D'Cruz O F, Swisher C N, Jaradeh S, Tang T, Konkol R J
Department of Neurology, University of North Carolina, Chapel Hill 27599-7025.
J Child Neurol. 1993 Jul;8(3):260-5. doi: 10.1177/088307389300800310.
We report five infants with restricted lateral gaze, facial diplegia, feeding difficulty, and/or respiratory disorders without significant pulmonary disease. Viral studies were negative in all patients. Two children had radiologic findings that included brain-stem hypoplasia and symmetric calcification in the dorsal tectum at the junction of the midbrain and pons. Autopsy of one of these two children demonstrated capillary telangiectasia in the mesencephalon and pons. The other three children had normal computed tomographic (CT) scans. However, their autopsies revealed focal brain-stem necrosis with calcifications but without vascular malformation. We suggest that the capillary malformations in one of our patients directly resulted in a vascular-induced necrosis and the manifestation of Möbius sequence. The similarity of symmetric neuropathologic findings in the three other patients and the CT scan in the one surviving patient suggest focal hemodynamic changes restricted to the posterior circulation, indirectly supporting a vascular theory of embryopathogenesis.
我们报告了5例婴儿，他们存在侧向凝视受限、面瘫、喂养困难和/或呼吸障碍，但无明显肺部疾病。所有患者的病毒学检查均为阴性。2例患儿有影像学表现，包括脑干发育不全以及中脑与脑桥交界处背侧顶盖的对称性钙化。这2例患儿中的1例尸检显示中脑和脑桥有毛细血管扩张。另外3例患儿的计算机断层扫描（CT）结果正常。然而，他们的尸检显示有局灶性脑干坏死伴钙化，但无血管畸形。我们认为，我们其中1例患者的毛细血管畸形直接导致了血管性坏死以及莫比乌斯序列的表现。其他3例患者对称的神经病理学发现以及1例存活患者的CT扫描结果相似，提示局灶性血流动力学改变局限于后循环，间接支持了胚胎发病机制的血管理论。
