| Kuo HC |
------>authors3_c= ------>paper_class1=1 ------>Impact_Factor=1.464 ------>paper_class3=2 ------>paper_class2=1 ------>vol=27 ------>confirm_bywho=wingchan ------>insert_bywho=ed100975 ------>Jurnal_Rank=63.7 ------>authors4_c= ------>comm_author= ------>patent_EDate=None ------>authors5_c= ------>publish_day=1 ------>paper_class2Letter=None ------>page2=296 ------>medlineContent= ------>unit=E0119 ------>insert_date=20081223 ------>iam=3 ------>update_date=None ------>author=??? ------>change_event=4 ------>ISSN= ------>authors_c= ------>score=500 ------>journal_name=Brain Dev ------>paper_name=Brain magnetic resonance image changes in a family with congenital and classic myotonic dystrophy ------>confirm_date=20081224 ------>tch_id=097094 ------>pmid=15862193 ------>page1=291 ------>fullAbstract=We present the clinical manifestations, brain magnetic resonance images (MRI), and genetic analysis of a family with 2 siblings with congenital myotonic dystrophy type 1 (DM1) and 4 patients with classic DM1. These 2 patients with congenital DM1 had severe mental retardation and a characteristic feature of hyperintensity of white matter at the posterior-superior trigone (HWMPST), in addition to ventricular dilatation in T2-weighted images (T2WI) of brain MRI. In 2 of the 4 classic DM1 patients, brain T2WI MRI showed hyperintensity lesions in the bilateral frontal and/or temporal regions, which were absent in congenital DM1. In conclusion, we suggest that the HWMPST in brain MRI is a characteristic finding in congenital DM1, and that the severe cognitive impairments are not only attributable to the subcortical white matter lesions. In congenital DM1, the cognitive function is a diffuse impairment, which is different from that in classic DM1. ------>tmu_sno=None ------>sno=20925 ------>authors2=Hsiao KM ------>authors3=Chen CJ ------>authors4=Hsieh YC ------>authors5=Huang CC ------>authors6= ------>authors6_c= ------>authors=Kuo HC ------>delete_flag=0 ------>SCI_JNo=None ------>authors2_c= ------>publish_area=0 ------>updateTitle=Brain magnetic resonance image changes in a family with congenital and classic myotonic dystrophy. ------>language=2 ------>check_flag=None ------>submit_date=None ------>country=None ------>no=4 ------>patent_SDate=None ------>update_bywho=None ------>publish_year=2005 ------>submit_flag=None ------>publish_month=6 |