Respiratory Failure due to Glial Fibrillary Acidic Protein Antibody-Associated Disorders: Recovery Following Plasma Exchange.

Glial fibrillary acidic protein antibody-associated disorders (GFAP-ADs) generally respond to steroid treatment but may become fulminant when brainstem lesions cause central hypoventilation. A 56-year-old man developed fever, myoclonus, gait instability, urinary retention, and altered consciousness. An analysis of cerebrospinal fluid (CSF) showed pleocytosis and high protein levels, and magnetic resonance imaging (MRI) demonstrated diffuse leptomeningeal enhancement of the brainstem, cerebral cortex, and spinal cord. Despite two courses of intravenous methylprednisolone, he developed respiratory arrest and required mechanical ventilation. Plasma exchange (PE) resulted in rapid extubation and neurological recovery. Anti-GFAPα antibodies were detected in the CSF. Early PE may represent a crucial therapeutic option for steroid-refractory GFAP-ADs with medullary involvement.