Journal
PEDIATRIC RADIOLOGY
Volume 37, Issue 11, Pages 1166-1170Publisher
SPRINGER
DOI: 10.1007/s00247-007-0592-0
Keywords
Revesz syndrome; dyskeratosis congenita; aplastic anemia; child
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Revesz syndrome is a variant of dyskeratosis congenita characterized by aplastic anemia, retinopathy, and central nervous system abnormalities. We describe a 3-year-old boy in whom the spectrum of neuroimaging findings, including intracranial calcifications, cerebellar hypoplasia and unusual brain lesions were found by biopsy to be gliosis despite their enhancement and progression. In patients with dyskeratosis-related syndromes, non-neoplastic parenchymal brain lesions occur and gliosis should be considered in the differential diagnosis for progressive enhancing brain lesions. Should this finding be confirmed consistently in additional cases, brain biopsy could potentially be avoided.
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