2018
DOI: 10.1007/s00330-018-5543-7
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Imaging spectrum of Bing–Neel syndrome: how can a radiologist recognise this rare neurological complication of Waldenström’s macroglobulinemia?

Abstract: • 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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Cited by 28 publications
(19 citation statements)
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“…22 Although MYD88 L265P mutation is the most common somatic mutation in WM, this is followed closely by CXCR4 WHIM-like frameshift and nonsense mutations. 23,24 Plerixafor is an FDA-approved CXCR4 partial agonist and allosteric antagonist of CXCR7, which has been studied for clinical efficacy and safety in treatment of WHIM syndrome. [25][26][27] In vitro studies have demonstrated that ibrutinib resistance can be potentially reversed by CXCR4 inhibition, yet MYD88 inhibition superseded the survival benefits provided by CXCR4 frameshift mutations.…”
Section: Discussionmentioning
confidence: 99%
“…22 Although MYD88 L265P mutation is the most common somatic mutation in WM, this is followed closely by CXCR4 WHIM-like frameshift and nonsense mutations. 23,24 Plerixafor is an FDA-approved CXCR4 partial agonist and allosteric antagonist of CXCR7, which has been studied for clinical efficacy and safety in treatment of WHIM syndrome. [25][26][27] In vitro studies have demonstrated that ibrutinib resistance can be potentially reversed by CXCR4 inhibition, yet MYD88 inhibition superseded the survival benefits provided by CXCR4 frameshift mutations.…”
Section: Discussionmentioning
confidence: 99%
“…3 Bing-Neel syndrome (BNS) is a rare and probably underdiagnosed neurological complication of Waldenström macroglobulinemia (WM) with infiltration of the central nervous system (CNS) by malignant lymphoplasmacytic cells. [4][5][6] Herein, we report the case of a 69-year-old Caucasian male patient presenting atypical manifestations of a small B-cell lymphoma, presumed to be a marginal zone lymphoma with plasmacytic differentiation and IgM paraprotein, complicated by CSH and neurological impairment similar to BNS.…”
Section: Introductionmentioning
confidence: 99%
“…Gadolinium-enhanced T1-weighted and post-contrast FLAIR MRI remain the most sensitive imaging techniques for diagnosing LMD [3,7,9] as tumor cells adhere to the leptomeninges [10][11][12]. However, in the literature the sensitivity of MRI in this setting ranges between 53 and 79% [13][14][15][16][17][18].…”
Section: Introductionmentioning
confidence: 99%