Routon M C, Expert-Bezançon M C, Bursztyn J, Mselati J C, Robain O
Laboratoire de Neuropathologie, Hôpital St-Vincent-de-Paul.
Rev Neurol (Paris). 1994;150(5):363-9.
Developmental pseudobulbar palsies seem to be different from the adult form described by Foix, Chavany and Marie. They usually include a major speech delay and severe epileptic seizures. In one clinicopathological case, neuroradiological imaging showed a macrogyric aspect of both rolandic operculi and unilateral destruction of pes pedunculari. Microscopic examination showed a four-layered polymicrogyria involving the first temporal gyrus and in the brainstem a selective destruction of the left oculomotor nucleus. Thus, the macrogyric aspect could be related to post migratory disorder occurring late in the cortical development. The brain stem lesion, destroying unilaterally the third cranial nerve nucleus gives a good example of the complex somatotopia of this oculomotor nucleus.
发育性假性延髓麻痹似乎与福瓦、沙瓦尼和玛丽所描述的成人型不同。它们通常包括严重的语言发育迟缓以及严重的癫痫发作。在一个临床病理病例中,神经放射学成像显示中央前回盖部呈巨脑回形态,以及大脑脚单侧破坏。显微镜检查显示涉及第一颞回的四层多小脑回,并且在脑干中左侧动眼神经核有选择性破坏。因此,巨脑回形态可能与皮质发育后期发生的迁移后障碍有关。脑干病变单侧破坏第三脑神经核,很好地说明了该动眼神经核复杂的躯体定位。