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  • Updated 02.25.2021
  • Released 01.22.2002
  • Expires For CME 02.25.2024

Neurolymphomatosis

Introduction

This article includes discussion of neurolymphomatosis, Marek disease, peripheral lymphomatosis, and reticulosis of nervous system. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Historical note and terminology

The term neurolymphomatosis is used to describe peripheral nervous system or spinal root disease resulting from infiltration by malignant lymphoma cells (14; 30). This phenomenon was originally recognized by Marek, who observed a neuropathy in 4 chickens and found demyelination and lymphomatous nerve infiltrates with sparing of internal organs in 1 autopsy (46). Now referred to as Marek disease, it is recognized as a T-cell lymphoma in fowl associated with a Herpes virus (42). Pappenheimer and colleagues attempted to reproduce this condition in experimental chickens in order to identify its etiology; they coined the term “neurolymphomatosis gallinarum” (53). Association with leukemia was described in a report of a young man who died 1 month after presenting with sensorimotor polyneuropathy, anemia, and splenomegaly; autopsy showed leukemic involvement of nerves, liver, kidney, and spleen (26). The term "peripheral lymphomatosis" was proposed by Lhermitte and Trelles for the case of 67-year-old woman with malignant neuropathy and lymphoid nerve infiltrates but no systemic or brain involvement (43).

A histologically similar case of multiple relapsing-progressive mononeuropathies was described by Garcin and colleagues (19). They report that an autopsy 7 months after onset demonstrated generalized nerve enlargement and diffuse lymphomatous infiltration of essentially all nerves, dural spaces, and central nervous system with sparing of internal organs. Radnai and Takacs Nagy described a 36-year-old woman with multiple mononeuropathies and lymphomatous involvement of the brain and peripheral nervous system but not the spinal cord, and of internal organs but not lymphoid organs (57).

Allison and Gordon used the term "reticulosis of nervous system" for the case of a 56-year-old woman with progressive sensorimotor peripheral and cranial neuropathies, characterized by lymphomatous nerve infiltrates (03). Barron and colleagues described a case associated with Hodgkin disease (07). Gherardi and colleagues presented 2 patients with T cell lymphomatous infiltrates (20). Diaz-Arrastia and colleagues presented a literature review dedicated to neurolymphomatosis (14). Vital and colleagues detected retrovirus-like particles consistent with HTLV-1 on electron microscopy of peripheral nerves with T-cell lymphomatous infiltrates (77). Abad and colleagues described 4 cases of neurolymphomatosis with malignant infiltration in Waldenstrom macroglobulinemia (01).

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