Clinical and Experimental Neuroimmunology, Journal Year: 2025, Volume and Issue: unknown
Published: April 17, 2025
ABSTRACT Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP‐A) is an autoimmune inflammatory central nervous system disease. Recent neuropathological findings indicate that GFAP‐specific cluster of differentiation (CD)8 + T cells are likely the effectors GFAP‐A. Of 387 individuals in Japan identified as having GFAP‐A, most presented with headache and/or fever followed by neurological symptoms including consciousness disturbance, urinary dysfunction, hyperreflexia, movement disorders, and papilledema. Sixteen (5.9%) 270 GFAP‐A patients tested had coexisting antibodies. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis increased levels. Moreover, transiently CSF adenosine deaminase, decreased glucose, positive oligoclonal band results were sometimes observed. Brain magnetic resonance imaging (MRI) occasionally showed T2‐hyperintensity lesions. Linear perivascular radial gadolinium‐enhancement patterns observed may be hallmark Spinal cord MRI exhibited spinal lesions, which longitudinally extensive. Most treated immunotherapies, intravenous methylprednisolone pulse therapy or without immunoglobulin plasma exchange; this was oral corticosteroid therapy, gradually tapered. Some refractory received second‐line immunotherapies rituximab cyclophosphamide. In 203 follow‐up ≥ 6 months, median modified Rankin scale score at last 1 (range: 0–6); however, 44 (21.7%) scores 3 greater, six died. The common finding cognitive dysfunction; recurrence rate 10.5%. GFAP‐immunoglobulin G should examined who present these characteristic clinical radiological features. Trial Registration: GFAP registry (UMIN: 000054387).
Language: Английский