Sporadic late onset nemaline myopathy and immunoglobulin deposition disease.
Muscle Nerve
; 48(6): 983-8, 2013 Dec.
Article
em En
| MEDLINE
| ID: mdl-23873431
ABSTRACT
INTRODUCTION:
In monoclonal gammopathy, organ dysfunction can occur due to deposition of immunoglobulin fragments. A rare form of acquired myopathy often associated with monoclonal gammopathy is sporadic late onset nemaline myopathy (SLONM), which is characterized by nemaline rods in myofibers. The pathogenetic link between monoclonal gammopathy and SLONM has not yet been elucidated.METHODS:
Case report of a patient with monoclonal gammopathy who developed a progressive myopathy, finally diagnosed as SLONM.RESULTS:
A muscle biopsy showed mild myopathic changes. A second biopsy 1 year after clinical onset demonstrated deposition of immunoglobulin light and heavy chains and the presence of nemaline rods. The patient experienced marked improvement of muscle strength after autologous stem cell transplantation and treatment with bortezomib, a therapy that is known to be effective in light chain deposition disease.CONCLUSIONS:
We speculate that deposition of light and heavy chains, rather than nemaline bodies, has myotoxic effects on skeletal muscle.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Imunoglobulinas
/
Miopatias da Nemalina
/
Doenças do Sistema Imunitário
Limite:
Aged
/
Humans
/
Male
Idioma:
En
Revista:
Muscle Nerve
Ano de publicação:
2013
Tipo de documento:
Article
País de afiliação:
Alemanha