atypical neuroimaging manifestations of linear scleroderma “en coup de sabre”
نویسندگان
چکیده
how to cite this article: allmendinger am, ricci ja, desai ns, viswanadhan n, rodriguez d. atypical neuroimaging manifestations of linear scleroderma “en coup de sabre”. iran j child neurol. summer 2015;9(3):62-68. abstract linear scleroderma “en coup de sabre” is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. patients often present with neurologic symptoms. on imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. infratentorial lesions and other lesions not closely associated with the overlying scalp abnormality, such as those found in the cerebellum, have been reported, but are extremely uncommon. we present a case of an 8-year-old boy with a left fronto-parietal “en coup de sabre” scalp lesion and describe the neuroimaging findings of a progressively enlarging left cerebellar lesion discovered incidentally on routine magnetic resonance imaging. interestingly, the patient had no neurologic symptoms given the size of the mass identified.
منابع مشابه
Atypical Neuroimaging Manifestations of Linear Scleroderma “en coup de sabre”
Linear scleroderma "en coup de sabre" is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. Patients often present with neurologic symptoms. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Infratentorial lesions and other lesions not closely associated with the overlying sca...
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متن کاملLinear scleroderma en coup de sabre with associated neurologic abnormalities.
Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scle...
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Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bon...
متن کاملA familial case of scleroderma en coup de sabre.
Sir, Linear scleroderma represents a rare disease, more often observed in the pediatric age group. The lower extremities are usually involved; less commonly it affects the frontoparietal area and the anterior scalp. Recently, a large case series of frontoparietal scleroderma en coup de sabre has been reported (1), but in this series, as well as in the English language literature, to the best of...
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عنوان ژورنال:
iranian journal of child neurologyجلد ۹، شماره ۳، صفحات ۶۹-۷۲
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