title: Heterogeneous spectrum of neuropathies in Waldenström's macroglobulinemia: a diagnostic strategy to optimize their management.
abstract: Neuropathy in Waldenström's macroglobulinemia (WM) is very heterogeneous. We retrospectively studied 40 patients with WM and neuropathy to analyze the different presentations and mechanisms encountered and to propose a diagnostic strategy. Twenty-five patients (62.5%) had axonal neuropathy, related to the following mechanisms: amyloid neuropathy (n = 5), cryoglobulinemic neuropathy (n = 5), neuropathy associated with tumoral infiltration (n = 2), vasculitic neuropathy (n = 2), a clinical motor neuropathy possibly of dysimmune origin (n = 6), or an unclassified mechanism (n = 5). A demyelinating pattern was observed in 15 patients, 10 having anti-myelin-associated glycoprotein (anti-MAG) antibodies and 5 having neuropathy related to chronic inflammatory demyelinating polyradiculoneuropathy. On the basis of these results, we propose a diagnostic strategy combining: (1) an EMG to distinguish between a demyelinating and an axonal pattern; (2) measurement of anti-MAG and anti-ganglioside antibodies; (3) screening for "red flag" features to orientate further investigations. This strategy may help clinicians to identify the mechanism of neuropathy in order to adapt the therapeutic strategy.
authors.affiliation: Département de Neurophysiologie Clinique, Hôpital de la Salpêtrière, 47 bd de l'Hôpital, Paris Cedex 13, France. karine.viala@psl.aphp.fr
authors.forename: Karine
authors.initials: K
authors.lastname: Viala
authors.forename: Tanya
authors.initials: T
authors.lastname: Stojkovic
authors.forename: Anne-Violaine
authors.initials: AV
authors.lastname: Doncker
authors.forename: Thierry
authors.initials: T
authors.lastname: Maisonobe
authors.forename: Timothée
authors.initials: T
authors.lastname: Lenglet
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Bruneteau
authors.forename: Lucile
authors.initials: L
authors.lastname: Musset
authors.forename: Jean
authors.initials: J
authors.lastname: Neil
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Léger
authors.forename: Véronique
authors.initials: V
authors.lastname: Leblond
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology*; D012189:Retrospective Studies; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000473:pathology