中国进行性脊肌萎缩（PMA）患者的神经心

PLoSOne.2015Jun4;10(6):i:10.1371/ne..

NeuropsychologicalInvestigationinChinesePatientswithProgressiveMuscularAtrophy.

CuiB1,CuiL2,LiuM1,LiX1,MaJ1,FangJ1,DingQ1.

Authorinformation

1DepartmentofNeurology,PekingUnionMedicalCollegeHospital,ChineseAcademyofMedicalSciences,Beijing,China.

2DepartmentofNeurology,PekingUnionMedicalCollegeHospital,ChineseAcademyofMedicalSciences,Beijing,China;NeurosciencesCenter,ChineseAcademyofMedicalSciences,Beijing,China.

Abstract

BACKGROUND:

Progressivemuscularatrophy(PMA)isararetypeofdegenerativemotorneurondisease(MND)spiteitswell-definedclinicalcharacteristics,itsneuropsychologicalprofilehasremainedpoorlyunderstood,consideringtheconsensusofcognitiveandbehavioralimpairmentreachedinamyotrophiclateralsclerosis(ALS).

METHODS:

Weconductedacross-sectionalevaluationofChinesePMApatientswithaseriesofprehensivebatteriesemphasizingtheexecutiveandattentionfunction,andcoveringotherdomainsofmemory,language,visuospatialfunction,eirperformanceswereparedwiththoseofage-andeducation-matchedALSandhealthycontrols(HC).

RESULTS:

21patientsnewlydiagnosedwithPMAwereconsecutivelyenrolledintoourALSandotherMNDregistryplatform,accountingfor14.7paredwithHC,PMAperformedsignificantlyworseinmaintenancefunctionofattention,whiletheyexhibitedquantitativesimilaritytoALSinallbehavioralinventoriesandneuropsychologicaltestsexceptthetimeforStroopinterferenceeffect.

CONCLUSION:

PMAcoulddisplaymildcognitivedysfunctioninthesamefrontal-mediatedterritoryofALSbutinalesserdegree,whereastheydidnotdifferfromALSbehaviorally.

PMID: