Atypical Neuroimaging Manifestations of Linear Scleroderma “en coup de sabre”

Andrew M. ALLMENDINGER, Joseph A. RICCI, Naman S. DESAI, Narayan VISWANADHAN, Diana RODRIGUEZ

Abstract


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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.


Keywords


Linear Scleroderma “en coup de sabre”; Localized scleroderma; Morphea; Cerebellum; MRI

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References


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DOI: https://doi.org/10.22037/ijcn.v9i3.6897

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