title: Proteus syndrome associated with hemimegalencephaly and Ohtahara syndrome: report of two cases.
abstract: The authors report two cases of Brazilian children with most of the common syndromic features of Proteus syndrome, such as asymmetric overgrowth of tissues, skin abnormalities, hypotonia and mental retardation. In both patients, a refractory epilepsy, compatible with Ohtahara syndrome, as well as hemimegalencephaly, with asymmetric distribution of facial fat, were also diagnosed.
authors: Child Neurology, Hospital de Clínicas de Porto Alegre (HCPA), Universidade Federal do Rio Grande do Sul (UFRGS), Rua Ramiro Barcelos, 2350, CEP 90035-003, Porto Alegre, Brazil. halisson.bastos@gmail.com
authors: Halisson
authors: H
authors: Bastos
authors: Paula Fabiana Sobral
authors: PF
authors: da Silva
authors: Marco Antônio Veloso
authors: MA
authors: de Albuquerque
authors: Adriana
authors: A
authors: Mattos
authors: Rudimar Santos
authors: RS
authors: Riesgo
authors: Lygia
authors: L
authors: Ohlweiler
authors: Maria Isabel Bragatti
authors: MI
authors: Winckler
authors: José Augusto
authors: JA
authors: Bragatti
authors: Rodrigo Dias
authors: RD
authors: Duarte
authors: Denise Isabel
authors: DI
authors: Zandoná
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use; D004569:Electroencephalography; D004830:Epilepsy, Tonic-Clonic / Q000150:complications* / Q000473:pathology; D005260:Female; D005654:Fundus Oculi; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D054220:Malformations of Cortical Development / Q000150:complications* / Q000473:pathology; D009421:Nervous System Malformations / Q000150:complications* / Q000473:pathology; D011379:Prognosis; D016715:Proteus Syndrome / Q000150:complications* / Q000473:pathology; D012640:Seizures / Q000188:drug therapy / Q000209:etiology; D013577:Syndrome
chemical_list: D000927:Anticonvulsants