Human neurolymphomatosis.
A patient with a chronically progressive fatal sensorimotor neuropathy showed, at autopsy, extensive and selective lymphocytic infiltration of peripheral and cranial nerves and a segmental demyelinative process. A clinically occult retroperitoneal lymphoma without spread to other systemic organs was also present. The possibility of a selective infiltration of the peripheral nervous system by the retroperitoneal malignancy is rejected as unlikely. Our case bears a strong similarity to 3 other cases previously reported which may be grouped together under the heading "human neurolymphomatosis". The clinical and pathological features of this rare entity are discussed. It bears a resemblance to Marek's disease of chickens and seems to represent an unusual inflammatory neuropathy or form of malignancy.
D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D008223:Lymphoma / Q000473:pathology*; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009420:Nervous System / Q000473:pathology*; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000473:pathology; D012186:Retroperitoneal Neoplasms / Q000473:pathology*; D013116:Spinal Cord / Q000473:pathology