Palmini A, Andermann F, Olivier A, Tampieri D, Robitaille Y, Melanson D, Ethier R
Department of Neurology and Neurosurgery, McGill University, Montreal, Quebec, Canada.
Can J Neurol Sci. 1991 Nov;18(4 Suppl):580-7. doi: 10.1017/s0317167100032753.
Computed tomography and magnetic resonance imaging enable the identification of neuronal migration disorders during life. Several specific syndromes have been identified and early diagnosis of previously unrecognized entities is now possible. We report 51 patients with imaging. Thirty-two had a single widespread cortical dysplastic lesion. Twenty-eight had focal corticectomies. From a pathological standpoint, these encompassed focal cortical dysplasia (14 cases) and forme fruste of tuberous sclerosis (10 cases). These two groups of patients were indistinguishable from the clinical and radiological standpoint. In only two was the MRI examination normal. In addition, there were 10 with bilateral perisylvian dysplasia, four with diffuse cortical dysplasia or the "double cortex" syndrome, three with hemimegalencephaly, one with megalencephaly, and one with nodular neuronal heterotopia. The electroclinical and imaging findings led to the development of specific surgical strategies for the alleviation of the intractable seizures in each of these radiologically-defined syndromes.
计算机断层扫描和磁共振成像能够在患者生前识别神经元迁移障碍。现已确定了几种特定综合征，目前有可能对以前未被认识的病症进行早期诊断。我们报告了51例有影像学表现的患者。32例有单个广泛的皮质发育异常病灶。28例行局灶性皮质切除术。从病理学角度来看，这些包括局灶性皮质发育异常（14例）和结节性硬化症的顿挫型（10例）。从临床和放射学角度来看，这两组患者无法区分。仅2例MRI检查正常。此外，有10例双侧外侧裂周发育异常，4例弥漫性皮质发育异常或“双皮质”综合征，3例半侧巨脑症，1例巨头症，1例结节性神经元异位。电临床和影像学表现促使针对这些放射学定义综合征中的每一种制定了缓解难治性癫痫的特定手术策略。
