Widening of myelin lamellae in polyneuropathy with immunoglobulin-M monoclonal gammopathy, without activity against myelin-associated glycoprotein, responsive to treatment.
Neuromuscul Disord
; 32(8): 678-681, 2022 08.
Article
in En
| MEDLINE
| ID: mdl-35618575
ABSTRACT
We report the case of a patient with a very severe predominantly demyelinating sensorimotor polyneuropathy (with axonal loss) that had developed over several months, along with an immunoglobulin-M monoclonal gammopathy without anti-myelin associated glycoprotein antibodies (or other antibodies against myelin). Widening of myelin lamellae were frequently observed by electron microscopic examination of a nerve biopsy immunoglobulin-M targeting an unknown myelin antigen appears to be responsible for the nerve lesions similar to those observed in anti-myelin associated glycoprotein polyneuropathy. Usually, if in anti-myelin associated glycoprotein neuropathy the response to immunotherapies is not optimal, in this case the combination of plasma exchanges and rituximab was effective, suggesting an autoimmune origin.
Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Paraproteinemias
/
Polyneuropathies
/
Antibodies, Monoclonal
Type of study:
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
Neuromuscul Disord
Journal subject:
NEUROLOGIA
Year:
2022
Document type:
Article
Affiliation country:
France