Snijders Birgitta M G, Koek Huiberdina L, Peters Mike J L, Mali Willem P T M, van Beek Michelle M, Betman Merel J C, Golüke Nienke M S, Kruyswijk Tijl, de Lange Stéphanie V, Lith Bouke D W T, Pekelharing Ruth M, Roos Marvin J, Rutgers Dirk R, Venema Simone M Uniken, Verberne Wouter R, Emmelot-Vonk Marielle H, de Jong Pim A
From the Departments of Geriatrics (B.M.G.S., H.L.K., M.J.LP., M.M.v.B., T.K., B.D.W.T.L., R.M.P., M.J.R., W.R.V., M.H.E.-V.), University Medical Center Utrecht, Utrecht, the Netherlands
From the Departments of Geriatrics (B.M.G.S., H.L.K., M.J.LP., M.M.v.B., T.K., B.D.W.T.L., R.M.P., M.J.R., W.R.V., M.H.E.-V.), University Medical Center Utrecht, Utrecht, the Netherlands.
AJNR Am J Neuroradiol. 2025 Jan 8;46(1):147-152. doi: 10.3174/ajnr.A8446.
The total calcification score (TCS) is a visual rating scale to measure primary familial brain calcification (PFBC)-related calcification severity on CT. We investigated the inter- and intrarater agreement of a modified TCS.
Patients aged ≥18 years with PFBC or Fahr syndrome who visited the outpatient clinic of a Dutch academic hospital were included. The TCS was modified, for example, by adding hippocampal calcification, and ranged from 0 to 95 points. Fifteen raters evaluated all CTs, of whom 3 evaluated the CTs twice. Their entrustable professional activity (EPA) level ranged from II (medical student) to V (neuroradiologist). Agreement was assessed by using the intraclass correlation coefficient (ICC) for the total score. Kendall's W and weighted Cohen κ were used to determine the inter- and intrarater agreement for individual locations, respectively.
Forty patients were included (mean age 60 years, 53% women). The median modified TCS was 34 (range 4-76). For all EPA levels, the interrater agreement of the modified TCS was excellent (ICC = 0.97 [95% CI 0.95-0.98]). The Kendall W values were good to excellent for commonly affected locations, but poor to moderate for less commonly affected locations for raters with lower levels of expertise. The intrarater agreement of the modified TCS was excellent. The Cohen κ of most locations were substantial to almost perfect.
The modified TCS can be used with excellent reproducibility of the overall amount of brain calcifications and with limited training, although for some individual calcification locations more expertise is needed.
总钙化评分（TCS）是一种视觉评分量表，用于测量CT上原发性家族性脑钙化（PFBC）相关钙化的严重程度。我们研究了改良TCS的评分者间和评分者内一致性。
纳入年龄≥18岁、患有PFBC或法尔综合征且前往荷兰一家学术医院门诊就诊的患者。对TCS进行了改良，例如增加了海马钙化，评分范围为0至95分。15名评分者对所有CT进行评估，其中3名评分者对CT进行了两次评估。他们的可委托专业活动（EPA）水平从II级（医学生）到V级（神经放射科医生）不等。通过使用组内相关系数（ICC）评估总分的一致性。分别使用肯德尔W系数和加权科恩κ系数来确定各个部位的评分者间和评分者内一致性。
纳入40例患者（平均年龄60岁，53%为女性）。改良TCS的中位数为34（范围4 - 76）。对于所有EPA水平，改良TCS的评分者间一致性极佳（ICC = 0.97 [95% CI 0.95 - 0.98]）。对于常见受累部位，肯德尔W值良好至极佳，但对于专业水平较低的评分者，较少受累部位的肯德尔W值较差至中等。改良TCS的评分者内一致性极佳。大多数部位的科恩κ系数为实质性至几乎完美。
改良TCS可用于脑钙化总量的重复性极佳且所需培训有限，尽管对于某些个别钙化部位需要更多专业知识。
