Schreiber Jane E, Gurney James G, Palmer Shawna L, Bass Johnnie K, Wang Mingjuan, Chen Si, Zhang Hui, Swain Michelle, Chapieski Mary L, Bonner Melanie J, Mabbott Donald J, Knight Sarah J, Armstrong Carol L, Boyle Robyn, Gajjar Amar
Department of Psychology St. Jude Children's Research Hospital, Memphis, Tennessee (J.E.S., S.L.P.); Department of Epidemiology and Cancer Control, St. Jude Children's Research Hospital, Memphis, Tennessee (J.G.G.); School of Public Health, University of Memphis, Memphis, Tennessee (J.G.G.); Rehabilitation Services, St. Jude Children's Research Hospital, Memphis, Tennessee (J.K.B); Department of Biostatistics, St. Jude Children's Research Hospital, Memphis, Tennessee (M.W., S.C., H.Z.); Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee (A.G.); Royal Children's Hospital Brisbane, Herston, Australia (M.S.); Department of Pediatric Medicine, Texas Children's Hospital, Houston, Texas (M.L.C.); Department of Psychiatry, Duke University Medical Center, Durham, North Carolina (M.J.B.); Department of Psychology, The Hospital for Sick Children, Toronto, Canada (D.J.M.); Department of Psychology, The Royal Children's Hospital Melbourne, Victoria Australia (S.J.K.); Neuro-Oncology Program, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania (C.L.A); Psychology Service, Sydney Children's Hospital, Randwick, Australia (R.B.).
Neuro Oncol. 2014 Aug;16(8):1129-36. doi: 10.1093/neuonc/nou006. Epub 2014 Feb 3.
The aim of this study was to prospectively examine the effects of hearing loss and posterior fossa syndrome (PFS), in addition to age at diagnosis and disease risk status, on change in intellectual and academic outcomes following diagnosis and treatment in a large sample of medulloblastoma patients.
Data from at least 2 cognitive and academic assessments were available from 165 patients (ages 3-21 years) treated with surgery, risk-adapted craniospinal irradiation, and 4 courses of chemotherapy with stem cell support. Patients underwent serial evaluation of cognitive and academic functioning from baseline up to 5 years post diagnosis.
Serious hearing loss, PFS, younger age at diagnosis, and high-risk status were all significant risk factors for decline in intellectual and academic skills. Serious hearing loss and PFS independently predicted below-average estimated mean intellectual ability at 5 years post diagnosis. Patients with high-risk medulloblastoma and young age at diagnosis (<7 years) exhibited the largest drop in mean scores for intellectual and academic outcomes.
Despite a significant decline over time, intellectual and academic outcomes remained within the average range at 5 years post diagnosis for the majority of patients. Future studies should determine if scores remain within the average range at time points further out from treatment. Patients at heightened risk should be closely monitored and provided with recommendations for appropriate interventions.
本研究的目的是前瞻性地研究在一大群髓母细胞瘤患者中，除了诊断时的年龄和疾病风险状态外，听力丧失和后颅窝综合征（PFS）对诊断和治疗后智力和学业成绩变化的影响。
165例年龄在3至21岁之间的患者接受了手术、风险适应性颅脊髓照射以及4个疗程的化疗并伴有干细胞支持治疗，可获得至少2次认知和学业评估的数据。患者从基线到诊断后5年接受认知和学业功能的系列评估。
严重听力丧失、PFS、诊断时年龄较小以及高危状态均是智力和学业技能下降的显著风险因素。严重听力丧失和PFS独立预测诊断后5年估计平均智力能力低于平均水平。高危髓母细胞瘤患者和诊断时年龄较小（<7岁）的患者在智力和学业成绩方面的平均得分下降幅度最大。
尽管随着时间推移有显著下降，但大多数患者在诊断后5年时智力和学业成绩仍保持在平均范围内。未来的研究应确定在治疗后更远时间点的分数是否仍保持在平均范围内。高危患者应密切监测，并提供适当干预的建议。
