An Atypical Case of Neurolymphomatosis

The diagnosis of neurolymphomatosis can be highly challenging requiring a high index of suspicion. This paper discusses a clinical case and workup for secondary neurolymphomatosis. A patient with history of diffuse large B-cell lymphoma presented as an acute, ascending, progressive neuropathy mimicking acute inflammatory demyelinating polyradiculopathy with subtle differences. No response to intravenous immunoglobulins was seen though he had transient improvement after concurrent plasmapheresis and oral steroids. Extensive work-up eventually lead to a diagnosis of neurolymphomatosis by sural nerve biopsy. The patient was sent to hospice per family preference prior to initiation of the typical current treatment of chemotherapeutic agents. Neurolymphomatosis can present as a rapidly progressive neuropathy that can be diagnostically challenging, however, data is limited regarding treatment protocols. Multiple case reports suggest encouraging results from chemotherapy combinations which include high-dose methotrexate.