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Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis

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Characterization of monoclonal gammopathy in patients with amyotrophic lateral sclerosis

H-H Wolf¹, A Posa², S Panitz²

¹Department of Internal Medicine IV, Martin-Luther-University Halle-Wittenberg, Halle (Saale), Germany.

Acta Neurologica Scandinavica

|February 4, 2015

Abstract

Background:

Recently, new guidelines for the monitoring and the risk evaluation of monoclonal gammopathy with undetermined significance (MGUS) became available and the light chain MGUS subtype was defined.

Aims Of The Study:

To characterize the type, risk and diagnostic implications of MGUS in patients with amyotrophic lateral sclerosis.

Methods:

We screened 97 consecutive patients with ALS and 97 age- and gender-matched controls for MGUS by serum electrophoresis and immunofixation and compared the characteristics of MGUS with population-based data.

Results:

MGUS was identified in 8.2% of ALS patients and 6.2% of controls (mean age: 62.5 years both). Seven of eight ALS patients with MGUS had 'low-risk MGUS'. M-protein was moderately increased in one ALS patient. The immunoglobulin distribution in ALS patients with MGUS was IgG kappa (n = 2), IgM lambda (n = 1) and light chains of lambda type (n = 3). No differences in demographic and clinical parameters were found between patients with and without MGUS.

Conclusions:

The percentage of patients with MGUS is increased in ALS, but the immunoglobulin distribution is similar to that reported in the general population. MGUS in ALS mostly represents 'low-risk MGUS'; therefore, unnecessary diagnostic procedures should be avoided in ALS patients.

Keywords:

amyotrophic lateral sclerosis light chain MGUS low-risk MGUS monoclonal gammopathy motor neurone disease

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