Childhood-onset myasthenia gravis: the analysis of influencing factors of therapeutic effect and prognosis / 中华儿科杂志

<p><b>OBJECTIVE</b>Though myasthenia gravis (MG) is a typical autoimmune disorder, there was some controversy on the treatment of the childhood-onset MG. By observing the efficacy of different therapies, the authors analyzed the affecting factors of prognosis in childhood-onset MG.</p><p><b>METHOD</b>The retrospective data of 155 patients with childhood-onset MG (age of MG onset was less than 15 years) were collected from Department of Neurology, Beijing Tongren Hospital (January 2000 - February 2010). The patients were non-randomly divided according to their treatment into 3 groups (glucocorticoid, thymectomy and glucocorticoid combined with thymectomy groups). Postintervention status meeting the criteria of Myasthenia Gravis Foundation of America (MGFA) "complete stable remission, CSR", "pharmacologic remission, PR", "minimal manifestations, MM", or "Improved, I" was regarded as desirable response, which was used as primary indicator of observation. The authors assessed the efficacy of three therapies and analyzed the influencing factors of prognosis by using Chi-square test and Logistic regression.</p><p><b>RESULT</b>At 3 months of treatment, glucocorticoid group showed the highest effective rate. At the end of 1 year or 2 years of treatment, glucocorticoid combined with thymectomy group showed the highest effective rate respectively. The generalization rate of MG at 2 years, 10 years and 20 years in childhood-onset ocular MG patients were 4.3%, 10.7%, and 41.5%, respectively. Of patients with generalization of MG, 48.1% occurred within 2 years, 92.6% within 20 years. Univariate analysis showed that in childhood-onset ocular MG patients, variables such as age at onset (> 10 years), LG-MG and with chronic fatigue were significantly associated with general MG conversion. Whereas multivariate analysis showed that patients with age at onset (> 10 years) and chronic muscle fatigue were apt to convert to generalized MG.</p><p><b>CONCLUSION</b>Glucocorticoid appeared to have an effect that leads to early remission of symptoms in childhood-onset MG patients and glucocorticoid combined with thymectomy appeared to have better long-term effect. For those childhood-onset ocular MG patients with longer course of disease, older age of onset, chronic fatigue, or LG-MG, physicians should try to prevent the generalization of MG by immunosuppressive therapies.</p>

Application of informant questionnaire on cognitive decline in the elderly in the screening of cognitive impairment in the elderly / 中华老年医学杂志

Objective To evaluate the reliability and validity of informant questionnaire on cognitive decline in the elderly(IQCODE) in Chinese elderly patients and to estimate the efficacy of IQCODE in the screening of patients with cognitive impairment.Methods Ninety three patients with cognitive impairment and 128 community-dwelling healthy elderly over 60 years old were assessed.Informants were interviewed with the short version of the IQCODE.Results The internal consistency coefficient of IQCODE was 0.83.The correlation coefficient of test-retest reliability was 0.86.The validity coefficient of IQCODE with mini-mental state examination and activities of daily living were-0.78 and 0.71 respectively.The scores of IQCODE of the patients varied with the degree of cognitive deficits,and the differences had statistical significance.But there was no significant difference among different types of senile dementia.The scores of IQCODE of the patients with cognitive impairment were closely correlated with the degree of cognitive deficits.Conclusions IQCODE is a reliable and effective measurement for the patients with cognitive impairment.It could discriminate the cognitive impairment patients from the normal cognitive ones,and is practical in screening.But it can not distinguish different kinds of dementia.