Linear scleroderma associated with progressive brain atrophy.
Linear scleroderma (LS) is characterized by scleroatrophic lesions affecting limbs and legs, unilaterally. Neurological involvement may be associated with ipsilateral facial and skull involvement in disorders referred to clinically as LS 'en coup de sabre', and Parry-Romberg syndrome. We report a child with LS presenting with a severe neurological disorder characterized by epilepsy, progressive mental deterioration and a rapid process of atrophy involving the ipsilateral cerebral hemisphere, but not associated with an overlying facial structure involvement. Functional brain studies showed a reduction in the diameter of the left internal carotid and of the left middle cerebral artery. Our observations suggest that neuroimaging studies should be considered in all patients with linear scleroderma, and such studies become necessary when neurological symptoms occur.
Grosso
Salvatore
S
Department of Pediatrics, Obstetrics and Reproductive Medicine, University of Siena, Viale M. Bracci - Le scotte, 53100 Siena, Italy.
Fioravanti
Antonella
A
Biasi
Giovanni
G
Conversano
Elvira
E
Marcolongo
Roberto
R
Morgese
Guido
G
Balestri
Paolo
P
D001927:Brain Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D002343:Carotid Artery, Internal / Q000473:pathology; D002533:Cerebral Angiography; D002648:Child; D003937:Diagnosis, Differential; D005150:Facial Hemiatrophy / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D020768:Middle Cerebral Artery / Q000473:pathology; D012594:Scleroderma, Localized / Q000150:complications* / Q000473:pathology / Q000503:physiopathology*; D015899:Tomography, Emission-Computed, Single-Photon; D014057:Tomography, X-Ray Computed