AI Article Synopsis

  • Waldenstrom's macroglobulinemia (WM) is a rare low-grade B cell cancer that can lead to a rare condition called Bing Neel syndrome, which affects the central nervous system.
  • A 64-year-old man experienced worsening double vision and drooping eyelids over four years, along with various neurological symptoms, which led to further tests revealing anemia and elevated protein levels associated with WM.
  • Despite treatment with bendamustine and rituximab, the patient's neurological condition did not improve, emphasizing the need to consider WM in older patients presenting with isolated eye movement issues.

Article Abstract

Background And Aims: Waldenstroms macroglobulinemia (WM) is a low-grade B cell neoplasm. Bing Neel syndrome is a rare manifestation of WM characterized by infiltrative involvement of the central nervous system.

Case Report: 64-year-old man, presented with 4 years history of slowly progressive diplopia and ptosis of eyes. Examination showed left oculomotor (internal and external ophthalmoplegia), with trochlear, abducens, and right partial oculomotor and abducens nerve involvement. Evaluation showed anemia of hemoglobin 10.7 g/dL, raised erythrocyte sedimentation rate of 120 mm/h and plasma albumin:globulin reversal. Serum protein electrophoresis showed a paraprotein peak in the early gamma region with elevated IgM level (3810 mg/dL) and elevated free kappa light chain level (70.1 mg/L). Bone marrow aspiration from posterior iliac crest revealed mature small lymphocytes with positive immunohistochemical markers of CD5, CD10 negativity and MYD88 mutation positivity suggestive of WM. Patient was treated with bendamustine and rituximab regimen, with no neurological improvement at the end of one year.

Conclusion: This case expands spectrum of paraproteinemic neuropathy to include cranial nerve palsy. Thus, plasma cell dyscrasias have to be considered in patients with isolated ophthalmoparesis especially in elderly patients, even with other comorbidities such as diabetes mellitus.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11112000PMC
http://dx.doi.org/10.1016/j.ensci.2024.100505DOI Listing

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