M.T. Palmisani

Long-Term Results of Corticosteroid Therapy in Patients with Myasthenia Gravis

We studied the long-term outcome of prednisone therapy in 104 patients with myasthenia gravis (MG). At the end of the follow-up period, good therapeutic results were recorded in 85 patients (81.7%), poor results in 13 (12.5%) and no significant change of MG status in 6 (5.8%). We found a correlation between the duration of treatment and the incidence of steroid side effects. The presence of thymoma and severe forms of MG were associated with relapsing disease requiring prolonged corticosteroid regimens. The age at the start of therapy did not influence significantly the response to treatment.

Myasthenia gravis during interferon alfa therapy

Article abstract—Patients treated with interferon alfa can develop autoantibodies and autoimmune diseases. We describe two patients who developed myasthenia gravis with abnormal levels of serum anti-acetyl-choline receptor antibodies during interferon alfa-2b treatment for malignancies. One of these patients had myopathic findings on EMG and focal deficiency of cytochrome c oxidase on muscle biopsy.

Early-onset myasthenia gravis: clinical characteristics and response to therapy

We studied 59 children with myasthenia gravis (MG). Disease onset was pre-pubertal in 26 patients and post-pubertal in 33. The male to female ratio was 0.62 in the early- and 0.17 in the late-onset groups. The frequency of ocular MG was higher in patients with prepubertal onset. Patients with generalized MG generally showed a good response to thymectomy and corticosteroid therapy proved effective with no major side-effects. In our experience early-onset MG has the same course as in adult life. We recommend thymectomy for generalized disease in childhood, except in very young children on account of possible long-term effects on immunological development. Immunosuppressive therapy should be considered in severely affected patients who do not respond adequately to other therapies.

Familial autoimmune myasthenia gravis: report of four families

Four families each with two patients with autoimmune myasthenia gravis or related conditions are reported. All clinical forms of myasthenia gravis were represented and different disease types were found within the same family. Either one or two generations could be affected and no association with a single HLA haplotype was found. The frequency of familial autoimmune myasthenia gravis is very low and the genetic factors involved seem to be different from MHC genes.

Myasthenia gravis Associated with Thymoma: Clinical Characteristics and Long-Term Outcome

We studied 111 myasthenic patients with thymoma followed over the last 20 years. Most patients were affected with severe myasthenia gravis (MG) and had been treated with immunosuppressive drugs. At the end of the follow-up, we observed good therapeutic results in 74% of patients, but a complete remission in only 10% of cases. The survival rate in thymoma patients was significantly lower than in nonthymoma cases. Clinical parameters, MG response to treatment and survival rate did not differ in patients with invasive and noninvasive thymic neoplasms. Patients with invasive thymoma showed slightly higher surgical mortality and a 10% incidence of tumor relapse.

Thymoma and Myasthenia Gravis

The prognosis of thymoma appears to be correlated mainly with the extent of the tumor, the radically of surgical exeresis1,2 and, as it has recently been reported, with the histological type.3 Association with myasthenia gravis (MG) does not adversely affect patients’ survival.4