Presurgical evaluation and cognitive functional reorganization in Fishman syndrome.
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.
Donaire
Antonio
A
Hospital Clinic, Barcelona, Spain. ajdonaire@yahoo.com
Carreno
Mar
M
Bargalló
Nuria
N
Setoaín
Xavier
X
Agudo
Rolando
R
Martín
Gisela
G
Boget
Teresa
T
Raspall
Toni
T
Pintor
Luis
L
Rumiá
Jordi
J
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