title: Longstanding epileptic encephalopathy and linear localized scleroderma: two distinct pathologic processes in an adolescent.
abstract: We report a 7-year-old boy who unexpectedly developed a multi-drug resistant epilepsy with negative neuroimaging results, followed by the insidious appearance of linear localized scleroderma involving the right leg. When the boy was 16 and severely affected by epileptic encephalopathy, we have evaluated this case for the first time: his localized scleroderma had reached the right buttock and positive anti-nuclear antibody was the only positive laboratory test. Methotrexate administered for 12 months was ineffective in improving both the organization of his electroencephalographic pattern and seizure control, though seemed to stabilize the progression of linear scleroderma. This report suggests that neurological abnormality and extracranial scleroderma might represent two own distinct processes in a same patient.
authors.affiliation: Center of Periodic Fevers, Department of Pediatric Sciences, Università Cattolica Sacro Cuore, Largo A. Gemelli no. 8, 00168, Rome, Italy. drigante@gmail.com
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.forename: Domenica
authors.initials: D
authors.lastname: Battaglia
authors.forename: Ilaria
authors.initials: I
authors.lastname: Contaldo
authors.forename: Ilaria
authors.initials: I
authors.lastname: La Torraca
authors.forename: Laura
authors.initials: L
authors.lastname: Avallone
authors.forename: Stefania
authors.initials: S
authors.lastname: Gaspari
authors.forename: Giulia
authors.initials: G
authors.lastname: Bersani
authors.forename: Achille
authors.initials: A
authors.lastname: Stabile
mesh_terms: D000293:Adolescent; D018501:Antirheumatic Agents / Q000627:therapeutic use; D002648:Child; D004351:Drug Resistance; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D012594:Scleroderma, Localized / Q000150:complications / Q000188:drug therapy
chemical_list: D018501:Antirheumatic Agents; D008727:Methotrexate