A case of autoimmune glial fibrillary acidic protein astrocytopathy with concurrent renal cell carcinoma

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel central nervous system disorder characterized by subacute neuropsychiatric symptom development and rapid remission with corticosteroid therapy. In this paper, we describe a case of GFAP encephalitis found to have renal cell carcinoma. The patient was a 71-year-old man with a history of transient ischemic attacks, severe diabetic retinopathy and monoclonal gammopathy of undetermined significance, who presented with subacute cognitive decline. Anti-GFAP antibodies were detected in the cerebrospinal fluid. Computer Tomography (CT) of chest, abdomen and pelvis identified a renal mass that was resected. Subsequent pathology confirmed clear cell renal cell carcinoma. The patient was treated with corticosteroids with cognitive recovery near to baseline. In conclusion, we encourage the early consideration of autoimmune GFAP astrocytopathy for patients with clinical manifestation of subacute neuropsychiatric symptoms after other common conditions have been ruled out, as it usually has a great response to immunosuppressive therapy. In addition, this case highlights the importance of full body scan to identify possible underlying malignancy as a possible precipitating factor.

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Journal
Neuroimmunology Reports
Published
2024-09-08
DOI
https://doi.org/10.1016/j.nerep.2024.100226
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
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article

A case of autoimmune glial fibrillary acidic protein astrocytopathy with concurrent renal cell carcinoma

Wilson Rodriguez, Christopher Tapia, Krithika Arrabothu, Srinivas Govindan et al.
Neuroimmunology Reports
Autoimmune Neurological Disorders and Treatments
article

A case of autoimmune glial fibrillary acidic protein astrocytopathy with concurrent renal cell carcinoma

Wilson Rodriguez, Christopher Tapia, Krithika Arrabothu, Srinivas Govindan, Robert L. White
article en

Abstract

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel central nervous system disorder characterized by subacute neuropsychiatric symptom development and rapid remission with corticosteroid therapy. In this paper, we describe a case of GFAP encephalitis found to have renal cell carcinoma. The patient was a 71-year-old man with a history of transient ischemic attacks, severe diabetic retinopathy and monoclonal gammopathy of undetermined significance, who presented with subacute cognitive decline. Anti-GFAP antibodies were detected in the cerebrospinal fluid. Computer Tomography (CT) of chest, abdomen and pelvis identified a renal mass that was resected. Subsequent pathology confirmed clear cell renal cell carcinoma. The patient was treated with corticosteroids with cognitive recovery near to baseline. In conclusion, we encourage the early consideration of autoimmune GFAP astrocytopathy for patients with clinical manifestation of subacute neuropsychiatric symptoms after other common conditions have been ruled out, as it usually has a great response to immunosuppressive therapy. In addition, this case highlights the importance of full body scan to identify possible underlying malignancy as a possible precipitating factor.

Neuroimmunology ReportsVol. 6
Saint Louis University (ES), University of Washington (US), Washington University in St. Louis (US), VA Puget Sound Health Care System (US), VA St. Louis Health Care System (US), Saint Louis University (US)
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Openalex Percentile: Top 76%
Autoimmune Neurological Disorders and Treatments
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