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Focal cortical dysplasia - review.

Kabat J, Król P - Pol J Radiol (2012)

Bottom Line: A complete resection of the epileptogenic zone is required for seizure-free life.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Diagnostic Imaging, Mazowiecki Regional Hospital in Siedlce, Siedlce, Poland.

ABSTRACT
Focal 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.

No MeSH data available.


Related in: MedlinePlus

Focal cortical dysplasia type Ia with ipsilateral hippocampal sclerosis (“dual pathology”) in a 31-year-old female by Blumcke IIIa. Coronal MR images: turbo spin-echo inversion-recovery T1-weighted (A,D), turbo spin-echo T2-weighted (B,E), turbo spin-echo FLAIR T2-weighted (C,F) obtained respectively at the level of the temporal pole and the head of the hippocampus. Hypoplasia of the right temporal pole is recognizable, with volume loss of the white matter which leads to mild hyperintensity on T2-weighted images. Mild blurring of the cortical-white matter junction is visible on both T1- and T2-weighted images (A–C). The right hippocampus is atrophic, with decreased signal on IR-T1W images and increased signal on T2W images, consistent with hippocampal sclerosis. Courtesy of dr Norico Salamon. Epileptic Disorders, 2009; 11, 194–205.
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f1-poljradiol-77-2-35: Focal cortical dysplasia type Ia with ipsilateral hippocampal sclerosis (“dual pathology”) in a 31-year-old female by Blumcke IIIa. Coronal MR images: turbo spin-echo inversion-recovery T1-weighted (A,D), turbo spin-echo T2-weighted (B,E), turbo spin-echo FLAIR T2-weighted (C,F) obtained respectively at the level of the temporal pole and the head of the hippocampus. Hypoplasia of the right temporal pole is recognizable, with volume loss of the white matter which leads to mild hyperintensity on T2-weighted images. Mild blurring of the cortical-white matter junction is visible on both T1- and T2-weighted images (A–C). The right hippocampus is atrophic, with decreased signal on IR-T1W images and increased signal on T2W images, consistent with hippocampal sclerosis. Courtesy of dr Norico Salamon. Epileptic Disorders, 2009; 11, 194–205.

Mentions: – FCD type I – significant segmental or lobar hypoplasia/atrophy often coexistent with reduced volume of subcortical white matter, which may reveal moderately increased signal on T2-weighted images, significantly increased on FLAIR and decreased on T1, 3D volume GE T1, and significantly decreased on T1 IR. Slight blurring of GM/WM junction and abnormal sulcal and gyral pattern may be observed as well. Focal cortical dysplasia type I is frequently found in the temporal lobe with coexistent hippocampal atrophy according to Palmini et al. (2005), while Blumcke et al. (2011) distinguished a new type – IIIa (Figure 1).


Focal cortical dysplasia - review.

Kabat J, Król P - Pol J Radiol (2012)

Focal cortical dysplasia type Ia with ipsilateral hippocampal sclerosis (“dual pathology”) in a 31-year-old female by Blumcke IIIa. Coronal MR images: turbo spin-echo inversion-recovery T1-weighted (A,D), turbo spin-echo T2-weighted (B,E), turbo spin-echo FLAIR T2-weighted (C,F) obtained respectively at the level of the temporal pole and the head of the hippocampus. Hypoplasia of the right temporal pole is recognizable, with volume loss of the white matter which leads to mild hyperintensity on T2-weighted images. Mild blurring of the cortical-white matter junction is visible on both T1- and T2-weighted images (A–C). The right hippocampus is atrophic, with decreased signal on IR-T1W images and increased signal on T2W images, consistent with hippocampal sclerosis. Courtesy of dr Norico Salamon. Epileptic Disorders, 2009; 11, 194–205.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3403799&req=5

f1-poljradiol-77-2-35: Focal cortical dysplasia type Ia with ipsilateral hippocampal sclerosis (“dual pathology”) in a 31-year-old female by Blumcke IIIa. Coronal MR images: turbo spin-echo inversion-recovery T1-weighted (A,D), turbo spin-echo T2-weighted (B,E), turbo spin-echo FLAIR T2-weighted (C,F) obtained respectively at the level of the temporal pole and the head of the hippocampus. Hypoplasia of the right temporal pole is recognizable, with volume loss of the white matter which leads to mild hyperintensity on T2-weighted images. Mild blurring of the cortical-white matter junction is visible on both T1- and T2-weighted images (A–C). The right hippocampus is atrophic, with decreased signal on IR-T1W images and increased signal on T2W images, consistent with hippocampal sclerosis. Courtesy of dr Norico Salamon. Epileptic Disorders, 2009; 11, 194–205.
Mentions: – FCD type I – significant segmental or lobar hypoplasia/atrophy often coexistent with reduced volume of subcortical white matter, which may reveal moderately increased signal on T2-weighted images, significantly increased on FLAIR and decreased on T1, 3D volume GE T1, and significantly decreased on T1 IR. Slight blurring of GM/WM junction and abnormal sulcal and gyral pattern may be observed as well. Focal cortical dysplasia type I is frequently found in the temporal lobe with coexistent hippocampal atrophy according to Palmini et al. (2005), while Blumcke et al. (2011) distinguished a new type – IIIa (Figure 1).

Bottom Line: A complete resection of the epileptogenic zone is required for seizure-free life.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Diagnostic Imaging, Mazowiecki Regional Hospital in Siedlce, Siedlce, Poland.

ABSTRACT
Focal 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.

No MeSH data available.


Related in: MedlinePlus