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Rev Neurol (Paris). 2011 Jan;167(1):57-63. doi: 10.1016/j.neurol.2010.07.042. Epub 2010 Dec 28.

[New elements in the diagnosis and the treatment of primary AL amyloid polyneuropathy and neuropathy due to POEMS syndrome].

[Article in French]

Author information

1
Service de neurologie, CHU de Bicêtre, AP-HP, université Paris-Sud 11, 78, rue du Général-Leclerc, 94275 Le Kremlin Bicêtre, France. david.adams@bct.aphp.fr

Abstract

INTRODUCTION:

Primary AL amyloid polyneuropathy (AL-PN) and neuropathy due to POEMS syndrome (POEMS-N) are rare, associated with a monoclonal gammopathy (MG) IgGλ or IgAλ at a low rate and systemic manifestations. They are invalidating and life-threatening.

STATE OF THE ART:

AL-PN usually mimics small fiber length-dependent axonal polyneuropathies, but also multifocal or painful neuropathies, POEMS-N corresponds to a rapid ascending CIDP with MG. To confirm the diagnosis of AL-PN, initial investigations should identify amyloidosis on nerve or accessory salivary glands, to establish the type of amyloid after serum free light-chain (FLC) measurements. For the diagnosis of N-POEMS, diagnosis is based on the presence of four criteria proposed by Dispenzieri. These neuropathies are associated with biomarkers, useful for diagnosis and treatment monitoring: elevated serum level of FLC monoclonal in (AL-PN) or VEGF (N-POEMS).

PERSPECTIVES:

Early diagnosis of these neuropathies and early treatment using high-dose melphalan associated with an autologous hematopoietic stem cell graft or low monthly doses can improve the clinical manifestations and patient survival.

CONCLUSIONS:

Systematic search for monoclonal gammopathy by immunofixation and serum free light chains is very useful for the management of progressive peripheral neuropathies of unknown origin.

PMID:
21190703
DOI:
10.1016/j.neurol.2010.07.042
[Indexed for MEDLINE]
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