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Objective To develop new scales on the severity of myasthenia gravis ( MG) which are suitable for clinical practice in China .Methods A final version of new comprehensive scale was developed by item optimization from a preliminary scale established by combination of Delphi method and previous evaluation of items from source scales .With the original quantitative data from 60 MG patients, items were selected on the basis of interobserver and test-retest reliability of items , the contributions of items on internal consistency and construct validity of the preliminary scale .Evaluation of this final scale includes internal consistency, interobserver and test-retest reliability, construct validity and correlation with currently used scales.A self-rating version was simplified from the final scale by including items that can be measured by patients, which was then evaluated in 57 generalized MG patients.Results One item ( palpebral fissure ) was deleted from the preliminary scale due to poor interobserver and test-retest reliability.The remaining nine items constituted the final comprehensive scale which covered six commonly involved muscle groups in MG.All items in the comprehensive scale had fair to excellent interobserver reliability ( K=0.43-0.80, P<0.01) and fair to excellent test-retest reliability ( K =0.41 -1.00, P<0.01) .The comprehensive scale had acceptable internal consistency (Cronbach ɑ=0.63), excellent interobserver reliability (intraclass correlation coefficient (ICC) =0.88, P<0.01) and test-retest reliability (ICC=0.86, P<0.01).The comprehensive scale had four common factors and good construct validity ( cumulative dedication rate 68.46％).There was high correlation between the comprehensive scale and Myasthenia Gravis Composite ( MGC ) , Absolute and Relative Score of MG ( ARS-MG ) and Quantitative Myasthenia Gravis score (QMGs) ( r=0.87 -0.90, P<0.01), and moderate correlation between the comprehensive scale and Myasthenia Gravis Activities of Daily Living (MG-ADL) (r=0.68, P<0.01).A self-rating scale was developed by deleting ocular and facial items and keeping the remaining six items , which covers the bulbar , limb, neck and respiratory muscles.Internal consistency (Cronbach ɑ=0.69), interobserver reliability and test-retest reliability (ICC=0.90 for both, P<0.01) of the self-rating scale were better than those of the comprehensive scale.The self-rating scale had two common factors and good construct validity ( cumulative dedication rate 64.24％) .There was high correlation between the self-rating scale and the comprehensive scale, MGC and ARS-MG (r=0.74-0.86, P<0.01), and moderate correlation between the self-rating scale and QMGs and MG-ADL ( r=0.55 -0.68, P<0.01 ) .Conclusions Item optimization was performed from a preliminary scale established by Delphi method .A new comprehensive scale and a self-rating scale were developed .The comprehensive scale and self-rating scale had good reliability and validity , which can be used to assess the severity of MG reliably and effectively .
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Objective To explore the associations between vitamin D receptor ( VDR) Fok- Ⅰ and Apa- Ⅰ polymorphisms and myasthenia gravis (MG) in Chinese Han population.Methods Polymorphisms of VDR Fok- Ⅰ and Apa-Ⅰ were determined by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) method.The frequencies of genotypes and hyplotypes were compared among 286 normal controls and 267 MG patients in different subgroups classified by gender,age of onset,presence of thymoma,and Osserman classification sat maximal severity in the follow-up.The association between the genotypes and maximal severity of MG and short-term glucocorticoid treatment were also investigated.Results There were no significant differences in frequencies of genotypes and hyplotypes of both Fok-Ⅰ and Apa-Ⅰ between MG group and control group,and among subgroups of MG.The Fok- Ⅰ showed no statistical difference between the patients with better and less improvement after short-term treatment of glucocorticoid.The frequency of Apa-Ⅰ alleles in the patients with better improvement (55/186,29.6% ) significantly differed from the less improved group ( 7/48,14.6%,OR = 2.46,95% CI 1.04-10.43,x2 = 4.400,P = 0.036).The patients with the genotype A A/Aa were more likely to improve better after the treatment(48/93,51.6%) than in the worse group(7/24,29.2%,OR =2.59,95% CI 0.98-14.60,x2 =3.858,P= 0.049).Conclusions Alleles and genotypes of VDR-Fok- Ⅰ and Apa-Ⅰ were not found to be related with MG onset and severity.MG patients with VDR-Apa-A allele may have better improvement short-term treatment of glucocorticoid.
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Objective:To investigate the imaging and clinical feature of leukoencephalopathy with hearing loss first complaint.Method:The head MRI and clinical feature of 7 cases leukoencephalopathy with hearing loss first complaint were retrospectively analysed,including their place,shape,range and blood plasma very-long-chain fatty acid(VLCFA).Result:The head MRI of 2 cases shows symmetry distributed long T_1 and T_2 signal like butterfly aliform in white matter beside both cornu posterious ventriculi lateralis.The head MRI of 5 cases shows abnormal long T_1 and T_2 signal in different part in Alba.The pathological changes often located in white matter of frontal lobe and secondly beside both eornu posterious ventriculi lateralis.The shape shows little patching in 7 cases.The range only shows beside cornu posterious ventriculi lateralis in 2 cases.The range only shows in frontal lobe in 1 cases.Two or more than two parts shows in the rest 5 cases.The result of VLCFA is normal in 3 cases.The rest 4 cases refused to have examination of VLCFA. Conclusion:The patient who firstly complained of hearing loss should be routinely done head MRI.The head MRI imaging could clearly show abnormal white matter in order to avoid omitting existent pathological changes.
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OBJECTIVE@#To investigate the imaging and clinical feature of leukoencephalopathy with hearing loss first complaint.@*METHOD@#The head MRI and clinical feature of 7 cases leukoencephalopathy with hearing loss first complaint were retrospectively analysed, including their place, shape, range and blood plasma very-long-chain fatty acid (VLCFA).@*RESULT@#The head MRI of 2 cases shows symmetry distributed long T, and T2 signal like butterfly aliform in white matter beside both cornu posterior ventriculi lateralis. The head MRI of 5 cases shows abnormal long T1 and T2 signal in different part in Alba. The pathological changes often located in white matter of frontal lobe and secondly beside both cornu posterior ventriculi lateralis. The shape shows little patching in 7 cases. The range only shows beside cornu posterior ventriculi lateralis in 2 cases. The range only shows in frontal lobe in 1 cases. Two or more than two parts shows in the rest 5 cases. The result of VLCFA is normal in 3 cases. The rest 4 cases refused to have examination of VLCFA.@*CONCLUSION@#The patient who firstly complained of hearing loss should be routinely done head MRI. The head MRI imaging could clearly show abnormal white matter in order to avoid omitting existent pathological changes.
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Adolescent , Child , Child, Preschool , Female , Humans , Male , Hearing Loss , Diagnosis , Leukoencephalopathies , Diagnosis , Magnetic Resonance Imaging , Retrospective Studies , Tomography, X-Ray ComputedABSTRACT
0.05).Conclusion:The lymphoid tissues can not only express the ACTH-R?5-HT_ 1A-R protein but also synthesize their mRNA.ACTH and 5-HT can regulate the functions of the immune system through their receptors on the membrane of the immunocytes.
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Objective To investigate the change of adhesive properties of polymorphonuclear neutrophils(PMN) and endothelial cells (EC) in patients with cerebral infarction (CI), and define the effects of antibodies to intercellular adhesion molecular 1 (ICAM 1, anti CD54 antibodies) upon the adhesion.Methods We detected the adhesive rate between human umbilical vein endothelial cells (ECV 304) and PMN of patients with CI within 1 week and at 21 days.Results (1) The adhesive rate of ECV 304 to PMN of 30 patients with CI within 1 week increased significantly ( P