Bing-Neel syndrome is an extremely rare neurological complication of Waldenström macroglobulinemia where there is malignant lymphocyte infiltration into the central nervous system (CNS).
The exact incidence is unknown, however, in one study of patients with Waldenström macroglobulinemia, it was found that only 0.8% had the additional complication of Bing-Neel syndrome 1,2. This suggests that Bing-Neel syndrome is very rare 1,2.
The clinical presentation can be extremely varied and tend to develop over a period of weeks or months 2-4. Common features that have described, depending on the region of the CNS primarily affected, include 2-4:
- mainly meningeal involvement: headache, nausea and vomiting, cranial neuropathies
- mainly parenchymal involvement: cognitive decline, seizures, aphasia, weakness, coma, psychiatric symptoms
- mainly spinal cord involvement: weakness, sensory changes
Bing-Neel syndrome is characterized by lymphoplasmacytoid infiltration of the brain parenchyma and IgM deposition in the CNS 2. Two distinct forms have been described: diffuse infiltrative form and tumoral form.
Diffuse infiltrative form
The lymphoplasmacytoid infiltration is primarily of the leptomeninges, perivascular spaces, periventricular white matter, brainstem, and spinal cord 2.
Unifocal or multifocal tumor-like regions of lymphoplasmacytoid infiltration in the subcortical regions 2.
MRI is the imaging modality of choice to investigate Bing-Neel syndrome.
The two distinct pathological forms of Bing-Neel syndrome have distinct radiographic patterns.
Diffuse infiltrative form
- typically contrast-enhancement of affected regions, best appreciated in T1-weighted gadolinium-enhanced sequences, accompanied by thickening of the affected leptomeninges and cauda equina 2,5
- these lesions otherwise demonstrate hyperintensity on T2-weighted sequences and iso- or hypointensity on T1-weighted sequences 2,5
- characterized by unifocal or multifocal mass-like lesions in the subcortical regions of the brain that similarly demonstrate hyperintensity on T2-weighted sequences and iso- or hypointensity on T1-weighted sequences with contrast enhancement on T1-weighted gadolinium-enhanced sequences 2,5
Treatment and prognosis
It is considered potentially treatable with cranial radiation therapy alone, or in combination with intrathecal chemotherapy 2-4.
History and etymology
It was first described in 1936 by Jens Bing and Axel Valdemar Neel, several years before the first description of Waldenström macroglobulinemia 6.
- 1. Kulkarni T, Treon SP, Manning R, Xu L, Rinne M, Lee EQ, Ghobrial IM, Norden A, Kluk MJ, Nayak L. Clinical Characteristics and Treatment Outcome Of CNS Involvement (Bing-Neel Syndrome) In Waldenstrom’s Macroglobulinemia. (2013) Blood. 122 (21): 5090.
- 2. Minnema MC, Kimby E, D’Sa S, Fornecker LM, Poulain S, Snijders TJ, Kastritis E, Kremer S, Fitsiori A, Simon L, Davi F, Lunn M, Castillo JJ, Patterson CJ, Garff-Tavernier ML, Costopoulos M, Leblond V, Kersten MJ, Dimopoulos MA, Treon SP. Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome. (2017) Haematologica. 102 (1): 43. doi:10.3324/haematol.2016.147728 - Pubmed
- 3. Simon L, Fitsiori A, Lema Rl, Dupuis J, Carpentier B, Boudin L, Corby A, Aurran-Schleinitz T, Gastaud L, Talbot A, Leprêtre S, Mahe B, Payet C, Soussain C, Bonnet C, Vincent L, Lissandre S, Herbrecht R, Kremer S, Leblond V, Fornecker LM. Bing-Neel syndrome, a rare complication of Waldenström macroglobulinemia: analysis of 44 cases and review of the literature. A study on behalf of the French Innovative Leukemia Organization (FILO). (2015) Haematologica. 100 (12): 1587. doi:10.3324/haematol.2015.133744 - Pubmed
- 4. Malkani RG, Tallman M, Gottardi-Littell N, Karpus W, Marszalek L, Variakojis D, Kaden B, Walker M, Levy RM, Raizer JJ. Bing-Neel syndrome: an illustrative case and a comprehensive review of the published literature. (2010) Journal of neuro-oncology. 96 (3): 301-12. doi:10.1007/s11060-009-9968-3 - Pubmed
- 5. Kim HJ, Suh SI, Kim JH, Kim BJ. Brain magnetic resolution imaging to diagnose Bing-Neel syndrome. (2009) Journal of Korean Neurosurgical Society. 46 (6): 588-91. doi:10.3340/jkns.2009.46.6.588 - Pubmed
- 6. Bing J, von Neel A. Two cases of hyperglobulinaemia with affection of the central nervous system on a toxiinfectious basis. (1936) Acta Med Scand. 88:492–506.
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