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Despite this progress, a lack of standardized clinical definitions, variable presentations, and heterogeneous calcification patterns has perpetuated inconsistencies in diagnosis and patient care. To address these challenges, an international expert panel undertook a comprehensive process\u2014combining systematic literature review, virtual and in\u2010person expert discussions, and iterative Delphi consensus questionnaires\u2014to develop unified recommendations. These consensus guidelines encompass terminology, diagnostic criteria, neuroimaging protocols, clinical evaluation standards, genetic testing approaches, and management strategies. Notably, PBC is recommended as the clinical standard, with new diagnostic criteria, including the use of a computed tomography\u2013based total calcification score and a three\u2010tiered diagnostic algorithm (possible, probable, definite PBC). A systematic review of 27 symptoms and signs identified 19 as most closely linked with PBC, guiding more focused clinical assessment. Recommendations strongly support comprehensive next\u2010generation sequencing for genetic testing, favoring whole genome over exome or targeted panels to maximize diagnostic yield. Multidisciplinary management priorities are outlined in four key principles centering on individualized care, symptom relief, genetic counseling, and ongoing monitoring. Widespread adoption of these unified guidelines will facilitate consistent diagnosis, enable comparative international data collection, and lay the groundwork for large\u2010scale collaborative research\u2014including future randomized controlled trials of symptomatic and disease\u2010modifying PBC therapies. This consensus fosters clarity and consistency, creating a framework for improved patient care and scientific discovery. \u00a9 2025 International Parkinson and Movement Disorder Society.<\/jats:p>","DOI":"10.1002\/mds.70140","type":"journal-article","created":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T06:30:29Z","timestamp":1764916229000},"page":"315-336","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Primary Brain Calcification: An International Consensus on Nomenclature, Diagnosis, Evaluation, and Management"],"prefix":"10.1002","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6951-7698","authenticated-orcid":false,"given":"Wei","family":"Luo","sequence":"first","affiliation":[{"name":"Department of Neurology, The Second Affiliated Hospital Zhejiang University School of Medicine  Hangzhou China"},{"name":"Liangzhu Laboratory Zhejiang University School of Medicine  Hangzhou 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