Wisniewski K E, Schmidt-Sidor B
New York State Office of Mental Retardation and Developmental Disabilities, Institute for Basic Research in Developmental Disabilities, Staten Island 10314.
Clin Neuropathol. 1989 Mar-Apr;8(2):55-62.
Myelination up to the age of 6 years was studied in two groups consisting of 129 Down syndrome (DS) 17/129 fetuses and 112/129 postnatal) and 73 non-DS cases (10/73 fetuses and 63/73 postnatal). In both groups studied a similar number of congenital heart disease (CHD), gastrointestinal (GI) malformations and infections were diagnosed. Paraffin or celloidin brain sections were stained with Klüver-Barrera, Heidenhain or Loyez method. The myelination was found to be delayed in 29/129 (22.5%) DS and only in 5/73 (6.8%) non-DS cases. Myelination in fetuses and newborns in the DS and non-DS groups was not delayed. In DS the myelination was delayed between ages 2 months-6 years (17/29; 58.6%) up to 12 months, and 12/29 (41.4%) aged 2-6 years, while in non-DS aged 2-6 months only. The myelination delay affected tracts with late beginning and slow cycle of myelination, mainly the associated and intercortical fibers of the fronto-temporal lobes. In 3/7 of DS cases (ages 3, 4, 6 years) less advanced myelination of U fibers was noted. In both groups the myelination delay seemed also to depend on the systemic diseases which affected the subjects during the time of myelination. In DS where CHD was present the myelination delay was found in 14/29 (48.2%) in contrast to 3/5 (60%) non-DS. Also, in 28/129 (23%) DS cases dates regarding the developmental milestones were available and some correlation was found between developmental and myelination delay.(ABSTRACT TRUNCATED AT 250 WORDS)
对两组对象进行了研究，以观察6岁前的髓鞘形成情况。第一组包括129例唐氏综合征（DS）患者（其中17例为胎儿，112例为出生后患者），第二组包括73例非DS患者（其中10例为胎儿，63例为出生后患者）。在两组研究对象中，先天性心脏病（CHD）、胃肠道（GI）畸形和感染的诊断病例数相似。用Klüver-Barrera、Heidenhain或Loyez方法对石蜡或火棉胶包埋的脑切片进行染色。结果发现，129例DS患者中有29例（22.5%）髓鞘形成延迟，而73例非DS患者中只有5例（6.8%）髓鞘形成延迟。DS组和非DS组胎儿及新生儿的髓鞘形成未延迟。在DS组中，2个月至6岁（17/29；58.6%）直至12个月时髓鞘形成延迟，2至6岁时为12/29（41.4%），而非DS组仅在2至6个月时髓鞘形成延迟。髓鞘形成延迟影响了起始较晚且髓鞘形成周期缓慢的神经束，主要是额颞叶的联合纤维和皮质间纤维。在7例DS患者中的3例（年龄分别为3岁、4岁、6岁）中，发现U纤维的髓鞘形成进展较慢。在两组中，髓鞘形成延迟似乎也取决于在髓鞘形成期间影响研究对象的全身性疾病。在患有CHD的DS患者中，14/29（48.2%）出现髓鞘形成延迟，相比之下，非DS患者中这一比例为3/5（60%）。此外，在129例DS患者中的28例（23%）中可获得发育里程碑数据，并且发现发育延迟与髓鞘形成延迟之间存在一定相关性。（摘要截断于250字）
