Epidemiology of Myasthenia gravis: A Population-Based Study in Stockholm, Sweden

Kalb, Bobby; Matell, G.; Pirskanen, Ritva; Lambe, Mats

doi:10.1159/000065639

Cited by 73 publications

(53 citation statements)

References 19 publications

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“…The present estimate (24 per 100,000 inhabitants) is the highest reported to date [1-2, 4, 7-12, 28-32] ( fig. 3 ) and clearly indicates an increasing prevalence in the province of Pavia, in accordance with other studies showing that prevalence rates for MG have increased over time [32] . A possible explanation for the high prevalence we found with respect to other studies is a greater reliability of the case-finding method, which should allow the identification of almost all MG patients resident in the study area even if we cannot exclude that some patients remain unrecognized.…”

Section: Discussionsupporting

confidence: 90%

Epidemiology and Geographical Variation of Myasthenia Gravis in the Province of Pavia, Italy

et al. 2012

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Background and Aims: Previous studies have reported a prevalence estimate of myasthenia gravis (MG) from 7.7 to 11.1 per 100,000 inhabitants in Europe. Moreover, the study of the geographical distribution of MG should be useful to generate specific hypotheses. The aims are to estimate MG prevalence and to investigate its geographical variation in a delimited area in Northern Italy. Methods: The primary source of data was the MG database of the Neurological Institute of Pavia and all other sources of case collection in and outside the province. We adopted a Bayesian approach to analyze MG geographical variation within the finest geographical grid. Results: We identified 119 live MG prevalent cases resident in the province of Pavia on December 31, 2008. The overall crude prevalence was 24 per 100,000 inhabitants. The Bayesian analysis identified a small cluster of higher MG prevalence in the northern area of the province. Conclusions: The estimated MG prevalence sets the province of Pavia among the high-risk areas. The identification of high/low MG risk areas deserves further investigation of genetic and environmental factors possibly related to a major risk of the disease in that area.

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Section: Discussionsupporting

confidence: 90%

Epidemiology and Geographical Variation of Myasthenia Gravis in the Province of Pavia, Italy

et al. 2012

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“…Transformation to a generalized form from OMG has not been as clearly delineated in CMG patients as in AMG cases [20]. Interestingly, the rate of transformation (12.4 %) in the present study was significantly lower than the rate (32.5 %) in the 220 AMG patients who have been followed up in the same way at the Hospital (Table 5), and by far lower than the expected rate (up to 80 %) in AMG population [21]. This difference between the CMG and AMG highlights the idea that CMG may constitute a distinctive clinical entity different from AMG patients in some aspects such as the rare presence of thymoma, high percentage of OMG and a tendency to relapse in CMG patients.…”

Section: Discussioncontrasting

confidence: 77%

Long-term outcome of 424 childhood-onset myasthenia gravis patients

et al. 2015

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The objective of this study was to describe the clinical characteristics, outcome and factors that may affect the outcome of childhood-onset myasthenia gravis (CMG) patients in China. We have followed up 424 patients with CMG for at least 5 years at Tongji Hospital. At the end of follow-up, the outcome of all the patients was measured according to MGFA Post-intervention Status. In this study, the patients have been followed up for 9.8 ± 5.4 years. The mean onset age was 5.4 ± 3.6 years. Ocular myasthenia gravis (OMG) was the major type of CMG within 2 years after onset (95%). Thymic hyperplasia was found in 116 patients, and thymoma was confirmed in 6 patients. Acetylcholine receptor antibodies were elevated in 69.5% of the patients. All the patients were routinely treated. Thymectomy was performed in 34 patients (8.0%). At the end of follow-up, seventy-one patients (16.7%) were significantly improved, 66 patients (15.6%) remained unchanged, 53 patients (12.5%) were worsened, and 234 patients (55.2%) were exacerbated. Importantly, fifty OMG patients (12.4%) had transformed into generalized myasthenia gravis (GMG) over 2 years after onset. Thymectomy did not effectively reduce the transformation from OMG to GMG. However, GMG cases significantly benefited from the surgery. This study indicated that the cases with autoimmune CMG account for over 50% in Chinese MG population. The long-term follow-up discloses that CMG patients have a low percentage of improvement, and a high percentage of worsening and exacerbation. The treatment should not be withdrawn too early after the patients obtain complete stable remission. More studies are needed to gain better control of CMG symptoms.

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“…Autoantibodies to the AChR, MuSK, or Lrp4 are responsible for MG, an autoimmune disease with a prevalence of 1-2 in 10,000 (Kalb et al 2002;Murai et al 2011). Intermittent muscle weakness, which worsens after activity, is the hallmark feature of MG. Autoantibodies to the AChR are responsible for most ( 80%) cases of MG. A preponderance of these antibodies are directed to the main immunogenic region in the AChR a subunit, which can block AChR function, increase AChR turnover, and/or stimulate complement-mediated damage (Fig.…”

Section: Autoimmune Mg and Myastheniamentioning

confidence: 99%