ACR Appropriateness Criteria® Demyelinating Diseases

Abstract

Demyelinating diseases of the central nervous system represent a diverse spectrum of inflammatory disorders affecting myelin sheaths, including multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), and myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD). MRI is the cornerstone imaging modality, providing superior sensitivity over CT for detecting demyelinating lesions in the brain and spinal cord. Advanced MRI techniques such as diffusion tensor imaging, magnetization transfer imaging, and AI applications enhance diagnostic accuracy. The 2024 McDonald criteria integrate new imaging features such as central vein sign and paramagnetic rim lesions, improving MS diagnosis with 94% accuracy at 3T MRI. Gadolinium enhancement patterns distinguish active inflammatory lesions from chronic plaques, with specific morphologic characteristics differentiating MS from NMOSD and MOGAD. Spinal cord MRI reveals characteristic lesion patterns: short peripheral lesions in MS versus longitudinally extensive lesions in NMOSD. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer-reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Keywords: AUC; Appropriate Use Criteria; Appropriateness Criteria; MRI; central nervous system (CNS); demyelinating diseases; inflammatory disorders; multiple sclerosis (MS); neuromyelitis optica spectrum disorder (NMOSD).