Suppiej Agnese, Cainelli Elisa, Casara Giulia, Cappellari Ambra, Nosadini Margherita, Sartori Stefano
Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy.
Child Neurology and Clinical Neurophysiology, Paediatric University Hospital, Padua, Italy; Lifespan Cognitive Neuroscience Laboratory, Department of General Psychology, University of Padua, Padua, Italy.
Pediatr Neurol. 2014 Apr;50(4):363-7. doi: 10.1016/j.pediatrneurol.2013.12.006. Epub 2013 Dec 12.
Acute disseminated encephalomyelitis is an inflammatory-demyelinating disorder of the central nervous system usually with a monophasic course and a favorable neurological outcome. Long-term neurocognitive sequelae and quality of life have not yet been fully investigated.
To examine neurocognitive outcome and quality of life in pediatric monophasic acute disseminated encephalomyelitis.
Of the 36 patients diagnosed with acute disseminated encephalomyelitis at our institution, six were lost to follow-up and eight relapsed (two with multiphasic forms and six with multiple sclerosis). The outcome of the 22 remaining patients was evaluated using four subscales of the Wechsler Intelligence Scales for estimation of IQ, a battery of neuropsychological tests, and semistructured and PedsQL questionnaires for quality of life. The effect of age at onset, neuroradiological recovery, and time elapsed from the acute event on outcome was also investigated.
Estimated IQ, neuropsychological mean group scores, and quality of life at follow-up were within the normal range, but 23% of the patients had pathological scores in various neuropsychological functions, among which attention was the most clearly affected. The neuroradiological recovery was not correlated with the result of the neuropsychological tests. Age at onset correlated with linguistic skills, whereas the time elapsed from the acute event had a significant effect on attention tasks: scores were worse in the group of patients with a follow-up shorter than 7 years.
Our results suggest that pediatric monophasic acute disseminated encephalomyelitis has a favorable neurocognitive outcome. Patients with longer follow-up had a better outcome, suggesting a neurocognitive course that is different from that of multiple sclerosis and a potential for long-term recovery of affected functions.
急性播散性脑脊髓炎是一种中枢神经系统的炎症性脱髓鞘疾病，通常病程为单相，神经功能预后良好。长期神经认知后遗症和生活质量尚未得到充分研究。
研究儿童单相急性播散性脑脊髓炎的神经认知结局和生活质量。
在我院诊断为急性播散性脑脊髓炎的36例患者中，6例失访，8例复发（2例为多相型，6例为多发性硬化）。使用韦氏智力量表的四个分量表评估剩余22例患者的智商，进行一系列神经心理学测试，并使用半结构化问卷和儿童生活质量量表（PedsQL）评估生活质量。还研究了发病年龄、神经影像学恢复情况以及急性事件发生后的时间对结局的影响。
随访时的估计智商、神经心理学平均组分数和生活质量均在正常范围内，但23%的患者在各种神经心理学功能方面存在病理分数，其中注意力受影响最明显。神经影像学恢复与神经心理学测试结果无关。发病年龄与语言技能相关，而急性事件发生后的时间对注意力任务有显著影响：随访时间短于7年的患者组分数较差。
我们的结果表明，儿童单相急性播散性脑脊髓炎具有良好的神经认知结局。随访时间较长的患者结局较好，这表明其神经认知过程与多发性硬化不同，受影响功能有长期恢复的潜力。
