Cognitive changes and cranial MRI changes in patients with myotonic dystrophy type 1: a study of 4 cases
10.3760/cma.j.issn.1671-8925.2012.05.016
- VernacularTitle:四例强直性肌营养不良1型患者认知功能及头颅MRI研究
- Author:
Qing-Hua HOU
1
;
Yi XU
;
Gui-Hua JIANG
;
Wen-Feng ZHAN
;
Hai-Lang HUANG
;
Hai-Run LIU
;
Long-Chang XIE
;
Qing-Chun GAO
Author Information
1. 广州医学院附属第二医院
- Keywords:
Myotonic dystrophy type 1;
Intelligent test;
Cognition function disorder;
Magnetic resonance imaging
- From:
Chinese Journal of Neuromedicine
2012;11(5):502-507
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the cognitive, intelligent and cranial MRI status of 4 patients with myotonic dystrophy type 1 (DM1). Methods A pedigree of 4 patients,admitted to our hospital from September 2011 to December 2011 and diagnosed as having DM1, were chosen in our study; Chinese edition tests of Wechsler adult intelligence scale and Wechsler memory scale were used to test the intelligence of these patients; Montreal cognitive assessment (MoCA) and Neurobehavioral cognitive status examination (NCSE) were applied to evaluate the patients' cognitive function.All of the 4 patients received a cranial MRI scan. Results Except for 1 patient,the other 3 patients had low intelligent quotient (IQ) and memory quotient (MQ),and their verbal IQs were all lower than performance IQs.Three patients showed impaired visual space/executive skills,calculation,memory,and conceptual thinking abilities; 1 patient showed impaired sentence repetition and verbal fluency on MoCA,though his final scores were within normal range. Three patients had brain atrophy to some extent on MRI scan,prominently in the frontal lobe and temporal lobe; white matter lesions and thickened skull were founded in all 4 patients. Conclusion Multiple cognition disorders and brain involvements can be found in DM1 patients and deserve further investigation.
