Bogousslavsky J, Miklossy J, Regli F, Deruaz J P, Despland P A
J Neurol Neurosurg Psychiatry. 1984 Sep;47(9):927-35. doi: 10.1136/jnnp.47.9.927.
Five patients with "locked-in" syndrome and dysconjugate palsy of horizontal gaze were studied. In all cases internuclear ophthalmoplegia due to dysfunction or destruction of the median longitudinal fasciculus was combined with an ipsilateral gaze palsy, producing the "one-and-a-half" syndrome. Clinical and electro-oculographic examination suggested involvement of the paramedian pontine reticular formation when all ipsilateral saccades were abolished, when exotropia of the contralateral eye was present, and when vestibular stimulation showed full conjugate deviation to the damaged side. Involvement of the abducens nucleus was suggested when the palsy of ipsilateral gaze was not dissociated on vestibular stimulation. In three cases these clinical deductions were confirmed by the pathological study, which showed a corresponding destruction of the median longitudinal fasciculus, paramedian pontine reticular formation and abducens nucleus. In one case the one-and-a-half syndrome evolved into a total horizontal gaze palsy, which corresponded to involvement of the abducens nucleus contralateral to the initially destroyed paramedian pontine reticular formation. Vertical oculocephalic response disappeared, because of destruction of the median longitudinal fasciculus on both sides (bilateral internuclear ophthalmoplegia). Patients with the locked-in syndrome provide a unique situation in which complex pontine oculomotor disturbances may be studied, because consciousness is preserved. In these patients, dissociated and dysconjugate oculomotor palsy may have been underestimated.
对5例患有“闭锁综合征”及水平凝视分离性麻痹的患者进行了研究。在所有病例中，因内侧纵束功能障碍或破坏所致的核间性眼肌麻痹与同侧凝视麻痹合并存在，产生了“一个半”综合征。临床和眼震电图检查表明，当所有同侧扫视均消失、对侧眼出现外斜视、前庭刺激显示向受损侧的完全共轭偏斜时，脑桥旁正中网状结构受累。当前庭刺激时同侧凝视麻痹未分离时，提示展神经核受累。3例患者的这些临床推断经病理研究证实，病理显示内侧纵束、脑桥旁正中网状结构及展神经核有相应破坏。1例患者的一个半综合征演变为完全性水平凝视麻痹，这与最初受损的脑桥旁正中网状结构对侧的展神经核受累相对应。垂直眼前庭反射消失，系因双侧内侧纵束破坏（双侧核间性眼肌麻痹）。患有闭锁综合征的患者提供了一种独特的情况，可借此研究复杂的脑桥动眼神经障碍，因为意识得以保留。在这些患者中，分离性及非共轭性动眼神经麻痹可能一直未得到充分认识。
