Tabarki B, Durand P, Husson B, Loiseau-Corvez M N, Tardieu M
Service de neurologie, hôpital de Bicêtre, Le Kremlin-Bicêtre, France.
Arch Pediatr. 1997 Jul;4(7):645-9. doi: 10.1016/s0929-693x(97)83364-7.
Central pontine myelinolysis has been rarely reported in children. This report concerns two children, aged 4 1/2 years.
The main clinical features were altered level of consciousness, tetraplegia and signs of brain stem dysfunctions. In both cases, magnetic resonance imaging (MRI) revealed hypersignal of protuberance in association with cerebellar lesion in one case and occipital lesion in the other, compatible with central and extrapontine myelinolysis. No underlying disease or electrolytic disorders was identified. Clinical outcome was good. Mental status was normal after 9 months for patient one and 4 months for patient two. The MRI abnormality was reduced at 9-month follow-up in patient two and unchanged at 4 months in patient one.
The incidence of central pontine myelinolysis is probably underestimated in children. MRI allows a greater sensitivity in identifying areas of focal demyelination where diagnosis was previously only established by post mortem studies.
儿童脑桥中央髓鞘溶解症鲜有报道。本报告涉及两名4岁半的儿童。
主要临床特征为意识水平改变、四肢瘫痪及脑干功能障碍体征。两例中,磁共振成像(MRI)均显示脑桥突出部高信号,其中一例合并小脑病变,另一例合并枕叶病变,符合脑桥中央髓鞘溶解症及脑桥外髓鞘溶解症。未发现潜在疾病或电解质紊乱。临床结局良好。患者一9个月后精神状态恢复正常,患者二4个月后恢复正常。患者二在9个月随访时MRI异常减轻,患者一在4个月时未变。
儿童脑桥中央髓鞘溶解症的发病率可能被低估。MRI在识别局灶性脱髓鞘区域方面具有更高的敏感性,此前仅通过尸检研究才能确诊。