Mateos V, Campos D M, Colosía V P, Salas-Puig J, Fernández J M, Lahoz C H
Servicio de Neurología y Radiodiagnóstico, Hospital General de Asturias, Oviedo.
Arch Neurobiol (Madr). 1992 Jul-Aug;55(4):183-7.
The nuclear syndrome of the third nerve was first described in 1981. It has the very characteristic disturbance of an ophthalmoplegia with complete ipsilateral third nerve palsy associated with paresis of elevation in contralateral eye. This particularly presentation is due to the innervation of the superior rectus which comes mainly from the contralateral oculomotor nucleus. As associated signs were described contralateral cerebellar and or pyramidal syndromes, uni or bilateral parasympathetic disfunction and sometimes gaze disorders. The etiology es usually a vascular damage (ischemic most frequently) located in mesencephalon. We report on a case of a 60 years old man who developed acute nuclear ophthalmoplegia of the third right nerve accompanied with cerebellar and pyramidal syndrome and focal asterixis in left extremities. MRI showed an ischemic lesion in right paramedial mesencephalic territory with extension to the ipsilateral thalamic region. Pyramidal and cerebellar syndromes and asterixis disappeared in a few weeks, while ophthalmoplegia remained unchanged. Semiologic characteristics and anatomic basis of the nuclear oculomotor syndrome which allow to make the differential diagnosis between this syndrome and intra-axial fascicular disturbances of the third nerve (Weber, Claude and Benedikt syndromes) are discuss.
动眼神经核综合征于1981年首次被描述。其具有非常典型的动眼神经麻痹表现,即同侧动眼神经完全麻痹伴有对侧眼上抬无力。这种特殊表现是由于上直肌的神经支配主要来自对侧动眼神经核。还描述了相关体征,如对侧小脑和/或锥体束综合征、单侧或双侧副交感神经功能障碍,有时还有凝视障碍。病因通常是中脑的血管损伤(最常见的是缺血性损伤)。我们报告一例60岁男性,其出现右侧动眼神经急性核性麻痹,伴有小脑和锥体束综合征以及左侧肢体的局灶性扑翼样震颤。MRI显示右侧中脑旁正中区域有缺血性病变,并延伸至同侧丘脑区域。锥体束和小脑综合征以及扑翼样震颤在几周内消失,而动眼神经麻痹仍无变化。文中讨论了动眼神经核综合征的症状学特征和解剖学基础,这有助于该综合征与动眼神经轴内束性病变(韦伯综合征、克洛德综合征和贝内迪克特综合征)进行鉴别诊断。