Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2023.420
Autopsy and Case Reports
Autopsy Case Report

Focal Cortical Dysplasia with hippocampal sclerosis

Devendra Jadav; Vaibhav Gupta; Sudeep Khera; Vikas Meshram

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Abstract

Focal Cortical Dysplasia (FCD) is a group of focal developmental malformations of the cerebral cortex cytoarchitecture. FCD usually manifests as medically intractable epilepsy, especially in young children. Live patients are diagnosed by radiological examination such as magnetic resonance imaging (MRI), fluorodeoxyglucose positron emission tomography (FDG PET), magnetoencephalography (MEG), diffusion-tensor imaging (DTI), and intracranial electroencephalogram (EEG). While some cases can be missed by radiological examination, they are usually diagnosed on the histopathological examination of the surgically removed specimens of medically intractable epilepsy patients. We report a case of a young girl with cerebral palsy, mental retardation, and seizure disorder who died in her sleep. The deceased was diagnosed with FCD type III with hippocampal sclerosis on histopathological examination at autopsy. H & E stain and NeuN immunohistochemistry neuronal cell marker were used to demonstrate the findings of FCD.

Keywords

Cortical Dysplasia- Focal Epilepsy Syndrome Histopathology, Immunohistochemistry, Forensic Pathology, Autopsy

References

1 Crino PB. Focal cortical dysplasia. Semin Neurol. 2015;35(3):201-8. http://dx.doi.org/10.1055/s-0035-1552617. PMid:26060899.

2 Pascual-Castroviejo I, Hernández-Moneo JL, Gutiérrez-Molina ML, et al. Focal cortical dysplasia. Clinical-radiological-pathological associations. Neurologia. 2012;27(8):472-80. PMid:22217526.

3 Shaker T, Bernier A, Carmant L. Focal cortical dysplasia in childhood epilepsy. Semin Pediatr Neurol. 2016;23(2):108-19. http://dx.doi.org/10.1016/j.spen.2016.06.007. PMid:27544467.

4 Fabera P, Krijtova H, Tomasek M, et al. Familial temporal lobe epilepsy due to focal cortical dysplasia type IIIa. Seizure. 2015;31:120-3. http://dx.doi.org/10.1016/j.seizure.2015.07.014. PMid:26362388.

5 Mischel PS, Nguyen LP, Vinters HV. Cerebral cortical dysplasia associated with pediatric epilepsy. Review of neuropathologic features and proposal for a grading system. J Neuropathol Exp Neurol. 1995;54(2):137-53. http://dx.doi.org/10.1097/00005072-199503000-00001. PMid:7876884.

6 Palmini A, Najm I, Avanzini G, et al. Terminology and classification of the cortical dysplasias. Neurology. 2004;62(6, Suppl 3):S2-8. http://dx.doi.org/10.1212/01.WNL.0000114507.30388.7E. PMid:15037671.

7 Blümcke I, Thom M, Aronica E, et al. The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission. Epilepsia. 2011;52(1):158-74. http://dx.doi.org/10.1111/j.1528-1167.2010.02777.x. PMid:21219302.

8 Marucci G, Farnedi A, Giulioni M. Reelin: a possible link between hippocampal sclerosis and cortical dyslamination in the setting of FCD type IIIa. Neurol Sci. 2012;33(6):1479-81. http://dx.doi.org/10.1007/s10072-011-0895-7. PMid:22203331.

9 Blumcke I, Spreafico R, Haaker G, et al. Histopathological findings in brain tissue obtained during epilepsy surgery. N Engl J Med. 2017;377(17):1648-56. http://dx.doi.org/10.1056/NEJMoa1703784. PMid:29069555.

10 Wang X, Deng D, Zhou C, et al. Focal cortical dysplasia type iii related medically refractory epilepsy: mri findings and potential predictors of surgery outcome. Diagnostics (Basel). 2021;11(12):2225. http://dx.doi.org/10.3390/diagnostics11122225. PMid:34943462.

11 Najm I, Lal D, Alonso Vanegas M, et al. The ILAE consensus classification of focal cortical dysplasia: an update proposed by an ad hoc task force of the ILAE diagnostic methods commission. Epilepsia. 2022;63(8):1899-919. http://dx.doi.org/10.1111/epi.17301. PMid:35706131.

12 Thom M. The autopsy in sudden unexpected adult death: epilepsy. Curr Diagn Pathol. 2007;13(5):389-400. http://dx.doi.org/10.1016/j.cdip.2007.05.004.

13 Kabat J, Król P. Focal cortical dysplasia: review. Pol J Radiol. 2012;77(2):35-43. http://dx.doi.org/10.12659/PJR.882968. PMid:22844307.

14 Giulioni M, Vornetti G, Marucci G. Letter to the Editor. Focal cortical dysplasia type IIIa and isolated hippocampal sclerosis. J Neurosurg. 2018;128(6):1898-9. http://dx.doi.org/10.3171/2017.8.JNS171954. PMid:29424649.

15 Thom M, Zhou J, Martinian L, Sisodiya S. Quantitative post-mortem study of the hippocampus in chronic epilepsy: seizures do not inevitably cause neuronal loss. Brain. 2005;128(Pt 6):1344-57. http://dx.doi.org/10.1093/brain/awh475. PMid:15758032.
 


Submitted date:
07/30/2022

Accepted date:
01/02/2023

Publication date:
01/20/2023

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