Biomaterial Database

Congenital muscular dystrophy of the Fukuyama type (FCMD) with severe myocardial fibrosis. A case report with postmortem angiography. 1987

K Miura, and H Shirasawa

Department of Pathology, Hamamatsu University School of Medicine, Japan.

Autopsy findings of a 17-year-old Japanese male with the typical features of congenital muscular dystrophy of the Fukuyama type (FCMD) are presented. This case showed progressive muscular dystrophy and central nervous system malformation, accompanied by severe myocardial fibrosis. Large mitochondrial dense bodies were found in both the cardiac and skeletal muscle cells. Postmortem coronary angiography failed to reveal the presence of any obstructive lesions responsible for myocardial fibrosis. It was also demonstrated that abnormal vessels proliferating in an area of micropolygyria (MPG) in the cerebrum were branches of the cortical artery originating from the pia mater. The cerebral malformation was accompanied by basal skull deformities.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009132	Muscles	Contractile tissue that produces movement in animals.	Muscle Tissue,Muscle,Muscle Tissues,Tissue, Muscle,Tissues, Muscle
D009136	Muscular Dystrophies	A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS.	Muscular Dystrophy,Myodystrophica,Myodystrophy,Dystrophies, Muscular,Dystrophy, Muscular,Myodystrophicas,Myodystrophies
D009206	Myocardium	The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.	Muscle, Cardiac,Muscle, Heart,Cardiac Muscle,Myocardia,Cardiac Muscles,Heart Muscle,Heart Muscles,Muscles, Cardiac,Muscles, Heart
D001921	Brain	The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.	Encephalon
D005355	Fibrosis	Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.	Cirrhosis,Fibroses
D005837	Genitalia, Male	The male reproductive organs. They are divided into the external organs (PENIS; SCROTUM; and URETHRA) and the internal organs (TESTIS; EPIDIDYMIS; VAS DEFERENS; SEMINAL VESICLES; EJACULATORY DUCTS; PROSTATE; and BULBOURETHRAL GLANDS).	Accessory Sex Organs, Male,Genital Organs, Male,Sex Organs, Accessory, Male,Genitals, Male,Reproductive System, Male,Genital, Male,Male Genital,Male Genital Organs,Male Genitalia,Male Genitals,Male Reproductive System,Male Reproductive Systems,Reproductive Systems, Male
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000792	Angiography	Radiography of blood vessels after injection of a contrast medium.	Arteriography,Angiogram,Angiograms,Angiographies,Arteriographies