[Ocular myasthenia gravis: diagnostic aspects and evolution]. 2007

M de Entrambasaguas, and R López-Bernabé, and M López-Alemany
Servicio de Neurofisiología Clínica, Hospital General de Castellón, 12004 Castellón de la Plana, España. entrambasaguas_man@gva.es

BACKGROUND Myasthenia gravis lacks a diagnostic gold standard, so diagnosis is supported by the findings of several tests. Conversion from ocular myasthenia gravis (OMG) to a more widespread disease is frequently early. AIMS. To describe and compare the clinical data and findings of the diagnostic tests of patients with OMG and those with a different diagnosis, and to know the conversion rate from OMG to generalized myasthenia. METHODS Descriptive and analytic retrospective study of 44 patients referred for neurophysiological testing through 4 years because of suspected OMG. RESULTS 12 patients (27%) were diagnosed as having OMG. Evolution time prior to diagnosis tended to be shorter in OMG patients. Isolated ptosis or combined with diplopia was more frequent in OMG, while isolated diplopia was so in other diagnoses (<I>p</I> = 0.003). No thymoma was found. SFEMG jitter of facial muscles was abnormal, including blocking, in all OMG patients (8/8) and normal in the rest (30/30). Edrophonium test was positive in all OMG patients (7/7) and doubtful in another one (1/7). Anti-AChR titers were initially positive in 10/11 OMG patients and 0/17 with other diagnoses. Aponeurotic ptosis and strabismus were the most frequent non-myasthenic etiologies. OMG showed an early generalization in two patients who developed dysarthria. CONCLUSIONS The low ratio of diagnostic confirmation suggests that in the face of ptosis or diplopia diagnostic testing with a high sensibility for OMG is favoured. Jitter showed the best initial diagnostic performance.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009157 Myasthenia Gravis A disorder of neuromuscular transmission characterized by fatigable weakness of cranial and skeletal muscles with elevated titers of ACETYLCHOLINE RECEPTORS or muscle-specific receptor tyrosine kinase (MuSK) autoantibodies. Clinical manifestations may include ocular muscle weakness (fluctuating, asymmetric, external ophthalmoplegia; diplopia; ptosis; and weakness of eye closure) and extraocular fatigable weakness of facial, bulbar, respiratory, and proximal limb muscles. The disease may remain limited to the ocular muscles (ocular myasthenia). THYMOMA is commonly associated with this condition. Anti-MuSK Myasthenia Gravis,MuSK MG,MuSK Myasthenia Gravis,Muscle-Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle-Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Generalized,Myasthenia Gravis, Ocular,Anti MuSK Myasthenia Gravis,Generalized Myasthenia Gravis,Muscle Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Anti-MuSK,Myasthenia Gravis, MuSK,Ocular Myasthenia Gravis
D009460 Neurologic Examination Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system. Examination, Neurologic,Neurological Examination,Examination, Neurological,Examinations, Neurologic,Examinations, Neurological,Neurologic Examinations,Neurological Examinations
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D000369 Aged, 80 and over Persons 80 years of age and older. Oldest Old
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective

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