Distal acquired demyelinating symmetric neuropathy

Abstract

Distinguishing acquired demyelinating neuropathies by phenotype can often predict the presence of IgM kappa M-proteins, anti-MAG antibodies, and responses to immunomodulating therapy.

Incidence and prevalence of CIDP and the association of diabetes mellitus

“…From the onset, we aimed to include only patients with "classic" CIDP, having the typical, symmetric, polyradiculoneuropathy, rather than cases of focal or multifocal CIDP (e.g., Lewis-Sumner syndrome) 28 or a distal, sensory predominant form of CIDP (distal acquired demyelinating symmetric neuropathy). 29 In comparing our results with prior observations, our prevalence of 8.9 in 100,000 is higher than most. The 5-year prospective Norwegian study 8 with a CIDP prevalence of 7.7 in 100,000 most closely aligns with our findings and patient demographics.…”

Length dependence in polyneuropathy associated with IgM gammopathy

“…[1][2][3][4][5][6] The IgM may be pathogenic by binding to myelin-associated glycoprotein (MAG), peripheral myelin protein 22 (PMP22), protein zero (P0), or sulphated glycolipids; these molecules are involved in membrane adhesion and Schwann cell-axon signaling and display a carbohydrate epitope to which the IgM may bind. 1,2,5,[7][8][9][10][11][12][13][14] Nerve conduction studies may show a typical pattern of pronounced distal slowing and less pronounced or no slowing in adjacent lower arm or leg segments.…”

Paraproteinemic neuropathy

“…A terminal latency index (TLI = distal distance/[forearm motor conduction velocity × distal motor latency]) of <0.26 is highly specific for anti-MAG antibody neuropathy with a demyelinating pattern. 16,22 The TLI has previously been found to be considerably lower in anti-MAG antibody neuropathy than in other demyelinating neuropathies such as CIDP and Charcot Marie-Tooth neuropathy type 1a. 26,27 This characteristic feature means that an electrophysiologic test is a suitable first step for distinguishing anti-MAG antibody neuropathy from the other demyelinating neuropathies.…”