Mh. Chung et al., INTRACEREBRAL INVOLVEMENT IN SCLERODERMA EN COUP DE SABRE - REPORT OFA CASE WITH NEUROPATHOLOGIC FINDINGS, Annals of neurology, 37(5), 1995, pp. 679-681
Linear scleroderma en coup de sabre (LScs) is a rare disorder not infr
equently associated with neurologic symptoms, notably epilepsy. Howeve
r, histopathologic documentation of intracerebral lesions in LScs is v
ery limited and the etiology of the central nervous system symptoms ha
s therefore never been convincingly established. We describe a 27-year
-old woman with LScs and a longstanding history of epilepsy. Radiograp
hic studies demonstrated a focal, intraparenchymal lesion in the left
frontal lobe directly subjacent to the area of scleroderma on the fore
head and scalp. The resected cerebral lesion revealed localized band-l
ike sclerosis of the leptomeninges and associated vessels, as well as
intraparenchymal calcifications and anomalous, ectatic vessels. These
findings suggest that LScs may represent a neurocutaneous syndrome of
vascular dysplasia similar to the Sturge-Weber syndrome, rather than a
localized form of collagen vascular disease, as suggested by some.