Back to Search
Start Over
Myelin uncompaction and axo-glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b.
- Source :
-
Journal of the peripheral nervous system : JPNS [J Peripher Nerv Syst] 2020 Mar; Vol. 25 (1), pp. 54-59. Date of Electronic Publication: 2019 Dec 19. - Publication Year :
- 2020
-
Abstract
- 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.<br /> (© 2019 Peripheral Nerve Society.)
- Subjects :
- Ataxia immunology
Ataxia pathology
Ataxia physiopathology
Autoantibodies
Humans
Immunoglobulin M
Male
Middle Aged
Peripheral Nervous System Diseases immunology
Peripheral Nervous System Diseases pathology
Peripheral Nervous System Diseases physiopathology
Ataxia diagnosis
Axons pathology
Gangliosides immunology
Myelin Sheath pathology
Neuroglia pathology
Peripheral Nervous System Diseases diagnosis
Subjects
Details
- Language :
- English
- ISSN :
- 1529-8027
- Volume :
- 25
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Journal of the peripheral nervous system : JPNS
- Publication Type :
- Report
- Accession number :
- 31837178
- Full Text :
- https://doi.org/10.1111/jns.12359