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Vol. 33. Issue 5.
Pages 250-253 (September - October 2022)
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Vol. 33. Issue 5.
Pages 250-253 (September - October 2022)
Case Report
Bing–Neel syndrome presenting with bilateral lumbar radiculopathy: A case report of a rare clinical entity
Síndrome de Bing-Neel que se presenta con radiculopatía lumbar bilateral: reporte de caso de una entidad clínica rara
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Alexandros G. Brotisa,
Corresponding author
amprotis@med.uth.gr

Corresponding author.
, Maria Palassopouloub, Eftychia Z. Kapsalakic,d, Thanos Paschalisa, Vassilis Papastergioua, Konstantinos N. Fountasa,d
a Department of Neurosurgery, General University Hospital of Larissa, Larissa, Greece
b Department of Haematology, General University Hospital of Larissa, Larissa, Greece
c Department of Radiology, General University Hospital of Larissa, Larissa, Greece
d School of Medicine, University of Thessaly, Larissa, Greece
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Abstract

The differential diagnosis of bilateral lower extremity weakness is broad. We present a very rare case of a 48-year old male patient, with walking difficulties due to Bing–Neel syndrome. On clinical examination, there was a significant loss of muscle power in all his lower extremities key-muscle groups. The lumbar spine magnetic resonance imaging (MRI) showed only mild degenerative changes, whereas the MRI of the head indicated a diffuse meningeal thickening at the right temporal region, characterized by significant enhancement after contrast administration. Serum protein electrophoresis detected an IgM-kappa monoclonal protein. The patient received intrathecal chemotherapy with methotrexate and cytarabine, and was started on oral ibrutinib 420mg daily. In conclusion, a past medical history of Waldenstrom macroglobulinemia in conjunction with neurological manifestations should alert the treating physician for Bing–Neel syndrome. A complete diagnostic imaging and serologic protocol helps in setting the final diagnosis. Steroids are part of the treatment, but should be given after the diagnosis is set. Neurosurgical intervention is indicated for histologic confirmation in the case of diagnostic uncertainty.

Keywords:
Bing–Neel syndrome
Waldenstrom macroglobulinemia
Walking difficulty
Central nervous system
CSF analysis
Abbreviations:
BNS
WM
R-CHO
FCR
MRI
CT
CSF
PCR
LPC
CNS
Resumen

El diagnóstico diferencial de la debilidad bilateral de las extremidades inferiores es amplio. Presentamos un caso muy raro de un paciente masculino de 48 años, con dificultad para caminar debido al síndrome de Bing-Neel. El examen clínico reveló una pérdida significativa de fuerza muscular en todos los grupos de músculos clave de sus extremidades inferiores. La resonancia magnética de la columna lumbar reveló solamente cambios degenerativos leves. Sin embargo, la resonancia magnética de cerebro detectó engrosamiento meníngeo difuso en la región temporal derecha caracterizado por realce significativo tras la administración del contraste. La electroforesis de proteínas en suero detectó una proteína monoclonal IgM-Kappa. Inicialmente, el paciente recibió dexametasona y su situación neurológica mejoró excepcionalmente. Además, se le administró quimioterapia intratecal con metotrexato y citarabina y empezó a tomar ibrutinib oral 420mg a diario. En conclusión, un historial médico anterior de macroglobulinemia de Waldenström junto con manifestaciones neurológicas debe alertar al médico tratante sobre síndrome de Bing-Neel. Un protocolo completo de diagnóstico por imágenes y serológico ayuda a establecer el diagnóstico final. Los esteroides son parte del tratamiento, pero deben administrarse después de establecer el diagnóstico. La intervención neuroquirúrgica está indicada para confirmación histológica en caso de incertidumbre diagnóstica.

Palabras clave:
Síndrome de Bing-Neel
Macroglobulinemia de Waldenström
Dificultades al caminar
Sistema nervioso central
Análisis de LCR

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