A Case of Nemaline Myopathy With Associated Dilated Cardiomyopathy and Respiratory Failure

Rihito Nagata; Daisuke Kamimura; Yoji Suzuki; Toshihiko Saito; Hideshi Toyama; Tohru Dejima; Haruhiko Inada; Yoshiyuki Miwa; Kazuaki Uchino; Satoshi Umemura; Makoto Shimizu

doi:10.1536/ihj.52.401

Case Reports

A Case of Nemaline Myopathy With Associated Dilated Cardiomyopathy and Respiratory Failure

Rihito Nagata, Daisuke Kamimura, Yoji Suzuki, Toshihiko Saito, Hideshi Toyama, Tohru Dejima, Haruhiko Inada, Yoshiyuki Miwa, Kazuaki Uchino, Satoshi Umemura, Makoto Shimizu

Author information

Rihito Nagata
Department of Cardiology, Yaizu City Hospital
Daisuke Kamimura
Department of Cardiology, Yaizu City Hospital
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine
Yoji Suzuki
Department of Neurology, Yaizu City Hospital
Toshihiko Saito
Department of Cardiology, Yaizu City Hospital
Hideshi Toyama
Department of Cardiology, Yaizu City Hospital
Tohru Dejima
Department of Cardiology, Yaizu City Hospital
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine
Haruhiko Inada
Department of Cardiology, Yaizu City Hospital
Yoshiyuki Miwa
Department of Cardiology, Yaizu City Hospital
Kazuaki Uchino
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine
Satoshi Umemura
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine
Makoto Shimizu
Department of Cardiology, Yaizu City Hospital

Keywords: Nemaline myopathy, Dilated cardiomyopathy, Respiratory failure, Heart failure, Noninvasive positive pressure ventilation

JOURNAL FREE ACCESS

2011 Volume 52 Issue 6 Pages 401-405

DOI https://doi.org/10.1536/ihj.52.401

Details

Abstract

Nemaline myopathy is a representative form of congenital myopathy, and is characterized by nemaline bodies in muscle fibers. Here we report a 47-year-old man with congenital nemaline myopathy complicated with dilated cardiomyopathy-related heart failure, and restrictive respiratory failure. The complication of dilated cardiomyopathy in nemaline myopathy has rarely been reported. In this case, nemaline bodies were detected in the cardiac muscle fibers, demonstrating the presence of underlying disease-related myocardial degeneration. The patient responded to the combination of conventional therapy for heart failure including β-blocker and noninvasive continuous positive-pressure ventilation for respiratory failure. His general condition has been stable during a 10-month follow up period.

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