Myoclonic epilepsy with photosensitivity in infants with Pallister-Killian Syndrome.


Journal

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
ISSN: 1532-2130
Titre abrégé: Eur J Paediatr Neurol
Pays: England
ID NLM: 9715169

Informations de publication

Date de publication:
Jul 2019
Historique:
received: 02 04 2019
revised: 15 05 2019
accepted: 20 05 2019
pubmed: 11 6 2019
medline: 19 11 2019
entrez: 11 6 2019
Statut: ppublish

Résumé

Pallister-Killian Syndrome (PKS) (OMIM #601803) is a rare genetic disorder caused by a mosaic tetrasomy of the short arm of chromosome 12. Epilepsy is a frequent concern in PKS patients. we report 3 PKS patients, with early-onset myoclonic epilepsy and photosensitivity. In these children, we analysed epileptic history and the EEG phenotype. Epilepsy onset was in the first 2 years of life in all patients and in 2 of them myoclonic seizures were the only seizure type. In all children photosensitivity was observed and myoclonic seizures were mainly related to low-frequency (1-6 Hz) intermittent photic stimulation. Levetiracetam was effective and well tolerated in the 2 treated patients. early-onset myoclonic epilepsy is a possible clinical manifestation of PKS. Low-frequency photosensitivity is a peculiar bioelectrical marker in these children.

Identifiants

pubmed: 31178275
pii: S1090-3798(19)30132-1
doi: 10.1016/j.ejpn.2019.05.012
pii:
doi:

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

653-656

Informations de copyright

Copyright © 2019 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Auteurs

Emilia Ricci (E)

Child Neurology and Psychiatry Unit, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Bologna, Italy; Epilepsy Center, Health Sciences Department, Università degli Studi di Milano, San Paolo Hospital, Milan, Italy. Electronic address: emilia.ricci2@unibo.it.

Rocco Bonfatti (R)

Child Neurology and Psychiatry Unit, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: rocco.bonfatti@studio.unibo.it.

Alessandro Rocca (A)

Neonatology Unit, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: alessandro.rocca@studio.unibo.it.

Giacomo Sperti (G)

Neonatology Unit, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: giacomo.sperti@studio.unibo.it.

Valeria Cagnazzo (V)

Neonatology Unit, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: valeria.cagnazzo@studio.unibo.it.

Aglaia Vignoli (A)

Epilepsy Center, Health Sciences Department, Università degli Studi di Milano, San Paolo Hospital, Milan, Italy. Electronic address: aglaia.vignoli@ao-sanpaolo.it.

Guido Cocchi (G)

Neonatology Unit, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: guido.cocchi@unibo.it.

Duccio Maria Cordelli (DM)

Child Neurology and Psychiatry Unit, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Bologna, Italy. Electronic address: ducciomaria.cordelli@unibo.it.

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