title: Demyelination as a harbinger of lymphoma: a case report and review of primary central nervous system lymphoma preceded by multifocal sentinel demyelination.
abstract: BACKGROUND
  Primary central nervous system lymphoma (PCNSL) may rarely be preceded by "sentinel demyelination," a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). Interpreting the overlapping radiologic and clinical characteristics associated with each of these conditions-contrast-enhancing demyelination of white matter and relapsing and remitting steroid-responsive symptoms respectively-can be a significant diagnostic challenge.
  
  
  METHODS
  We describe a 57-year-old woman with an unusual clinical course who presented with multi-focal enhancing white matter lesions demonstrated to be inflammatory demyelination by brain biopsy. Despite a good initial response to steroids and rituximab for treatment of presumed tumefactive multiple sclerosis, the patient's condition rapidly deteriorated, and a repeat brain biopsy six months later was consistent with a diagnosis of diffuse large B-cell lymphoma.
  
  
  CONCLUSIONS
  Early clinical suspicion for PCNSL and awareness that biopsied lesions may initially show sentinel demyelination suggestive of alternate diagnoses may be essential for early initiation of appropriate therapies and mitigation of disease progression. Clinical, pathophysiological, and diagnostic aspects of sentinel demyelination and PCNSL are discussed.
authors.affiliation: Program in Neuroscience and Medical Scientist Training Program, University of Maryland School of Medicine, Baltimore, Maryland.|Departments of Physiology, University of Maryland School of Medicine, Baltimore, Maryland, USA.
authors.forename: Mark D
authors.initials: MD
authors.lastname: Kvarta
authors.affiliation: Vascular Medicine Institute, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
authors.forename: Deva
authors.initials: D
authors.lastname: Sharma
authors.affiliation: Department of Pathology, University of Maryland School of Medicine, Baltimore, Maryland, USA.
authors.forename: Rudolph J
authors.initials: RJ
authors.lastname: Castellani
authors.affiliation: Department of Diagnostic Radiology, University of Maryland School of Medicine, Baltimore, Maryland, USA.
authors.forename: Robert E
authors.initials: RE
authors.lastname: Morales
authors.affiliation: Department of Neurology, University of Maryland School of Medicine, Baltimore, Maryland, USA.
authors.forename: Stephen G
authors.initials: SG
authors.lastname: Reich
authors.affiliation: Department of Internal Medicine, University of Maryland School of Medicine, Baltimore, Maryland, USA.
authors.forename: Amy S
authors.initials: AS
authors.lastname: Kimball
authors.affiliation: Department of Neurology, MedStar Georgetown University Hospital, District of Columbia, Washington, USA. robert.k.shin@gunet.georgetown.edu.
authors.forename: Robert K
authors.initials: RK
authors.lastname: Shin
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D016543:Central Nervous System Neoplasms / Q000150:complications / Q000473:pathology*; D003711:Demyelinating Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000473:pathology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D016393:Lymphoma, B-Cell / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D000069283:Rituximab / Q000627:therapeutic use; D066127:White Matter / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones; D000069283:Rituximab
keywords: Demyelination; Multiple Sclerosis; Pre-operative steroids; Primary CNS Lymphoma