title: Neurolymphomatosis: a case series of clinical manifestations, treatments, and outcomes.
abstract: BACKGROUND
  Neurolymphomatosis (NL) is a rare clinical entity characterized by infiltration of malignant lymphocytes into the peripheral nervous system. We analyzed the clinicoradiological features, treatments, and outcomes in NL patients.
  
  
  METHODS
  We identified six patients with NL seen at The University of Texas MD Anderson Cancer Center from 01/2010 to 10/2012. We extracted clinical presentations, imagings, CSF cytology, and electrodiagnostic studies from medical records. One patient had a nerve biopsy. We defined therapy response as clinical improvement of neurological deficits.
  
  
  RESULTS
  The mean age at onset was 57.1 years. Most were predominantly men with non-Hodgkin lymphoma. Positron emission tomography (PET) was positive in five patients. Nerve conduction demonstrated mononeuritis multiplex, plexopathy, demyelination, and axonal polyradiculoneuropathy, whereas electromyography was nonspecific. All patients received systemic chemotherapy, four intrathecal chemotherapy, and three intravenous immunoglobulin, plasma exchange or both. One patient who received intravenous immunoglobulin showed mild neurological improvement. Two patients responded, and the median overall survival was 15 months.
  
  
  CONCLUSIONS
  NL is an increasingly recognized complication of NHL and leukemia. A high clinical suspicion is necessary for correct diagnosis. In the present series, patients with leukemia had mononeuritis multiplex, whereas those with lymphoma had plexopathy. Electrodiagnosis and PET scans were useful diagnostic tools. No factors correlated with poorer prognosis. International collaborative studies will help to better determine the risk factors of NL, response to treatment and outcomes.
authors.affiliation: Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; Department of Neurology, The University of Texas Medical Branch at Galveston, Galveston, TX, USA. Electronic address: ckamiya@mdanderson.org.
authors.forename: Carlos
authors.initials: C
authors.lastname: Kamiya-Matsuoka
authors.affiliation: Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; Department of Neurology, The University of Texas Medical Branch at Galveston, Galveston, TX, USA.
authors.forename: Sheetal
authors.initials: S
authors.lastname: Shroff
authors.affiliation: Department of Neurology, Baylor College of Medicine, Houston, TX, USA.
authors.forename: Kasey
authors.initials: K
authors.lastname: Gildersleeve
authors.affiliation: Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; Department of Neurology, The University of Texas Medical Branch at Galveston, Galveston, TX, USA.
authors.forename: Bahram
authors.initials: B
authors.lastname: Hormozdi
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: John T
authors.initials: JT
authors.lastname: Manning
authors.affiliation: Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Karin H
authors.initials: KH
authors.lastname: Woodman
mesh_terms: D000328:Adult; D000368:Aged; D000818:Animals; D001717:Birds; D001917:Brachial Plexus / Q000473:pathology; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008380:Marek Disease / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000628:therapy*; D008875:Middle Aged; D010951:Plasma Exchange; D049268:Positron-Emission Tomography; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D019788:Fluorodeoxyglucose F18
keywords: Electrodiagnostic studies; Intravenous immunoglobulin; Leukemia; Lymphoma; Neurolymphomatosis; Plasma exchange