[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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A 2-year-3-month-old girl developed suddenly ocular ptosis following flu-like symptoms. She was diagnosed as myasthenia gravis by positive tensilon test. However, there was no improvement in spite of an adequate dose of anticholinesterase medication. She was admitted to our hospital, and received an alternative-day treatment of 2 mg/kg of prednisolone, because of ineffectiveness by anticholinesterase treatment, and of a low level of T cells. Prednisolone was increased to 3mg/kg, and it was continued for 2 months, but there was no improvement of ptosis. Three months later, she developed a struma and exophthtalmos, and showed abnormal thyroid function tests. T₃ was 493ng/dl, T₄ was 18.0μg/dl. She was diagnosed as myasthenia gravis associated with hyperthyroidism. Prednisolone was tapered down, and a combined therapy by mercaptoimidazole, neostigmine bromide, and ambenonium chloride was started. Her ptosis and ophthalmoplegia were gradually improved by this therapy, which was continued for 10 months. She is now doing well without anticholinesterase.
Association of myasthenia gravis and hyperthyroidism has been occasionally reported. The incidence of hyperthroidism before, during or after myasthenia gravis has been roughly estimated as 5%. In the pediatric age group, this association is relatively uncommon, probably because of the rarity of hyperthyroidism in the first decade of life. The present patient is probably the youngest ever reported.
In these two diseases respectively, abnormalities of the receptor relation to autoimmune mechanism seemed to be related with the pathogenesis, and steroid therapy was considered to be useful. However, steroid by itself was not effective at all.
In myasthenia gravis, it has been generally reported that the levels of T cells are low, and those of IgGFc receptor⁺ T cells are high. In cases of myasthenia gravis associated with hyperthyroidism, no such data have been available.
In the present case, T cell was 45-55% on the course, IgGFcR+ T cell was 2.6% after the discontinuance of the steroid therapy. The reason of low level of IgGFcR⁺ T cell was unknown.

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Computed tomography (CT) scans of the proximal and distal limb muscles were evaluated on three children with congenital muscular dystrophy and two with Duchenne dystrophy, in reference to the degree of muscular degeneration. We found a decrease of the attenuation values in those patients, especially those with congenital muscular dystrophy. The decrease of the attenuation value on CT scans was related to the degree of muscular degeneration and replacement of fatty tissue histologically.

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