European Federation of Neurological Societies/Peripheral Nerve Society guideline

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Eur J Neurol. 2006 Aug;13(8):809-18.

European Federation of Neurological Societies/Peripheral Nerve

Society guideline on management of paraproteinaemic demyelinating

neuropathies: report of a joint task force of the European Federation

of Neurological Societies and the Peripheral Nerve Society*

Hadden RD, Nobile-Orazio E, Sommer C, Hahn A, Illa I, Morra E,

Pollard J, RA, Bouche P, Cornblath D, Evers E, Koski CL, Leger

JM, Van den Bergh P, van Doorn P, van Schaik IN.

Department of Neurology, King's College Hospital, London, UK.

Background. Paraprotein-associated neuropathies have heterogeneous

clinical, neurophysiological, neuropathological and haematological

features. Objectives. To prepare evidence-based and consensus

guidelines on the clinical management of patients with both a

demyelinating neuropathy and a paraprotein (paraproteinaemic

demyelinating neuropathy, PDN).

Methods. Search of MEDLINE and the Cochrane library, review of

evidence and consensus agreement of an expert panel.

Recommendations. In the absence of adequate data, evidence based

recommendations were not possible but the panel agreed the following

good practice points: (1) Patients with PDN should be investigated

for a malignant plasma cell dyscrasia. (2) The paraprotein is more

likely to be causing the neuropathy if the paraprotein is

immunoglobulin (Ig)M, antibodies are present in serum or on biopsy,

or the clinical phenotype is chronic distal sensory neuropathy. (3)

Patients with IgM PDN usually have predominantly distal and sensory

impairment, with prolonged distal motor latencies, and often anti-

myelin associated glycoprotein antibodies. (4) IgM PDN sometimes

responds to immune therapies. Their potential benefit should be

balanced against their possible side-effects and the usually slow

disease progression. (5) IgG and IgA PDN may be indistinguishable

from chronic inflammatory demyelinating polyradiculoneuropathy,

clinically, electrophysiologically, and in response to treatment. (6)

For POEMS syndrome, local irradiation or resection of an isolated

plasmacytoma, or melphalan with or without corticosteroids, should be

considered, with haemato-oncology advice.