title: Hemispherectomy Procedure in Proteus Syndrome.
abstract: Objective Proteus syndrome is a rare overgrowth disorder including bone, soft tissue, and skin. Central nervous system manifestations were reported in about 40% of the patients including hemimegalencephaly and the resultant hemicranial hyperplasia, convulsions and mental deficiency. We report a 1-month-old male baby referred to Pediatric Neurology Clinic Soetomo Hospital, Surabaya, Indonesia in 2014 presented recurrent seizures since birth with asymmetric dysmorphic face with the right side larger than the left, subcutaneous mass and linear nevi. Craniocervical MRI revealed hemimegalencephaly right cerebral hemisphere. Triple antiepileptic drugs were already given as well as the ketogenic diet, but the seizures persisted. The seizure then was resolved after hemispherectomy procedure.
authors.affiliation: Airlangga University College of Medicine, Jl. Prof DrMoestopo, 6-8 Surabaya, Indonesia.
authors.forename: PrastiyaIndra
authors.initials: P
authors.lastname: Gunawan
authors.affiliation: Airlangga University College of Medicine, Jl. Prof DrMoestopo, 6-8 Surabaya, Indonesia.
authors.forename: Lusiana
authors.initials: L
authors.lastname: Lusiana
authors.affiliation: Airlangga University College of Medicine, Jl. Prof DrMoestopo, 6-8 Surabaya, Indonesia.
authors.forename: Darto
authors.initials: D
authors.lastname: Saharso
keywords: Hemimegalencephaly; Hemispherectomy; Intractable epilepsy; Linear nevi; Proteus syndrome