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).
Epidemiology
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 . This suggests that Bing-Neel syndrome is very rare .
Clinical presentation
The clinical presentation can be extremely varied and tend to develop over a period of weeks or months . Common features that have described, depending on the region of the CNS primarily affected, include :
- mainly meningeal involvement: headache, nausea and vomiting, cranial neuropathies
- mainly parenchymal involvement: cognitive decline, seizures, aphasia, weakness, coma, psychiatric symptoms
- mainly spinal cord involvement: weakness, sensory changes
Pathology
Bing-Neel syndrome is characterized by lymphoplasmacytoid infiltration of the brain parenchyma and IgM deposition in the CNS . Two distinct forms have been described: diffuse infiltrative form and tumoral form.
Diffuse infiltrative form
The lymphoplasmacytoid infiltration is primarily of the leptomeninges, perivascular spaces, periventricular white matter, brainstem, and spinal cord .
Tumoral form
Unifocal or multifocal tumor-like regions of lymphoplasmacytoid infiltration in the subcortical regions .
Radiographic features
MRI is the imaging modality of choice to investigate Bing-Neel syndrome.
MRI
The two distinct pathological forms of Bing-Neel syndrome have distinct radiographic patterns.
Diffuse infiltrative form
- typically contrast-enhancement of affected regions, best appreciated in T1-weighted gadolinium-enhanced sequences, accompanied by thickening of the affected leptomeninges and cauda equina
- these lesions otherwise demonstrate hyperintensity on T2-weighted sequences and iso- or hypointensity on T1-weighted sequences
Tumoral form
- characterized by unifocal or multifocal mass-like lesions in the subcortical regions of the brain that similarly demonstrate hyperintensity on T2-weighted sequences and iso- or hypointensity on T1-weighted sequences with contrast enhancement on T1-weighted gadolinium-enhanced sequences
Treatment and prognosis
It is considered potentially treatable with cranial radiation therapy alone, or in combination with intrathecal chemotherapy .
History and etymology
It was first described in 1936 by Jens Bing and Axel Valdemar Neel, several years before the first description of Waldenström macroglobulinemia .
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