[Nervous system and lymphomatoid granulomatosis. Clinical, pathological and nosological considerations (author's...

This paper describes the first French case of lymphomatoid granulomatosis (LYG) in 46-year-old male. Autopsy revealed bilateral pulmonary involvement and multiple central nervous system (CNS) localizations. The originality of this study is due to 1st the scarcity of reported cases of LYG in Europe 2nd a detailed neuropathological study permitting diagnosis of multifocal central nervous involvement by LYG. In the literature only 12 previous cases of LYG have histologically proven CNS lesions. These lesions correspond to; 8 instances of LYG, one progressive multifocal leukencephalopathy,, one post-therapeutic disseminated necrotizing leukoencephalopathy, one immunoblastic sarcoma and one case of LYG associated with histiocytic malignant lymphoma. This paper also evokes clinical, histological, ultrastructural and prognostic characteristics of LYG gathered from the available literature. From a nosological point of view the relationship of LYG with Wegener granulomatosis, polymorphic reticulosis and certain lymphomas is still, to this day, not clearly established.