Spinal Bulbar Muscular Atrophy: Canadian Best Practice Recommendations

This episode reviews clinical practice guideline developed for the management of Spinal Bulbar Muscular Atrophy (SBMA), also known as Kennedy disease, in Canada. The guideline is prompted by the discovery of a substantially elevated prevalence of this rare X-linked recessive neuromuscular disorder among First Nations and Métis people in western Canada. It addresses the diagnosis and comprehensive, multidisciplinary management of SBMA, including motor, cardiac, and endocrine complications, while emphasizing the critical need for culturally appropriate care for Indigenous populations. The recommendations, which cover 41 aspects of care, were created using rigorous methodological frameworks by an interdisciplinary working group and informed by existing guidelines for SBMA (from France) and Amyotrophic Lateral Sclerosis (ALS).