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Large Epileptogenic Type IIIb Dysplasia: A Radiological and Anatomopathological Challenge.
World Neurosurg 2019; 129:330-333WN

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%-87% of Engel Ia at 5-year follow-up.

CASE DESCRIPTION

A 4-year-old boy presented with a 1-year history of severe, invalidating, drug-resistant epilepsy. Imaging workup 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.). Postoperative magnetic resonance imaging assessed uncomplicated near-total resection. Histopathological analysis showed combined features of a DNET of nonspecific type and a focal cortical dysplasia.

CONCLUSION

We describe a rare condition of type IIIb dysplasia combining a focal cortical dysplasia with a DNET. Preoperative diagnosis of the lesion was of utmost difficultly, thereby rendering mandatory a thorough histopathological examination of resected specimen in the vast majority of cases. Increased recognition of the condition brings up the hypothesis of a genetic continuum or linkage between the 2 conditions. Functional results on seizure activity after ablative surgery are good and maximal safe resection should be the goal.

Authors+Show Affiliations

Department of Neurosurgery - Cliniques Universitaires St-Luc, Brussels, Belgium.Department of Neurosurgery - Cliniques Universitaires St-Luc, Brussels, Belgium.Department of Pathology - Cliniques Universitaires St-Luc, Brussels, Belgium.Department of Neuroradiology - Cliniques Universitaires St-Luc, Brussels, Belgium.Department of Neurosurgery - Cliniques Universitaires St-Luc, Brussels, Belgium. Electronic address: christian.raftopoulos@uclouvain.be.

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, vol. 129, 2019, pp. 330-333.
Joris V, Ribeiro Vaz JG, Lelotte J, et al. Large Epileptogenic Type IIIb Dysplasia: A Radiological and Anatomopathological Challenge. World Neurosurg. 2019;129:330-333.
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, 129, pp. 330-333. 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;129:330-333. 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/pubmed PY - 2019/6/25/medline PY - 2019/6/25/entrez KW - Differential diagnosis KW - Drug-resistant epilepsy KW - IIIb dysplasia SP - 330 EP - 333 JF - World neurosurgery JO - World Neurosurg VL - 129 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%-87% of Engel Ia at 5-year follow-up. CASE DESCRIPTION: A 4-year-old boy presented with a 1-year history of severe, invalidating, drug-resistant epilepsy. Imaging workup 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.). Postoperative magnetic resonance imaging assessed uncomplicated near-total resection. Histopathological analysis showed combined features of a DNET of nonspecific type and a focal cortical dysplasia. CONCLUSION: We describe a rare condition of type IIIb dysplasia combining a focal cortical dysplasia with a DNET. Preoperative diagnosis of the lesion was of utmost difficultly, thereby rendering mandatory a thorough histopathological examination of resected specimen in the vast majority of cases. Increased recognition of the condition brings up the hypothesis of a genetic continuum or linkage between the 2 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 -