Puget Stephanie, Boddaert Nathalie, Viguier Delphine, Kieffer Virginie, Bulteau Christine, Garnett Matthew, Callu Delphine, Sainte-Rose Christian, Kalifa Chantal, Dellatolas Georges, Grill Jacques
Department of Neurosurgery, Necker-Enfants Malades Hospital, Paris, France.
Cancer. 2009 Mar 15;115(6):1338-47. doi: 10.1002/cncr.24150.
Children treated for a malignant posterior fossa tumor (PFT) are at risk of intellectual impairment. Its severity is not explained by age and radiotherapy alone. The current study was designed to define the correlations between the anatomical damage and the neurological/neuropsychological deficits in children with a malignant PFT.
Sixty-one consecutive children (mean age, 6.0 years) treated for a malignant PFT with surgery, chemotherapy, and radiotherapy underwent a detailed neuropsychological evaluation, including a full-scale intelligence quotient (FSIQ), on average 5.6 years after the diagnosis. The neurological examination was recorded 1 month after surgery and at the time of the neuropsychological evaluation. Cerebellar and brain injuries were scored based on the magnetic resonance imaging (MRI). Correlation of these injuries with neurological and cognitive outcome were performed after adjustment for other potential risk factors (radiotherapy schedule, age, hydrocephalus, duration of symptoms, socioeconomic status, and surgical complications).
Neurological deficits were strong predictors of low cognitive performances irrespective of the other risk factors. The extent of cerebellar deficits and fine motor dexterity impairment were correlated with the degree of damage to the dentate nuclei and inferior vermis. The IQ scores were inversely correlated with the severity of the damage to the dentate nuclei; mean FSIQ was 83 if they were both intact and 65 in the case of bilateral damage (P=.009).
Damage to the dentate nuclei and to the inferior vermis, observed on MRI, predict the degree of impairment of neurological and neuropsychological functions in children treated for a malignant PFT.
接受恶性后颅窝肿瘤（PFT）治疗的儿童存在智力损害风险。其严重程度不能仅用年龄和放疗来解释。本研究旨在确定恶性PFT患儿解剖损伤与神经/神经心理缺陷之间的相关性。
61例连续接受手术、化疗和放疗的恶性PFT患儿（平均年龄6.0岁），在诊断后平均5.6年接受了详细的神经心理评估，包括全量表智商（FSIQ）。术后1个月和神经心理评估时记录神经学检查结果。根据磁共振成像（MRI）对小脑和脑损伤进行评分。在对其他潜在风险因素（放疗方案、年龄、脑积水、症状持续时间、社会经济状况和手术并发症）进行调整后，对这些损伤与神经和认知结果的相关性进行分析。
无论其他风险因素如何，神经缺陷都是低认知表现的强有力预测因素。小脑缺陷程度和精细运动灵活性损害与齿状核和下蚓部的损伤程度相关。智商得分与齿状核损伤严重程度呈负相关；两者均完整时平均FSIQ为83，双侧损伤时为65（P = 0.009）。
MRI观察到的齿状核和下蚓部损伤可预测接受恶性PFT治疗的儿童神经和神经心理功能的损害程度。
