Neurolymphomatosis

The term neurolymphomatosis (NL) encompasses nerve infiltration by neurotropic neoplastic cells in the setting of an unknown or a known hematologic malignancy. It is a rare neurologic manifestation of non-Hodgkin lymphoma (NHL) and leukemia with a poorly defined incidence. The typical manifestations of NL are of a neuropathy that may affect peripheral nerves, nerve roots, plexus, or cranial nerves. The most common presentations include painful peripheral neuropathy or radiculopathy, cranial neuropathy, painless polyneuropathy and peripheral mononeuropathy or a mononeuropathy multiplex. Successful therapy is contingent upon the recognition of this unique neurological complication, yet the diagnosis is difficult and often elusive. Of all diagnostic tools, imaging studies are of greatest clinical utility. MRI yields abnormal findings in almost 80% of affected patients and FDG-PETappears to be a highly sensitive diagnostic method facilitating identification of NL. There is no known standard treatment for NL and therefore, optimal management is ill defined. Treatment of NL consists of either chemotherapy alone or combined with radiotherapy. An aggressive multimodality therapy can prevent neurological deterioration and is associated with a prolonged survival in a subset of patients.