Tics, tremors and trusses

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• Tics, tremors and trusses

• A very brief history of movement disorders
• Dr Graham LennoxConsultant NeurologistGreat
  Western Hospitals NHS Foundation Trust

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What are movement disorders?

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

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Medieval movement disorders

• Tremor and palpitations
• Chorea and convulsions

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Tremor

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

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Chorea

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

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

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Thomas Sydenham 1624-1689
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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.

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Thomas Sydenham

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

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Thomas Sydenham
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.
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Thomas Sydenham

• Sydenhams chorea mentioned in an aside
• Link to rheumatic fever not noted

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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.

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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."

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Chorea classification
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Modern view of Sydenhams

• Mixture of chorea and tics
• Often psychiatric features
• Antibiotics
• Dopamine blocking drugs

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Samuel Johnson 1709-1784
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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

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Samuel Johnson
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Johnsons movement disorder

• Rejected as schoolmaster
• He has such a way of distorting his face
  which though he cant 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

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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)

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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)

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

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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

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Johnsons diagnosis?
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Gilles de la Tourette syndrome

• Early onset
• Multiple motor and phonic tics
• Obsessive-compulsive disorder

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Gilles de la Tourette syndrome

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

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Thomas Willis 1621-1675
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Thomas Willis

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

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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

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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)

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Georges Gilles de la Tourette 1857-1904
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Johnsons diagnosis?
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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

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Gilles de la Tourette

• Translated Beards 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

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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)

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1893
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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

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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

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Subsequent thinking

• Psychodynamic interpretations remained popular
  during early 20th century
• Similar phenomena (acquired Tourettism)
  following von Economos encephalitis
• Turning point in 1961 with reports of response to
  haloperidol (and frontal lobectomy)

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Now

• Genetics
• Drug treatments
• Neurosurgical treatments

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James Parkinson 1755-1824
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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

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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

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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)

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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

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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

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• 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

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• 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

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• 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

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London Corresponding Society

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

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• Subpoenad 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

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

• Does not describe
• Rigidity
• Dementia (beyond terminal slight delirium)

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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

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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
  Parkinsons disease

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Impact

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

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Subsequent developments

• Lewy bodies
• Dopamine
• Drug treatments
• Neurosurgical treatments

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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

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Summary

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

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

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