A Mystery in a Case: Unraveling the Complexity of Bing-Neel Syndrome

DOI: 10.7759/cureus.65042 A Mystery in a Case: Unraveling the Complexity of Bing-Neel Syndrome Payton N. [...] First identified by Jens Bing and Axel Valdemar Neel in 1936, Bing-Neel syndrome (BNS) is a rare clinicopathological complication of WM that results from the direct involvement of the central nervous system (CNS) with IgM-secreting lymphoplasmacytic cells [1-4]. [...] The presence of an MYD88 mutation, in combination with the patient’s MRI results, CSF analysis, and chemoresistance, strongly supported a diagnosis of BNS. [...] Additionally, meningitis, inflammation of the meninges, and encephalitis, inflammation of the brain itself, may present with symptoms like those in BNS, particularly in patients with underlying malignancies or immunosuppression [7]. [...] DOI 10.7759/cureus.65042 6 of 9suspicion of these differential diagnoses prompted thorough laboratory tests and imaging studies, contributing to the delayed diagnosis. The diagnosis of BNS relies on a comprehensive diagnostic approach beginning with an MRI of the brain and spinal cord [3,4,10]. [...] Notably, a gain-of-function mutation in the MYD88 gene leads to dysregulated inflammation, tissue damage, apoptosis inhibition, and upregulation of pro-survival genes [15]. [...] In patients with a history of WM, the time to BNS diagnosis varies across literature, ranging from 3.5 to seven years [11,17]. [...] Conclusions As demonstrated, the nonspecific and diverse symptoms associated with BNS present a complex diagnostic challenge, with no single clinical presentation including or excluding the diagnosis. This case posed unique challenges, as the patient experienced clinical symptoms despite the absence of MRI abnormalities. Consequently, it is crucial to maintain a high index of suspicion for BNS in [...] Such an approach is essential for preventing diagnostic oversights and improving the quality of life for 2024 Kotlarz et al. [...] Simon L, Fitsiori A, Lemal R, et al.: Bing-Neel syndrome, a rare complication of Waldenström macroglobulinemia: analysis of 44 cases and review of the literature.