Clinicopathological study of an autopsy case with sensory-dominant polyradiculoneuropathy with antiganglioside antibodies

Abstract

A previously reported patient presenting sensory‐dominant neuropathy with antiganglioside antibodies, bound preferentially to polysi‐ alogangliosides including GD1b, was autopsied. While axonal degeneration was predominant in the sural nerve, many demyelinated fibers were present in the spinal roots. Dorsal roots had undergone significant damage. These pathological findings were well correlated with the electrophysiological results showing decreased F‐wave conduction velocities and conduction blocks in motor nerves and decreased or absent sensory action potentials in sensory nerves, with distribution of GD1b in nerve tissues such as dorsal root ganglia and paranodal myelin in the ventral and dorsal roots. © 1999 John Wiley & Sons, Inc. Muscle Nerve 22: 1426–1431, 1999

Myelin uncompaction and axo‐glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b

“…Pathologic investigations on nerve tissues in CANOMAD/CANDA are still very limited. Neuropathologic findings described in previous reports include moderate to severe loss of large fibers, regeneration clusters, or early onion bulb formation . Apart from a single case showing a few endoneurial inflammatory cells, no inflammation or macrophagic activation was detected.…”

“…A further peculiarity of this case lies in the opportunity to substantiate the role of IgM monospecific to GD1b in peripheral nerve pathology, differently from most of previous pathologic studies where IgM reactivity was extended to other b‐series gangliosides, sulfatides, or other gangliosides lacking disialosyl epitope. Pathologic investigations on nerve tissues in CANOMAD/CANDA are still very limited.…”

“…Apart from a single case showing a few endoneurial inflammatory cells, no inflammation or macrophagic activation was detected. Intriguingly, in cases in which nerve tissues were investigated by immunocytochemistry, no immunoglobulin or complement deposition have been found …”

Chronic ataxic neuropathies associated with anti-GD1b IgM antibodies: response to IVIg therapy

“…Neuropathies associated with immunoglobulin M (IgM) antibodies against disialosyl residues have been defined1 2 3 4 5 6 7 8 as either sensory ataxic neuropathy associated with anti-GD1b IgM antibody,9 chronic sensory ataxic neuropathy associated with anti-disialosyl IgM antibodies, or chronic ataxic neuropathy involving ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies (CANOMAD) 10. The most prominent feature of these neuropathies is sensory ataxia with relatively well-preserved muscle strength and small-fibre sensation.…”

“…Reports of the therapeutic response of patients with GD1b associated sensory neuropathies are even rarer. Plasmapheresis improved in some of the patients treated,1 14 whereas only a few of the patients treated with corticosteroids improved 5 9 11 15 16 17 18 19 20 21. Intravenous immunoglobulin (IVIg) has been used in a few cases,9 16 17 19 21 and half of these patients improved.…”