Guillaume Benjamin Amand Duchenne de Boulogne
- Aran-Duchenne spinal muscular atrophy
- Becker's muscular dystrophy
- Duchenne's disease
- Duchenne's dystrophy in females
- Duchenne's posture
- Duchenne's syndrome
- Duchenne's trocar
- Duchenne-Erb paralysis
- Duchenne-Griesinger disease
- Trendelenburg's symptom
Biography of Guillaume Benjamin Amand Duchenne de Boulogne
Life and career
Guillaume Benjamin Amand Duchenne descended from a family of fishermen, traders and sea captains who had resided in the harbour city Boulogne-sur-Mer in Northern France since the first half of the 18th century. He was predestined for a career at seas, as his father was the commander Jean Duchenne who had been a ships captain during the Napoleonic wars, and expected his son to follow in his keel waters.
Despite his father’s efforts to induce him to follow the family seafaring tradition, however, it was his love of science that prevailed. Duchenne went to a local college at Douai, where he received his baccalauréat at the age of 19. From 1827, aged twenty-one, he studied medicine under teachers like René-Théophile-Hyacinthe Laënnec (1781-1826), Baron Guillaume Dupuytren (1777-1835), François Magendie (1783-1855), and Léon Cruveilhier (1791-1874). He graduated in medicine in Paris in 1831 and, probably influenced by Dupuytren, presented his Thèse de médecine, a monograph on burns.
Duchenne had an undistinguished undergraduate career and as he failed to obtain an academic post, and because of his father’s death he returned to Boulogne where he practised medicine for ten years. Duchenne married in 1831, but his wife died in childbirth already to years later - of puerperal fever. Duchenne’s mother in law spread rumours that the death of his wife was caused by the fact that only he was present at the delivery, and after this he was separated from his only son by his wife’s family.
As a consequence of this his patients the following years avoided his previously blossoming practice. After a new short and unhappy marriage he returned to Paris penniless in 1842 in order to pursue his ambition of doing medical research. His interest was particularly directed towards electropuncture, which had recently been invented by Magendie and Jean-Baptiste Sarlandière (1787-1838).
In Paris Duchenne met with a rather cool reception, being ridiculed for his provincial accent and his course manners. He was known under the name of Duchenne de Boulogne to avoid confusion with Édouard Adolphe Duchesne (1894-1869), a fashionable society physician. Having arrived alone and without funds, he set to work in charity clinics and hospitals, and gained his livelihood from private practice, which presumably became adequate to supply his limited needs. He seemed to live only for his patients and his scholarship, pursuing his clinical neurological studies in a very unorthodox but effective fashion. He was described perhaps somewhat romantically as a strange mariner-like figure that every morning used to haunt one or two major Parisian teaching hospitals in order to study the most interesting cases, then to make them an object of his electrotherapeutic detailed studies.
Duchenne was a diligent investigator and meticulous at recording clinical histories. When necessary he would follow his patients from hospital to hospital to complete his studies. In this way he achieved an exceptionally rich and exquisite research material, to a degree far superior to that available to a single clinician and hospital physician.
The lone wolf and the monarchs of the ward
Because of resistance from the established medical circles Duchenne was never offered, and never applied for, an appointment at a Paris teaching hospital or at the university. He probably avoided such positions of fear that it might inhibit his self-imposed activity.
Duchenne was a lonely figure at the wards of the Paris hospitals, mocked by the interns and rebuffed by the senior medical staff, whom he called «monarchs» of the ward». However, he demonstrated an enormous personal courage and a singleness of purpose, and soon obtained a reputation as an outstanding neurologist and thus achieved some degree of academic recognition. The absence of daily hospital routines gave him the time and opportunity to develop his interest in muscular diseases, electrodiagnostics and electrical stimulation. However unambitious Duchenne may have been, he was frequently involved in disputes concerning forms of diseases that he had discovered, and he was never short of enemies and protagonists.
Toward the end of his life Duchenne became established and popular, among others with Jean-Martin Charcot (1825-1893), at the Hôtel de Salpêtrière. Charcot was among Duchenne’s friends and they held each other in considerable esteem. His clinical ability was such that the great Charcot dubbed him «The Master». Despite his lack of recognition in France, Duchenne at this stage of his career had become an international celebrity.
Besides his daily round tour of the Paris hospitals, Duchenne had a free reception for the indigenous twice a week, and every Monday he had a thorough discussion with students and foreign physicians. Every month he gave several dinner parties for his colleagues, among them Charcot, Pierre Paul Broca (1824-1880), Auguste Nélaton (1807-1873), and Edmé Félix Alfred Vulpian (1826-1887). During these come togethers histological slides were projected and discussed - mixed with funny pictures to please Duchenne’s grandchild.
In the early 1870’s Duchenne made several journeys, being received at the court of Queen Victoria in England and the court of Philip IV in Spain. He lived a quiet and lonesome life, completely dedicated to one task. He died of a haemorrhagic bleeding in 1875, following several years of illness.
The great pioneer of electro physiology
More than any other person of his day Duchenne was responsible for developing the technique of the meticulous neurological examination, and took an early interest in electrophysiology. In 1835 he happened to try electric stimulation of a patient with neuralgia. The scientist that had previously tried this had been scared off, and there was a lot of charlatanry.
Duchenne built his own machine for neuromuscular stimulation and developed the technique of using surface electrodes. Previous electric stimulation had caused a lot of tissue damages. The power source, portable in order to be usable at bedside, consisted of a coal-zinc battery. Exactly how the stimulation was performed is not known, but it resulted in either repeated contractions or tetanus.
Duchenne discovered that external electrical stimulation could cause muscle movements and initially used this as a form of therapy, but then appreciated its possibilities as a diagnostic method. He employed this technique to analyse the mechanism of facial expression, which was published and illustrated by many striking photographs. He was one of the first to use photographs to illustrate disease processes.
Duchenne separated between «indirect stimulation via the nerve» and direct stimulation of the muscle. He also performed electro stimulation of rectum and the bladder in incontinence and of uterus in amenorrhoea. The first results were described in a report to the Académie de Médecine in Paris in 1848.
It was largely Duchenne who introduced the painstaking and at times flamboyant physical examination and interrogation, which has become the stamp of all modern neurologists. He would cap a satisfactory answer to one of his queries or performance of a neurological manoeuvre with an explosive «bon».
Duchenne laid the foundation for Robert Remak’s (1815-1865) and Hugo Wilhelm von Ziemssen’s (1829-1902) subsequent work in physical diagnostics. It is therefore strange that the French school remained so discouraging to the great advances achieved in this field in Germany, and Duchenne ended up in an aggressive polemics with Remak.
Duchenne was probably the first person to use biopsy procedure to obtain tissue from a living patient for microscopic examination. This aroused a deal of controversial discussion in the lay press concerning the morality of examining living tissues. In order to perform histopathological diagnostics Duchenne constructed a biopsy needle, which made possible percutaneous muscle biopsies without anaesthesia.
«I have tried to create a sort of anatomy of the living (anatomie vivante). I have defined precisely the isolated and individual action of every muscle using exact methods. It is not for me to judge the value of these investigations, but I dare say that if my results are corroborated, muscular physiology will have a whole new look.» Later Duchenne’s research interest turned more towards pathology, and eventually to the pathological anatomy of the nervous system - and also in this field he achieved significant results.
Duchenne-Griesinger muscular atrophy
In 1861 in the second edition of his book Paraplégie Hypertrophique de l’enfance de cause cérébrale Duchenne described a boy with the form of muscular dystrophy that now bears his name. A keen photographer, he depicted his patient a year later in his Album de photographies pathologiques. Duchenne considered this to be a juvenile form of spinal muscular atrophy. In the second edition (1861) of his book De l’Electrisation localisée et de son application à la pathologie et à la thérapeutique he first described «hypertrophic paraplegia in child age». This was met with much attention, and histopathological findings - in particular of changes in the fatty tissue of the musculature - was shortly afterwards described by German investigators.
Duchenne gave a detailed account of 13 affected children in 1868 in a series of articles using the designation "paralysie musculaire pseudohypertrophique". In 1872, in the third edition of his book L'Electrisation Localisée, he provided an extensive, illustrated account of the disorder.
Duchenne also noted fatty change and an increase of it in interstitial tissue. He concluded that the disease was localised particularly to the interstitial tissue of the muscle. In the third edition of L'Electrisation Localisée, were presented results of a post-mortem investigation conducted by Duchenne with Charcot. In an appendix to this paper Duchenne also renders a deliberate description of a familial disease with, among other things, facies myopathica, which was later designated facio-scapulo-humeral muscular dystrophy, also known as Landouzy-Dejerine syndrome
In 1982 Dubowitz pointed out that two brothers described by Coste and Gioja in 1838 and recorded by Karl Christian Schmidt (1792-1855) in 1839 in his Jahrbücher der in- und ausländischen gesammten Medicin, represented the first reports of the dystrophy. The first definite description of the condition is attributed to Meryon, a London physician who reported in 1852 four affected brothers whom he had studied for several years. The title of his paper On fatty and granular degeneration of the voluntary muscles reflects the findings that he observed in autopsy.
The term «pseudohypertrophic dystrophy» was firmly established in 1879 when Gowers gave a series of five lectures on this topic to the students of the University College Hospital, London, describing 21 personal cases and reviewing 139 from the literature.
Duchenne’s name is also associated with progressive spinal muscular dystrophy that is a variant of amyotrophic lateral sclerosis. Francois Amilcar Aran published a description of the disease in 1850, based among other things on Duchenne’s methods of electro diagnostics. Duchenne later demanded priority in describing the disease, referring to a report to the French academy of sciences in 1849. This has not been found, however. Charcot called this condition progressive muscular atrophy type Duchenne-Aran, while Pierre Marie later termed it Aran-Duchenne’s type.
Apart from Duchenne’s description of the disorders to which his name is attached, he was also the first to distinguish upper and lower motor neurone causes of 7th nerve paralysis, and he made several important contributions to our understanding of lead palsy. He made exact descriptions of cerebellar and sensory ataxia and syringomyelia. He further studied the normal physiology of movement; periferal nerve damages like facialis and radialis pareses, and constructed mechanical prosthesis, orthoses and dynamometers.
In the first two editions of De l'Electrisation localisée Duchenne renders exact clinical descriptions of the natural course of polio. By a series of observations and deductions he proved that acute poliomyelitis (previously called ”paralysie essentielle de l’enfance,” localisation unknown) was a disease of motor nerve cells in the spinal cord. He also clarified the various forms of lead poisoning and revealed their electrical reactions.
He also made prognostic evaluations on the basis of the contractional capacity of the muscle in connection with electric stimulation, and suggested - without having any histological founding - that the profound paralysis of poliomyelitis must be due to a lesion that he located in the anterior horn cells of the spinal cord. This hypothesis proved correct when André Victor Cornil (1837-1898) in 1863 and Charcot in 1870 demonstrated histological changes.
Happiness, tragedy and honour
During the later part of his life Duchenne was a happy man, particularly after he had had a moving reunion with the son he had hardly seen, and his son commenced neurological practice in Paris. Tragedy, however, struck once more, when his son died in 1871 from typhoid fever. Following this, Duchenne apparently deteriorated rapidly with cerebral atherosclerosis and he died in 1875 from a cerebral haemorrhage. During his last days he recorded with his still sound hand his observations on the progressive hemiparesis. The famous monograph on poliomyelitis his son wrote was incorporated in the third edition of De l’électrisation . . ..
After his death Duchenne was honoured with a plaquette with his picture in deep relief at the Salpêtrière. Duchenne received several honours from foreign institutes, universities, and academies, but himself neither belonged to the French academy of sciences or a French University. He had set the stage for what was to become one of the most exciting eras of clinical neurology anywhere in the world.
Duchenne studied the mechanism of facial expression during emotion; his atlas of photographs is a most important contribution to medical photography. Darwin reproduced a number of his photographs in The Expression of the Emotions in Man and Animals.
According to Ernest-Charles Lasègue (1816-1883), and Isidor Straus (1845-1896) he was ”of medium height, thickset, active in movement and slow in speech,” with a faint provincial accent, and he closely resembled his father who had received the Croix de la Légion d’Honneur from Napoleon for valour as a sea captain in the French-English wars.
Although Duchenne was given no official recognition by the Académie de Médecine and the Institut de France, he was made honorary or corresponding member of academies in Rome, Madrid, Stockholm, St. Petersburg, Geneva and Leipzig, to his great satisfaction.