Abstract
Alexander's disease is a rare and progressive neurodegenerative disorder that presents significant challenges for anesthetic management due to its effects on the central nervous system. The disease stems from mutations in the glial fibrillary acidic protein (i.e., GFAP) gene, leading to impaired myelination and neurological complications such as bulbar dysfunction, spasticity, and autonomic instability. This case report presents the anesthetic management of a 34-year-old female patient diagnosed with Alexander's disease type II (i.e., adult-onset), cerebellar ataxia, and dysarthria. She underwent bilateral laparoscopic tubal sterilization and intrauterine device removal. After a thorough evaluation of the anesthetic options and consideration of the neurological complications associated with her condition, the procedure was successfully performed without complications under general anesthesia. To the best of our knowledge, this is the first documented case of a patient with Alexander's disease type II undergoing a gynecological procedure under general anesthesia. This case highlights the complex considerations necessary in anesthetic care for patients with Alexander's disease type II, including aspiration risk from bulbar dysfunction, altered response to neuromuscular blocking agents due to spasticity, and the potential for autonomic instability during surgical stimulation. Furthermore, it emphasizes the importance of a tailored anesthetic plan combined with vigilant perioperative monitoring in the management of these patients.