Exclusive CNS involvement by lymphomatoid granulomatosis in a 12-year-old boy: A case report

BACKGROUND Lymphomatoid granulomatosis [LYG] is an angiocentric, angiodestr uctive disease at the higher grade end of the spectrum of angiocentric immu noproliferative lesions. It primarily involves the lungs, but it may also i nvolve several extrapulmonary sites including the central nervous system (C NS), skin, and kidneys. CASE DESCRIPTION Clinical presentation, radiology and treatment of LYG in a 12-year-old male child with multiple intracranial extraaxial lesions is de scribed. A 12-year-old boy presented with sudden onset of left focal motor seizures with associated history of headache and vomiting. Computerized tomographic scan of the brain suggested high-density, bilateral, parietal extraaxial le sions. On magnetic resonance imaging, the lesions were iso- to hyperintense on T1-weighted images and hyperintense on T2-weighted images. The lesions were excised in two stages and histopathological examination confirmed the diagnosis. CONCLUSION LYG seldom involves the CNS exclusively. The present case demons trates exclusive CNS involvement by LYG in a young boy. (C) 1999 by Elsevie r Science Inc.