Neurolymphomatosis

Abstract

We describe a patient with sensorimotor peripheral neuropathy and cranial neuropathy due to autopsy-proven neurolymphomatosis defined by infiltration of peripheral nerves by tumor cells and review the findings in 39 previously reported patients. The cause of the neuropathy is not known. The association with immune-deficient states suggests virally mediated pathogenesis, possibly a retrovirus.

High‐dose intravenous methotrexate followed by autologous stem cell transplantation as a potentially effective therapy for neurolymphomatosis

“…Because these patients may have comorbid conditions such as diabetes (and diabetic neuropathy) or chemotherapy-induced neuropathy, the EMG findings may be misleading. 16,23 The premortem diagnosis is confirmed by nerve biopsy. The first case diagnosed by nerve biopsy was in 1975 by Vital et al 24 Before that, all cases were diagnosed at autopsy.…”

“…To our knowledge, the first large series of NL was reported by Diaz-Arrastia et al, 16 who described 1 case and reviewed 39 other reported cases. Twentyeight patients (72%) presented with subacute progressive neuropathy.…”

“…In addition, approximately one-half of the cases had widespread systemic lymphoma found at the time of autopsy and 5% had central nervous system (CNS) involvement. 16,19 Most cases of NL presented with a subacute progressive sensorimotor polyneuropathy. However, other presentations include cranial neuropathy, mononeuropathy multiplex, and an isolated median or sciatic nerve palsy.…”

“…The first case diagnosed by nerve biopsy was in 1975 by Vital et al 24 Before that, all cases were diagnosed at autopsy. Approximately 83percent of nerve biopsies are reported to demonstrate lymphomatous infiltration, 16,19,25 whereas the rest may only demonstrate nonspecific findings. The most common pathologic finding is infiltration of lymphoma cells in all compartments of the nerve, especially in the endoneurium.…”

Increased Serum Thymidine Kinase Activity in Acute Sarcoidosis.

“…We considered sarcoidosis as the most plausible diagnosis, but could not initially rule out ML,because MLcan also present all of the above findings; Fever and lymphadenopathy are the most common signs of ML.MLcan affect the salivary glands, especially in patients with Sjogren's syndrome (8) and it has been reported that MLhas involved bilateral parotid glands (8,9). MLcan present a variety of neurological signs (10). Consequently, we measured serum ACE,sIL2R and TK, the markers of sarcoidosis and/or ML.…”