Transcript A shorty history of Tics, Twitches and Tremors

Tics, tremors and trusses
A very brief history of movement disorders
Dr Graham Lennox
Consultant Neurologist
Great Western Hospitals NHS Foundation Trust
What are movement disorders?
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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.
Thomas Sydenham
• Mainly famous for his treatment of
infectious diseases
• Laudanum (opium)
• Cinchona (quinine) for malaria
• Detailed description of gout
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.”
Thomas Sydenham
• Sydenham’s chorea mentioned in an
aside
• Link to rheumatic fever not noted
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.”
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
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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:
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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:
Tremor
Slowness, difficulty writing
Posture
Gait
Sleep disturbance
Constipation
Drooling
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