Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); GFAP astrocytopathy is characterized by optic neuritis and meningoencephalomyelitis. We report the case of a 55-year-old man, otherwise healthy, who present...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2024-04-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2024.1366263/full |
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| author | Eslam Shosha Colleen Connolly Adrian Budhram Adrian Budhram |
| author_facet | Eslam Shosha Colleen Connolly Adrian Budhram Adrian Budhram |
| author_sort | Eslam Shosha |
| collection | DOAJ |
| description | Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); GFAP astrocytopathy is characterized by optic neuritis and meningoencephalomyelitis. We report the case of a 55-year-old man, otherwise healthy, who presented with isolated headaches for three months, without other features of meningoencephalitis or myelitis. His neurological examination and fundoscopy were unremarkable. Gadolinium-enhanced brain MRI demonstrated increased T2 hyperintensity within the right sub-lenticular basal ganglia, with additional leptomeningeal enhancement along the bilateral perisylvian regions and mesial temporal lobes. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, elevated protein, matching oligoclonal bands, and a negative infectious and cytological workup. Cell-based assays for anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein, autoimmune encephalitis panel, and vasculitis workup were all negative, except for CSF positivity for GFAP α antibody. Oncological screening, including CT of the chest, abdomen, pelvis, and scrotal US, was unremarkable. Immunotherapy with high-dose intravenous steroids for five days and subsequent single four-weekly doses resulted in the resolution of both clinical and radiographic features, with a maintained status 24 months after onset. This case highlights isolated headache and basal ganglia, mesial temporal lobe involvement as a rare presentation of autoimmune GFAP astrocytopathy. |
| first_indexed | 2024-04-24T07:57:27Z |
| format | Article |
| id | doaj.art-a47f3e0a23f0467ea92aae20e9566b1a |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-24T07:57:27Z |
| publishDate | 2024-04-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-a47f3e0a23f0467ea92aae20e9566b1a2024-04-18T04:42:01ZengFrontiers Media S.A.Frontiers in Neurology1664-22952024-04-011510.3389/fneur.2024.13662631366263Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathyEslam Shosha0Colleen Connolly1Adrian Budhram2Adrian Budhram3Neurology Division, Department of Medicine, McMaster University, Hamilton Health Science Center, Hamilton, ON, CanadaNeurology Division, Department of Medicine, McMaster University, Hamilton Health Science Center, Hamilton, ON, CanadaDepartment of Clinical Neurological Sciences, Western University, London Health Science Center, London, ON, CanadaDepartment of Pathology and Laboratory Medicine, Western University, London Health Science Center, London, ON, CanadaAutoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a recently emerging autoimmune disease of the central nervous system (CNS); GFAP astrocytopathy is characterized by optic neuritis and meningoencephalomyelitis. We report the case of a 55-year-old man, otherwise healthy, who presented with isolated headaches for three months, without other features of meningoencephalitis or myelitis. His neurological examination and fundoscopy were unremarkable. Gadolinium-enhanced brain MRI demonstrated increased T2 hyperintensity within the right sub-lenticular basal ganglia, with additional leptomeningeal enhancement along the bilateral perisylvian regions and mesial temporal lobes. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, elevated protein, matching oligoclonal bands, and a negative infectious and cytological workup. Cell-based assays for anti-aquaporin-4, anti-myelin oligodendrocyte glycoprotein, autoimmune encephalitis panel, and vasculitis workup were all negative, except for CSF positivity for GFAP α antibody. Oncological screening, including CT of the chest, abdomen, pelvis, and scrotal US, was unremarkable. Immunotherapy with high-dose intravenous steroids for five days and subsequent single four-weekly doses resulted in the resolution of both clinical and radiographic features, with a maintained status 24 months after onset. This case highlights isolated headache and basal ganglia, mesial temporal lobe involvement as a rare presentation of autoimmune GFAP astrocytopathy.https://www.frontiersin.org/articles/10.3389/fneur.2024.1366263/fullheadachemeningoencephalitisglial fibrillary acidic proteinastrocytopathyGFAP |
| spellingShingle | Eslam Shosha Colleen Connolly Adrian Budhram Adrian Budhram Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy Frontiers in Neurology headache meningoencephalitis glial fibrillary acidic protein astrocytopathy GFAP |
| title | Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy |
| title_full | Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy |
| title_fullStr | Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy |
| title_full_unstemmed | Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy |
| title_short | Case report: Headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy |
| title_sort | case report headache as the sole neurological symptom in autoimmune glial fibrillary acidic protein gfap astrocytopathy |
| topic | headache meningoencephalitis glial fibrillary acidic protein astrocytopathy GFAP |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2024.1366263/full |
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