From the Departments of Diagnostic Imaging (Q.J., S.S.M., M.S., J.H., J.O.G., A.E., D.S., W.E.R., Z.P., A.K.B.), Biostatistics (X.Y., J.L., Y.L.), Pathology (J.C., B.A.O.), Epidemiology and Cancer Control (T.B.), Oncology (G.W.R., A.G.), Neurology (R.B.K.), and Pediatric Medicine (R.B.K.), St. Jude Children's Research Hospital, 262 Danny Thomas Pl, MS 220, Memphis, TN 38105.
Radiology. 2024 May;311(2):e232521. doi: 10.1148/radiol.232521.
Background Cerebellar mutism syndrome (CMS), a complication following medulloblastoma surgery, has been linked to dentato-thalamo-cortical tract (DTCT) injury; the association of the degree of DTCT injury with severity of CMS-related symptoms has not been investigated. Purpose To investigate the association between severity of CMS-related symptoms and degree and patterns of DTCT injury with use of diffusion tensor imaging (DTI), and if laterality of injury influences neurologic symptoms. Materials and Methods This retrospective case-control study used prospectively collected clinical and DTI data on patients with medulloblastoma enrolled in a clinical trial (between July 2016 and February 2020) and healthy controls (between April and November 2017), matched with the age range of the participants with medulloblastoma. CMS was divided into types 1 (CMS1) and 2 (CMS2). Multivariable logistic regression was used to investigate the relationship between CMS likelihood and DTCT injury. Results Overall, 82 participants with medulloblastoma (mean age, 11.0 years ± 5.2 [SD]; 53 male) and 35 healthy controls (mean age, 18.0 years ± 3.06; 18 female) were included. In participants with medulloblastoma, DTCT was absent bilaterally (AB), absent on the right side (AR), absent on the left side (AL), or present bilaterally (PB), while it was PB in all healthy controls. Odds of having CMS were associated with higher degree of DTCT damage (AB, odds ratio = 272.7 [95% CI: 269.68, 275.75; < .001]; AR, odds ratio = 14.40 [95% CI: 2.84, 101.48; < .001]; and AL, odds ratio = 8.55 [95% CI: 1.15, 74.14; < .001). Left (coefficient = -0.07, χ = 12.4, < .001) and right (coefficient = -0.15, χ = 33.82, < .001) DTCT volumes were negatively associated with the odds of CMS. More participants with medulloblastoma with AB showed CMS1; unilateral DTCT absence prevailed in CMS2. Lower DTCT volumes correlated with more severe ataxia. Unilateral DTCT injury caused ipsilateral dysmetria; AB caused symmetric dysmetria. PB indicated better neurologic outcome. Conclusion The severity of CMS-associated mutism, ataxia, and dysmetria was associated with DTCT damage severity. DTCT damage patterns differed between CMS1 and CMS2. © RSNA, 2024 See also the editorial by Dorigatti Soldatelli and Ertl-Wagner in this issue.
背景 小脑缄默症综合征（CMS）是髓母细胞瘤手术后的一种并发症，与齿状核-丘脑-皮质束（DTCT）损伤有关；DTCT 损伤的程度与 CMS 相关症状的严重程度之间的关系尚未得到研究。目的 用弥散张量成像（DTI）研究 CMS 相关症状的严重程度与 DTCT 损伤程度和模式之间的关系，并探讨损伤的偏侧性是否影响神经症状。材料与方法 本回顾性病例对照研究使用前瞻性收集的髓母细胞瘤患者的临床和 DTI 数据（2016 年 7 月至 2020 年 2 月）和健康对照者（2017 年 4 月至 11 月），与髓母细胞瘤患者的年龄范围相匹配。CMS 分为 1 型（CMS1）和 2 型（CMS2）。采用多变量逻辑回归分析 CMS 发生的可能性与 DTCT 损伤之间的关系。结果 共有 82 例髓母细胞瘤患者（平均年龄，11.0 岁±5.2[标准差]；53 例男性）和 35 例健康对照者（平均年龄，18.0 岁±3.06；18 例女性）纳入研究。在髓母细胞瘤患者中，DTCT 双侧缺失（AB）、右侧缺失（AR）、左侧缺失（AL）或双侧存在（PB），而所有健康对照者均为 PB。发生 CMS 的可能性与更高程度的 DTCT 损伤相关（AB，比值比=272.7[95%CI：269.68，275.75；<.001]；AR，比值比=14.40[95%CI：2.84，101.48；<.001]；AL，比值比=8.55[95%CI：1.15，74.14；<.001]）。左（系数=-0.07，χ=12.4，<.001）和右（系数=-0.15，χ=33.82，<.001）DTCT 体积与 CMS 的可能性呈负相关。更多 AB 的髓母细胞瘤患者出现 CMS1；CMS2 中单侧 DTCT 缺失更为常见。较低的 DTCT 体积与更严重的共济失调相关。单侧 DTCT 损伤导致同侧运动失调；AB 导致对称运动失调。PB 表明更好的神经功能结局。结论 CMS 相关缄默、共济失调和运动失调的严重程度与 DTCT 损伤严重程度相关。CMS1 和 CMS2 之间的 DTCT 损伤模式不同。©RSNA，2024 另见本期 Dorigatti Soldatelli 和 Ertl-Wagner 的社论。
