Ogniskowa dysplazja korowa – stan obecny wiedzy

Abstract

SummaryFocal cortical dysplasia is a malformation of cortical development, which is the most common cause of medically refractory epilepsy in the pediatric population and the second/third most common etiology of medically intractable seizures in adults.Both genetic and acquired factors are involved in the pathogenesis of cortical dysplasia. Numerous classifications of the complex structural abnormalities of focal cortical dysplasia have been proposed – from Taylor et al. in 1971 to the last modification of Palmini classification made by Blumcke in 2011. In general, three types of cortical dysplasia are recognized.Type I focal cortical dysplasia with mild symptomatic expression and late onset, is more often seen in adults, with changes present in the temporal lobe.Clinical symptoms are more severe in type II of cortical dysplasia usually seen in children. In this type, more extensive changes occur outside the temporal lobe with predilection for the frontal lobes.New type III is one of the above dysplasias with associated another principal lesion as hippocampal sclerosis, tumor, vascular malformation or acquired pathology during early life.Brain MRI imaging shows abnormalities in the majority of type II dysplasias and in only some of type I cortical dysplasias.The most common findings on MRI imaging include: focal cortical thickening or thinning, areas of focal brain atrophy, blurring of the gray-white junction, increased signal on T2- and FLAIR-weighted images in the gray and subcortical white matter often tapering toward the ventricle. On the basis of the MRI findings, it is possible to differentiate between type I and type II cortical dysplasia. A complete resection of the epileptogenic zone is required for seizure-free life. MRI imaging is very helpful to identify those patients who are likely to benefit from surgical treatment in a group of patients with drug-resistant epilepsy.However, in type I cortical dysplasia, MR imaging is often normal, and also in both types the lesion seen on MRI may be smaller than the seizure-generating region seen in the EEG. The abnormalities may also involve vital for life brain parts, where curative surgery will not be an option. Therefore, other diagnostic imaging techniques such as FDG PET, MEG, DTI and intra-cranial EEG are widely used to establish the diagnosis and to decide on management.With advances in both genetics and neuroimaging, we may develop a better understanding of patients with drug-resistant epilepsy, which will help us to provide more successful pharmacological and/or surgical treatment in the future.

Emerging Surgical Strategies of Intractable Frontal Lobe Epilepsy with Cortical Dysplasia in Terms of Extent of Resection

“…A total of 4 patients with definitive lesion in this study's series buttressed these results through excellent postoperative outcomes (Engel's class I for 3 patients, II for 1 patient). With regard to pathology, Kabat and Król 23). presented that the specific MRI feature for CD was more evident in type II than type I.…”

Malformations of cortical development: 3T magnetic resonance imaging features

“…FCD-Ⅱ is characterized by focal area of cortical thickening, marked blurring of gray and white matter junction, and in some cases signal change in adjacent white matter on T2-weighted and FLAIR images [17] . On the other hand, it is harder to identify FCD-IIa than FCD-Ⅱb, and FCD-IIa cannot be always detected on MRI [16] .…”

“…MRI examination of FCD-Ⅰ cases is usually normal [15] . Hippocampal atrophy is frequently coexistent with FCD-Ⅰ [17] . In some cases, prominent segmental or lobar hypoplastic/atrophic changes and decrease the volume of subcortical white matter (Figure 12), sulcal and gyral pattern abnormalities may be seen.…”

“…In some cases, prominent segmental or lobar hypoplastic/atrophic changes and decrease the volume of subcortical white matter (Figure 12), sulcal and gyral pattern abnormalities may be seen. The most common location of FCD-Ⅰ is the temporal lobe [1,17] . FCD-Ⅲ is divided into four subgroups; FCD-Ⅲa is in combination with hippocampal sclerosis.…”

“…FCD-Ⅲb is associated with adjacent glial or glioneuronal tumors (i.e., DNET, ganglioglioma). FCD-Ⅲc is characterized with cortical lamination abnormalities adjacent to vascular malformations, whereas FCD-Ⅲd is associated with any other acquired lesions in early life period (i.e., posttraumatic lesions, ischemic injury, encephalitis) [3,16,17] .…”