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Primary angiitis of the central nervous system associated with Hodgkin's disease.
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1996
Year
Primary AngiitisVasculitisSclerodermaMedicineSurgical PathologyPathologyNeurologyCentral Nervous SystemNodular Sclerosing-type HodgkinNeuropathologyNeuroimmunologyPathologic Lesion
An unusual case of primary angiitis of the central nervous system associated with Hodgkin's disease in a 55-year-old-man is reported. After a 10-month history of acute transverse myelitis, the patient was diagnosed as having nodular sclerosing-type Hodgkin's disease involving the retroperitoneal lymph nodes. The patient died of a brainstem hemorrhage 1 week after a 15-day course of chemotherapy. Primary angiitis was documented on autopsy examination. To our knowledge, only nine similar cases have been reported in the literature, and none of them was associated with a sole initial spinal cord presentation. Owing to the rarity of this disease entity, a high index of suspicion and awareness of the association between primary angiitis and Hodgkin's disease are essential for early diagnosis.