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目的应用磁共振成像(MRI)对原发性婴儿痉挛与苯丙酮尿症(PKU)合并婴儿痉挛患儿脑髓鞘病变进行观察并比较。方法 2002年8月至2009年7月中日友好医院确诊的婴儿痉挛患儿19例,其中原发性婴儿痉挛(原发组)8例、PKU合并婴儿痉挛(PKU组)11例。分别进行头颅MRI检查,脑髓鞘发育按Staudt标准对不同年龄段患儿脑10个区域:小脑(CL)、桥脑(PO)、中脑(MES)、内囊后肢(ICPL)、内囊前肢(ICAL)、枕叶(OL)、额叶(FL)、顶叶(PL)、颞叶(TL)、胼胝体(CC)进行量化评估。结果 19例中髓鞘化延迟发生率为89.5%(17/19),其中原发组髓鞘化延迟发生率为75.0%(6/8),PKU组延迟发生率为100.0%(11/11),原发组发生髓鞘化延迟范围较普遍,10个区域均有发生,PKU组髓鞘化延迟的主要部位在FL、DL和CC,髓鞘化延迟发生区域数≥5个的PKU组与原发组比较,差异有统计学意义(P<0.05)。结论婴儿痉挛有较高的髓鞘化延迟发生率,其中PKU合并婴儿痉挛髓鞘化延迟发生率更高,且发生区域更集中,可能与血中苯丙氨酸增高影响脑髓鞘形成有关。
Objective To observe and compare the myelin sheath lesions in children with infantile spasms and primary infantile spasms and phenylketonuria (PKU) by using magnetic resonance imaging (MRI). Methods Nineteen infantile spasms were diagnosed in the Sino-Japanese Friendship Hospital from August 2002 to July 2009. Among them, 8 were primary spasticity (primary group) and 11 were PKU combined with infantile spasms (PKU group). The head MRI was performed respectively. According to Staudt criteria, 10 cases of cerebrum (CL), pons (MES), midgut (ICPL) (ICAL), occipital lobe (OL), frontal lobe (FL), parietal lobe (PL), temporal lobe (TL) and corpus callosum (CC). Results The incidence of delayed myelination in 19 patients was 89.5% (17/19). The incidence of delayed myelination in primary group was 75.0% (6/8) and that in PKU group was 100.0% (11/11) ). The delayed onset of myelination in the primary group was common and occurred in 10 regions. The major sites of delayed myelination in PKU group were FLUID, DL and CC, and the number of myelinization delayed onset regions was more than 5 in PKU group Compared with the primary group, the difference was statistically significant (P <0.05). Conclusions Infantile spasms have a higher incidence of delayed myelination. The incidence of delayed myelination in PKU with infantile spasm is higher, and the occurrence of myelination is more concentrated, which may be related to the increase of phenylalanine in blood and the formation of myelin sheath.