Miastenia gravis «seronegativa» y anticuerpos antiMuSK positivos: descripción de una serie española / «Seronegative» myasthenia gravis and antiMuSK positive antibodies: description of Spanish series
Med. clín (Ed. impr.)
; 125(3): 100-102, jun. 2005. tab, graf
Article
in Es
| IBECS
| ID: ibc-036675
Responsible library:
ES1.1
Localization: ES1.1 - BNCS
RESUMEN
Fundamento y objetivo:
Recientemente se ha descrito que un subgrupo de pacientes con miastenia gravis generalizada seronegativa tienen anticuerpos frente a la molécula tirosincinasa muscular específica (AcMuSK). Nuestro objetivo es describir nuestra serie de pacientes con miastenia generalizada y AcMuSK positivo. Pacientes ymétodo:
Mediante radioinmunoanálisis se determinaron los anticuerpos en el suero de 26 pacientes con miastenia generalizada seronegativa. Se identificaron 9 pacientes con AcMuSK, cuyos datos clínicos y terapéuticos se revisaron.Resultados:
De los 9 pacientes, 8 eran mujeres de entre 20 y 40 años. Todos presentaron debilidad bulbar grave, bien inicialmente o durante la evolución. El 77% presentó también clínica ocular y un 44%, fatigabilidad leve en las extremidades. Ninguno mejoró tras la timectomía. La respuesta a anticolinesterásicos fue variable. Todos respondieron a inmunoterapia, aunque un 30% precisó politerapia.Conclusiones:
Los AcMuSK distinguen a un subgrupo de pacientes, el 34,61% en esta serie, con miastenia generalizada seronegativa que presentan una clínica homogénea con debilidad de predominio bulbar. Conocer los mecanismos inmunopatológicos de los AcMuSK permitirá entender la variabilidad de respuesta a los anticolinesterásicos, así como la falta de respuesta a la timectomía, en estos pacientesABSTRACT
Background and objective:
Recently, the presence of antibodies to a muscle-specific tyrosine kinase (MuSK) has been reported in some patients with seronegative generalized myasthenia gravis. Our objective was to describe a group of patients who were positive for anti-MuSK antibodies. Patients andmethod:
Detection of antibodies using a radioimmunoassay was performed in the serum of 26 patients with generalized myasthenia gravis. We identified 9 patients with anti-MuSK antibodies (MuSK+). Clinical and therapeutic data from these patients were reviewed.Results:
Eight of nine patients were women aged between 20 and 40 years. Clinically, all of them showed prevalent bulbar signs, initially or during the progression of the disease. 77% of patients showed ocular involvement and 44% had symptoms of fatigability involving limbs. None of them improved upon thymectomy. Response to acetylcholinesterase inhibitors was variable. All of them responded to immunotherapy, although 30% required polytherapy.Conclusions:
The study of anti-MuSK antibodies defines a subgroup of patients, 34,61% in our series, with seronegative generalized myasthenia. This group is characterized by an homogenous clinical presentation with prevalent bulbar symptoms. The knowledge of the immunopathogenic mechanisms of anti-MuSK antibodies will allow a better understanding of both the variable response to acetylcholinesterase inhibitors and the absence of response to thymectomy in these patients
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Collection:
National databases
/
Spain
Database:
IBECS
Main subject:
Myasthenia Gravis
Type of study:
Prognostic study
Limits:
Female
/
Humans
/
Male
Language:
Spanish
Journal:
Med. clín (Ed. impr.)
Year:
2005
Document type:
Article
Institution/Affiliation country:
Hospital de la Santa Creu i Sant Pau/España