Hemorrhagic encephalitis as a primary manifestation of lymphomatoid granulomatosis: case report

Lymphomatoid granulomatosis is an angiocentric, angiodestructive lymphoproliferative disorder that affects multiple organs, but is most common in the lungs. It is pathologically characterised as being associated with a proliferation of B lymphocytes, related to the Epstein-Barr virus. Lymphomatoid granulomatosis affects the central nervous system secondarily in 25-30% of cases, but lymphomatoid granulomatosis involving primarily and only the CNS is extremely rare, with only 49 cases reported in the literature. We report the case of a 21-year-old boy with isolated primary lymphomatoid granulomatosis of the central nervous system, who presented with a clinical manifestation of his pathology; his diagnostic approach was made by diagnostic imaging and confirmed by biopsy.