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Focal cortical dysplasia

Subtype of malformative brain lesion associated with medication-resistant epilepsy.


Focal cortical dysplasias (FCDs) are mainly located in the frontal region, with a particular tropism for the central sulcus. Up to 30% of lesions are undetected (magnetic resonance [MR]-negative FCD patients) or belatedly diagnosed by visual analysis of MR images.

Roca et al. identified abnormal sulcal patterns in patients with FCD of the central region compared with healthy controls. The abnormal sulcal patterns ipsilateral to the FCD and the link between sulcus energy and the FCD location strengthen the interest of sulcal abnormalities in FCD patients 1).


International League Against Epilepsy (ILAE) classifications of FCD.

Type II focal cortical dysplasia (FCD II) is a malformation of cortical development, frequently associated with intractable epilepsy, characterised by cortical dyslamination, dysmorphic neurons (DNs) and balloon cells (BCs).

Rossini et al., investigated the expression of pS6 (downstream target) and pPDK1-pAkt (upstream targets) as evidence for mTOR pathway activation and their co-expression with Interleukin-1β in FCD II surgical specimens and compared the findings with control non-epileptic tissue, non-malformed epileptic tissue or acquired epilepsy-Rasmussen's Encephalitis (RE) occasionally presenting pS6 and Interleukin-1β positive abnormal neurons. Downstream mTOR activation was demonstrated in almost all abnormal cells in both FCD II and RE. Conversely, upstream activation in FCD II was observed in the majority of BCs, in a proportion of DNs, not presenting Interleukin-1β expression, but not at all in RE scattered abnormal neurons. Based on these findings we suggest that the presence of BCs and DNs in FCD II could be due to a first upstream mTOR pathway PI3K-Akt-mediate event occurring very early during cortical development in the large proportion of abnormal cells; followed by the appearance of additional pS6 positive DNs promoted by the presence of a later inflammatory processes 2).



The most important factor in seizure freedom following surgery for focal cortical dysplasia (FCD) is completeness of resection. However, intraoperative detection of epileptogenic dysplastic cortical tissue remains a challenge, potentially leading to a partial resection and the need for reoperation.

iMRI-guided resection of FCD in pediatric patients precluded the need for repeat surgery. Furthermore, it resulted in the achievement of complete resection in all the patients, leading to a high rate of postoperative seizure freedom 3).

Case series


A study included 71 patients who had a presurgical evaluation workup performed due to drug resistant epilepsys, who underwent epilepsy surgery, and who were histopathologically diagnosed with focal cortical dysplasia (FCD). Relationships involving MRI and 18F positron emission tomography (FDG-PET) findings and clinical data from pathological subgroups and patients were assessed.

According to the International League Against Epilepsy (ILAE) classifications of FCD, 28 of the patients were type I and 43 were type II. FCD was visible on the MRI scans of 53 patients, and a majority of this group was classified as type II FCD (n=34). Of these 53 patients, FCD was located in the temporal area of 21 patients, the extratemporal area of 29 patients. Of the patients who exhibited FDG-PET hypometabolism (PET-positive), 23 were classified as temporal, 17 as frontal, 11 showed involvement of the posterior cortex. The age of seizure onset was younger in PET-positive patients (p=0.032), and histopathological analyses revealed that 23 patients had type I FCD and 30 patients had type II FCD.

PET scans reveal a lesion by showing hypometabolism in patients who have refractory epilepsy and an early age of onset with FCD. The lesions of MRI-negative/PET-positive FCD patients tend to be localized in the temporal lobe and that FCD may be localized in the frontal lobe of MRI-negative/PET-negative patients. However, the histopathological examinations of MRI-positive/PET-positive, MRI-negative/PET-positive, and MRI-negative/PET-negative patients did not exhibit a particular histopathological subtype 4).

Sacino et al retrospectively reviewed the medical records of pediatric patients who underwent Intraoperative magnetic resonance imaging-assisted resection of FCD at the Children's National Health System between January 2014 and April 2015. Data reviewed included demographics, length of surgery, details of iMRI acquisition, postoperative seizure freedom, and complications. Postsurgical seizure outcome was assessed utilizing the Engel Epilepsy Surgery Outcome Scale.

Twelve consecutive pediatric patients (8 females and 4 males) underwent iMRI-guided resection of FCD lesions. The mean age at the time of surgery was 8.8 years ± 1.6 years (range 0.7 to 18.8 years), and the mean duration of follow up was 3.5 months ± 1.0 month. The mean age at seizure onset was 2.8 years ± 1.0 year (range birth to 9.0 years). Two patients had Type 1 FCD, 5 patients had Type 2A FCD, 2 patients had Type 2B FCD, and 3 patients had FCD of undetermined classification. iMRI findings impacted intraoperative surgical decision making in 5 (42%) of the 12 patients, who then underwent further exploration of the resection cavity. At the time of the last postoperative follow-up, 11 (92%) of the 12 patients were seizure free (Engel Class I). No patients underwent reoperation following iMRI-guided surgery.

iMRI-guided resection of FCD in pediatric patients precluded the need for repeat surgery. Furthermore, it resulted in the achievement of complete resection in all the patients, leading to a high rate of postoperative seizure freedom 5).

Roca P, Mellerio C, Chassoux F, Rivière D, Cachia A, Charron S, Lion S, Mangin JF, Devaux B, Meder JF, Oppenheim C. Sulcus-Based MR Analysis of Focal Cortical Dysplasia Located in the Central Region. PLoS One. 2015 Mar 30;10(3):e0122252. doi: 10.1371/journal.pone.0122252. eCollection 2015. PubMed PMID: 25822985.
Rossini L, Villani F, Granata T, Tassi L, Tringali G, Cardinale F, Aronica E, Spreafico R, Garbelli R. FCD Type II and mTOR pathway: Evidence for different mechanisms involved in the pathogenesis of dysmorphic neurons. Epilepsy Res. 2016 Dec 7;129:146-156. doi: 10.1016/j.eplepsyres.2016.12.002. [Epub ahead of print] PubMed PMID: 28056425.
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Sacino MF, Ho CY, Murnick J, Tsuchida T, Magge SN, Keating RF, Gaillard WD, Oluigbo CO. Intraoperative MRI-guided resection of focal cortical dysplasia in pediatric patients: technique and outcomes. J Neurosurg Pediatr. 2016 Jun;17(6):672-8. doi: 10.3171/2015.10.PEDS15512. Epub 2016 Feb 26. PubMed PMID: 26919314.
Halac G, Delil S, Zafer D, Isler C, Uzan M, Comunoglu N, Oz B, Yeni SN, Vatankulu B, Halac M, Ozkara C. Compatibility of MRI and FDG-PET findings with histopathological results in patients with focal cortical dysplasia. Seizure. 2016 Dec 6;45:80-86. doi: 10.1016/j.seizure.2016.11.024. [Epub ahead of print] PubMed PMID: 27960132.
focal_cortical_dysplasia.txt · Last modified: 2017/05/17 10:24 by administrador