ABSTRACT
INTRODUCTION: Myasthenia is an autoimmune disease, being generalized muscular weakness, with important participation of facial muscles, a prominent feature. Signs of muscular fatigue arise, worsened by exercise and alleviated by rest. Clinical symptoms are less marked before noon, and get worse as the day advances, through the afternoon and evening. A clear relationship between myasthenia and thymic abnormalities does exist, being glandular hyperplasia and tumours the commonest underlying pathologic findings. Initial treatment is based on anticholinesterase drugs and steroids. Non respondents should be treated with immunoglobulins, immunosuppresses, plasmapheresis and surgical removal of the thymus, according to the symptoms control. CASE REPORT: We present the case of a seven years old girl with generalized muscular weakness, worsening through the day, being the diagnosis of myasthenia confirmed by the high level of acetylcholine antireceptors antibodies and the neurophysiologic study. Imaging study of the mediastinum showed a thymic mass located in the right lobe. CONCLUSION: It is therefore most important to rule out these conditions when myasthenia is suspected.
Subject(s)
Myasthenia Gravis/etiology , Thymoma/complications , Thymus Neoplasms/complications , Child , Female , Humans , Myasthenia Gravis/diagnosis , Myasthenia Gravis/diagnostic imaging , Myasthenia Gravis/surgery , Radiography , Thymoma/diagnosis , Thymoma/diagnostic imaging , Thymoma/surgery , Thymus Neoplasms/diagnosis , Thymus Neoplasms/diagnostic imaging , Thymus Neoplasms/surgeryABSTRACT
Introducción. La miastenia es una enfermedad autoinmune, caracterizada por debilidad muscular generalizada, con importante participación de la musculatura facial. Aparecen signos de fatiga muscular, que empeora con el ejercicio y mejora tras el reposo. Las manifestaciones clínicas son menos llamativas por la mañana y empeoran a medida que avanza el día o tras el ejercicio. Existe una relación importante entre la miastenia y la patología del timo, y se asocia con frecuencia a hiperplasias glandulares y a tumores tímicos. El tratamiento inicial se compone de anticolinesterásicos y corticoides, y, si la respuesta no es buena, se utilizan inmunoglobulinas, inmunosupresores, plasmaféresis y la extirpación quirúrgica del timo, todo ello en función del control de los síntomas. Caso clínico. Presentamos una niña de 7 años, con debilidad muscular generalizada, que empeoraba en el transcurso del día, y en la que el estudio neurofisiológico y los títulos de anticuerpos antirreceptores de acetilcolina confirman el diagnóstico de miastenia. Se practica estudio de imagen de mediastino y se demuestra la existencia de una masa tumoral que depende del lóbulo tímico derecho. Conclusiones. Es obligado investigar la patología tímica ante todo paciente sospechoso de la enfermedad (AU)
Subject(s)
Child , Female , Humans , Thymoma , Myasthenia Gravis , Thymus NeoplasmsABSTRACT
OBJECTIVE: The aim of this study was to evaluate methods of hypoacusis screening. PATIENTS AND METHODS: The early detection of audition problems is vital for quick rehabilitation. For this reason, resting on the criteria of the Comisión Española para la Detección Precoz de la Hipoacusia (Spanish Commission for the Early Detection of Hypoacusis), we have carried out a prospective study, from January to May 1998, evaluating patients at risk of suffering from hypoacusis. The study included 151 patients with ages between birth and 14 years. Medical records and brainstem auditory evoked responses (BAER) were carried out. RESULTS: The most common reason for requesting a consultation for the 151 patients included in our study was the suspicion of hypoacusis. Seventy-one (47%) presented pathological BAER, 37 of them were bilateral. In most cases the loss of audition was of cochlear origin, with 11 patients having a serious deafness, 4 with bilateral affection (3 suspicious of hypoacusis and 1 of hyperbilirubinemia) and 7 unilateral deafness. CONCLUSIONS: BAER is a good screening method for children at risk. It is an innocuous, objective and specific test that does not require the patient's collaboration. The level of positives is high (47%).
