SAHZU neurologist leads an international consensus on primary brain calcification

An international consensus on primary brain calcification (PBC) was recently published online in Movement Disorders, the official journal of the International Parkinson and Movement Disorder Society (MDS). The consensus, titled Primary Brain Calcification: An International Consensus on Nomenclature, Diagnosis, Evaluation, and Management, was developed under the leadership of Prof. LUO Wei from the Department of Neurology at SAHZU, who also serves as an Executive Committee Member of the MDS-Asian and Oceanian Section.

This landmark document marks the first global expert consensus on PBC since the disease was first described in 1850. By synthesizing the knowledge and experience of researchers worldwide, it establishes a unified, standardized framework for clinical diagnosis, treatment, and translational research of PBC.

PBC, or Primary Brain Calcification, presents significant clinical challenges due to the high heterogeneity of its symptoms—including movement disorders, psychiatric symptoms, cognitive impairment, and other manifestations—coupled with complex and variable imaging features. Treatment responses are highly inconsistent, and disease-modifying therapies remain unavailable. For a long time, the lack of unified standards for disease nomenclature, diagnosis, evaluation, and management principles has prevented data from international multi-center cohorts or case reports from being compared or integrated. These challenges have become a major obstacle to disease management, leaving patients and their families with few effective options.

Based on his team's long-term clinical and genetic research on PBC and experience from managing the world's largest PBC patient cohort, Prof. LUO first proposed the idea of developing an international expert consensus for PBC as early as 2020. This initiative received encouragement and support from globally recognized PBC authorities: Prof. Christine Klein from the University of Lübeck, Germany, and Prof. Gaël Nicolas from the University of Rouen Normandy, France.

In April 2023, Prof. LUO's team spearheaded the effort by convening 23 PBC clinical experts and researchers from 10 countries—Brazil, China, France, Germany, Italy, Japan, the Netherlands, Switzerland, the United Kingdom, and the United States—to organize and advance the development of the international consensus.

The consensus expert panel was divided into six subgroups, covering disease nomenclature, diagnostic criteria, imaging evaluation, clinical assessment, genetic testing and counseling, and disease management.

Guided by subgroup leaders, each team conducted systematic literature reviews to draft statements. These were discussed and refined in online meetings, then formalized into draft statements suitable for the Delphi questionnaires for anonymous voting. From September 2023 to June 2025, the panel held 10 online meetings and conducted 4 rounds of Delphi voting on a total of 61 statements. Based on the voting results, the most comprehensive, widely participated, and authoritative international expert consensus on PBC to date was consolidated.

The consensus-building process also fostered the formation of a closely collaborative international PBC researcher group. It is hoped that through sustained and in-depth global collaboration that this disease can be ultimately overcome within the next 5 to 10 years.

Source: The Second Affiliated Hospital, Zhejiang University School of Medicine
Editor: HAN Xiao