Paraproteinemic neuropathy: a practical review

Rison, Richard A.; Beydoun, Said R.

doi:10.1186/s12883-016-0532-4

Cited by 61 publications

(81 citation statements)

References 64 publications

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“…Plasma cells can be detected in the blood of up to 45% of patients while 90% of patients have immature plasma cells in their cerebrospinal fluid, indicating a poor prognosis [8] . (3) Peripheral neuropathy may cause plasma cells to increase abnormally, leading to the binding of M-protein to the myelin of peripheral nerve, particularly myelin-associated glycoprotein [9] . M-protein can be deposited in between myelin sheath gaps and causes secondary demyelination [10] .…”

Section: Discussionmentioning

confidence: 99%

Anterior horn lesion of spinal cord as the initial manifestation of multiple myeloma: A case report

et al. 2016

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Multiple myeloma (MM) is a malignant proliferation of abnormal plasma cell tumour that often violates multiple body systems. Its clinical manifestations vary greatly, thus leading to a high rate of misdiagnosis. Herein, we report a clinical case of non-compressive spinal cord lesion, which manifested early signs of multiple myeloma. Retrospective analyses of MM pathogenesis, clinical manifestation, and diagnosis were based on the clinical features and diagnostic criteria of MM that have been published in the literature. This study was initiated to improve current awareness of MM and to reduce the rate of misdiagnosis.

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Section: Discussionmentioning

confidence: 99%

Anterior horn lesion of spinal cord as the initial manifestation of multiple myeloma: A case report

et al. 2016

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“…This requires detection of monoclonal proteins (paraproteins) formed by a single heavy chain (M, G or A) and a light chain (kappa or lambda), which is usually but not always secreted. Around 10% of patients with polyneuropathy of otherwise unknown cause have a monoclonal gammopathy 17. A low-level stable paraprotein is occasionally discovered in older persons, and often this is attributed to an insignificant monoclonal gammopathy of unknown significance (MGUS).…”

Section: The Typically Atypical Presentation Of Poems Syndromementioning

confidence: 99%

POEMS neuropathy: optimising diagnosis and management

Keddie¹,

D’Sa

Foldes

et al. 2018

Pract Neurol

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POEMS syndrome is a rare and disabling autoinflammatory condition characterised by a typical peripheral neuropathy and the presence of a monoclonal plasma cell disorder. The acronym ‘POEMS’ represents the complex and multisystem features of the disease, including polyneuropathy, organomegaly, endocrinopathy, a monoclonal plasma cell disorder and skin disease. The diagnosis of POEMS is a significant challenge because of the heterogeneity of clinical presentations and variation of POEMS features. Patients are often misdiagnosed with another cause of inflammatory neuropathy and receive one or more ineffective immunomodulatory medications, resulting in delayed diagnosis and further clinical deterioration before a diagnosis is made. University College London Hospitals sees one of the largest reported POEMS cohorts in Europe, and runs a multispecialist clinic to assist with diagnosis, treatment and ongoing support. This review draws upon our experience to present the typical features of POEMS syndrome and highlight diagnostic conundrums commonly experienced, supplemented with clinical cases. We provide an investigative guide for clinicians when considering POEMS as the diagnosis, and propose a treatment algorithm that centres on the site and degree of monoclonal cell proliferation.

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“…The following conditions are suspected: (1) IgM paraproteinemic demyelinating neuropathy with negativity for anti-MAG antibodies, or IgG or IgA paraproteinemic demyelinating neuropathy with a chronic progressive course, and with the discovery of widely spaced myelin on electron microscopy or deposits of Ig and/or complement bound to myelin; (2) amyloidosis; and (3) malignant lymphoproliferative infiltration of nerves. General ideas were derived from "Raheja et al 8 " and "Rison et al 99 ".…”

Section: Neuropathy Associated With Mgusmentioning

confidence: 99%

Paraproteinemic neuropathy

Pyun

Kim

2017

Ann Clin Neurophysiol

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Paraproteinemia is caused by a proliferation of monoclonal plasma cells or B lymphocytes. Approximately 10% of idiopathic neuropathies are associated with paraproteinemia, where a certain paraprotein acts like an antibody targeted at constituents of myelin or axolemma in peripheral nerves. The relationship between paraproteinemia and peripheral neuropathy remains unclear despite this being of interest for a long time. Neurologists frequently find paraproteinemia during laboratory examinations of patients presenting with peripheral neuropathy, especially in the elderly. The possibility of a relationship with paraproteinemia should be considered in cases without an explainable cause. We review the causal association between paraproteinemia and neuropathy as well as clinical, laboratory, and electrophysiologic features, and the treatment options for paraproteinemic neuropathy.

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Paraproteinemic neuropathy: a practical review

Cited by 61 publications

References 64 publications

Anterior horn lesion of spinal cord as the initial manifestation of multiple myeloma: A case report

Anterior horn lesion of spinal cord as the initial manifestation of multiple myeloma: A case report

POEMS neuropathy: optimising diagnosis and management

Paraproteinemic neuropathy

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