Autoimmune glial fibrillary acidic protein astrocytopathy associated with breast cancer: a case report.
Astrocytopathy
Autoantibody
Breast cancer
Glial fibrillary acidic protein (GFAP)
Journal
BMC neurology
ISSN: 1471-2377
Titre abrégé: BMC Neurol
Pays: England
ID NLM: 100968555
Informations de publication
Date de publication:
04 Apr 2023
04 Apr 2023
Historique:
received:
10
12
2022
accepted:
30
03
2023
medline:
6
4
2023
entrez:
4
4
2023
pubmed:
5
4
2023
Statut:
epublish
Résumé
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is an autoimmune inflammatory central nervous system disorder characterized by the detection of autoantibodies that recognize GFAP in CSF. The pathogenesis of GFAP-A is poorly understood. Some patients had a neoplasm detected and GFAP expressed by neoplasms is plausible as immunogen triggering paraneoplastic neurological autoimmunity. We report a case of 76-year-old female patient with GFAP-A complicated with breast cancer. She presented with altered consciousness, nuchal rigidity, speech disturbances, and weakness. Her clinical symptoms were improved by immunotherapy and cancer treatments. Immunohistochemical analysis showed that the restricted tumor expressed GFAP. The infiltration of CD3 + T cells were observed in the peritumoral and intratumoral areas. The most common infiltrating lymphocytes were CD8 + T cells. CD4 + T cells and CD20 + B cells were also observed in the predominant peritumoral area. These results suggest that GFAP-A may occur in a paraneoplastic neurological syndrome associated with breast cancer.
Sections du résumé
BACKGROUND
BACKGROUND
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is an autoimmune inflammatory central nervous system disorder characterized by the detection of autoantibodies that recognize GFAP in CSF. The pathogenesis of GFAP-A is poorly understood. Some patients had a neoplasm detected and GFAP expressed by neoplasms is plausible as immunogen triggering paraneoplastic neurological autoimmunity.
CASE PRESENTATION
METHODS
We report a case of 76-year-old female patient with GFAP-A complicated with breast cancer. She presented with altered consciousness, nuchal rigidity, speech disturbances, and weakness. Her clinical symptoms were improved by immunotherapy and cancer treatments. Immunohistochemical analysis showed that the restricted tumor expressed GFAP. The infiltration of CD3 + T cells were observed in the peritumoral and intratumoral areas. The most common infiltrating lymphocytes were CD8 + T cells. CD4 + T cells and CD20 + B cells were also observed in the predominant peritumoral area.
CONCLUSIONS
CONCLUSIONS
These results suggest that GFAP-A may occur in a paraneoplastic neurological syndrome associated with breast cancer.
Identifiants
pubmed: 37016352
doi: 10.1186/s12883-023-03194-7
pii: 10.1186/s12883-023-03194-7
pmc: PMC10071775
doi:
Substances chimiques
Glial Fibrillary Acidic Protein
0
Autoantibodies
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
145Subventions
Organisme : JSPS KAKENHI
ID : JP21K07457
Informations de copyright
© 2023. The Author(s).
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