Symptoma

Bing-Neel Syndrome

Finally, dura matter involvement, better visible after gadolinium injection, was present in six patients (6/10). [haematologica.org]

Eighteen months later, the patient presented with complaints of headache and blurry vision. Physical examination revealed aphasia, paraparesis, and nuchal rigidity. [bloodjournal.org]

Funding and Disclosures Presented in part at the Annual Meeting of the American Society of Hematology, Atlanta, December 10–13, 2011. [nejm.org]

  • Hodgkin Lymphoma

    Frequent mutation of histone modifying genes in non-Hodgkin lymphoma. Nature 2011 ;476: 298 - 303 29. Lohr JG, Stjanov P, Lawrence MS, et al. [nejm.org]

  • Meningism

    One patient who received intrathecal MTX developed treatment related “chemical meningitis”, and was subsequently treated with HD-MTX to which she had a CSF response. The median overall survival was not reached. [bloodjournal.org]

    Response of meningeal Waldenström’s macroglobulinemia to 2-chlorodeoxyadenosine. J Clin Oncol. 1995; 13 ( 9 ):2476. [ PubMed ] [ Google Scholar ] 8. Vos JM, Kersten M-J, Kraan W, et al. [ncbi.nlm.nih.gov]

    The diagnosis was assessed by the histopathology of a meningeal or a brain biopsy in eight cases. No data are available in this series regarding electrophoresis of CSF as this was not performed in routine practice. [haematologica.org]

  • Convulsions

    Bing-Neel syndrome the central nervous system manifestations of Waldenstrцm macroglobulinemia; symptoms may include encephalopathy, hemorrhage, stroke, convulsions, delirium, and coma. Medical dictionary. 2011. [medicine.academic.ru]

    Four patients with a tumoral form presented with convulsions, hemiparesis or aphasia. No intraocular involvement was documented in this series. Biological results CSF analysis was performed for all patients. [haematologica.org]

  • Dysarthria

    […] dementia (12/44, 27%), sensory deficit with hypoesthesia, dysesthesia or paresthesia (11/44, 25%), headache (8/44, 18%), pain (mainly localized in the back, neck or limbs) (8/44, 18%), cauda equina syndrome (6/44, 14%), motor deficit (6/44, 14%) and dysarthria [haematologica.org]

  • Dysesthesia

    Other signs were poor performance status ( 2) (12/44, 27%), cognitive impairment with frontal syndrome, memory loss or dementia (12/44, 27%), sensory deficit with hypoesthesia, dysesthesia or paresthesia (11/44, 25%), headache (8/44, 18%), pain (mainly [haematologica.org]

  • Cranial Nerve Involvement

    The symptoms or signs that led to the diagnosis of BNS were extremely heterogeneous, the most common being a balance disorder or disturbed gait [21 patients (48%), with ataxia described in 15 patients and dizziness in 6 patients] and cranial nerve involvement [haematologica.org]

[…] double vision) Follow up (6 months – 9 years) 1 patient with 2 relapses, second relapse CNS only 60 Giampaolo Merlini [email protected] 8th International Workshop on Waldenström’s Macroglobulinemia August 14-16, 2014 London, United Kingdom Diagnosis and workup [slideplayer.com]

Rituximab was part of the first-line treatment in 20 (45%) patients. [haematologica.org]

HD-MTX is active in the treatment of BNS, though prospective studies are required to standardize treatment and improve outcomes. [bloodjournal.org]

Treatment is with cranial irradiation and/or intrathecal chemotherapy. [emedicine.medscape.com]

The pathophysiology of BNS remains unexplained, but the role of hyperviscosity has been raised to explain the disruption of the blood-brain barrier. [haematologica.org]

Single-agent therapy Single-agent rituximab Plasmapheresis Low Ig M and cytopenias Plasmapheresis High Ig M - risk of “flare” Single-agent chlorambucil Old age and slow progression. 29 Plasmapheresis for removal of IgM Hyperviscosity-related symptoms Prevention [slideplayer.com]