Atypical neuroimaging manifestations of linear scleroderma “en coup de sabre”

Andrew M. Allmendingerdo, Joseph A. Ricci, Naman S. Desai, Narayan Viswanadhan, Diana Rodriguez

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)62-68
Number of pages7
JournalIranian Journal of Child Neurology
Volume9
Issue number3
StatePublished - Aug 23 2015

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology

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