We describe the follow-up of a patient with Waldenström's macroglobulinemia who developed mild predominantly sensory peripheral neuropathy, Bing-Neel syndrome, and, after 17 years, acute mononeuropathy multiplex associated with increasing paraprotein levels. Nerve biopsy demonstrated deposition of IgM in the endoneurium and perineurium. Magnetic resonance imaging showed extension of the cerebral white-matter abnormality. We suggest that the pathogenetic mechanism of the mononeuropathy multiplex may include direct IgM deposition. Late peripheral nerve complications appeared to be related to the paraprotein level.
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Waldenstrom's macroglobulinemia (WM) or lymphoplasmacytic lymphoma is a B-cell malignancy characterized by lymphoplasmacytic cells in the bone marrow that secrete high amounts of immunoglobulin (Ig) M. The large pentameric structure of IgM leads to a variety of unique complications in WM, such as hyperviscosity syndrome, cryoglobulinemia and sensory neuropathy. Furthermore, malignant cells can infiltrate the central nervous system and lead to a variety of neurological complications, also known as Bing Neel Syndrome.
View Article and Find Full Text PDFWaldenström Macroglobulinemia - A State-of-the-Art Review: Part 1: Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Risk Stratification, and Clinical Problems.
Mediterr J Hematol Infect Dis
July 2024
Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA.
Article Synopsis
- Waldenström macroglobulinemia (WM) is a rare B-cell lymphoma characterized by IgM paraprotein and the MYD88 L265P mutation, affecting the bone marrow and leading to various health complications.
- Common complications from WM include cytopenias, hyperviscosity, and peripheral neuropathy, resulting from malignant cell invasion and immune response.
- Chemoimmunotherapy, especially combining rituximab with other treatments, has been the standard, but recent studies show Bruton Tyrosine Kinase inhibitors (BTKIs) are also effective, with a median life expectancy of 10 to 12 years.
Bing Neel syndrome presenting as isolated cranial nerve palsies - a case report.
eNeurologicalSci
June 2024
Department of Neurology, National Institute of Mental Health and Neuro Sciences (NIMHANS), Bengaluru, India.
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.
Waldenström Macroglobulinemia-Associated Peripheral Neuropathy in the Brachial Plexus With Bing-Neel Syndrome Diagnosed Through Repeated Cerebrospinal Tests.
Cureus
January 2024
Department of Hematology and Oncology, Toyohashi Municipal Hospital, Toyohashi, JPN.
In Waldenström macroglobulinemia (WM), confirming the presence of Bing-Neel syndrome (BNS) is important because drugs that penetrate the central nervous system (CNS) must be selected. We report the case of a 75-year-old man for whom tirabrutinib, a second-generation Bruton's tyrosine kinase inhibitor (BTKi), was useful in treating WM-associated peripheral neuropathy (PN) with BNS. Numbness and muscle weakness in the fingers occurred three years after the initial treatment of WM.
View Article and Find Full Text PDFBing-Neel syndrome: a rare neurological complication of Waldenström macroglobulinaemia.
BMJ Case Rep
January 2024
Ophthalmology, North Florida Regional Medical Center, Gainesville, Florida, USA.
Bing-Neel syndrome (BNS) is a very rare manifestation of Waldenström macroglobulinaemia (WM), in which lymphoplasmacytic cells invade the central nervous system. The clinical presentation includes symptoms of headaches, visual floaters, neuropathy, seizures and gait abnormalities. Here, we describe an elderly woman, who presented with complaints of visual floaters, progressive neuropathy and cognitive changes.
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