Guzzetta Francesco, Cioni Giovanni, Mercuri Eugenio, Fazzi Elisa, Biagioni Enrico, Veggiotti Pierangelo, Bancale Adina, Baranello Giovanni, Epifanio Roberta, Frisone Maria Flavia, Guzzetta Andrea, La Torre Giuseppe, Mannocci Alice, Randò Teresa, Ricci Daniela, Signorini Sabrina, Tinelli Francesca
Developmental Neuroscience Department, Catholic University, Rome, Italy.
Eur J Paediatr Neurol. 2008 Sep;12(5):387-97. doi: 10.1016/j.ejpn.2007.10.008. Epub 2007 Dec 11.
The aim of this study was to evaluate the epileptic and developmental evolution in infants with West syndrome.
A prospective study of 21 infants was performed, with a follow-up at 2 years. Serial assessment included long-term EEG monitoring, visual and auditory evaluation and assessment of neurodevelopment.
Neurosensory and developmental impairments at the spasm onset were transitory in seven cases, including four cryptogenic forms. In all other cases, there was a progressive worsening in neurosensory and developmental impairments. The epileptic evolution was generally better: in 11 of the 16 infants without seizures at outcome, spasms had already disappeared by 2 months after disease onset. Statistic analysis of results showed a correlation between neurosensory impairment and development throughout the whole follow-up. In addition, visual function at T1 resulted significant predictor of developmental outcome. Among the epileptic features, disorganization of slow sleep was an unfavorable prognostic factor.
Some forms of West syndrome are confirmed to have a benign evolution: among them there are not only cryptogenic cases but also symptomatic ones without significant neurodevelopmental impairment. Abnormalities of sleep organization, expression of the pervasive epileptic disorder, seem to play a role in determining a developmental deterioration. Neurosensory impairment since the onset of the disease could be a relevant cause of the developmental disorder.
本研究旨在评估婴儿痉挛症患儿的癫痫及发育演变情况。
对21例婴儿进行前瞻性研究，随访2年。系列评估包括长期脑电图监测、视觉和听觉评估以及神经发育评估。
7例患儿（包括4例隐源性类型）在痉挛发作时的神经感觉和发育障碍为暂时性。在所有其他病例中，神经感觉和发育障碍呈进行性加重。癫痫演变情况总体较好：在最终无癫痫发作的16例婴儿中，11例在疾病发作后2个月时痉挛已消失。结果的统计分析表明，在整个随访过程中，神经感觉障碍与发育之间存在相关性。此外，T1时的视觉功能是发育结局的显著预测因素。在癫痫特征中，慢波睡眠紊乱是不良预后因素。
证实某些类型的婴儿痉挛症具有良性演变：其中不仅有隐源性病例，还有无明显神经发育障碍的症状性病例。睡眠结构异常，即普遍性癫痫障碍的表现，似乎在决定发育恶化方面起作用。疾病发作时的神经感觉障碍可能是发育障碍的一个相关原因。
