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Large epileptogenic type IIIb dysplasia: a radiological and anatomopathological challenge.

Abstract

BACKGROUND

Type IIIb dysplasia is a subtype of focal cortical dysplasia associated with a tumor, most frequently with gangliogliomas then with dysembryoplastic neuroepithelial tumors (DNETs). Their preoperative diagnosis often remains equivocal since specific features are missing. The functional results (i.e. seizure free) is good with 81 to 87% of Engel Ia at 5-year follow-up.

CASE DESCRIPTION

A 4-year-old boy presented with a one-year history of severe, invalidating, drug-resistant epilepsy. Imaging work-up demonstrated a huge left limbic lesion of which diagnosis remained speculative. Because of worsening neurological status, resective surgery was recommended after multidisciplinary discussion. The resection was performed through left transtemporal approach under neuronavigation (C.R.). Post-operative MRI assessed uncomplicated near-total resection. Histopathological analysis showed combined features of a DNET of non-specific type and a focal cortical dysplasia.

CONCLUSION

We describe a rare condition of type IIIb dysplasia combining a focal cortical dysplasia with a DNET. Pre-operative diagnosis of such lesion is utmost difficult, thereby rendering mandatory a thorough histopathological examination of resected specimens in the vast majority of cases. Increased recognition of the condition arises the hypothesis of a genetic continuum or linkage between the two conditions. Functional results on seizure activity after ablative surgery are good and maximal safe resection should be the goal.

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  • Authors+Show Affiliations

    ,

    Department of neurosurgery- Cliniques Universitaires St-Luc, avenue Hippocrate, 10 - 1200 Brussels - BELGIUM.

    ,

    Department of neurosurgery- Cliniques Universitaires St-Luc, avenue Hippocrate, 10 - 1200 Brussels - BELGIUM.

    ,

    Department of pathology - Cliniques Universitaires St-Luc, avenue Hippocrate, 10 - 1200 Brussels - BELGIUM.

    ,

    Department of neuroradiology- Cliniques Universitaires St-Luc, avenue Hippocrate, 10 - 1200 Brussels - BELGIUM.

    Department of neurosurgery- Cliniques Universitaires St-Luc, avenue Hippocrate, 10 - 1200 Brussels - BELGIUM.

    Source

    World neurosurgery : 2019 Jun 21 pg

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    31233930

    Citation

    Joris, Vincent, et al. "Large Epileptogenic Type IIIb Dysplasia: a Radiological and Anatomopathological Challenge." World Neurosurgery, 2019.
    Joris V, Ribeiro Vaz JG, Lelotte J, et al. Large epileptogenic type IIIb dysplasia: a radiological and anatomopathological challenge. World Neurosurg. 2019.
    Joris, V., Ribeiro Vaz, J. G., Lelotte, J., Duprez, T., & Raftopoulos, C. (2019). Large epileptogenic type IIIb dysplasia: a radiological and anatomopathological challenge. World Neurosurgery, doi:10.1016/j.wneu.2019.06.106.
    Joris V, et al. Large Epileptogenic Type IIIb Dysplasia: a Radiological and Anatomopathological Challenge. World Neurosurg. 2019 Jun 21; PubMed PMID: 31233930.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Large epileptogenic type IIIb dysplasia: a radiological and anatomopathological challenge. AU - Joris,Vincent, AU - Ribeiro Vaz,Jose-Geraldo, AU - Lelotte,Julie, AU - Duprez,Thierry, AU - Raftopoulos,Christian, Y1 - 2019/06/21/ PY - 2019/04/15/received PY - 2019/06/12/revised PY - 2019/06/13/accepted PY - 2019/6/25/entrez PY - 2019/6/25/pubmed PY - 2019/6/25/medline KW - IIIb dysplasia KW - differential diagnosis KW - drug-resistant epilepsy JF - World neurosurgery JO - World Neurosurg N2 - BACKGROUND: Type IIIb dysplasia is a subtype of focal cortical dysplasia associated with a tumor, most frequently with gangliogliomas then with dysembryoplastic neuroepithelial tumors (DNETs). Their preoperative diagnosis often remains equivocal since specific features are missing. The functional results (i.e. seizure free) is good with 81 to 87% of Engel Ia at 5-year follow-up. CASE DESCRIPTION: A 4-year-old boy presented with a one-year history of severe, invalidating, drug-resistant epilepsy. Imaging work-up demonstrated a huge left limbic lesion of which diagnosis remained speculative. Because of worsening neurological status, resective surgery was recommended after multidisciplinary discussion. The resection was performed through left transtemporal approach under neuronavigation (C.R.). Post-operative MRI assessed uncomplicated near-total resection. Histopathological analysis showed combined features of a DNET of non-specific type and a focal cortical dysplasia. CONCLUSION: We describe a rare condition of type IIIb dysplasia combining a focal cortical dysplasia with a DNET. Pre-operative diagnosis of such lesion is utmost difficult, thereby rendering mandatory a thorough histopathological examination of resected specimens in the vast majority of cases. Increased recognition of the condition arises the hypothesis of a genetic continuum or linkage between the two conditions. Functional results on seizure activity after ablative surgery are good and maximal safe resection should be the goal. SN - 1878-8769 UR - https://www.unboundmedicine.com/medline/citation/31233930/Large_epileptogenic_type_IIIb_dysplasia:_a_radiological_and_anatomopathological_challenge L2 - https://linkinghub.elsevier.com/retrieve/pii/S1878-8750(19)31658-4 DB - PRIME DP - Unbound Medicine ER -