Pubblicazioni

Myelin uncompaction and axo-glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b  (2020)

Autori:
Tagliapietra, Matteo; Zanusso, Gianluigi; Ferrari, Sergio; Orlandi, Riccardo; Bertolasi, Laura; Cavallaro, Tiziana; Monaco, Salvatore
Titolo:
Myelin uncompaction and axo-glial detachment in chronic ataxic neuropathy with monospecific IgM antibody to ganglioside GD1b
Anno:
2020
Tipologia prodotto:
Articolo in Rivista
Tipologia ANVUR:
Articolo su rivista
Lingua:
Inglese
Formato:
A Stampa
Referee:
Nome rivista:
Journal of the peripheral nervous system
ISSN Rivista:
1085-9489
N° Volume:
25
Numero o Fascicolo:
1
Intervallo pagine:
54-59
Parole chiave:
anti-GD1b IgM; anti-disialosyl ganglioside antibodies; chronic lymphocytic leukemia; lipid rafts; myelin vacuolation; sensory ataxic neuropathy
Breve descrizione dei contenuti:
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.
Pagina Web:
https://doi.org/10.1111/jns.12359
Id prodotto:
111509
Handle IRIS:
11562/1008528
ultima modifica:
15 novembre 2022
Citazione bibliografica:
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. 12020pp. 54-59

Consulta la scheda completa presente nel repository istituzionale della Ricerca di Ateneo IRIS

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