DiseasesDB 20944 | ||
Bing–Neel syndrome (BNS) is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM). It involves central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia. This increases blood viscosity, which impairs its circulation through small brain and eye blood vessels. Patients with BNS can be classified into Group A and Group B based on the presence of these cells within the brain parenchyma, leptomeninges, dura, and/or the CSF.
Contents
Discovery
It was first described in 1936 by Jens Bing and Axel Valdemar Neel, who observed a case of 2 women, 56 and 39 years old, presenting with rapid neurodegeneration in the setting of hyperglobulinemia.
Symptoms
Symptoms include episodes of confusion, slurred speech, headache, fatigue, ataxia, memory problems, nausea, vomiting, and extremity numbness.
Diagnosis
Complete blood count, electrolytes, and liver enzymes are normal in patients with Bing–Neel syndrome. Diagnosis can include lumbar puncture and magnetic resonance imaging (MRI) of the brain and spinal cord.
Treatment
Treatment involves central nervous system penetrating chemotherapy. Some significant improvement has been shown in patients as a result of cranial radiation treatment preceding a brief course of intrathecal chemotherapy. In Arkansas, a patient was treated with "intrathecal chemotherapy with several cycles of systemic chemotherapy followed by autologous stem cell-supported high-dose therapy transplant". The patient discontinued all treatment in 2009 and was still asymptomatic by the time a follow-up report was published in 2013.