title: Presurgical evaluation and cognitive functional reorganization in Fishman syndrome.
abstract: Fishman syndrome, also known as encephalocraniocutaneous lipomatosis (ECCL), is a rare, congenital neurocutaneous syndrome characterized by unilateral skin, eye, and brain abnormalities. Epileptic seizures and developmental delay are usually present. We report the clinical, radiological, and, for the first time, neurophysiological findings in a 24-year-old woman diagnosed with ECCL who was evaluated for epilepsy surgery. Functional magnetic resonance imaging revealed transfer of memory and language functions to the nonaffected hemisphere, providing evidence that functional reorganization and restoration of cognitive functions may occur in the context of extensive malformations, such as neurocutaneous syndromes.
authors.affiliation: Hospital Clinic, Barcelona, Spain. ajdonaire@yahoo.com
authors.forename: Antonio
authors.initials: A
authors.lastname: Donaire
authors.forename: Mar
authors.initials: M
authors.lastname: Carreno
authors.forename: Nuria
authors.initials: N
authors.lastname: Bargalló
authors.forename: Xavier
authors.initials: X
authors.lastname: Setoaín
authors.forename: Rolando
authors.initials: R
authors.lastname: Agudo
authors.forename: Gisela
authors.initials: G
authors.lastname: Martín
authors.forename: Teresa
authors.initials: T
authors.lastname: Boget
authors.forename: Toni
authors.initials: T
authors.lastname: Raspall
authors.forename: Luis
authors.initials: L
authors.lastname: Pintor
authors.forename: Jordi
authors.initials: J
authors.lastname: Rumiá
mesh_terms: D000328:Adult; D001931:Brain Mapping; D002540:Cerebral Cortex / Q000098:blood supply / Q000473:pathology; D003071:Cognition / Q000502:physiology*; D004827:Epilepsy / Q000209:etiology; D005260:Female; D006801:Humans; D008068:Lipomatosis / Q000473:pathology / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging / Q000379:methods; D020752:Neurocutaneous Syndromes / Q000473:pathology / Q000503:physiopathology*; D009483:Neuropsychological Tests; D015835:Ocular Motility Disorders / Q000209:etiology