Human neurolymphomatosis

Abstract

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.