Tics, tremors and trusses

A very brief history of movement disordersDr Graham Lennox

Consultant NeurologistGreat Western Hospitals NHS Foundation Trust

What are movement disorders?

• Parkinsonism• Tremor• Chorea• Dystonia • Myoclonus• Tics• Stereotypies• Restless legs

Medieval movement disorders

• Tremor and palpitations

• Chorea and convulsions

Tremor

• Galen, Sylvius and others distinguished between action tremor and rest tremor

Chorea

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

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

Thomas Sydenham 1624-1689

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.

• Mainly famous for his treatment of infectious diseases

• Laudanum (opium)

• Cinchona (quinine) for malaria

• Detailed description of gout

Thomas Sydenham

Thomas Sydenham

“Among the remedies which it has pleasedAlmighty God to give to man to relieve hissufferings, none is so universal and soefficacious as opium.”

“A man is as old as his arteries.”

• Sydenham’s chorea mentioned in an aside

• Link to rheumatic fever not noted

Thomas Sydenham

• “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)

Schedula Monitoria de Novae Febris Ingressa

(1686) • “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."

Chorea classification

Modern view of Sydenham’s

• Mixture of chorea and tics

• Often psychiatric features

• Antibiotics

• Dopamine blocking drugs

Samuel Johnson 1709-1784

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

Samuel Johnson

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”

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)

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)

Suppressibility

“He could sit motionless, when he was told to do so, as well as any other man.”

(Sir Joshua Reynolds)

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

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”

Johnson’s diagnosis?

Gilles de la Tourette syndrome

• Early onset

• Multiple motor and phonic tics

• Obsessive-compulsive disorder

Gilles de la Tourette syndrome

• Brief descriptions in 1489 in a priest and more completely by Thomas Willis in 1701 in an Oxfordshire family

Thomas Willis 1621-1675

Thomas Willis

• Local boy, born on a farm in Great Bedwyn

• Moved to North Hinksey then Oxford

• Initially consulted in the Abingdon market place

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

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)

Georges Gilles de la Tourette 1857-1904

Johnson’s diagnosis?

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

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

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)

1893

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

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

Subsequent thinking

• Psychodynamic interpretations remained popular during early 20th century

• Similar phenomena (‘acquired Tourettism’) following von Economo’s encephalitis

• Turning point in 1961 with reports of response to haloperidol (and frontal lobectomy)

Now

• Genetics

• Drug treatments

• Neurosurgical treatments

James Parkinson 1755-1824

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

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

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)”

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

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:

• 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

• 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

• 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

London Corresponding Society

• Popgun plot (1794) allegedly to kill King George III with a poison dart

• 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

An essay on the shaking palsy

An essay on the shaking palsy (1817)Describes:TremorSlowness, difficulty writingPostureGaitSleep disturbanceConstipationDrooling

An essay on the shaking palsy (1817)

Does not describe:

• Rigidity

• Dementia (beyond terminal ‘slight delirium’)

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”

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

Impact

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

Subsequent developments

• Lewy bodies• Dopamine

• Drug treatments• Neurosurgical

treatments

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

Summary

• Sydenham and the choreas

• Willis, Gilles de la Tourette and the tics

• Parkinson and his disease

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

Isaac Newton

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

Hal Abelson