Frontiers of the Brain and Nervous System(UCTV)

This is my lovely one of the introductory lecture in Neurology.

Migraine: Interview Channel 11: Dr. Surat and Asso.Prof. Siwaporn

Video นี้เป็น Video ที่ผม และ อาจารย์ศิวาพรออกอากาศให้ความรู้เรื่องโรคปวดศีรษะแก่ผู้ป่วยครับ คิดว่าเป็นการแนะนำที่มีประโยชน์มากครับ

Charles Bell

Sir Charles Bell (Born November 1774, in Doun in Monteath, Edinburgh – Died April 28, 1842[1], in North Hallow,Worcestershire) was a Scottish anatomist, surgeon, physiologist and natural theologian. He was the younger brother ofJohn Bell (1763-1820), also a noted surgeon and writer.

Life

Bell lived and studied in Edinburgh, where he got his medical degree in 1799. He and his brother had extraordinary drawing talents, and together they taught anatomy and illustrated and published two volumes of A System of Dissection Explaining the Anatomy of the Human Body.

Soon after his graduation he was admitted to the Royal College of Surgeons, where he operated and taught anatomy. He and his brother published two additional volumes of their anatomical treatise in 1802 and 1804. His success, however, led to jealous opposition of local physicians, and he was barred from practice at the Edinburgh Royal Infirmary. He then moved to London in 1804, where he held a private surgery and school of anatomy. From 1812 to 1825, he ran, with his brother, the Great Windmill Street School of Anatomy, which had been founded by the great anatomist William Hunter (1718-1783). He also served as a military surgeon and famously documented his experiences at Waterloo in words and drawings. In 1835 he was instrumental in the creation of the Middlesex Hospital Medical School, and became, in 1824, the first professor of anatomy and surgery of the College of Surgeons in London. In 1829, the Windmill Street School of Anatomy was incorporated to the new King’s College London. Bell was invited to be its first professor of physiology, but resigned shortly afterwards.

Bestowed with honors and national and international recognition (he was made a Fellow of the Royal Society in 1826 and was knighted in 1831), Bell wished to return to Scotland. So, in 1836 he accepted the position of professor of surgery at the University of Edinburgh. He died in his homeland six years later, in 1842.

Work

Charles Bell was a prolific researcher and author. He first published detailed studies of the nervous system and brain in 1811, in his book An Idea of a New Anatomy of the Brain. He described his experiments with animals and how he was the first to distinguish between sensory and motor nerves. This book is considered by many the founding stone of clinical neurology.

He was one of the first physicians to combine the scientific study of neuroanatomy with clinical practice. He described in 1821 the trajectory of the facial nerve and a disease which led to the unilateral palsy (paralysis) of facial muscles, in one of the classics of neurology, a paper to the Royal Society entitled On the Nerves: Giving an Account of some Experiments on Their Structure an Functions, Which Lead to a New Arrangement of the System.

He also combined his many artistic, scientific, literary and teaching talents in a number of wax preparations and detailed anatomical and surgical illustrations, paintings and engravings in his several books on these subjects, such as in his beautiful book Illustrations of the Great Operations of Surgery: Trepan, Hernia, Amputation, Aneurism, and Lithotomy(1821). He wrote also the first treatise on notions of anatomy and physiology of facial expression for painters and illustrators, titled Essays on the Anatomy of Expression in Painting (1806). In 1833 he published the fourth Bridgewater Treatise, The Hand: Its Mechanism and Vital Endowments as Evincing Design.

A number of discoveries received his name:

  • Bell’s nerve: The posterior thoracic nerve.
  • Bell’s palsy: a unilateral idiopathic paralysis of facial muscles due to a lesion of the facial nerve.
  • Bell’s phenomenon: An upward movement of the eye and the eyelid which occurs when a person affected with Bell’s paralysis tries to close the eye.
  • Bell’s spasm: Involuntary twitching of the facial muscles.
  • Bell-Magendie law or Bell’s Law: States that the anterior branch of spinal nerve roots contain only motor fibers and the posterior roots contain only sensory fibers.

Charcot-Marie-Tooth syndrome(Historical note)

โรคนี้เป็นโรคทางระบบประสาท โดยเป็นกลุ่ม Chronic polyneuropathy

โดย แพทย์ชาวฝรั่งเศษ 2 ท่านคือ Jean-Martin Charcot (1825-1893) และลูกศิษย์ของเค้า Pierre Marie (1853-1940) (“Sur une forme particulière d’atrophie musculaire progressive, souvent familiale débutant par les pieds et les jambes et atteignant plus tard les mains”, Revue médicale, Paris, 1886; 6: 97-138.)

และในปีเดียวกัน นายแพทย์ Howard Henry Tooth (1856-1925) แพทย์ชาวอังกฤษ ได้บรรยายโรคนี้เช่นเดียวกัน(“The peroneal type of progressive muscular atrophy”, dissertation, London, 1886.)

เพื่อเป็นเกียรติแก่แพทย์ทั้ง 3  ท่าน จึงตั้งชื่อ CMT หรือ Charcot-Marie-Tooth syndrome

Guillain-Barre’ syndrome(Historical note)

GBS ได้ถูกบันทึกเป็นคร้ังแรกโดย แพทย์ชาวฝรั่งเศษ  Jean Landry in 1859 แต่อย่างไรก็ตาม เมื่อปี 1916, Georges Guillain, Jean Alexandre Barré, and Andre Strohl ได้บันทึกการวินิจฉัยโรค โดยมีผู้ป่วยทหาร 2 ราย ที่มีอาการป่วยแล้วมีอาการอ่อนแรง ร่วมกับ CSF มี protein สูงแต่ normal cell count ถือว่าเป็นการบันทึกแยกโรคเป็นครั้งแรก

GBS อาจเรียกด้วยชื่ออื่น ว่า acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio,Landry’s ascending paralysis และ Landry Guillain Barre syndrome แต่ GBS ถือว่าเป็นชื่อที่ได้รับความนิยมมากที่สุด

Joseph Jules François Félix Babinski

French neurologist, born November 17, 1857, Paris; died October 29, 1932, Paris.

Biography:
Joseph Jules François Félix Babinski was one of a handful of French neurologists and other medical scientists who made Hôpital de Salpêtrière in Paris a world famous medical centre in the late nineteenth and early twentieth century. Both his name, and those of many of his colleagues, are still of eponymic fame. The others being, among others, Jean-Martin Charcot, Claude Bernard, and Joseph Jules Dejerine,

Joseph Babinski was the son of a polish engineer and his wife who in 1848 flew Warsaw for Paris because of a Russian reign of terror with the purpose of stalling Polish attempts at achieving independence. Joseph, who grew up on the Montparnasse, as a child was described as rather heavy and docile in temper, though being lively and intense when engaged. It was also said that Joseph showed signs of a constitutional neurosis, and it is conceivable that an element of obsession may have contributed to his relentless search for any imaginable deviations in neurological status.

As a young student of medicine Babinsky came early to Charcot at the Salpêtrière in Paris. Charcot soon recognised the greatness of this distinct observer, who was soon to become his favourite student. Babinski received his medical degree from the University of Paris in 1884 with a thesis – of outstanding merit – on multiple sclerosis, a topic suggested to him by Edmé Félix Vulpian (1826-1887). Babinsky became Médecin des hôpitaux in 1890.

In the daily clinical work Babinski was an extremely untalkative loner who, during his minute neurological examinations more often than not never uttered a word, sometimes not even afterward. His working manner was characterised by exceptional observational powers and thorough discussions of the neurological systemathology. He strived not so much to map the theoretical background, but was above all a clinical descriptive observer.

An academic row and its consequences
Another of Charcot’s numerous students was Charles-Joseph Bouchard (1837-1915). He had first read medicine in Lyon, then at the age of 25 went to Paris to continue his studies under Charcot, who then, in 1862, was 37 years old. Bouchard was ambitious, gifted, and energetic, and, under Charcot’s supervision his career pointed straight up. His efforts were finally crowned with a professorship of medicine. When Bouchard was well established as a professor the relationship to his tutor and mentor deteriorated. Slowly they became enemies, each establishing his school, instead of completing each other and cooperate to the benefit of French Neurology.

This slowly growing enmity culminated when Bouchard by a skilful play of academic intrigue succeeded in outmanoeuvring Babinski, Charcot’s favourite student, at the appointment of a “professeur agrégé”, a meriting necessary for further advancement to “professeur de la chair”, full professor.

Charcot’s death left Babinski without support, and he subsequently never participated at qualifying competitions for “professeur agrégé”. Instead, in 1895, he became “chef de clinique” for the neighbouring Hôpital de la Pitié, where he worked until his retirement in 1922.

Deus ex machina
Babinski’s failure to climb the academic ladder was to become of fundamental importance to French neurology. Lacking of an academic position he was passed over for appointments and lacked the opportunity to establish his own school. Of all Charcot’s students Pierre Marie was, eventually, the only to be appointed full professor of neurology – a nomination that came 25 years after the death of Charcot. This appointment also caused an angry attack, this time from Dejerine, who came from the school of Bouchard. Babinski was, though, a member of the Académie de médecine from 1914. Charcot did not live to experience reconciliatory words from Bouchard. Reminded of who had been his teacher, however, Bouchard a decade after the death of Charcot expressed:

“It was Charcot who shaped our intellects; it was he who opened the gates to scientific work, it was he who took my hand and led me to the highest academic position I could reach. Confronted with his memory I shall always feel the deepest gratitude”.

Ironically the names of the two antagonists are still mentioned in one breath today at the mention of the Charcot-Bouchard aneurysm, i.e. microaneurysm on small cerebral perforated vessels that may cause intracranial bleedings.

Free of teaching duties, Babinski’s work at the Hôpital de la Pitié left him ample time to devote himself to the symptomathology of neurology. He was a masterly clinician, considerably less dependent on neuropathological examinations and laboratory tests than most of his contemporary colleagues. He chose the path of the unassumpting search, which may not so often give results, but that, with a scientist of an extraordinary intellect and intuition may lead to epoch making discoveries.

In 1900, a year before Alfred Fröhlich, Babinski described the adiposo-genital syndrome in a case of pituitary tumour, a condition still termed Babinski-Fröhlich syndrome. The following year, 1901, he reported with Augustin Charpentier (1852-1916) on the Argyll-Robertson’s pupil in neuro-syphilis as an expression of a lesion of the central nervous system. In 1902, with Jean Nageotte (1866-1948) he described the clinical symptoms caused by lesions in the postero-lateral part of pons, a complex of symptoms that still bears the name Babinski-Nageotte syndrome. In 1905 he described with insight the neurophysiological background of tabes dorsalis. He concerned himself with the pathology of the cerebellum and introduced the terms ataxia and dysdiakonesia as cardinal symptoms of cerebral lesions.

Farewell to hysteria
In the footsteps of his teacher, Jean-Martin Charcot, Babinski also took an interest in the pathogenesis of hysteria and was the first to present acceptable differential diagnostical criteria for separating hysteria from organic diseases. Jean-Martin Charcot thought he had discovered a new disease he called “hystero-epilepsy,” a disorder of mind and brain combining features of hysteria and epilepsy. The patients displayed a variety of symptoms, including convulsions, contortions, fainting, and transient impairment of consciousness.

Joseph Babinski decided that Charcot had invented rather than discovered hystero-epilepsy. The patients had come to the hospital with vague complaints of distress and demoralization. Charcot had persuaded them that they were victims of hystero-epilepsy and should join the others under his care. Charcot’s interest in their problems, the encouragement of attendants, and the example of others on the same ward prompted patients to accept Charcot’s view of them and eventually to display the expected symptoms. These symptoms resembled epilepsy, Babinski believed, because of a municipal decision to house epileptic and hysterical patients together (both having “episodic” conditions). The hysterical patients, already vulnerable to suggestion and persuasion, were continually subjected to life on the ward and to Charcot’s neuropsychiatric examinations. They began to imitate the epileptic attacks they repeatedly witnessed.

Babinski eventually won the argument. In fact, he persuaded Charcot that doctors could induce a variety of physical and mental disorders, especially in young, inexperienced, emotionally troubled women. There was no “hystero-epilepsy.” These patients were afflicted not by a disease but by an idea. With this understanding, Charcot and Babinski devised a two-stage treatment consisting of isolation and counter suggestion.

Babinski’s sign
When Babinski published it was most often commendably short and concise. Such was also the case when in 1896, at a meeting of the Société de Biologie in a 26 line presentation for the first time reported his “phenomène des orteils”, i.e. the discovery that while the normal reflex of the sole of the foot consists of a plantar reflex of the toes, an injury to the pyramidal tract will show up in an isolated dorsal flexion of the great toe – Babinski’s sign.

Although this sign had been reported three years before by Ernst Julius Remak (1849-1911), it was Babinski who first realized its diagnostic significance. In its simplicity, clinical importance, and physiological implications, Babinski’s sign has hardly an equal in medicine. The number of works devoted exclusively to ”Babinski” runs into the hundreds, yet in 1900 its diagnostic importance was declared to be ”minimal.” His description of the associated fanning of the toes, subsequently referred to as the signe de l’éventail, was published in 1903.

Two years later he published, in La Semaine médicale, a particularly complete description of the phenomena exemplified by case stories of patients with hemiplegia, Jackson’s epilepsy, encephalitis and strychnine poisoning. The clinical description in this works even today stands out as remarkably complete. Even here Babinski drew the correct conclusion that the sign was to be explained by affection of the pyramidal tract (not necessarily structural) and also noticed that it is to be found in healthy infants. In 1903 he followed up his observations with another article containing a description of sign de léventail, the fanning of the toes often occurring simultaneously with the extension of the great toe in affections of the pyramidal tract.

In the name of justice it must be pointed out that Félix Alfred Vulpian, neuropathologist at the Salpêtrière, half a century earlier had observed the extension of the great toe in certain types of brain damage. It was, however, first through Babinski’s brilliant observations and analysis of the phenomena that the clinician recognised the importance of this sign in lesions of the pyramidal tract. Babinski’s sign received much international interest and was surprisingly soon to achieve routine neurological status worldwide. Babinski’s pathogenetic conclusions could also be verified by Fulton in studies of chimpanzees, later also by Swedish neurologists, among them Erik Klas Henrik Kugelberg (born 1913).

Diagnostics of tumours in the spinal canal
On June 9th, 1887, Victor Horsley successfully conducted the first extirpation of a tumour in the spinal canal. At the turn of the century many attempts were made to surgically operate tumours in the spinal canal, but mostly the laminectomies were made at the wrong level – too low. In 1910 Babinsky demonstrated that a careful study of sensibility conducted according to specified principles without exception led to a diagnosis of level without correction. These observations were subsequently proved right.

In 1911 Babinski diagnosed a tumor in the spinal canal and sent the patient to Victor Horsley. Later the same year Babinski diagnoses one more spinal tumor, but remitted his patient to the then very well known French surgeon Paul Lecène (1878-1929). Babinski was, however, not satisfied with Lecène’s dexterity and decided to find another surgeon for his next case.

Rebirth of French neurosurgery
Through his student Clovis Vincent (1879-1947), later to become a famous neurosurgeon, Babinski became acquainted with Thierry de Martel, who declared himself willing to operate the next patient with a suspected spinal tumor. When Babinski towards the end of 1911 diagnosed a third such case, he therefore remitted his patient to Martel – who successfully removed a tumor in the spinal canal – localised exactly as foretold by Babinski.

This operation marks the rebirth of French neurosurgery. From then on Martel preferably devoted himself to neurosurgical cases, and soon other French neurologists, like Pierre Marie, Théophile Alajouanine (born 1880), Jacques Jean Lhermitte (1877-1959), Jean Alexandre Barré (1880-1967), and Auguste Tournay (1878-1969) followed Babinski’s example in sending their patients in need of neurosurgical treatment to Martel.

Six days prior to his death a close friend asked Babinski what he considered his most lasting achievement in science. “Of your contributions “the sign” will certainly persist”, uttered his friend. “Yes, the “sign” will be remembered”, answered Babinsky, “but it was not my greatest contribution – that was my showing the direction for Martel and Vincent”.

Babinski’s last year
Babinski lived with his younger brother by twelve years, Henri Babinski, a distinguished engineer who was also a famous cook, well known with all food artists. Under the pseudonym of Ali Baba, Henri Babinski published several now classic works on cooking. It is said that Babinski once interrupted his ward round when the nurse in charge whispered to him that the soufflé was nearly perfect. He spent the evenings at the theatre, opera and ballet.

Joseph Babinski died on December 13th 1932. The last years of his life he suffered from Parkinson’s disease, but he lived to see his achievements in French neurology internationally acknowledged. Neurologic profiles like Robert Wartenberg (1887-1956) and Samuel Alexander Kinnier Wilson (1878-1937) came to work and study with him. He was honoured by the American Neurological Society and several other foreign societies.

Babinski’s obituary in The Lancet ended with the following words: “None of Charcot’s pupils is surer to be remembered for his achievements in the field of neurology.” Babinski is buried on the Cimetière des Champeaux at Montmorency, about 13 km north of Paris, France.

With Édouard Brissaud (1852-1909), Pierre Marie (1853-1940), Joseph Jules Dejerine (1849-1917), Alexandre-Achille Souques (1860-1944), and others, Babinski founded the Société de neurologie de Paris, to which he was profoundly devoted.

One of those drawn to Babinsky was Antonio Caetano de Abreu Freire Egas Moniz (1875-1955), the Portuguese who was later to receive the Nobel Prize for physiology or medicine for his discovery of prefrontal lobotomy as a method of treatment in schizophrenia. Today, however, Egas Moniz is more remembered for his introduction of cerebral contrast-angiography in humans. The medical establishment rather coolly received this revolutionizing method of neurological diagnostisation, first adapted in 1927 after exhaustive studies of cadavers. Babinski, however, made an exemption in writing the preface to Egas Moniz monograph, published in 1931. Engaged and vividly he describes how Egas Moniz plans and carries out an exceptionally large project with an insight, a courage, and a single-mindedness leading his thought to his great fellow citizens, Bartolomeo Diaz and Vasco da Gama, as they embarked on their voyages to find the sea route to India. The description might as well go for Babinski’s life-achievement.

We thank Peter Ziffling for information submited.

Bibliography:

    Babinski’s bibliography contains 288 items

  • Oeuvre scientifique: recueil des principaux travaux.
    Publié par les soins de Barré, Chaillous, Charpentier, et al. Paris, Masson, 1934.
  • J. Babinski:
    Étude anatomique et clinique sur la sclérose en plaques. Paris, 1885.
  • Hystérie-pithiatisme et troubles nerveux d’ordre réflexe en neurologie de guerre.
    With Jules Froment (1878-1946). Paris, 1917; 2nd edition, 1918; translated into English.

Jean-Martin Charcot

French neurologist, born November 29, 1825 Paris; died August 16, 1893, Lac des Settons, Nièvre.

Biography:
Charcot was one of a select group of physicians who made the Salpêtrière hospital in Paris a world medical centre, founding the famous neurological clinique there. He is considered the founder of modern neurology, and is remembered as the first professor of neurology. His name is associated with at least 15 medical eponyms, of which the most known is Charcot-Marie-Tooth disease. Another is amyotrophic lateral sclerosis, in America particularly known as Lou Gehrig’s disease.

Jean Martin Charcot was born in Paris in 1825, the son and grandson of a coach-builder. The family originally came from Champagne. In childhood he manifested a taciturn personality which persisted throughout his life, and while still a boy demonstrated an early interest in medicine, but was no less interested in drawing and painting which taught him the importance of making careful observations – from which he was later to benefit greatly, both as a teacher and a scientist.

Charcot at an early age decided upon a career in medicine. He qualified in 1853, at the age of 23, and gained a junior post at the Salpêtrière. He became interne des hôpitaux in 1848 and was appointed chef de clinique in 1853, after defending an outstanding doctoral thesis on gout and chronic rheumatism (arthritis nodosa), in which he differentiated gout from other forms of chronic rheumatism He became médecin des hôpitaux de Paris (Bureau Central des hôpitaux de Paris) in 1856.

No gunpowder plot, just nerves
In 1862, at the age of 37 years Charcot was appointed senior physician at the Salpêtrière. This famous hospital on the left bank of the Seine is frequently mentioned in the history of great physicians. Among Charcot’s predecessors here were Léon Jean Baptiste Cruveilhier (1791-1874) and Ernest-Charles Lasègue (1816-1883), but it was under the carismatic leadership of Charcot that classic French neurology was founded.

The name Salpêtrière dates back to the time when the building was the arsenal and gunpowder store of Louis XIII. Now it was a hospice for more than 5000 indigent patients. Charcot life’s work revolved around the diagnosis and classification of these patients, and he gave definitive descriptions of numerous disorders, correlating their clinical and pathological findings. From 1862 he was active in the women’s clinic at the Salpêtrière. Between 1866 and 1878 he gave regular annual lectures on chronic diseases, diseases of old age and, in particular, on diseases of the nervous system.

Charcot became professeur agrégé in 1860.

War and peace
Charcots work was temporarily interrupted during the Franco-Prussian war of 1870-1871. He was forced to send his family to England and, in 1871, following the war and the strife engendered by the Commune of Paris, occupied himself with epidemics of typhoid and smallpox.

Thereafter his interest moved to hysteria and he advanced the revolutionary concept that affected persons had diseases of the brain which were functional rather than structural; in this respect he is regarded as a founder of the science of psycopathology.

Charcot’s career prospered and he was made professor of pathological anatomy at the Faculty of Medicine at the University of Paris in 1872, and in 1882 was appointed to the first chair of neurology, established especially for him, as professor of diseases of the nervous system. This year, at the Salpêtrière, he opened what was to become the greatest neurological clinic of his time in Europe.

During this period Charcot published a series of memoirs that attracted wide attention among neurologists. He also practiced extensively the clinical-anatomical method that correlated the symptoms observed in the sick patient and the lesions discovered at the time of autopsy. Through these he proved that the cells of the dorsal horn of the spinal cord possesses certain trophic properties and then analyzed the lesions found in theses cells as a result of infantile paralysis. In collaboration with Charles-Joseph Bouchard (1837-1915) he studied the secondary degeneration of the spinal cord.

Farewell to hysteria
Jean-Martin Charcot vigorously supported and defended the theory of cerebral localizations in man; several of his outstanding courses dealt with this theory and its application to Jacksonian epilepsy (John Hughlings Jackson, 1835-1911), aphasia, and Beard’s neurasthenia (George Miller Beard, 1839-1883). In 1872 he initiated work on hysteria and hysterical hemianesthesia, and on the link between traumatism and local hysteria. Indeed, Charcot must be considered as one of the first to demonstrate the clear and fruitful relationship between psychology and physiology.

Charcot, thought he had discovered a new disease he called “hystero-epilepsy,” a disorder of mind and brain combining features of hysteria and epilepsy. The patients displayed a variety of symptoms, including convulsions, contortions, fainting, and transient impairment of consciousness.

Joseph Babinski, his student, however, decided that Charcot had invented rather than discovered hystero-epilepsy. The patients had come to the hospital with vague complaints of distress and demoralization. Charcot had persuaded them that they were victims of hystero-epilepsy and should join the others under his care. Charcot’s interest in their problems, the encouragement of attendants, and the example of others on the same ward prompted patients to accept Charcot’s view of them and eventually to display the expected symptoms. These symptoms resembled epilepsy, Babinski believed, because of a municipal decision to house epileptic and hysterical patients together (both having “episodic” conditions). The hysterical patients, already vulnerable to suggestion and persuasion, were continually subjected to life on the ward and to Charcot’s neuropsychiatric examinations. They began to imitate the epileptic attacks they repeatedly witnessed.

Babinski eventually won the argument. In fact, he persuaded Charcot that doctors can induce a variety of physical and mental disorders, especially in young, inexperienced, emotionally troubled women. There was no “hystero-epilepsy.” These patients were afflicted not by a disease but by an idea. With this understanding, Charcot and Babinski devised a two-stage treatment consisting of isolation and counter suggestion.

First, “hystero-epileptic” patients were transferred to the general wards of the hospital and kept apart from one another. Thus they were separated from everyone else who was behaving in the same way and also from staff members who had been induced by sympathy or investigatory zeal to show great interest in the symptoms. The success of this first step was remarkable. Babinski and Charcot were reminded of the rare but impressive epidemic of fainting, convulsions, and wild screaming in convents and boarding schools that ended when the group of afflicted persons was broken up and scattered.

The second step, countersuggestion, was designed to give the patients a view of themselves that would persuade them to abandon their symptoms. Dramatic countersuggestions, such as electrical stimulation of “paralyzed” muscles, proved to be unreliable. The most effective technique was simply ignoring the hysterical behavior and concentrating on the present circumstances of these patients. They were suffering from many forms of stress, including sexual feelings and traumas, economic fears, religious conflicts, and a conviction (perhaps correct) that they were being exploited or neglected by their families. In some cases their distress had been provoked by a mental or physical illness. The hysterical symptoms obscured the underlying emotional conflicts and traumas. How trivial a sexual fear seemed to a patient in whom convulsive attacks produced paralysis and temporary blindness every day!

Staff members expressed their withdrawal of interest in hysterical behavior subtly, in such words as, “You’re in recovery now and we will give you some physiotherapy, but let us concentrate on the home situation that may have brought this on.” These face-saving countersuggestions reduced a patient’s need to go on producing hysteroepileptic symptoms in order to certify that her problems were real. The symptoms then gradually withered from lack of nourishing attention. Patients began to take a more coherent and disciplined approach to their problems and found a resolution more appropriate than hysterical displays.

Charcot removed his patients from the special wards when he realised what he had been inventing.

The great teacher
It was perhaps his incomparable qualities as teacher, writer and organizer that contributed most to the great reputation of this gifted clinician. As a teacher he was not afraid of employing theatrical techniques during his lecture-demonstrations on a floodlit stage in the amphitheatre of the Salpêtrière. As a lecturer he spoke rather slowly and articulated, often staying quiet for several minutes while observing the patient being demonstrated to the auditorium. The discussion of the neurologal status progressed during minutious observations of the patient’s physical signs. On tuesday mornings he held his famous clinical demonstrations, and, on fridays, his well prepared and repeated lectures connected to clinical cases.

Charcot’s experiments in hypnosis and his clinical demonstrations were open to the lay public, attracting the famous, the fashionable, and the aristocratic. At this stage of his career he was known as the “Caesar of the Salpêtrière”. Among his students were Pierre Marie, Joseph Jules Babinski, Wladimir Michailowich Bekhterev, Desiré-Magloire Bourneville, and George Albert Edouard Brutus Gilles de la Tourette. In 1885 one of his students was Sigmund Freud, and it was Charcot’s employment of hypnosis in an attempt to discover an organic basis for hysteria that stimulated Freud’s interest in the psychological origins of neurosis.

Axel Munthe on Jean-Martin Charcot
In his book on San Michele Axel Munthe describes daily life and the athmosphere at La Salpêtrière. In it, Munthe is highly critical of Charcot’s theories of hypnotism and his treatment of conditons of hysteric conversions. Munthe declares Charcot’s famous “Leçons du Mardi” at the Salpêtrière as a confused mixture of truth and fraud”. He considered the hysteric female patients to be “pure impostors knowing what was expected of them and delighted to demonstrate their skills to the public”.

Munthe also describes Charcot as a person: “Short, with the chest of an athlet and the neck of a bull, he was an impressing appearance. A pale, smoothly shaved face, a low forehead, cold, piercing eyes, aquiline nose, sensitive lips: the mask of a Roman Caesar. Charcot was also a rather charismatic character, but Munthe believed him to be callous and indifferent to the sufferings of his patients. Both his appearance and his personality, however, must have been inspiring confidence in his patients, as he had an enormous practice.

During his visit at the Salpêtrière Munthe happened to interfere in the treatment of one of Charcot’s favourite patients, causing his fall from grace and eventually being fired from his position.

Subsequent readers may accept parts of Munthe’s critisism of the methods of treatment at the Salpêtrière. This does not, however, diminish Charcot’s importance as a clinician. It is worth reminding that he was able to distinguish between conditions of neurological disease, although methods for testing muscular reflexes were still not available.

A hard working artist with no dog clinic
Charcot was very hard-working: “he worked uninterruptedly all day, and the lamp on the desk in his large library was still to be seen burning at two o’clock in the night. His only leisurely pleasure was music, Ludvig van Beethoven being his favourite composer. On his Thursday evenings, which were devoted entirely to music, nobody was allowed to utter a single word on medicine.”

Charcot was also a talented artist and a great lover of animals, always avoiding experiments on animals. He had inscribed on his door: “Vous ne trouverez pas une clinique des chiens chez moi” (you find no dog clinic with me). Every morning when he stepped out of his landauer in the inner yard of the Salpêtrière, he took a piece of bread from his pocket and and gave it to his two Rosinantes. He interrupted all conversations on hunting, and his antipathy against Englishmen was probably caused by his disgust of fox hunting.

Later years
Charcot was disturbed by the idea of disease running in families and when speaking of such conditions he would repeat the quotation “What have we done, Oh Zeus, to deserve this destiny? Our fathers were wanting but we, what have we done?”

The famous Charcot had preferred to see his favourite pupil, Joseph Babinsky, succeed him in the chair of neurology. Babinsky, however, was outmaneuvered through academic intrigue, and the chair thus was given to Fulgence Raymond (1844-1910), a somewhat distinguished neurologist, also of eponymic fame, but a man of much lesser format than Babinski.

From 1890 Charcot’s health began to deteriorate. He had several attacks of angina and died suddenly from pulmonary oedema in 1893, during a journey in the Morvan, at the age of 68 years. His funeral service was held in the Chapel of the Salpêtrière and he was buried at the Montmartre churchyard. Beside him rests his son, Jean Baptiste, also a physician, and a well known scientific traveller.

Charcot’s tremendous influence as a founder of neurology was recognised after his death by the erection of his bronze statue at the Salpêtrière. Regrettably it was destroyed in 1942 during the Nazi occupation of Paris.

Charcot was an officer of the Légion d’Honneur, vice president of the Société de biologie from 1860, honorary member of the Société anatomique (of which he became president in 1872) from 1882, member of the Académie de médecine from 1872, member of the Académie des sciences (elected with 46 against 12 votes) from November 12, 1883. He was created doctor of honour at the University of Würzburg in 1882 on the occasion of its 300th anniversary. He left a very great number of publications.

Just work, dear maid
In his immensely productive period between 1862 and 1870 Charcot gave a series of masterly clinical descriptions. He elucidated the adult progressive neuromuscular atrophy described by Guillaume Benjamin Amand Duchenne de Boulogne (1806-1875) in 1849 and François-Amilcar Aran (1817-1861) in 1850, demonstrating that the disease could also attack the lateral strings of the spinal marrow. In the same period he described a severe complication in with tabes dorsalis, known as Charcot’s joints. He gave clinical-anatomical description of multiple sclerosis unequelled to his day. Charcot’s triade classically comprises nystagmys, intention tremor and scandering speech. Among his other important contributions were clinical-pathological localisations of lesions to the spinal marrow.

Charcot recognised disseminated sclerosis as a distinct disease and was the first to diagose it on a living patient – previously it had been confused with Parkinsonism. Being a pragmatic, Charcot employed a housemaid with disseminated sclerosis in order to facilitate continuous close scrutiny. He noticed that her tremor was intentional and not static, and kept her employed until she had to be admitted to the the Salpêtrière, where his clinical diagnosis of disseminated sclerosis was confirmed at autopsy.

Charcot localised the motor centres of the cerebral cortex, he was the first to describe syphillitic and amyotrphic lateral sclerosis, and with Vulpian, first described ankle clonus. He is credited introducing geriatics as a separate educational specialty and in 1859 was among the first to recognise intermittent claudication. Besides having made excellent descriptions of neurological diseases, Charcot also made valuable contributions to the understanding of the pathology of the liver, kidneys and the organs of the chest, as well as the nature of tuberculosis and rheumatism. He is also famous for the investigations into the V. B. Burq’s (1823-1884) metaloscopy and metalltotherapy.

Charcot was one of the «grandes gloires» of the French nation, elevating its scientific medicine to a level it had not seen for one or two decades. In his capacity as pioneering representative of moderne nervous pathology, he gave numerous important contributions to medical science.

Charcot was a formidable writer, the author of many articles and books. He founded the journal Archives de neurologie, which he edited until his death.

“Vous ne trouveres pas une clinique des chiens chez moi”.
“You find no dog clinic with me”.

“Six mois, un an après début, tous les symptômes se son accumulés et plus ou moins fortement accentués. La mort arrive au bout de deux ou trois ans en moyenne par le fait des symptômes bulbaires.” Charcot in his lecture summarizing his observations
concerning the disease which he called
amyotrophic lateral sclerosis; May 18, 1874.

«Clinical medicine is made up of anomalies, while nosography is the description of phenomena that occur regularly. What we look for in the clinics is almost always exceptional; what we study in nosography is the rule. It is well to know that, in the practice of medicine, a nosographer is not always a clinician.»

«To learn how to treat a disease, one must learn how to recognize it. The diagnosis is the best trump in the scheme of treatment.»

«Disease is very old, and nothing about it has changed. It is we who change, as we learn to recognize what was formerly imperceptible.» De l’expectation en médecine.

«Why do we have to go over the same set of symptoms twenty times before we understand them? Why does the first statement of a new fact always leave us cold? Because our minds have to take in something which deranges our original set of ideas, but we are all like that in this miserable world.»

«It is the mind which is really alive and sees things, yet it hardly sees anything without preliminary instruction.»

«If the clinician, as observer wishes to see things as they really are, he must make a tabula rasa of his mind and procees without any preconceived notions whatever.»

«In the last analysis, we see only what we are ready to see, what we have been taught to see. We eliminate and ignore everything that is not a part of our prejudices.»

«How is that, one fine morning, Duchenne discovered a disease which probably existed in the time of Hoppocrates?»

«In dealing with a nervous patient, you should regard the malady before you merely as an episode. Thus, in a case of chorea, it is only necessary to inquire how long it has existed. The condition of the patient is only an accident in the history of the disease, just as each of us is only an accident in the history of humanity. De l’expectation en médecine

«Common-place scepticism, which is so readily opposed to all progress of the human mind, is a convenient pillow for lazy heads; but in this epoch there is no longer time to go to sleep.»
Lecons cliniques sur les maladies des vieillards et les maladies chroniques.
Introduction, Sect. 5.

«Symptoms then, are in reality nothing but the cry from suffering organs.»
Leçons cliniques sur les maladies des vieillards et les maladies chroniques.
Introduction, Sect. 1.

«In medicine . . . one never sees even the most stoic intellects limit themselves to stating the facts without looking for a way to relate them by some sort of theory: from the outset, one sees minds occupied more with the subjective relationships of things than with their reality itself; the empirical results of observation, scarcely acquired, are brought together, tested one against the other, to evolve thories or systems. There, one must recognize, is a necessity of the human mind.»
Leçons cliniques sur les maladies des vieillards et les maladies chroniques.
Introduction.

«There is, in any well executed description of disease, a remarkable power of transmission. If made at the right time, it will penetrate even the least prepared minds. What had hitherto remained in the womb of nothingness has begun to live. A description of a hitherto unknown species of disease is an event, a very great event, in pathology.»
Quoted by F. H. Garrison in Bulletin of New York Academy of Medicine, 1928; 4: 1000.

Bibliography:

  • Études pour servir à l’histoire de l’affection décrite sous les noms de goutte asthénique primitive, nodosités des jointures, rhumatisme articulaire chronique (forme primitive). Doctoral thesis, 1853.
  • Observation de leucocythémie. Written with Charles Philippe Robin (1821-1885).
    Comptes rendus de la Société de biologie
    . Paris, 1853; 44.
    Charcot-Leyden crystals.
  • De l’expectoration en médecine. Paris, 1857. Charcot-Leyden crystals.
  • Note sur les cristaux particuliers trouvés dans le sang et les viscères d’un sujet leucémique.
    Written with E. F. A. Vulpian. Gazette médicale de Paris, 1860; 7: 755.
    Charcot-Leyden crystals.
  • De la pneumonie chronique. Paris, 1860.
  • Contribution à l’étude des altérations anatomiques de la goutte.
    Written with André Victor Cornil. Comptes rendus de la Société de biologie (Mémoires). Paris, (1863); 1864, 3rd series, 5: 139-163.
  • Douleurs fulgurantes de l’ataxie sans incoordination des mouvements; sclérose commençante des cordons postérieurs de la moëlle épinière. Written with Abel Bouchard (1833-1899).
    (Maybe this should be Charles-Joseph Bouchard, French pathologist, 1837-1915).
    Gazette médicale de Paris, 1866, 3rd series; 21: 122-124.
    First clinical description of the electric pains in tabes.
  • Leçons cliniques sur les maladies des vieillards et les maladies chroniques. Paris, 1868.
  • Histologie de la sclérose en plaques.
    Gazette des hôpitaux, Paris, 1868; 41: 554-555.
  • Deuz cas d’atrophie musculaire progressive avec lésions de la substance grise et des faisceaux antéro-latéraux de la moëlle épinière.
    Archives de physiologie normale et pathologique, Paris, 1869; 2: 744-760.
    Description of the lesions of the spinal cord in muscular atrophy.
  • Une observation de paralysie infantile s’accompagnement d’une altération des cornes antérieures de la substance grise de la moëlle.
    Written with Alex Joffroy (1844-1908).
    Comptes rendus de la Société de biologie, Paris, (1869), 1870, 5th series; 1: 312-315.
    First demonstration of the atrophy of the anterior horns of the spinal cord in infantile paralysis, confirming earlier suggestions of Jacob von Heine (1800-1879) and Guillaume Benjamin Amand Duchenne de Boulogne (1806-1875).
  • Leçons sur les localisations dans les maladies du cerveau.
    2 volumes, Paris, Progrés médical & V. A. Delahaye, 1876-1880. Volume 2 (1880) under the title of Localisation dans les maladies du cerveau et de la moelle épinière.
    English translation (New Sydenham Society), 1883.
    With this work, one of his most important, Charcot won the Prix Monthyon of the Académie des sciences.
  • Leçons sur les maladies du système nerveux faites à la Salpêtrière.
    5 volumes, Paris, A. Delahaye, 1872-1887. English translation, 1877-1889.
    With Charcot-Weiss-baker syndrome.
  • Leçons sur les maladies du foie, des voies biliares et des reins faites à la Faculté de Médecine de Paris.
    Paris: Progrés Médical & Adrien Delahaye, 1877.
    English translation, New York, 1878.
  • Oeuvres complètes. 9 volumes. Paris, Bureaux de Progrés Médical [and other publishers], 1886-1890.
  • Sur une forme particulière d’atrophie musculaire progressive, souvent familiale débutant par les pieds et les jambes et atteignant plus tard les mains.
    Written with Pierre Marie.
    Revue médicale, Paris, 1886, 6: 97-138.
    Charcot-Marie-Tooth disease or syndrome.
  • Neue Vorlesungen über die Krankheiten des Nervensystems insbesondere über Hysterie. Autorisirte deutsche Ausgabe von Sigm. Freud. Leipzig, Toeplitz, 1886.
  • Sur un cas de coecite’ verbales. Ouvres Completes de Charcot.
    Paris, Delehaye, Lecrosnier, 1887. Charcot-Wilbrand syndrome.
  • Les démoniaques dans l’art.
    Produced with Paul Marie Louis Pierre Richer (1849-1933). Paris, A. Delahaye & E. Lecrosnier, 1887. Reprinted, Amsterdam, B. M. Israël, 1972.
    Charcot was a talented artist; he collaborated with Richer, artist at la Salpêtrière, in the production of interesting books on disease and deformity as portrayed by artists, books which have put the study of medicine in relation to art upon a sound footing.
  • Leçons du mardi à la Salpêtrière.
    Policlinique 1888-1889. Progrès médical, Paris, 1889.
  • Les difformes et les maladies dans l’art. Produced with Paul Marie Louis Pierre Richer (1849-1933). Paris, Lecrosnier & Babe, 1889. Reprinted, Amsterdam, B. M. Israël, 1972.
  • Leçons du mardi à la Salpêtrière. 2 volumes; Paris, 1889-1890.
  • Les centres moteurs corticaux chez l’homme.
    With Jean Albert Pitres (1848-1927). Paris, Rueff & Cie., 1895.
    This work conclusively proved the existence of cortical motor centres in man. It consists of three papers by Charcot and Albert of 1877, 1878, and 1883.

Work referred to:

  • Charles-Joseph Bouchard:
    Des dégenerations secondaires de la moëlle épinière.
    Archives génerales de médecine, Paris, 1866. English translation by E. R. Hun, Utica, 1869.

Reference

www.whonamedit.com

“The man who mistook his wife for a hat”

The man who mistook his wife for a hat

ผู้ป่วย Neurology เป็นผู้่ป่วยที่น่าสงสารนะครับ ทำไมหนะเหรอ ก็สิ่งที่ทำเจริญ พัฒนา และทำให้มนุษย์ต่างจากสัตว์ก็คือสมองนี้แหละครับ ผมได้ไปอ่านหนังสือของ Oliver Sack มา อ่านที่ Wellcome Library ที่ London ครับ เขียนเรื่อง “The man who mistook his wife for a hat” โอ้ พระเจ้า นึกไม่ออกเลยว่าถ้าผมเห็นภรรยาเป็นหมวกบ้างจะเป็นอย่างไร

“The man who mistook his wife for a hat” เขียนโดย Oliver Sack ซึ่งได้นำโครงเรื่องมาจาก case study เรื่อง a man with visual agnosia

The man who mistook his wife for a hat trailer

เรื่องนี้ได้สร้างเป็นหนังเมื่อปี 1987 โดย Rawlence ครับ

Progressive Multifocal Leukoencephalopathy(Research Channel)

Driving Bell and the Butterfly: ร่างกายที่ถูกขัง กับหัวใจที่โบยบิน

The Diving Bell and the Butterfly Trailer

The Diving Bell and The Butterfly- Official Trailer

หนังเรื่องนี้สร้างมาจากหนังสือฝรั่งเศษเรื่อง “The Driving Bell and the Butterfly” ซึ่งได้แปลมาจากสมุดบันทึก  Le scaphandre et le papillon โดยนักเขียนหนังสือ Jean-Dominique Bauby

เรื่องราวชีวิตของผู้เขียนหลังจากที่ป่วยเป็น Stroke ทำให้ผู้เขียนตกอยู่ในกลุ่มอาการที่เรียกว่า “Locked-in syndrome”

ในวันที่ 8 เดือนธันวาคม ค.ศ. 1995, Bauby, บรรณาธิการของวารสาร Elle magazine ได้ป่วยอย่างกระทันหันและอยู่ในอาการโคม่า หลังจากนั้น 20 วัน เค้าตื่นขึ้นมาด้วยสติสัมปชัญญะพร้อมมูลแต่ไม่สามารถขยับแขนขาได้ ส่วนที่พอขยับได้บ้างคือตาเท่านั้น Bauby เขียนหนังสือเล่มนี้ด้วยการกระพริบตา 4 ชั่วโมงต่อวัน เป็นเวลาถึง 10 เดือน โดย Bauby ใช้วิธีการ ส่งสัญญาณ French language frequency-ordered alphabet (E, S, A, R, I, N, T, U, L, etc.)

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Films

หนังเรื่อง The Driving Bell and the Butterfly ได้รับการสร้างเป็นภาพยนต์ ในชื่อเรื่องเดียวกับต้นฉบับหนังสือเมื่อปี 2007 โดยนักแสดงนำ Julian Schnabel ได้รับรางวัลผู้กำกับยอดเยืี่ยมใน Cannes Film Festival

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