Modha A, Vassilyadi M, Keene D, Jimenez C, Michaud J, Matzinger M A, Ventureyra E C
Department of Surgery, Children's Hospital of Eastern Ontario, University of Ottawa, Canada.
Childs Nerv Syst. 2000 May;16(5):269-77. doi: 10.1007/s003810050513.
Focal cortical dysplasia (FCD), a form of neuronal migration disorder, is a malformative lesion of the neocortex that occurs during development of the brain. It can cause partial and generalized epilepsy. Seizures occur at an early age and are often resistant to medication. Surgical resection has been found to be beneficial in these patients. Dual pathology, in the form of mesial temporal sclerosis, has been associated with FCD. At the Children's Hospital of Eastern Ontario, four patients with temporal lobe FCD have recently, been identified. This paper discusses how these children presented and how they were managed, with particular emphasis on their MRI findings and differential diagnoses. In three of the four patients neuroimaging studies showed lesions consistent with a neoplastic process because of the large volume and mass effect. Radiologically, FCD may mimic the MRI appearance of tumors, such as dysembryoplastic neuroepithelial tumors, primitive neuroectodermal tumors, gangliogliomas, oligodendrogliomas, and astrocytomas. These lesions are best visualized on fluid-attenuated inversion recovery (FLAIR) imaging, a technique that has recently become applicable in the clinical setting, as we help demonstrate in this series. With better MRI capability, milder forms of FCD and microdysplasia may be distinguished.
局灶性皮质发育不良(FCD)是神经元迁移障碍的一种形式,是大脑发育过程中发生的新皮质畸形性病变。它可导致部分性和全身性癫痫。癫痫发作在幼年时出现,且通常对药物治疗耐药。已发现手术切除对这些患者有益。内侧颞叶硬化形式的双重病理改变与FCD有关。在安大略东部儿童医院,最近已确诊4例颞叶FCD患者。本文讨论了这些患儿的临床表现及治疗方法,特别强调了他们的MRI表现和鉴别诊断。在这4例患者中的3例中,神经影像学研究显示病变与肿瘤性病变一致,这是由于病变体积大及占位效应所致。在放射学上,FCD可能类似于肿瘤的MRI表现,如胚胎发育不良性神经上皮肿瘤、原始神经外胚层肿瘤、神经节胶质瘤、少突胶质细胞瘤和星形细胞瘤。这些病变在液体衰减反转恢复(FLAIR)成像上显示最佳,正如我们在本系列研究中所展示的,这项技术最近已应用于临床。随着MRI能力的提高,可能会鉴别出更轻微形式的FCD和微小发育异常。