Bing–Neel syndrome
| Bing-Neel syndrome | |
|---|---|
| Classification and external resources | |
| DiseasesDB | 20944 |
Bing–Neel syndrome (BNS) is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM).
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.[1]
It involves central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia.[2] This increases blood viscosity, which impairs its circulation through small brain and eye blood vessels.[3] 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.[4]
Symptoms include episodes of confusion, slurred speech, headache, fatigue, ataxia, memory problems, nausea, vomiting, and extremity numbness.[5]
Complete blood count, electrolytes, and liver enzymes are normal in patients with Bing–Neel syndrome.[5] Diagnosis can include lumbar puncture and magnetic resonance imaging (MRI) of the brain and spinal cord.[4]
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.[5] 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.[6]
References
- ↑ Bing J, Neel AV. Two cases of hyperglobulinemia with affection of the central nervous system on a toxi-infectious basis. Acta Medica Scandinavica 1936; LXXXVIII(V-VI):492-506.
- ↑ Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH (March 2009). "Bing-Neel Syndrome revisited". Clin Lymphoma Myeloma. 9 (1): 104–6. doi:10.3816/CLM.2009.n.028. PMID 19362988.
- ↑ "Bing-Neel syndrome". Right Diagnosis. Health Grades. 7 May 2013. Retrieved 13 February 2014.
- 1 2 Ly KI, Fintelmann F, Forghani R, Schaefer PW, Hochberg EP, Hochberg FH. Novel diagnostic approaches in Bing-Neel syndrome. Clin Lymphoma Myeloma Leuk. 2011;11(1):180-3.
- 1 2 3 Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG. Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options. Clin Lymphoma Myeloma. 2009;9(6):462-6.
- ↑ Abdallah AO, Atrash S, Muzaffar J, et al. Successful treatment of Bing-Neel syndrome using intrathecal chemotherapy and systemic combination chemotherapy followed by BEAM auto-transplant: a case report and review of literature. Clin Lymphoma Myeloma Leuk. 2013;13(4):502-6.
Further reading
- Malkani RG, Tallman M, Gottardi-Littell N, et al. (February 2010). "Bing-Neel syndrome: an illustrative case and a comprehensive review of the published literature". J. Neurooncol. 96 (3): 301–12. doi:10.1007/s11060-009-9968-3. PMID 19618118.