Rajat Lahoria1, P James B Dyck1, William R Macon2, Brian A Crum1, Robert J Spinner3, Kimberly K Amrami4, Steven R Zeldenrust5, Jennifer A Tracy1. 1. Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Rochester, Minnesota, 55905, USA. 2. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. 3. Department of Neurosurgery, Mayo Clinic, Rochester, Minnesota, USA. 4. Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA. 5. Department of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Abstract
INTRODUCTION: Neurolymphomatosis (NL) is a rare disorder characterized by invasion of cranial or peripheral nerves, nerve roots, or plexi, usually by aggressive subtypes of non-Hodgkin lymphoma (NHL). The most common clinical presentation is that of a painful polyneuropathy or polyradiculopathy, followed by cranial neuropathy and, less frequently, by painless polyneuropathy. METHODS: Clinical and pathologic findings are reported for 2 NL cases. RESULTS: The following 2 patients with NL, with disparate clinical presentations, are presented: a patient with subacute onset, painful, multifocal, mixed axonal and demyelinating radiculoplexus neuropathy due to a large B-cell NHL, who required 2 targeted fascicular nerve biopsies to demonstrate NL; and a patient with a slowly progressive, length-dependent axonal polyneuropathy due to a low-grade B-cell lymphoproliferative disorder, as shown on a diagnostic sural nerve biopsy. CONCLUSIONS: The cases described illustrate the wide clinical spectrum of NL.
INTRODUCTION:Neurolymphomatosis (NL) is a rare disorder characterized by invasion of cranial or peripheral nerves, nerve roots, or plexi, usually by aggressive subtypes of non-Hodgkin lymphoma (NHL). The most common clinical presentation is that of a painful polyneuropathy or polyradiculopathy, followed by cranial neuropathy and, less frequently, by painless polyneuropathy. METHODS: Clinical and pathologic findings are reported for 2 NL cases. RESULTS: The following 2 patients with NL, with disparate clinical presentations, are presented: a patient with subacute onset, painful, multifocal, mixed axonal and demyelinating radiculoplexus neuropathy due to a large B-cell NHL, who required 2 targeted fascicular nerve biopsies to demonstrate NL; and a patient with a slowly progressive, length-dependent axonal polyneuropathy due to a low-grade B-cell lymphoproliferative disorder, as shown on a diagnostic sural nerve biopsy. CONCLUSIONS: The cases described illustrate the wide clinical spectrum of NL.