Linear scleroderma en coup de sabre associated with epileptic seizures: a case report
Introduction. Linear scleroderma is a rare form of localized scleroderma that mostly affects pediatric population. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre (LScs). Al most one-fourth of children with LScs may develop extracutaneus manifestation. Nervous system in volve ment includes seizures, headache, focal neurologic deficits and neuropsychiatric symptoms. Case. 4-years-old previously healthy girl developed first episode of febrile seizures. On the next day skin lesion on left side of fore head has appeared. During the next six months skin lesion has progressed with the development of ivory colored sclerotic plague on the left fore head extending to medial angle of the eye and mouth as well as alopecia. She was diagnosed with linear scleroderma and received topical treatment. At the same time shehas experienced 2 non provoked fo cal seizures. EEG showed a single spike-wave pattern in the left frontal area and treatment with valproates was initiated. Physical examination showed normal neurological status and other systemic findings were un remarkable. Complete blood count, erythrocyte sedimentation rate and biochemical findings were normal. Rheumatoid factor, antinuclear antibodies and antibodies for chronic infections (cytomegalovirus, Epstein-Barr virus) were negative. Head CT showed no abnormalities. Head MRI showed ipsilateral T2 hyperintensities in periventricular region ... [...].