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Neurolymphomatosis

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Lymphoma and Leukemia of the Nervous System

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Abstract

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.

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Author information

Authors and Affiliations

  1. Gaffin Center for Neuro-Oncology, Hadassah Hebrew-University Medical Center, Jerusalem, Israel

    Tali Siegal

  2. Department of Hematology, Hadassah Hebrew-University Medical Center, Jerusalem, Israel

    Sigal Grisariu & Batia Avni

  3. Departments of Neurology, Medicine and Neurosurgery, Yale University School of Medicine, New Haven, CT, 06520-8082, USA

    Joachim M. Baehring

Authors
  1. Tali Siegal
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  2. Sigal Grisariu
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  3. Batia Avni
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  4. Joachim M. Baehring
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Correspondence to Tali Siegal .

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Editors and Affiliations

  1. , Stephen E. and Catherine Pappas Center f, Massachusetts General Hospital, Fruit St. 55, Boston, 02114, Massachusetts, USA

    Tracy Batchelor

  2. Dept. Neurology, Memorial Sloan-Kettering Cancer Center, York Ave. 1275, New York, 10021-6007, New York, USA

    Lisa M. DeAngelis

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Siegal, T., Grisariu, S., Avni, B., Baehring, J.M. (2012). Neurolymphomatosis. In: Batchelor, T., DeAngelis, L. (eds) Lymphoma and Leukemia of the Nervous System. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-7668-0_13

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