Posterior quadrantic dysplasia or hemi-hemimegalencephaly
Posterior quadrantic dysplasia or hemi-hemimegalencephaly
复制标题
后象限发育不良或半侧巨脑畸形
作者：
Maria Daniela D'Agostino;A. Bastos;C. Piras;A. Bernasconi;T. Grisar;V. G. Tsur;J. Snipes;C. Juhász;H. Chugani;R. Guerrini;Helen Cross;E. Andermann;François Dubeau;José L. Montes;André Olivier;Frederick Andermann
简介：后象限发育不良（PQD）是一种累及一侧大脑半球颞叶、顶叶和枕叶的发育畸形，可导致难治性癫痫。目的：描述19例PQD患者的临床特征，并分析接受异型增生组织切除术的患者的术后结局。方法：采用高分辨率脑成像技术对畸形的范围和性质进行初步评估。14例患者接受了完全或部分颞顶枕切除术或颞叶切除伴顶枕分离术。术后随访8个月~7年。作者使用Engel分类评价术后结局。结果：所有患者均为散发。临床特征包括婴儿痉挛、部分性癫痫发作、智力迟钝、轻度轻偏瘫和视野缺损。神经影像学定位的神经功能缺损的对侧大脑后象限内的畸形，并表现出半侧-半侧巨脑畸形的19例患者中的14例和多叶皮质发育不良的19例患者中的5例。作者在6例患者中观察到I级结局。2例患者为II级，4例患者为III级结局。在2例患者中观察到IV级结局。手术后，两名患者出现轻度偏瘫，两名患者出现视野缺损。结论：广泛的皮质发育不良在后象限更常见。在我们的系列中，后象限发育不良代表半侧-半侧巨脑畸形或多叶皮质发育不良。后象限发育不良的个体具有一系列共同的临床特征。在这些患者的顽固性癫痫可能会减轻一个大的象限颞顶枕叶切除。
Introduction: Posterior quadrantic dysplasia (PQD), a developmental malformation involving the temporal, parietal, and occipital lobes of one cerebral hemisphere, leads to intractable epilepsy. Objective: To characterize the clinical features of 19 patients with PQD and analyze the postsurgical outcome of those who underwent resection of dysplastic tissue. Methods: The extent and nature of the malformation were primarily assessed with high-resolution brain imaging. Fourteen patients underwent complete or partial temporoparieto-occipital resection or temporal resection associated with parieto-occipital disconnection. Postoperative follow-up period ranged from 8 months to 7 years. The authors used the Engel classification for postoperative outcome. Results: All patients were sporadic. Clinical features included infantile spasms, partial seizures, mental retardation, mild hemiparesis, and visual field defects. Neuroimaging localized the malformation within the posterior cerebral quadrant contralateral to the neurologic deficit and demonstrated hemi-hemimegalencephaly in 14 of 19 patients and multilobar cortical dysplasia in 5 of 19 patients. The authors observed class I outcome in six patients. Two patients had class II and four patients had class III outcome. Class IV outcome was seen in two patients. After surgery, two patients developed mild hemiparesis, and two developed a visual field defect. Conclusions: Widespread cortical dysplasia is more frequent in the posterior quadrant. In our series, posterior quadrantic dysplasia represents either hemi-hemimegalencephaly or multilobar cortical dysplasia. Individuals with posterior quadrantic dysplasia share a spectrum of clinical features. The intractable epilepsy in these patients may be alleviated by a large quadrantic temporoparieto-occipital resection.