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European Radiology

, Volume 29, Issue 1, pp 102–114 | Cite as

Imaging spectrum of Bing–Neel syndrome: how can a radiologist recognise this rare neurological complication of Waldenström’s macroglobulinemia?

  • Aikaterini FitsioriEmail author
  • Luc-Matthieu Fornecker
  • Laurence Simon
  • Alexandros Karentzos
  • Damien Galanaud
  • Olivier Outteryck
  • Patrick Vermersch
  • Jean-Pierre Pruvo
  • Emmanuel Gerardin
  • Christine Lebrun-Frenay
  • Francois Lafitte
  • Jean-Philippe Cottier
  • Christine Clerc
  • Jerome de Seze
  • Jean-Louis Dietemann
  • Stephane Kremer
Neuro
  • 210 Downloads

Abstract

Objectives

Bing–Neel syndrome (BNS) is a rare neurological complication of Waldenström’s macroglobulinemia. The aim of this study is to describe the spectrum of radiological manifestations of this syndrome and their prevalence in order to facilitate its early diagnosis.

Methods

Twenty-four patients with BNS were diagnosed between 1994 and 2016 in eight centres in France. We retrospectively examined the medical records of these patients as well as the corresponding literature, focusing on imaging studies. Recorded data were statistically analysed and radiological findings described.

Results

The mean age of our patients was 62.4 years (35–80 years). The vast majority of patients were men, with a male to female ratio of 9:1. Findings included parenchymal or meningeal involvement or both. The most common finding was leptomeningeal infiltration, either intracranial or spinal, with a prevalence reaching 70.8%. Dural involvement was present in 37.5% of patients. In 41.7% (10/24) of patients, there was parenchymal involvement with a higher prevalence of brain comparing to medullar involvement (33.3% and 23.1% respectively). High T2 signal of the parenchyma was identified in 41.7% of patients and high signal in diffusion was evident in 25% of them. Intraorbital or periorbital involvement was also detected in four cases. A proposition regarding the appropriate imaging protocol completed our study.

Conclusion

BNS’s diagnosis remains challenging. Central nervous system MRI findings in the setting of known or suspected Waldenström’s macroglobulinemia appear to be highly suggestive of BNS and appropriate imaging protocols should be implemented for their depiction.

Key Points

• Diagnosis of Bing–Neel syndrome (BNS) remains challenging and recent expert recommendations include MRI in the diagnostic criteria for the syndrome.

• The most common radiological manifestations of BNS are leptomeningeal/dural infiltration or parenchymal involvement of brain or spinal cord, but many atypical forms may exist with various presentations.

• Appropriate imaging protocol for BNS should include enhanced MRI studies of both brain and spine.

Keywords

Magnetic resonance imaging Central nervous system Meninges Lymphocytes 

Abbreviations

BNS

Bing–Neel syndrome

CNS

Central nervous system

CSF

Cerebrospinal fluid

CT

Computed tomography

FLAIR

Fluid-attenuated inversion recovery

GRE

Gradient echo

MRI

Magnetic resonance imaging

PET-CT

Positron emission tomography–computed tomography

SD

Standard deviation

SE

Spin echo

SWI

Susceptibility-weighted imaging

WM

Waldenström’s macroglobulinemia

Notes

Funding

The authors state that this work has not received any funding.

Compliance with ethical standards

Guarantor

The scientific guarantor of this publication is Prof. Stephane Kremer.

Conflict of interest

The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Statistics and biometry

No complex statistical methods were necessary for this paper.

Informed consent

Written informed consent was waived by the institutional review board.

Ethical approval

Institutional review board approval was obtained.

Study subjects or cohorts overlap

Some study subjects (12 patients) have been previously reported in Haematologica by Simon et al.

Methodology

• retrospective

• multicentre study

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Copyright information

© European Society of Radiology 2018

Authors and Affiliations

  • Aikaterini Fitsiori
    • 1
    • 2
    Email author return OK on get
  • Luc-Matthieu Fornecker
    • 3
  • Laurence Simon
    • 3
  • Alexandros Karentzos
    • 4
  • Damien Galanaud
    • 5
  • Olivier Outteryck
    • 6
  • Patrick Vermersch
    • 6
  • Jean-Pierre Pruvo
    • 7
  • Emmanuel Gerardin
    • 8
  • Christine Lebrun-Frenay
    • 9
  • Francois Lafitte
    • 10
  • Jean-Philippe Cottier
    • 11
  • Christine Clerc
    • 12
  • Jerome de Seze
    • 13
  • Jean-Louis Dietemann
    • 1
  • Stephane Kremer
    • 1
  1. 1.From the 2nd Radiology DepartmentUniversity Hospital of StrasbourgStrasbourgFrance
  2. 2.Division of Neuroradiology, Department of Imaging and Medical Information SciencesGeneva University HospitalsGenevaSwitzerland
  3. 3.Haematology DepartmentUniversity Hospital of StrasbourgStrasbourgFrance
  4. 4.Department of Visceral SurgeryGeneva University HospitalsGenevaSwitzerland
  5. 5.Department of Diagnostic and Functional NeuroradiologyPitié-Salpêtrière Hospital of ParisParisFrance
  6. 6.Neurology DepartmentUniversity Hospital of LilleLilleFrance
  7. 7.Radiology DepartmentUniversity Hospital of LilleLilleFrance
  8. 8.Radiology DepartmentUniversity Hospital of RouenRouenFrance
  9. 9.Neurology DepartmentUniversity Hospital of NiceNiceFrance
  10. 10.Radiology DepartmentRothschild Foundation in ParisParisFrance
  11. 11.Radiology DepartmentUniversity Hospital of ToursToursFrance
  12. 12.Neurology DepartmentMontbéliard HospitalBelfortFrance
  13. 13.Neurology DepartmentUniversity Hospital of StrasbourgStrasbourgFrance

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