Palpebral ptosis and muscle fatiguability associated with perineurial cell ensheathment of muscle fibers: a new disease of the neuromuscular junction?
J Neurol Sci
; 149(2): 147-50, 1997 Aug.
Article
em En
| MEDLINE
| ID: mdl-9171322
ABSTRACT
Perineurial cell ensheathment of muscle fibers has been reported only in one patient. Here we describe a new case with identical morphologic features and a similar, but milder clinical course characterized by progressive muscle weakness and bilateral palpebral ptosis. EMG examination (including repetitive stimulation) and antibodies against acetylcholine receptors were normal. Muscle biopsy revealed several muscle fibers encircled by stratified rings of homogeneous material in which elongated nuclei were visible; this material was positively stained by antibodies directed at epithelial membrane antigen. On ultrastructural examination these encircling-fiber spirals had the characteristics of perineurial cells. It is not clear yet whether perineurial cell ensheathment of muscle fibers is an occasional feature, or whether it has a pathogenetic role in the clinical picture of both cases. The perineurial sheaths might alter the correct neuromuscular transmission mimicking a myasthenia-like disease, either by interfering with the neuromuscular junction, or by changing the microenvironment, and, thus, altering the general excitability of the muscle fibers.
Buscar no Google
Base de dados:
MEDLINE
Assunto principal:
Blefaroptose
/
Debilidade Muscular
/
Músculos Faciais
/
Junção Neuromuscular
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Risk_factors_studies
Limite:
Humans
/
Male
/
Middle aged
Idioma:
En
Revista:
J Neurol Sci
Ano de publicação:
1997
Tipo de documento:
Article
País de afiliação:
Itália