Myelin uncompaction and axo-glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b.
anti-GD1b IgM
anti-disialosyl ganglioside antibodies
chronic lymphocytic leukemia
lipid rafts
myelin vacuolation
sensory ataxic neuropathy
Journal
Journal of the peripheral nervous system : JPNS
ISSN: 1529-8027
Titre abrégé: J Peripher Nerv Syst
Pays: United States
ID NLM: 9704532
Informations de publication
Date de publication:
03 2020
03 2020
Historique:
received:
11
10
2019
revised:
01
12
2019
accepted:
04
12
2019
pubmed:
15
12
2019
medline:
20
5
2021
entrez:
15
12
2019
Statut:
ppublish
Résumé
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.
Substances chimiques
Autoantibodies
0
Gangliosides
0
Immunoglobulin M
0
ganglioside, GD1b
19553-76-5
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
54-59Informations de copyright
© 2019 Peripheral Nerve Society.
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