Epilepsy in paediatric patients with Parry-Romberg syndrome: A review of the literature

Rocha, Ruben; Kaliakatsos, Marios

Publication

Epilepsy in paediatric patients with Parry-Romberg syndrome: A review of the literature

2020Journal article

dc.contributor.author	Rocha, Ruben
dc.contributor.author	Kaliakatsos, Marios
dc.date.accessioned	2022-07-12T14:00:57Z
dc.date.available	2022-07-12T14:00:57Z
dc.date.issued	2020
dc.description.abstract	Background: Parry-Romberg syndrome (PRS) is a rare disorder characterized by unilateral slow progressive facial atrophy that can be associated with neurologic manifestations, namely seizures. There is scarce data about seizures in paediatric patients with PRS. The aim of our work was to clarify the clinical features of paediatric patients with PRS and seizures. Methods: We performed a literature review based on a literature search using PubMed and EMBASE databases. We included original articles in which the main diagnosis was PRS and the patients were 17 years old or less when the first seizure occurred. Results: We included 40 patients. Most of the patients had previously normal development and had their first seizure in the first decade of life. Neurologic examination was abnormal in 56 % of patients. Seizures are typically focal, frequently with impaired awareness, and became refractory in about 40 % of patients. Few patients have generalized seizures. On electroencephalogram, epileptic discharges are generally focal, on the same side as the facial atrophy, without a predominant cerebral lobe localization. Brain MRI is almost always abnormal, typically with T2 subcortical hyperintensities, and sometimes brain atrophy or calcifications. In addition to the classic antiepileptic drugs, immunosuppressive drugs should be considered as potential epilepsy treatment. Conclusion: To the best of our knowledge, this is the first review dedicated to the characteristics of paediatric patients with PRS and epilepsy. Seizures are usually focal, became refractory in 40 %, and have a significant impact on the quality of life and neurodevelopment of patients.	pt_PT
dc.description.version	info:eu-repo/semantics/publishedVersion	pt_PT
dc.identifier.citation	Rocha R, Kaliakatsos M. Epilepsy in paediatric patients with Parry-Romberg syndrome: A review of the literature. Seizure. 2020;76:89-95. doi:10.1016/j.seizure.2020.01.017	pt_PT
dc.identifier.doi	10.1016/j.seizure.2020.01.017	pt_PT
dc.identifier.issn	1059-1311
dc.identifier.issn	1532-2688
dc.identifier.uri	http://hdl.handle.net/10400.16/2719
dc.language.iso	eng	pt_PT
dc.peerreviewed	yes	pt_PT
dc.publisher	Elsevier	pt_PT
dc.relation.publisherversion	https://www.seizure-journal.com/article/S1059-1311(20)30036-4/fulltext	pt_PT
dc.rights.uri	http://creativecommons.org/licenses/by-nc-nd/4.0/	pt_PT
dc.subject	Epilepsy	pt_PT
dc.subject	Paediatric	pt_PT
dc.subject	Parry Romberg syndrome	pt_PT
dc.subject	Progressive facial atrophy	pt_PT
dc.subject	Seizure	pt_PT
dc.title	Epilepsy in paediatric patients with Parry-Romberg syndrome: A review of the literature	pt_PT
dc.type	journal article
dspace.entity.type	Publication
oaire.citation.conferencePlace	England	pt_PT
oaire.citation.endPage	95	pt_PT
oaire.citation.startPage	89	pt_PT
oaire.citation.title	Seizure	pt_PT
oaire.citation.volume	76	pt_PT
person.familyName	Rocha
person.givenName	Ruben
person.identifier.ciencia-id	731B-8049-FA18
person.identifier.orcid	0000-0002-5523-538X
rcaap.rights	openAccess	pt_PT
rcaap.type	article	pt_PT
relation.isAuthorOfPublication	77fc7317-407a-47e6-950b-626398af2d3f
relation.isAuthorOfPublication.latestForDiscovery	77fc7317-407a-47e6-950b-626398af2d3f