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PubMedDate not supplied

[Thymectomy in juvenile myasthenia gravis].

INTRODUCTION: Juvenile myasthenia gravis (JMG) is an infrequent autoimmune disease, the symptoms and therapeutic handling of which do not differ from those of the adult forms. Chronic treatment with corticoids very often causes side effects in childhood, which is why patients are being submitted to thymectomies at younger ages with better results. AIMS. To analyse the clinical and evolutionary profile of JMG treated by thymectomy in our centre. PATIENTS AND METHODS: We report the case of four girls aged between 5 and 13 who were diagnosed as suffering from generalised myasthenia gravis (MG) with bulbar affectation. One of them started with a myasthenic crisis. The four of them were submitted to Tensilon s test, an electrophysiological study, determination of AChR, thoracic CT, and study of autoimmunity and thyroid functioning. After surgery the thymus was analysed histologically. RESULTS: They all gave positive in Tensilon s test and were seropositive for AChR. They were treated with anticholinesterases, up to the maximum tolerated dose, and corticoids, without complete remission being accomplished and so they were submitted to a thymectomy in the first year of evolution. In three cases surgical approach was transsternal and in the other by means of a videothoracoscope. All the thymuses showed lymphoid hyperplasia. After a variable follow up the girls are at present asymptomatic, although none of them has been able to completely give up the pharmacological treatment. CONCLUSIONS: Thymectomy is one of the mainstays of treatment for JMG. The ever more frequent use of videothoracoscopic techniques achieves results that are similar to those obtained by conventional surgery but with fewer post operative and aesthetic problems

Adolescent↗
PubMed2002

Gender prevalence in childhood multiple sclerosis and myasthenia gravis.

Multiple sclerosis and myasthenia gravis are the most common autoimmune diseases affecting the nervous system, with generally a female predominance in adults. To determine the gender distribution in childhood, we reviewed 28 patients with definite multiple sclerosis and 44 patients with myasthenia gravis with onset before 16 years. In myasthenia gravis, a significant female preponderance was observed only with onset after 10 years of age; earlier disease showed no gender difference. Multiple sclerosis tended to affect boys more than girls before age 10 years and both sexes equally after this age. Gender prevalence in childhood multiple sclerosis and myasthenia gravis is different than in adult series; these results may provide insight into pathogenesis or clinical approach.

Adolescent↗
PubMedDate not supplied

[Association between mycosis fungoides and myasthenia gravis (author's transl)].

Myasthenia gravis characterized by physical examination, electrophysiologic abnormalities, ephedronium test, without thymoma, associated in a 47 year-old-man, with mycosis fungoides. Myasthenia gravis began two years after the onset of mycosis fungoides, and the patient died of infection four years after the development of the cutaneous lymphoma. At autopsy, there was no visceral involvement. Myasthenia gravis and lymphoma rarely coexist; the occurrence of myasthenia gravis shortly after the onset of mycosis fungoides suggests that the two disorders may be linked by immunological abnormalities.

Autoantibodies↗
PubMed1999

[Treatment of myasthenia gravis].

The strategy of myasthenia gravis has been progressed during recent 30 years. Extended thymectomy and alternate-day prednisolone administration remarkably improved therapeutic effect in myasthenia gravis. Besides of these therapies, plasmapheresis and immunoadsorption plasmapheresis extremely improved mortality in myasthenia gravis. 1. Corticosteroid therapy Usually initial doses of PSL is 20-30 mg every other day in the morning after thymectomy, and increased 5 mg every on-day. Maximum PSL doses is 80-100 mg alternate-day, then this doses will be maintained over 4 weeks. After these procedures, PSL is gyradually decreased and discontinued within 2-3 years. 2. Thymectomy Extended transsternal thymectomy is the most useful method, because many authors have reported that re-thymectomy reveals re-appearance of thymus. 3. Invasive thymoma Although this type of thymoma has been called malignant thymoma, it should be called as invasive thymoma since any thymoma can invade surrounding tissues for a long subclinical period. Strategy of treating invasive thymoma is PSL administration, radiation and chemotherapy. Chemotherapy should be performed depend on histopathological findings of thymoma. CHOP therapy is useful for the lymphocytic cell predominant type of thymoma, however CHOP + cis-platin therapy is useful for the epithelial cell predominant type. Treatment of myasthenia gravis has been improved, however it is still unclear that how to treat invasive thymoma, and how to prevent re-appearance of symptoms by decrease or discontinuity of PSL administration.

Adrenal Cortex Hormones↗
PubMed1971

Myasthenia gravis occurring in twins.

Myasthenia gravis in one member each of two sets of twins is described. A 17 year old girl developed generalized myasthenia gravis at the age of 14 years, while her monozygotic twin sister has remained in good health during a three year period of observation. Another patient was a 19 year old woman with the onset of generalized myasthenia gravis at the age of 11 years, and her dizygotic twin sister has been in good health.Myasthenia gravis has been described in 13 sets of monozygotic twins, seven sets of dizygotic twins, and one set of twins with undetermined zygosity. Both members of twins were affected in five sets of monozygotic twins and one set of twins with undetermined zygosity, and only one twin was affected in the remaining sets of twins. Occurrence of myasthenia gravis in both members of monozygotic twins and in none of dizygotic twins, and frequent familial occurrence of the disease suggest the role of a genetic factor in the pathogenesis of this disease.

Adolescent↗
PubMedDate not supplied

The effect of use of pyridostigmine and requirement of vecuronium in patients with myasthenia gravis.

CONTEXT: Patients with myasthenia gravis receive pyridostigmine, an anticholinesterase agent, as a part of therapy. These patients demonstrate a heightened sensitivity towards non-depolarising muscle relaxants. Continuing pyridostigmine till the day of the surgery or omitting it on the night before surgery could provide variable results with regards to the effect of vecuronium. AIMS: Myographic evaluation of a dose of vecuronium in patients with myasthenia gravis on pyridostigmine therapy. SETTING AND DESIGN: A randomised, double-blind, clinical study conducted in a teaching hospital. SUBJECTS AND METHODS: Medically (oral pyridostigmine) well-controlled adult patients with myasthenia gravis who were posted for thymectomy, were randomly divided into two groups. Patients in Group 1 received their last dose of pyridostigmine on the night before surgery while those in Group 2 received even the morning dose of the drug on the day of surgery. Neostigmine (1-2 mg) intravenously was used as rescue medication. Vecuronium (0.01 mg/kg) was used for intubation and muscle relaxation during trans-sternal thymectomy and its effect was reversed using neostigmine and atropine. RESULTS: Fourteen patients (7 in each group) belonging to both sexes were enrolled in the study. The intubating dose of vecuronium showed quicker onset time (155 sec or 2.7 min approx.) and peak effect (99% T1 suppression) in patients belonging to Group 1, and 3/7 (43%) complained of respiratory discomfort while waiting for surgery. By giving the morning dose of pyridostigmine (Group 2), an identical intubating dose of vecuronium showed relative resistance (peak effect-97% T1 suppression) and delayed onset time (198 sec approx.). However, the reversal was complete at the end of surgery in both the regimens. CONCLUSIONS: Omission of the pyridostigmine dose on the day of surgery predisposed patients with myasthenia gravis to the possibility of respiratory discomfort and sensitivity to vecuronium. Continued administration significantly prolonged the onset time of vecuronium and the patients required a higher dose of vecuronium.

Adult↗
PubMed1983

The relation of clinical disease to antibody titre, proliferative response and neurophysiology in murine experimental autoimmune myasthenia gravis.

Murine myasthenia is a relatively faithful model of the human disease. We studied anti-AChR antibody titres, lymphocyte proliferative response to AChR, characteristic electromyographic (EMG) abnormalities and muscle strength at weekly intervals after immunization of C57B16J mice with Torpedo AChR. The boosting immunization at 1 month after the primary immunization resulted in a progressive increase of anti-AChR IgG antibodies, but caused a marked drop in the proliferative response. Although characteristic EMG abnormalities occurred in nearly all immunized mice, clinical disease appeared very late or not at all. These data parallel findings in human myasthenia.

Acetylcholine↗
PubMed1979

Symptom changes during the psychotherapy of patients with myasthenia gravis.

Four patients with myasthenia gravis were treated psychotherapeutically: two who suffered from generalized myasthenia gravis were treated without additional medical treatment. During the course of the therapy a clear correspondence of the patients' behavior and the severity of symptoms could be observed. The symptoms would improve, or even disappear, depending on the developed 'ego-strength' and the possibility of being aggressive and creative during therapy and also depending on the degree to which the 'meaning' of the symptoms could be understood. There seems to be a possibility of influencing decisively the course of myasthenia gravis by psychotherapy.

Adult↗
PubMed1980

Coexistence of systemic lupus erythematosus and myasthenia gravis.

The coexistence of myasthenia gravis and systemic lupus erythematosus in a 52-year-old woman is reported. Review of the literature shows that systemic lupus erythematosus occurs much more often in the myasthenia gravis population than in the population in general. Myasthenia gravis should be considered in the differential diagnosis in a patient with weakness and systemic lupus erythematosus.

Diagnosis, Differential↗