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 Volume 71, Number 19, November 4, 2008
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NEUROLOGY 2008;71:1538-1545
© 2008 American Academy of Neurology
Views & Reviews

Neurologic manifestations of localized scleroderma

A case report and literature review Ilya Kister, MD, Matilde Inglese, MD, PhD, Ronald M. Laxer, MD and Joseph Herbert, MD

From the MS Care Center, Department of Neurology (I.K., J.H.), and Department of Radiology (M.I.), NYU School of Medicine, New York; and Department of Pediatrics and Medicine (R.M.L.), University of Toronto, Canada.

Address correspondence and reprint requests to Dr. Ilya Kister, NYU Hospital for Joint Diseases, 301 East 17th St., Suite 544, New York, NY 10003

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.

Abbreviations: ANA = antinuclear antibody; LScs = localized scleroderma en coup de sabre; MS = multiple sclerosis; PFH = progressive facial hemiatrophy.

ilya.kister{at}gmail.com

Disclosure: The authors report no disclosures.

Received April 10, 2008. Accepted in final form August 5, 2008.