Publications

Authors:

Tagliapietra, Matteo; Zanusso, Gianluigi; Ferrari, Sergio; Orlandi, Riccardo; Bertolasi, Laura; Cavallaro, Tiziana; Monaco, Salvatore

Title:

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

Year:

2020

Type of item:

Articolo in Rivista

Tipologia ANVUR:

Articolo su rivista

Language:

Inglese

Format:

A Stampa

Referee:

Sì

Name of journal:

Journal of the peripheral nervous system

ISSN of journal:

1085-9489

N° Volume:

25

Number or Folder:

1

Page numbers:

54-59

Keyword:

anti-GD1b IgM; anti-disialosyl ganglioside antibodies; chronic lymphocytic leukemia; lipid rafts; myelin vacuolation; sensory ataxic neuropathy

Short description of contents:

To describe clinical features, disease course, treatment response, and sural nerve biopsy findings in a patient with chronic sensory ataxic neuropathy, Binet stage A chronic lymphocytic leukemia, and monoclonal IgMλ paraprotein against ganglioside GD1b. During 9 months of hospitalization at two neurologic centers, the patient underwent serial neurologic examinations, neurophysiologic studies, imaging investigations, extensive laboratory work-up, bone marrow, and sural nerve biopsies. The patient had a severe progressive sensory neuropathy accompanied by motor involvement, dysautonomia, and marked bulbar weakness with preserved ocular movements. Conduction studies were characterized by prolonged F-wave minimal latencies, prolonged distal latencies, reduction of compound motor action potentials, and absence of sensory nerve action potentials. Sural nerve biopsy showed endoneurial edema, axonal degeneration, and regeneration, in the absence of cellular inflammation, macrophagic activation, and B-lymphocyte infiltration; no IgM or complement deposition was detected. Myelinated fibers showed redundant/abnormally thickened myelin, myelin vacuolation, and frank intramyelinic edema with condensed axoplasm. Ultrastructural features included axo-glial detachment, disruption of membrane integrity, and myelin uncompaction. This study shows that monospecific anti-GD1b IgM paraprotein is associated with non-inflammatory nerve damage. We suggest that the loss of myelin and axonal integrity reflects antibody-induced disruption of membrane lipid rafts.

Web page:

https://doi.org/10.1111/jns.12359

Product ID:

111509

Handle IRIS:

11562/1008528

Last Modified:

November 15, 2022

Bibliographic citation:

Tagliapietra, Matteo; Zanusso, Gianluigi; Ferrari, Sergio; Orlandi, Riccardo; Bertolasi, Laura; Cavallaro, Tiziana; Monaco, Salvatore, Myelin uncompaction and axo-glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b «Journal of the peripheral nervous system» , vol. 25 , n. 1 , 2020 , pp. 54-59

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