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

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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.

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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

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Funding

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

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Correspondence to Aikaterini Fitsiori.

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Guarantor

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

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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.

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Written informed consent was waived by the institutional review board.

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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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Fitsiori, A., Fornecker, LM., Simon, L. et al. Imaging spectrum of Bing–Neel syndrome: how can a radiologist recognise this rare neurological complication of Waldenström’s macroglobulinemia?. Eur Radiol 29, 102–114 (2019). https://doi.org/10.1007/s00330-018-5543-7

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