Distal acquired demyelinating symmetric neuropathy associated with anti-GM1 and anti-GD1b antibodies

Abstract

The clinical hallmark of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the presence of both proximal and distal weakness. Patients with both proximal and distal motor involvement are highly likely to respond to corticosteroids, irrespective of the presence or absence of a serum paraprotein.1 Another well-documented group of patients with symmetric, acquired, demyelinating polyneuropathies predominantly presents with sensory involvement. When weakness is present, it is limited to distal muscle groups in a length-dependent fashion. Distal acquired demyelinating symmetric (DADS) neuropathy is an acquired demyelinating polyneuropathy that presents with distal, symmetric, and predominantly sensory or sensorimotor involvement. 2 DADS neuropathy is usually most strongly associated with IgM anti-myelin associated glycoprotein (MAG) antibody at significant titers. 3 The role of antiglycolipid antibodies in the pathophysiology of peripheral neuropathy and their association with particular clinical phenotypes continues to be a source of informative case reports, clinical-serological studies and basic research. Antibodies to a wide range of gangliosides, including GM1 and GD1b, have been reported for Guillain-Barré syndrome and chronic inflammatory neuropathies. 4 To our knowledge, there are few case reports on the presence of anti-ganglioside antibodies in DADS neuropathy. 5,6Received: April 21, 2016 Distal acquired demyelinating symmetric (DADS) neuropathy is a variant form of chronic inflammatory demyelinating polyradiculoneuropathy. A 54-year-old man presented with gait disturbance owing to weakness in both legs. Nerve conduction studies showed demyelinating sensorimotor polyneuropathy, and laboratory studies demonstrated anti-GM1 and anti-GD1b IgG antibodies, but no anti-myelin associated glycoprotein activity. We suggest that an antiganglioside antibodies assay needs to be applied when DADS neuropathy is suspected in order to improve the classification of dysimmune neuropathies.