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Med Clin (Barc). 2005 Jun 18;125(3):100-2.

["Seronegative" myasthenia gravis and antiMuSK positive antibodies: description of Spanish series].

[Article in Spanish]

Author information

1
Unidad de Enfermedades Musculares, Servicio de Neurología, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. illa@hsp.santpau.es

Abstract

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 AND METHOD:

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.

PMID:
15989843
[Indexed for MEDLINE]

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