Neurologic manifestations of localized scleroderma: A case report and literature review

Ilya Kister, Matilde Inglese, Ronald M. Laxer, Joseph Herbert

Research output: Contribution to journalReview articlepeer-review

112 Scopus citations


We describe a young woman with localized scleroderma, seizures, numerous persistently enhancing white matter lesions on brain MRI, and oligoclonal bands in the CSF. The case is remarkable in the widespread bilateral distribution of the lesions and their enhancement during more than a year of follow-up despite immunosuppression. Literature search yielded 54 case descriptions of localized scleroderma associated with neurologic symptoms and neuroimaging findings. All patients had craniofacial scleroderma: linear scleroderma en coup de sabre (LScs), progressive facial hemiatrophy (PFH, or Parry-Romberg syndrome) or both. LScs and PFH should be viewed as variants of craniofacial localized scleroderma as they often manifest in the same patient, share the same neurologic manifestations and imaging features, and evidence pathologic inflammation in skin and CNS.

Original languageEnglish
Pages (from-to)1538-1545
Number of pages8
Issue number19
StatePublished - 4 Nov 2008
Externally publishedYes


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