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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 manifestation and imaging features, and evidence pathologic inflammation in skin and CNS. |
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cerebrospinal fluid,oligoclonal IgG in headache hemifacial atrophy MRI MRI,abnormal MRI,contrast enhanced multiple sclerosis,differential diagnosis of neurologic examination neurologic signs neurologic symptoms progressive hemifacial atrophy review article scleroderma scleroderma,neurologic involvement with seizure skin,lesions in neurologic disorders
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