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

  1. ↑ 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.
  2. ↑ 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.
  3. ↑ "Bing-Neel syndrome". Right Diagnosis. Health Grades. 7 May 2013. Retrieved 13 February 2014.
  4. 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.
  5. 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.
  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


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