A case of autoimmune glial fibrillary acidic protein astrocytopathy with excessive startle response and cortical hyperexcitability

Abstract

AbstractWe herein report a 60‐year‐old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP‐A) with excessive startle response (ESR), or hyperekplexia. Short‐latency somatosensory evoked potentials (SSEPs) revealed high‐amplitude potentials of the early cortical component. Anti‐GFAPα antibodies in the spinal fluid were positive. The hyperekplexia, other clinical symptoms, and SSEP abnormalities were resolved by corticosteroid therapy. The present case demonstrates that ESR can be associated with GFAP‐A, and its pathophysiology may be cortical hyperexcitability.