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ID 115572
Author
Adachi, Katsuhito National Hospital Organization Tokushima Hospital
Hashiguchi, Shuji National Hospital Organization Tokushima Hospital
Saito, Miho National Hospital Organization Tokushima Hospital
Kashiwagi, Setsuko National Hospital Organization Tokushima Hospital
Miyazaki, Tatsushi National Hospital Organization Tokushima Hospital
Kawai, Hisaomi Hiwasa Hospital
Kobayashi, Michio National Hospital Organization Akita Hospital
Ishizaki, Masatoshi National Hospital Organization Kumamoto-Minai Hospital
Matsumura, Tuyoshi National Hospital Organization Toneyama Hospital
Mori-Yoshimura, Madoka National Center of Neurology and Psychiatry
Kimura, En National Center of Neurology and Psychiatry
Keywords
Female dystrophinopathy carrier
Cardiomyopathy
Cardiac magnetic resonance imaging
Late gadolinium enhancement
Speckle tracking echocardiography
Content Type
Journal Article
Description
Regular health checkups for mothers of patients with Duchenne muscular dystrophy have been performed at National Hospital Organization Tokushima Hospital since 1994. Among 43 mothers participated in this study, 28 dystrophinopathy carriers were identified. Skeletal and cardiac muscle functions of these subjects were examined. High serum creatine kinase was found in 23 subjects (82.1%). Obvious muscle weakness was present in 5 (17.8%) and had progressed from 1994 to 2015. Cardiomyopathy was observed in 15 subjects (60.0%), including dilated cardiomyopathy-like damage that was more common in the left ventricular (LV) posterior wall. Late gadolinium enhancement on cardiac MRI was found in 5 of 6 subjects, suggesting fibrotic cardiac muscle. In speckle tracking echocardiography performed seven years later, global longitudinal strain was decreased in these subjects, indicating LV myocardial contractile abnormality. These results suggest that female dystrophinopathy carriers should receive regular checkups for detection and treatment of cardiomyopathy, even if they have no cardiac symptoms.
Journal Title
Journal of the Neurological Sciences
ISSN
0022510X
NCID
AA00703265
AA11534024
Publisher
Elsevier
Volume
386
Start Page
74
End Page
80
Published Date
2017-12-21
Rights
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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DOI (Published Version)
URL ( Publisher's Version )
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language
eng
TextVersion
Publisher
departments
University Hospital
Medical Sciences