Focal cortical dysplasia
Updates to Article Attributes
Focal cortical dysplasia is a disorders of cortical formation, which may demonstrate both architectural and proliferative features.
Clinical presentation
It's a frequent cause of refractory epilepsy.
Epidemiology
Age of presentation, especiallyusually with epilepsy depends to a degree on the type of cortical dysplasia, with type I (see below) more frequently presenting in childrenadulthood 4.
Classification
FocalA number of classification systems for focal cortical dysplasia can be divided into three main categories:have been devised over the years since first description in 1971 by Taylor et al 5.
The most common classification used until recently was the a histopathological system proposed by Pamini et al 6 in 2004 a genetic / imaging classification by Barkovich et al 2 in 2005.
The most recent classification system is that proposed by Blumcke in 2011 and has been widely accepted.
Unfortunately, as is the case with many classification systems that have developed in parallel with numerous iterations and revisions, there is significant overlap between the various classifications systems with the same terminology used slightly differently. As such it is safest to explicitly state which classification system is being used (e.g. "Blumcke Type IIB").
-
FCD type I(non-Taylor -
type Ia- dyslamination and mild malformation ofBarkovich classification of focal cortical
developmentdysplasia (2005) -
type Ib- isolated architectural abnormalities and cytoarchitectural dysplasia (without presence -
type Ic- combination of both Ia and Ib
-
type IIa- no balloon cells -
type IIb- balloon cells present
-
type IIIa- with hippocampal sclerosis -
type IIIb- with epilepsy associated tumours -
type IIIc- with vascular malformations
Radiographic features
MRI
MRI studies could be negative or show signs of:
- focal cortical thickening and abnormal gyration with or without T2/ FLAIR hyperintensity.
- blurring of cortex/white matter interface.
Differential diagnosis
Imaging differential considerations include:
- DNET
- ganglioglioma
- low grade glioma
- hamartoma of tuberous sclerosis
-<p><strong>Focal cortical dysplasia</strong> is a <a href="/articles/disorders-of-cortical-formation">disorders of cortical formation</a>, which may demonstrate both architectural and proliferative features.</p><h4>Clinical presentation</h4><p>It's a frequent cause of refractory epilepsy, especially in children. </p><h5>Classification</h5><p>Focal cortical dysplasia can be divided into three main categories: </p><ul>-<li>-<strong>FCD type I</strong> (<a href="/articles/non-taylor-dysplasia">non-Taylor dysplasia</a>)<ul>-<li>-<strong>type Ia</strong> - dyslamination and mild malformation of cortical development</li>-<li>-<strong>type Ib</strong> - isolated architectural abnormalities and cytoarchitectural dysplasia (without presence of dysmorphic neurons)</li>-<li>-<strong>type Ic</strong> - combination of both Ia and Ib</li>-</ul>-</li>-<li>-<strong>FCD type II</strong> (<a href="/articles/taylor-dysplasia">Taylor dysplasia</a>)- most common <ul>-<li>-<strong>type IIa</strong> - no balloon cells</li>-<li>-<strong>type IIb</strong> - balloon cells present</li>-</ul>-</li>-<li>-<strong>FCD type III</strong><ul>-<li>-<strong>type IIIa </strong>- with hippocampal sclerosis</li>-<li>-<strong>type IIIb </strong>- with epilepsy associated tumours</li>-<li>-<strong>type IIIc </strong>- with vascular malformations</li>-</ul>-</li>- +<p><strong>Focal cortical dysplasia</strong> is a <a href="/articles/disorders-of-cortical-formation">disorders of cortical formation</a>, which may demonstrate both architectural and proliferative features.</p><h4>Clinical presentation</h4><p>It's a frequent cause of refractory epilepsy. </p><h4>Epidemiology</h4><p>Age of presentation, usually with epilepsy depends to a degree on the type of cortical dysplasia, with type I (see below) more frequently presenting in adulthood <sup>4</sup>. </p><h4>Classification</h4><p>A number of classification systems for focal cortical dysplasia have been devised over the years since first description in 1971 by Taylor et al <sup>5</sup>. </p><p>The most common classification used until recently was the a histopathological system proposed by Pamini et al <sup>6</sup> in 2004 a genetic / imaging classification by Barkovich et al <sup>2</sup> in 2005.</p><p>The most recent classification system is that proposed by Blumcke in 2011 and has been widely accepted. </p><p>Unfortunately, as is the case with many classification systems that have developed in parallel with numerous iterations and revisions, there is significant overlap between the various classifications systems with the same terminology used slightly differently. As such it is safest to explicitly state which classification system is being used (e.g. "Blumcke Type IIB"). </p><ul>
- +<li><p><a href="/articles/palmini-classification-of-focal-cortical-dysplasia">Palmini </a><a href="/articles/barkovich-classification-of-focal-cortical-dysplasia">classification of focal cortical dysplasia</a> (2004)</p></li>
- +<li><p><a href="/articles/barkovich-classification-of-focal-cortical-dysplasia">Barkovich classification of focal cortical dysplasia</a> (2005)</p></li>
- +<li><p><a href="/articles/blumcke-classification-of-focal-cortical-dysplasia">Blumcke classification of focal cortical dysplasia</a> (2011)</p></li>
-</ul><h4><strong>Differential diagnosis</strong></h4><p>Imaging differential considerations include</p><ul>- +</ul><h4><strong>Differential diagnosis</strong></h4><p>Imaging differential considerations include:</p><ul>
- +<li>low grade glioma</li>
- +<li>hamartoma of <a href="/articles/tuberous-sclerosis">tuberous sclerosis</a>
- +</li>
References changed:
- 4. Kabat J & Król P. Focal Cortical Dysplasia - Review. Pol J Radiol. 2012;77(2):35-43. <a href="https://doi.org/10.12659/pjr.882968">doi:10.12659/pjr.882968</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/22844307">Pubmed</a>
- 5. Taylor D, Falconer M, Bruton C, Corsellis J. Focal Dysplasia of the Cerebral Cortex in Epilepsy. J Neurol Neurosurg Psychiatry. 1971;34(4):369-87. <a href="https://doi.org/10.1136/jnnp.34.4.369">doi:10.1136/jnnp.34.4.369</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/5096551">Pubmed</a>
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