Management of juvenile/adult-onset myasthenia gravis at the Queen Elizabeth Hospital, Barbados

This paper is retrospective study of the management of myasthenia gravis (MG) in Barbados. The clinical experience, including an analysis of the role of thymectomy in the management of its disease, has been reviewed. Of 41 patients who were diagnosed as having MG six were excluded because of the onset below the age of twelve. Of the 35 patients studied, 15 had severe disease (Classes III-V) and 10 of these severely affected patients underwent thymectomy, the majority (70 percent) by the trans-cervical route. Follow up was available on all of the patients undergoing thymectomy and 15 of the patients on medical therapy (Classes II-V). Patients undergoing trans-cervical thymectomy had a better overall response than those treated medically, as measured by being able to get off drugs completely or being asymptomatic on a reduced drug dosage. Two of the patients had thymomas, on benign and the other malignant. The patient with the benign thymoma is asymptomatic but still on drugs and the other patient is still in the postoperative phase. It is concluded that in severe cases trans-cervical thymectomy produces a better overall response than medical treatment alone, and carries significantly less morbidity than the transternal approach in non-thymomatous cases.(AU)

Management of juvenile/adult-onset myasthenia gravis at the Queen Elizabeth Hospital, Barbados

This retrospective study of the management of Myasthenia gravis(MG) in Barbados reviews clinical experience including an analysis of the role of thymectomy. pf 41 patients who were diagnosed as having MG six were excluded because of the onset below the age of twelve. Of the 35 patients studied, 15 had severe disease (Class III-V) and 10 of these severely affected patients underwent thymectomy, the majority (70 percent) by the trans-cervical route. Follow up was available on all of the patients undergoing thymectomy and 15 of the patients on medical therapy (Classes II-V). Patients undergoing trans-cervical thymectomy had a better overall response than those treated medically particularly in being able to get off drugs completely or being asymptomatic on a reduced drug dosage. Two of the patients had thymomas, one benign and the other malignant. The patient with the benign thymoma is asymptomatic but still on drugs and the other patient is still in the postoperative phase. It is concluded that in severe cases trans-cervical thymectomy produces a better overall response than medical treatment alone, and carries significantly less morbidity than the trans-sternal approach in non thymomatous cases. (AU)

Myasthenia gravis and HLA phenotypes in Jamaicans

We determined HLA-antigen frequencies and corresponding relative risks (RR) in 30 Jamaicans with myasthenia gravis (MG) and 40 normal controls.. using a microcytotoxicity assay and commercially prepared typing trays, we found that the strongest HLA associations with MG in Jamaicans were with HLA-A2 (RR = 6.15), HLA-B8 (RR = 3.4), HLA-B13 (RR=7.6), and DQw4 (RR = 3.8). After correction of the P value for the number of antigens tested, only HLA-A2 was stastistically significantly increased in MG patients. There was a statistically significant negative association between Mg and HLA-DR2, as well as as HLA-A9 and -B5. No correlation was observed between HLA phenotype, thymic disease, clinical grades, or disease course. HLA-A2 and sex were independent risk factors for MG, female patients having a higher risk (RR = 5.8). Further studies using larger patient and control groups, locally derived typing sera, and DNA probe analysis are indicated. (AU)

Thymectomy in myasthenia gravis: the Jamaican experience

Forty-five patients with myasthenia gravis (MG) were subjected to thymectomy by the median sternotomy technique and were followed up for 4,380 patient months. No operative deaths occurred and 93.3 percent of the patients benefited from surgery with 28.8 percent achieving remission. Forty patients (88.5 percent) showed improvement within one month, and 73 percent of those who achieved remission did so in the first 2 years. Outcome was not affected by thymic pathology except in one patient who had a thymoma removed. These results confirm the value of thymectomy in the management of MG patients with generalised disease and the efficacy of the simple median sternotomy procedure (AU)

HLA association in Jamaica patients with myasthenia gravis - abstract

Thirty-two (32) Jamaicans with ocular or generalised myasthenia gravis (MG) as well as 40 normal controls were investigated for HLA-A, -B, -C and -DR antigens. HLA antigens contribution to relative risk (RR) in MG patients included HLA -A2 (RR = 615), HLA -B8 (RR = 3.4), HLA-13 (RR=7.76), HLA -DRw8 (RR = 1.34), HLAw12(RR = 1.89), HLA-DQw2(RR = 2.0), HLA -DQw3 (RR = 2.1) and HLA -DRw4 (RR = 3.8). The strongest associations were, therefore, HLA, -A2, -B8, -B13, DQw4. After correction of the 'P value" for the number of antigens tested, only HLA-A2, was significantly increased in MG patients compared to normal Jamaicans. The -DR3 haplo-type which is usually associated with auto-immune diseases was absent in Jamaican MG patients and present in only 5 percent controls. There was a paucity of auto-immune conditions associated with MG in this study. Several of the HLA associations found in Jamaican MG patients correspond to those found in other ethnic groups. However, HLA antigens associated with MG in Jamaican patients were more in agreement with those found in Japanese patients. The HLA types found in Jamaican MG patients did not correlate with thymic pathology, serum concentrations of acetylcholine receptor antibodies, clinical grades or disease course (AU)

Myasthenia gravis in Jamaica: clinical, immunological and genetic studies

Clinical, immunological and genetic parameters were studied in 73 Jamaican patients with myasthenia gravis (Mg). The reported biomodal clinical distribution of females with early onset of disease and males with late onset was not observed. The female to male ratio was 2:1. The most frequent manifestations of disease were ptosis (84.9 percent), general muscle weakness (68.5 percent), bulbar symptoms (41.1 percent) and diplopia (32.9 percent). Unusual presenting features such as unilateral ptosis, recurrent chest infection and stumbling while walking resulted in diagnosis being missed in 5.8 percent of cases. The sensitivity of radiommunoassay in detecting acetylcholine receptor antibody (AchR-Ab) in sera from a subgroup of 35 MG patients was 71.4 percent whilst that of the ELISA was only 14.2 percent. There was no correlation between HLA-type, thymic pathology and course of disease. HTLV-I could not be implicated in the pathogenesis of this disease. There was a paucity of other associated autoimmune conditions among MG patients. Thymectomy was an important therapeutic modality in that improvement was observered in 22 cases and remission in 11. (AU)

Acute myasthenia gravis in pregnancy

Two cases of acute myasthensia gravis associated with pregnancy are presented. The first developed in the puerperium following normal labour, and the second was precipitated by Curare used in delivery by Caesarean section. Both were young females who had normal ante-natal courses. The association between pregnancy and myasthenia gravis is reviewed, and also the drugs which may precipate the disease. A plea is made for careful monitoriong of patients receiving these drugs, until their reaction is established (AU)

Myasthenia gravis in Jamaica - abstract

The purpose of this study is to assess the incidence, clinical features and results of treatment of myasthenia gravis as seen at the University Hospital in Jamaica. This paper deals with 50 cases. The clinical features and pitfalls in diagnosis are emphasized. Seven patients had thyrotoxicosis and seven showed muscular wasting. There were three with a thymoma only one of which was examined histologically and found to be malignant. Five patients became pregnant during their illness. Medical and surgical treatment are briefly discussed and the method of selection for each category is described. The best surgical result is obtained in young women with a rapidly progressive history. Thumectomy was performed in 19 patients with very good results. One patient died one year after surgery; he was a 60-year-old man with a malignant thymoma and severe generalized weakness. There was dramatic improvement after surgery in 15 patients. The remaining three improved slowly. Meticulous preoperative and post-operative managemant are essential(AU)

Myasthenia gravis in Kingston, Jamaica

Myasthenia Gravis has been described as a complete clinical entity only within the last 90 years, although there have been references to it as early as 1672. A brief historical review has been given. The condition is not common in Kingston, Jamaica, there being only 12 proven cases out of a total admission of 6,000 patients to two medical wards at the Kingston Public Hospital between January 1st, 1959 and December 31st, 1964. The clinical behaviour of these cases is reported in detail (AU)