Aikaterini Fitsiori1,2, Luc-Matthieu Fornecker3, Laurence Simon3, Alexandros Karentzos4, Damien Galanaud5, Olivier Outteryck6, Patrick Vermersch6, Jean-Pierre Pruvo7, Emmanuel Gerardin8, Christine Lebrun-Frenay9, Francois Lafitte10, Jean-Philippe Cottier11, Christine Clerc12, Jerome de Seze13, Jean-Louis Dietemann14, Stephane Kremer14. 1. From the 2nd Radiology Department, University Hospital of Strasbourg, Avenue Molière 1, 67098, Strasbourg, France. Aikaterini.Fitsiori@hcuge.ch. 2. Division of Neuroradiology, Department of Imaging and Medical Information Sciences, Geneva University Hospitals, Rue Gabrielle-Perret-Gentil 4, 1205, Geneva, Switzerland. Aikaterini.Fitsiori@hcuge.ch. 3. Haematology Department, University Hospital of Strasbourg, Strasbourg, France. 4. Department of Visceral Surgery, Geneva University Hospitals, Geneva, Switzerland. 5. Department of Diagnostic and Functional Neuroradiology, Pitié-Salpêtrière Hospital of Paris, Paris, France. 6. Neurology Department, University Hospital of Lille, Lille, France. 7. Radiology Department, University Hospital of Lille, Lille, France. 8. Radiology Department, University Hospital of Rouen, Rouen, France. 9. Neurology Department, University Hospital of Nice, Nice, France. 10. Radiology Department, Rothschild Foundation in Paris, Paris, France. 11. Radiology Department, University Hospital of Tours, Tours, France. 12. Neurology Department, Montbéliard Hospital, Belfort, France. 13. Neurology Department, University Hospital of Strasbourg, Strasbourg, France. 14. From the 2nd Radiology Department, University Hospital of Strasbourg, Avenue Molière 1, 67098, Strasbourg, France.
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.
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.
Entities:
Keywords:
Central nervous system; Lymphocytes; Magnetic resonance imaging; Meninges
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