|
Disclaimer:
Whonamedit.com does not give medical advice.
This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor.
|
A recommendation:
Hypography is an open community about science and all things related
|
|
|
Duchenne-Griesinger disease
Also known as:
Duchenne de Boulogne muscular dystrophy
Duchenne's muscular dystrophy
Duchenne's myodystrophy
Duchenne's pseudohypertrophic muscular dystrophy
Duchenne's syndrome
Griesinger's disease or syndrome
Associated persons:
Guillaume Benjamin Amand Duchenne de Boulogne
Wilhelm Griesinger
Description:
Condition characterized by weakness and pseudohypertrophy of the affected muscles. The disease begins in childhood, usually between 2 and 6 years, is progressive, and affects the shoulder and pelvic girdle muscles. Waddling gait, frequent falling, clumsiness and Gower’s manoeuvre to arise when lying on the floor, contractures develop. Frequent respiratory infections. Usually patients become incapacitated and are confined to wheelchair by adolescent age. Etiology Unknown. Sex-linked recessive inheritance. Affects only males, with an occurrence of 1 of 4800 boys born (see also Duchenne’s dystrophy in females). However, girls with Turner’s syndrome may acquire the disease. Death usually occurs at an early age, few patients survive past the second decade of life. A clinically similar autosomal recessive disease affecting both sexes equally occurs in the Mediterranean countries.
The British neurologist Sir William Richard Gowers (1845-1915) in 1879 recognised that only boys are affected, and that the disease is transmitted through healthy mothers to their sons. The German neurologist Wilhelm Heinrich Erb in 1890/1891 presented an extensive description of the clinical picture and histopathological findings.
Bibliography:
- W. Griesinger:
Über Muskelhypertrophie.
Archiv der Heilkunde, Leipzig, 1865, 6: 1-13.
- G. B. Duchenne:
Recherches sur la paralysie musculaire pseudo-hypertrophique, ou paralysie myo-sclérosique. Archives générales de médecine, Paris, 6 sér, 1868, 11: 5-25, 179-209, 305-321, 421-443, 552-588.
English translation of first portion in Bick, Classics of orthopaedics, 72-75.
- W. R. Gowers:
Pseudo-hypertrophic muscular paralysis. London, 1879.
|
|
|
