Late onset childhood occipital epilepsy-Gastaut type (LOCOE) is a rare idiopathic epilepsy syndrome with an uncertain long-term prognosis. Elementary visual hallucinations and interictal spike-and-wave complexes in the occipital areas represent the main electroclinical findings of the syndrome. The functional nature of LOCOE has been emphasized together with the presence of genetic predisposition in the affected patients. Here, we report on two families in which two patients, respectively, showed electroclinical features compatible with LOCOE. Although further studies are needed to validate our observations, the involvement of two generations in one of the families we studied may corroborate the previously formulated hypothesis of an autosomal dominant model of inheritance in LOCOE. Of course, the identification of larger families is propaedeutic to linkage analysis studies.

Grosso, S., Vivarelli, R., Gobbi, G., DI BARTOLO, R.M., Berardi, R., & Balestri, P. (2008). Late-onset childhood occipital epilepsy(Gastaut type): a family study. EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY, 12(5), 421-426 [10.1016/j.ejpn.2007.11.007].

Late-onset childhood occipital epilepsy(Gastaut type): a family study

GROSSO, S.;DI BARTOLO, R. M.;BERARDI, R.;BALESTRI, P.
2008

Abstract

Late onset childhood occipital epilepsy-Gastaut type (LOCOE) is a rare idiopathic epilepsy syndrome with an uncertain long-term prognosis. Elementary visual hallucinations and interictal spike-and-wave complexes in the occipital areas represent the main electroclinical findings of the syndrome. The functional nature of LOCOE has been emphasized together with the presence of genetic predisposition in the affected patients. Here, we report on two families in which two patients, respectively, showed electroclinical features compatible with LOCOE. Although further studies are needed to validate our observations, the involvement of two generations in one of the families we studied may corroborate the previously formulated hypothesis of an autosomal dominant model of inheritance in LOCOE. Of course, the identification of larger families is propaedeutic to linkage analysis studies.
Grosso, S., Vivarelli, R., Gobbi, G., DI BARTOLO, R.M., Berardi, R., & Balestri, P. (2008). Late-onset childhood occipital epilepsy(Gastaut type): a family study. EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY, 12(5), 421-426 [10.1016/j.ejpn.2007.11.007].
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11365/20659
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