A Case of MuSK Myasthenia Gravis Presenting With Persistent Respiratory Insufficiency.
J Clin Neuromuscul Dis
; 23(1): 39-42, 2021 Sep 01.
Article
en En
| MEDLINE
| ID: mdl-34431800
ABSTRACT: Muscle-specific kinase (MuSK) antibody is seen in 4%-10% of patients with myasthenia gravis (MG), with 40% of these patients reporting bulbar weakness as the initial symptom. We present the case of a 40-year-old woman with MuSK MG whose only presenting symptom was progressive respiratory insufficiency necessitating BiPAP use 16-24 hours daily. She was unresponsive to treatment for cardiac and pulmonary causes and thus referred to neurology. Initial workup directed toward autoimmune and genetic myopathies was unrevealing. MuSK antibodies were positive (60.7 nmol/L, nl 0.00-0.02). Electrodiagnostic studies were unremarkable other than single fiber electromyography which was consistent with a defect in neuromuscular transmission. Treatment with prednisone, plasma exchange, and rituximab led to improvement to reliance on BiPAP only at night. However, her most treatment refractory and quality of life limiting symptom continues to be respiratory insufficiency. Further investigation to better characterize differential response to treatment in this subset of patients with MuSK MG may be needed.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Insuficiencia Respiratoria
/
Miastenia Gravis
Tipo de estudio:
Etiology_studies
Aspecto:
Patient_preference
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J Clin Neuromuscul Dis
Asunto de la revista:
FISIOLOGIA
/
NEUROLOGIA
Año:
2021
Tipo del documento:
Article
Pais de publicación:
Estados Unidos