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Tics, tremors and trusses A very brief history of movement disorders Dr Graham Lennox Consultant Neurologist Great Western Hospitals NHS Foundation Trust.

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Presentation on theme: "Tics, tremors and trusses A very brief history of movement disorders Dr Graham Lennox Consultant Neurologist Great Western Hospitals NHS Foundation Trust."— Presentation transcript:

1 Tics, tremors and trusses A very brief history of movement disorders Dr Graham Lennox Consultant Neurologist Great Western Hospitals NHS Foundation Trust

2 What are movement disorders? Parkinsonism Tremor Chorea Dystonia Myoclonus Tics Stereotypies Restless legs

3 Medieval movement disorders Tremor and palpitations Chorea and convulsions

4 Tremor Galen, Sylvius and others distinguished between action tremor and rest tremor

5 Chorea Referred to a wide range of phenomena, including complex stereotyped movements such as St Vitus’ dance

6

7 Thomas Sydenham 1624-1689

8 Thomas Sydenham 1624 born into Dorset landed gentry 1642 Magdalen Hall, Oxford, then parliamentary army 1648 Oxford BM; elected a fellow of All Souls 1655 resigned from All Souls; later attended Montpellier 1663 licensed by Royal College of Physicians 1676 Cambridge MD; Pembroke Hall where his eldest son was by then an undergraduate.

9 Mainly famous for his treatment of infectious diseases Laudanum (opium) Cinchona (quinine) for malaria Detailed description of gout Thomas Sydenham

10 “Among the remedies which it has pleased Almighty God to give to man to relieve his sufferings, none is so universal and so efficacious as opium.” “A man is as old as his arteries.”

11 Sydenham’s chorea mentioned in an aside Link to rheumatic fever not noted Thomas Sydenham

12 “This is a kind of convulsion, which attacks boys and girls from the tenth year to the time of puberty. It first shows itself by limping or unsteadiness in one of the legs, which the patient drags. The hand cannot be steady for a moment. It passes from one position to another by a convulsive movement, however, much the patient may strive to the contrary.” Schedula Monitoria de Novae Febris Ingressa (1686)

13 “This is a kind of convulsion, which attacks boys and girls from the tenth year to the time of puberty. It first shows itself by limping or unsteadiness in one of the legs, which the patient drags. The hand cannot be steady for a moment. It passes from one position to another by a convulsive movement, however, much the patient may strive to the contrary.” "Before he can raise a cup to his lips, he makes as many gesticulations as a mountebank; since he does not move in a straight line, but has his hand drawn aside by spasms, until by some good fortune he brings it at last to his mouth. He then gulps it off at once, so suddenly and so greedily as if he were trying to amuse the lookers-on."

14 Chorea classification

15 Modern view of Sydenham’s Mixture of chorea and tics Often psychiatric features Antibiotics Dopamine blocking drugs

16 Samuel Johnson 1709-1784

17 Samuel Johnson Biographer of Sydenham (and many others) Poet, essayist, lexicographer, literary critic, hack and wit Son of a bookseller Childhood scrofula and myopia Briefly studied at Pembroke College, Oxford

18 Samuel Johnson

19 Johnson’s movement disorder Rejected as schoolmaster: “He has such a way of distorting his face which though he can’t help, the gent. think it may affect some young lads” Started his own school, teaching David Garrick: “He did not appear to have been profoundly reverenced by his pupils. His oddities of manner, and uncouth gesticulations, could not but be the subject of merriment to them”

20 Multiple movements “His mouth is continually opening and shutting, as if he were chewing something; he has a singular method of twirling his fingers, and twisting his hands: his vast body is in constant agitation, see-sawing backwards and forwards: his feet never a moment quiet” (Fanny Burney)

21 Vocalisations “In the intervals of articulating he made various sounds with his mouth, sometimes as if ruminating, or what is called chewing the cud, sometimes giving a half-whistle, sometimes making his tongue play backwards from the roof of his mouth, as if clucking like a hen, and sometimes protruding it against his upper gums in front too, as if pronouncing quickly under his breath too, too, too” (James Boswell)

22 Suppressibility “He could sit motionless, when he was told to do so, as well as any other man.” (Sir Joshua Reynolds)

23 Complex motor rituals and compulsions Twirling and leaping in doorways Standing with his feet at particular angles Touching posts in the street Avoiding cracks in the pavement Holding teacup in outstretched arm

24 Self-injurious behaviour Repetitive leg rubbing “Not only did he pare his nails to the quick, but scraped the joints of his fingers with a pen-knife until they seemed quite red and raw”

25 Johnson’s diagnosis?

26 Gilles de la Tourette syndrome Early onset Multiple motor and phonic tics Obsessive-compulsive disorder

27 Gilles de la Tourette syndrome Brief descriptions in 1489 in a priest and more completely by Thomas Willis in 1701 in an Oxfordshire family

28 Thomas Willis 1621-1675

29 Thomas Willis Local boy, born on a farm in Great Bedwyn Moved to North Hinksey then Oxford Initially consulted in the Abingdon market place

30

31 Thomas Willis “Father of neuroscience” Major contributions to neuroanatomy Many original descriptions of disorders such as restless legs syndrome, narcolepsy, achalasia of the oesophagus etc

32 Gilles de la Tourette syndrome Fuller description in 1825 by Itard of the Marquise de Dampierre, who had lifelong tics with coprolalia and whose case was subsequently re-reported in 1850, 1851, 1873 and 1885 (twice)

33 Georges Gilles de la Tourette 1857-1904

34 Johnson’s diagnosis?

35 Gilles de la Tourette Born into a provincial medical family, studied in Paris Described as having boundless energy and a very short-temper, prepared to argue over anything, and as ugly as a Papuan idol Many literary and artistic interests

36 Gilles de la Tourette Translated Beard’s description of the jumping Frenchmen of Maine Asked by Charcot to study the ‘chaos of the choreas’ Found no jumping Frenchmen but in 1885 described 9 patients, 6 of whom he had examined personally, with his syndrome

37 Gilles de la Tourette Drew attention to the association with learning difficulties, and a family history of ‘mental instability’ Emphasised the pathognomonic coprolalia (present in 5 of his cases)

38 1893

39 Georges Gilles de la Tourette Later (probably already ill) influenced by his contemporary Guinon, who thought that all cases progressed on to psychosis and who distinguished between TS (incurable) and hysterical tics (alleviated by hypnosis) Died, probably of neurosyphilis

40

41 Sigmund Freud Attended lectures by Charcot and Gilles de la Tourette on tics Attributed the multiple motor and phonic tics of Frau Emmy von N to hysteria resulting from repressed childhood trauma, and treated her with hypnosis and catharsis on two occasions with benefit

42 Subsequent thinking Psychodynamic interpretations remained popular during early 20 th century Similar phenomena (‘acquired Tourettism’) following von Economo’s encephalitis Turning point in 1961 with reports of response to haloperidol (and frontal lobectomy)

43 Now Genetics Drug treatments Neurosurgical treatments

44 James Parkinson 1755-1824

45 James Parkinson Born into a medical family, briefly studied at The London Hospital, apprenticed to his father who was a GP in Hoxton Industrial revolution and expansion of London French revolution and radical politics

46 Medical interests Busy GP Parish doctor (surgeon, apothecary and man-midwife) –First fever wards in London, improving outcome from typhus Medical attendant to local psychiatric hospitals –Campaigning for better conditions and against impressment into services

47

48 Medical Interests Papers on: Child care and child abuse Appendicitis Resuscitation from drowning Lightning injury Gout “Hints on the improvement of trusses (for the use of the labouring poor)”

49 Other scientific interests Textbook of chemistry Three volume textbook on palaeontology (‘Organic remains of a former world’), founder member of the London Geological Society, first description of the geological strata of London and its fossils

50

51 Parkinson the political radical Campaigner for parliamentary reform and the extension of suffrage Published many pamphlets as Old Hubert and under his own name In ‘Revolutions without bloodshed; or, reformation preferable to revolt’ (1794) called for wildly radical reforms:

52 Taxes might be proportioned to the abilities of those on whom they are levied, and not made to fall heavier on the poor than the rich The heavy excise taxes on the ‘necessities of life’ (soap, starch, candles, beer) be removed

53 Workmen might no longer be punished with imprisonment for uniting to obtain an increase in wages Some proportion might be preserved between crime and punishment

54 Children of the poor [might be given] such instruction as might enable them to earn their living, and form a just notion of their rights and duties as members of society

55 London Corresponding Society Popgun plot (1794) allegedly to kill King George III with a poison dart

56 Subpoena’d to attend the Privy Council and cross-examined by Pitt and the Attorney General when a fellow committee member was being tried for high treason Not charged but steered clear of politics thereafter

57 An essay on the shaking palsy

58 An essay on the shaking palsy (1817) Describes: Tremor Slowness, difficulty writing Posture Gait Sleep disturbance Constipation Drooling

59 An essay on the shaking palsy (1817) Does not describe: Rigidity Dementia (beyond terminal ‘slight delirium’)

60 An essay on the shaking palsy (1817) 6 cases: 1 followed in detail from onset to death 1 seen 12 years into the disease with a stroke, and then followed 1 seen briefly for treatment of pulmonary empyema 2 “casually met with in the street” 1 “only seen at a distance”

61 Impact Charcot amplified the description of the tremor, described cases without tremor, pointed out the core feature of rigidity, mentioned the association with dementia, and referred to it as Parkinson’s disease

62 Impact British authors (such as Gowers) continued to prefer the terms ‘shaking palsy’ and ‘paralysis agitans’ until late in the 19 th century

63 Subsequent developments Lewy bodies Dopamine Drug treatments Neurosurgical treatments

64 Is it a disease? Several genetic causes LRRK 2 is the commonest, and accounts for 3% of sporadic PD as well as much familial PD LRRK 2 cases have Lewy bodies or tau pathology

65 Summary Sydenham and the choreas Willis, Gilles de la Tourette and the tics Parkinson and his disease

66 “If I have seen further it is because I am standing on the shoulders of giants” Isaac Newton

67 “If I have not seen as far as others, it is because giants were standing on my shoulders.” Hal Abelson


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