Bing-Neel syndrome

Bing-Neel syndrome is an extremely rare neurological complication of Waldenström macroglobulinemia where there is malignant lymphocyte infiltration into the central nervous system (CNS).

The exact incidence is unknown, however in one study of patients with Waldenström macroglobulinemia, it was found that only 0.8% had the additional complication of Bing-Neel syndrome 1,2. This suggests that Bing-Neel syndrome is very rare 1,2.

The clinical presentation can be extremely varied and tend to develop over a period of weeks or months 2-4. Common features that have described, depending on region of the CNS primarily affected, include:

  • mainly meningeal involvement: headache, nausea and vomiting, cranial neuropathies 2-4
  • mainly parenchymal involvement: cognitive decline, seizures, aphasia, weakness, coma, psychiatric symptoms 2-4
  • mainly spinal cord involvement: weakness, sensory changes 2-4

Bing-Neel syndrome is characterised by lymphoplasmacytoid infiltration of the brain parenchyma and IgM deposition in the CNS 2. Two distinct forms have been described: diffuse infiltrative form and tumoural form. 

The lymphoplasmacytoid infiltration is primarily of the leptomeninges, perivascular spaces, periventricular white matter, brainstem, and spinal cord 2.

Unifocal or multifocal tumour-like regions of lymphoplasmacytoid infiltration in the subcortical regions 2.

MRI is the imaging modality of choice to investigate Bing-Neel syndrome.

The two distinct pathological forms of Bing-Neel syndrome have distinct radiographic patterns.

  • typically contrast-enhancement of affected regions, best appreciated in T1-weighted gadolinium-enhanced sequences, accompanied by thickening of the affected leptomeninges and cauda equina 2,5
  • these lesions otherwise demonstrate hyperintensity in T2-weighted sequences and iso- or hypointensity in T1-weighted sequences 2,5
  • characterised by unifocal or multifocal mass-like lesions in the subcortical regions of the brain that similarly demonstrate hyperintensity in T2-weighted sequences and iso- or hypointensity in T1-weighted sequences with contrast enhancement on T1-weighted gadolinium-enhanced sequences 2,5

It is considered potentially treatable with cranial radiation therapy alone, or in combination with intrathecal chemotherapy 2-4.

It was first described in 1936 by Jens Bing and Axel Valdemar Neel, several years before the first description of Waldenström macroglobulinaemia 6.

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Article information

rID: 51982
Tag: cases
Synonyms or Alternate Spellings:
  • Bing-Neel syndrome

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