Any disorder in which the structure and normal activity of cells within a tissue have been disrupted by inadequate nutrition. The usual cause is poor circulation of blood through the tissue, but dystrophy can also be due to nerve damage or deficiency of a specific enzyme in the tissue. Examples include muscular dystrophies and leukodystrophies. Corneal dystrophies, in which cells lining the cornea are damaged, are a rare cause of blindness.
Dystrophy means defective or faulty nutrition, and is a term applied to a group of developmental changes occurring in the muscles, independently of the nervous system (see MUSCLES, DISORDERS OF). The best-known form is progressive muscular dystrophy, a group of hereditary disorders characterised by symmetrical wasting and weakness, with no sensory loss. There are three types: Duchenne (usually occurring in boys within the ?rst three years of life); limb girdle (occurring in either sex in the second or third decade); and facio-scapulohumeral (either sex, any age). The three types have di?erent prognoses, but may lead to severe disability and premature death, often from respiratory failure. The third type progresses very slowly, however, and is compatible with a long life.
Diagnosis may be con?rmed by ELECTROMYOGRAPHY (EMG) or muscle biopsy. Although genetic research is pointing to possible treatment or prevention, at present no e?ective treatment is known, and deterioration may occur with excessive con?nement to bed. Physio-therapeutic and orthopaedic measures may be necessary to counteract deformities and contractures, and may help in coping with some disabilities.
(dystrophia) n. a disorder of an organ or tissue, usually muscle, due to impaired nourishment of the affected part. The term is applied to several unrelated conditions; for example, *muscular dystrophy, *Fuchs’ endothelial dystrophy, and dystrophia adiposogenitalis (see Fröhlich’s syndrome).
a *sex-linked (X-linked) disorder in which affected males develop an increase in muscle size followed by weakness and wasting. It usually starts between the ages of 5 and 15, and 25 years after onset most patients are wheelchair-bound. Although most men become severely disabled, life expectancy is close to normal. The disorder is similar to Duchenne *muscular dystrophy but less severe. [P. E. Becker (20th century), German geneticist]... becker muscular dystrophy
a hereditary condition in which the corneal endothelium loses its functional ability, usually with age. It may result in thickening and swelling of the cornea (*bullous keratopathy) and thus affect vision. Cornea guttata, small whitish deposits of hyalin, are seen on the inner surface of the cornea and signify a reduced number of endothelial cells. A corneal transplantation (see keratoplasty) may become necessary in certain cases. [E. Fuchs (1851–1913), German ophthalmologist]... fuchs’ endothelial dystrophy
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