D'Agostino M D, Bastos A, Piras C, Bernasconi A, Grisar T, Tsur V Gross, Snipes J, Juhasz C, Chugani H, Guerrini R, Cross H, Andermann E, Dubeau F, Montes J, Olivier A, Andermann F
Department of Neurology and Epilepsy Service, Montreal Neurological Hospital and Institute, McGill University, Montreal, Canada.
Neurology. 2004 Jun 22;62(12):2214-20. doi: 10.1212/01.wnl.0000130459.91445.91.
Posterior quadrantic dysplasia (PQD), a developmental malformation involving the temporal, parietal, and occipital lobes of one cerebral hemisphere, leads to intractable epilepsy.
To characterize the clinical features of 19 patients with PQD and analyze the postsurgical outcome of those who underwent resection of dysplastic tissue.
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.
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.
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.
后象限发育异常（PQD）是一种涉及一个大脑半球颞叶、顶叶和枕叶的发育畸形，可导致难治性癫痫。
描述19例后象限发育异常患者的临床特征，并分析接受发育异常组织切除手术患者的术后结果。
主要通过高分辨率脑成像评估畸形的范围和性质。14例患者接受了完全或部分颞顶枕叶切除术或与顶枕叶离断相关的颞叶切除术。术后随访时间为8个月至7年。作者采用恩格尔分类法评估术后结果。
所有患者均为散发性。临床特征包括婴儿痉挛、部分性发作、智力发育迟缓、轻度偏瘫和视野缺损。神经影像学检查将畸形定位于与神经功能缺损对侧的大脑后象限，19例患者中有14例表现为半侧巨脑回畸形，5例表现为多叶皮质发育异常。作者观察到6例患者预后为Ⅰ级。2例患者为Ⅱ级，4例患者为Ⅲ级。2例患者为Ⅳ级。术后，2例患者出现轻度偏瘫，2例患者出现视野缺损。
广泛的皮质发育异常在后象限更为常见。在我们的系列研究中，后象限发育异常表现为半侧巨脑回畸形或多叶皮质发育异常。后象限发育异常患者具有一系列临床特征。这些患者的难治性癫痫可通过大范围的象限性颞顶枕叶切除术得到缓解。
