Duchenne's muscular dystrophy

Duchenne's muscular dystrophy

Etymology: Guillaume B.A. Duchenne

an abnormal congenital condition characterized by progressive symmetric wasting of the leg and pelvic muscles. This disease predominantly affects males and accounts for 50% of all muscular dystrophy diseases. It is an X-linked recessive disease that appears insidiously between 3 and 5 years of age and spreads from the leg and pelvic muscles to the involuntary muscles. Associated muscle weakness produces a waddling gait and pronounced lordosis. Muscles rapidly deteriorate, and calf muscles become firm and enlarged as a result of fatty deposits. Affected children experience contractures, have difficulty climbing stairs, often stumble and fall, and display winged scapulae when they raise their arms. Such persons are usually confined to wheelchairs by 12 years of age, and progressive weakening of cardiac muscle causes tachycardia and pulmonary problems. The patients affected may also have cardiac murmurs, faint heart sounds, and chest pain and may suffer arrhythmias or infections that produce overt heart failure. Such complications, especially in the later stages of this disease, can cause sudden death. Duchenne's muscular dystrophy usually causes death within 10 to 15 years of symptom onset. There is no successful treatment of the disease. Orthopedic appliances, exercise, physical therapy, and surgery to correct contractures can help preserve mobility. Nursing care involves psychologic support of the patient and family and encouragement of the patient to prevent long periods of bed rest and inactivity and ensure maximum physical activity. Splints, braces, grab bars, and overhead slings help the patient exercise. A wheelchair helps preserve mobility. Other devices that can increase comfort and help prevent footdrop include footboards, high-topped sneakers, and foot cradles. Also called pseudohypertrophic muscular dystrophy.

---

dystrophy [dis´trah-fe]

any disorder due to defective or faulty nutrition, especially muscular dystrophy. adj., adj dystroph´ic.

adiposogenital dystrophy adiposity of the feminine type, genital hypoplasia, changes in secondary sex characters, and metabolic disturbances; seen with lesions of the hypothalamus; see also adiposogenital dystrophy.

Becker's muscular dystrophy (Becker type muscular dystrophy) a form closely resembling Duchenne's muscular dystrophy, but having a later onset and milder course; transmitted as an X-linked recessive trait.

distal muscular dystrophy distal myopathy.

Duchenne's muscular dystrophy (Duchenne type muscular dystrophy) The childhood type of muscular dystrophy.

facioscapulohumeral muscular dystrophy muscular dystrophy affecting the face, shoulder, and upper arm muscles; called also Landouzy-Dejerine muscular dystrophy.

Landouzy-Dejerine dystrophy (Landouzy-Dejerine muscular dystrophy) facioscapulohumeral muscular dystrophy.

muscular dystrophy see muscular dystrophy.

myotonic dystrophy a rare, slowly progressive, hereditary disease, marked by myotonia followed by muscular atrophy (especially of the face and neck), cataracts, hypogonadism, frontal balding, and cardiac disorders. Called also dystrophia myotonica, myotonia atrophica, and myotonia dystrophica.

progressive muscular dystrophy muscular dystrophy.

pseudohypertrophic muscular dystrophy muscular dystrophy affecting the shoulder and pelvic girdles, beginning in childhood and marked by increasing weakness, pseudohypertrophy of the muscles, followed by atrophy, and a peculiar swaying gait with the legs kept wide apart. Called also pseudohypertrophic muscular paralysis.

reflex sympathetic dystrophy a syndrome of chronic pain that usually develops after a trauma or noxious stimulus, although the nerve injury cannot be immediately identified. The pain is not limited to the distribution of a single nerve and is often out of proportion to the precipitating event. It is most often described as a burning pain, and is accompanied by swelling, sweating, sensitivity to touch, and sometimes changes in tissue growth. Called also chronic or complex regional pain syndrome. Clinical practice guidelines have been published by the Reflex Sympathetic Dystrophy Syndrome Association of America and are available on their web site at http://www.rsds.org or by writing to Reflex Sympathetic Dystrophy Syndrome Association of America, P.O. Box 502, Milford CT 06460.

---

Duchenne's muscular dystrophy,

n an X-linked recessive condition pres-ent at birth in which the muscles of the pelvis and legs waste away in a symmetric fashion. Appears in children between three to five years of age, with death occurring 10 to 15 years after the onset of symtoms. Also called
pseudohypertrophic muscular dystrophy.

Duchenne's muscular dystrophy.