Grill Jacques, Viguier Delphine, Kieffer Virginie, Bulteau Christine, Sainte-Rose Christian, Hartmann Olivier, Kalifa Chantal, Dellatolas Georges
Department of Paediatric and Adolescent Oncology, Gustave Roussy Institute, Villejuif, France.
J Neurosurg. 2004 Nov;101(2 Suppl):152-8. doi: 10.3171/ped.2004.101.2.0152.
Intellectual impairment is a major concern after treatment of malignant posterior fossa tumors in children. The effects of age at diagnosis and radiotherapy have been widely documented. Little is known, however, about perioperative factors, especially neurological damage to the cerebellum, the role of which in cognition and learning has been recently indicated. The authors studied the effects in 76 children treated for a malignant posterior fossa tumor in a cross-sectional study.
Two thirds of the tumors were medulloblastoma. Neuropsychological evaluation was performed at least 6 months after the end of treatment, and findings were correlated with clinical risk factors for intellectual impairment. The mean verbal intelligence quotient (VIQ) score was 87 +/- 19 (+/- standard deviation) and the mean performance IQ (PIQ) score was 76 +/- 17.5. A single neuropsychological test measuring hand skills (the Purdue Pegboard) was the strongest predictor of low IQ scores including items testing higher cognitive functions. A low VIQ was associated with impaired hand skills (p < 0.0001) and the presence of preoperative hydrocephalus (p = 0.02), whereas a low PIQ was associated with impaired hand skills (p < 0.0001) and incision of the vermis (p = 0.02). Impaired hand skills were associated with postoperative cerebellar mutism, oculomotor deficits, cerebellar syndrome, and therapeutic requirements.
When treatment schedules are adapted to risk of disease and age, surgery-related risk factors then become critical for predicting intellectual impairment. Children with cerebellar damage are particularly at risk for long-term neuropsychological dysfunction and require active rehabilitation measures. Reducing surgery-related morbidity should be the next goal to reduce posterior fossa surgery-specific deficits.
智力障碍是儿童恶性后颅窝肿瘤治疗后的一个主要问题。诊断时的年龄和放疗的影响已有大量文献记载。然而，关于围手术期因素，尤其是小脑的神经损伤，人们了解甚少，而最近已有研究表明其在认知和学习中的作用。作者在一项横断面研究中对76例接受恶性后颅窝肿瘤治疗的儿童的影响进行了研究。
三分之二的肿瘤为髓母细胞瘤。在治疗结束后至少6个月进行神经心理学评估，并将结果与智力障碍的临床风险因素相关联。平均言语智商（VIQ）得分为87±19（±标准差），平均操作智商（PIQ）得分为76±17.5。一项测量手部技能的单一神经心理学测试（普渡钉板测试）是低智商得分的最强预测指标，包括测试更高认知功能的项目。低VIQ与手部技能受损（p<0.0001）和术前脑积水的存在（p = 0.02）相关，而低PIQ与手部技能受损（p<0.0001）和蚓部切开（p = 0.02）相关。手部技能受损与术后小脑缄默症、动眼神经缺陷、小脑综合征及治疗需求相关。
当治疗方案根据疾病风险和年龄进行调整时，手术相关风险因素对于预测智力障碍就变得至关重要。小脑受损的儿童尤其有长期神经心理功能障碍的风险，需要积极的康复措施。降低手术相关发病率应是减少后颅窝手术特定缺陷的下一个目标。
