin oligodendrocyte glycoprotein and anti–aquaporin-4 antibodies, antinuclear antibodies, extractable nuclear antigen, antineutrophil cytoplasmic antibodies, angiotensin-converting enzyme and α-chitotriosidase levels, quantiferon, total body CT scan) was unrevealing. CSF analysis disclosed lymphocytic pleocytosis (204 leukocytes/μL), high protein levels (123 mg/dL), CSF-restricted oligoclonal bands, and anti–glial fibrillary acidic protein (GFAP) antibodies (cell-based assay), leading to the diagnosis of autoimmune GFAP astrocytopathy.2 After receiving IV methylprednisolone, the patient improved substantially.
Leptomeningeal Gadolinium Enhancement in Autoimmune GFAP Astrocytopathy / Azzolini F.; Farina A.; Gastaldi M.; Barilaro A.; Scotti V.; Falchetti G.; Fainardi E.; Moretti M.; Massacesi L.. - In: NEUROLOGY. - ISSN 1526-632X. - ELETTRONICO. - 98:(2022), pp. 720-722. [10.1212/WNL.0000000000200291]
Leptomeningeal Gadolinium Enhancement in Autoimmune GFAP Astrocytopathy
Azzolini F.;Farina A.;Gastaldi M.;Barilaro A.;Scotti V.;Falchetti G.;Fainardi E.;Moretti M.;Massacesi L.
2022
Abstract
A 46-year-old woman presented with urinary retention, headache, tremor, ataxia, mental slowness, and tetra-hyperreflexia following urinary infection. MRI showed signs of encephalomyelitis with diffuse postcontrast 3D fluid-attenuated inversion recovery leptomeningeal enhancement, a common finding in several neuroinflammatory conditions1 (Figures 1 and 2). An extensive diagnostic workup (serum and CSF neurotropic viruses, Borrelia burgdorferi, Treponema pallidum, serum anti–myelin oligodendrocyte glycoprotein and anti–aquaporin-4 antibodies, antinuclear antibodies, extractable nuclear antigen, antineutrophil cytoplasmic antibodies, angiotensin-converting enzyme and α-chitotriosidase levels, quantiferon, total body CT scan) was unrevealing. CSF analysis disclosed lymphocytic pleocytosis (204 leukocytes/μL), high protein levels (123 mg/dL), CSF-restricted oligoclonal bands, and anti–glial fibrillary acidic protein (GFAP) antibodies (cell-based assay), leading to the diagnosis of autoimmune GFAP astrocytopathy.2 After receiving IV methylprednisolone, the patient improved substantially.File | Dimensione | Formato | |
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