Alexander Disease
https://assets.cureus.com/uploads/case_report/pdf/339159/20250501-228466-xp6kfq.pdf
Alexander’s disease is a rare and progressive neurological condition belonging to the leukodystrophy group which affects the white matter in the brain and spinal cord. It is caused by mutations in the GFAP gene, which disrupt the normal function of cells that support the brain’s nerve fibres. Adult-onset Alexander’s disease (type II) is the least severe form, however it still presents complex symptoms such as ataxia (unsteady movement), dysarthria (difficulty speaking), spasticity (muscle stiffness), and bulbar dysfunction (problems with swallowing and breathing). These symptoms make surgical procedures especially challenging, particularly when anaesthesia is required. This case report describes the successful anaesthetic care of a 34-year-old woman with adult-onset Alexander’s disease who underwent laparoscopic gynaecological surgery to remove an intrauterine device and undergo sterilisation. The care team recognised the increased risk of complications, such as aspiration and unstable blood pressure, due to her underlying neurological condition. After a consultation with the neurologist, the team developed a tailored general anaesthetic plan that addressed these risks while using standard medications available in their hospital. Despite the challenges, the procedure was completed safely and without complications. This is the first known case describing this type of surgery and anaesthesia in someone with Alexander’s disease type II. This case highlights how with careful planning, monitoring, and a strong understanding of the disease’s specific effects on the body, patients with rare neurodegenerative conditions can receive safe and effective care, even when access to specialist drugs is limited.