EPONYM

Eponym

Stokes Adams attacks and cardiovascular syncope

J Harbison, J L Newton, C Seifer, R A Kenny

Case report
A 71–year-old woman was admitted for investigation of collapses of unknown cause. She had a history of occasional faints since
childhood, but in the previous 6 months the collapses had become more sinister. Collapses occurred without warning, at least once
per week, and were associated with transient loss of consciousness. A witness account stated that the woman became very pale
during the collapses but the witness did not recall the woman flushing on recovery. The patient was assigned a provisional diagnosis
of Stokes Adams attacks and referred for further cardiovascular investigations.
The woman’s previous history was unremarkable apart from a diagnosis of mild hypertension treated with bendroflumethiazide. She
was normotensive and results of cardiovascular examination were otherwise normal. Her electrocardiogram showed normal sinus
rhythm and no abnormalities. Cardiovascular investigations showed a transient, symptomless 15 mm Hg postural drop in systolic
blood pressure resulting from 3 min of standing unaided. Carotid sinus massage resulted in a sinus pause of 4·1 s, with reproduction
of the patient’s syncopal symptoms. A diagnosis of cardioinhibitory carotid sinus syndrome was made, the patient was referred for
insertion of a permanent pacemaker, and has had no further collapses since this procedure.

Stokes Adams Attacks Adams attack is still commonly used by non-specialist

The classical description of a Stokes Adams attack is of doctors although rarely used by cardiologists and doctors
collapse without warning, associated with loss of specialising in the management of syncope. Heart block still
consciousness lasting a few seconds. The affected individual causes syncope but is rare (<3% of cases)3 and does not
is pale initially, but can become flushed on recovery from often present with classical Stokes Adams attacks. By
the episode. This flushing does not always occur, and some contrast, various other cardiovascular disorders can present
seizure-like activity can be noted if the attack is prolonged. with sudden, transient loss of consciousness. These
The disorder is typically associated with complete heart collapses might previously have been described clinically as
block, but has also been described in other diseases such as Stokes Adams attacks, but improved understanding of
tachy-brady syndrome.1 cardiovascular physiology and pathology and techniques for
Stokes (figure 1) first described syncope associated with investigation, and the development of specific diagnostic
bradycardia in his 1846 article, “Observations of some cases of classifications have left the term increasingly redundant.
permanently slow pulse”,2 in which he also described what is Assessment of patients with collapse begins with a history
now known as periodic or detailing syncopal episodes, other problems, and potential
Cheyne Stokes breathing. culprit drugs. Thorough physical examination should be
In the same article, he done, including measurement of blood pressure and
acknowledged Adams, cardiovascular examination. In addition to a 12–lead
who had described a electrocardiogram, routine haematological and biochemical
similar case of syncope in investigations will identify potential contributory disorders
1827. These were not, such as anaemia, hyponatraemia, and hypothyroidism. The
however, the first table shows tests, treatments, and most common causes of
recorded descriptions of cardiovascular syncope.4
the syndrome. Giovanni Less common causes of cardiovascular syncope include
Battista Morgagni obstructive cardiac lesions (eg, aortic stenosis, atrial
(1682–1771) described a myxoma, and hypertrophic cardiomyopathy), cerebro-
very similar disorder in vascular insufficiency, subclavian steal syndrome, psycho-
“De sedibus et causis genic syncope, hyperventilation, and other situational
morborum” (1761), a work syncopes such as cough or sneeze syncope, and micturition
that also contains the first syncope. Many disorders can mimic syncope (panel).
known descriptions of
angina pectoris and Robert Adams and William Stokes
intracardiac thrombus. Figure 1: William Stokes The 19th century is often referred to as the golden age of
The term Stokes Courtesy of RCPI. Irish medicine. Substantial advances were made in scientific
and clinical aspects of medicine by doctors working in
Lancet 2002; 359: 158–60 Dublin hospitals, and these are reflected by an array of
eponyms. These physicians and surgeons include Robert
Cardiovascular Investigations Unit, Royal Victoria Infirmary, Graves, Dominic Corrigan, John Cheyne, and Abraham
Newcastle upon Tyne, UK (J Harbison MRCPI, J L Newton MRCP, Colles in addition to Robert Adams and William Stokes.
C Seifer MRCPI, R A Kenny FRCPI) Robert Adams (1791–1875) (figure 2) was born in
Correspondence to: Dr Joseph Harbison, Department of Geriatric Dublin, studied at Trinity College, and, despite his later
Medicine, Care of the Elderly Offices, Royal Victoria Infirmary, contributions to medicine, trained to be a surgeon. He did
Newcastle upon Tyne NE2 4LP, UK not achieve his MB degree until 1842, the year in which he
(e-mail: j.a.harbison@ncl.ac.uk) was also awarded an MD by Trinity College. He worked

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For personal use. Only reproduce with permission from The Lancet Publishing Group.
 EPONYM

Meath Hospital. He later attended the University of

Non-cardiovascular causes of syncope Edinburgh from which he obtained his MD in 1825. Stokes
then returned to the Meath Hospital in Dublin, where
Cause Effect
he became both a colleague and close friend of Graves,
Neurological Seizures with whom he developed a new system of clinical
Transient ischaemic attacks instruction for medical students that emphasised the
Raised intracranial pressure importance of bedside teaching. He researched the use
Narcolepsy or cataplexy of the stethoscope, and his book, “The diagnosis and
Metabolic Hypoglycaemia treatment of diseases of the chest” (1837), made his name as a
Alcohol or other drug intoxication physician. Stokes was also an internationally renowned
Psychiatric disorders Psychogenic collapse teacher. His edited lectures were published as “Clinical
Major depression lectures on the theory and practice of medicine” (1840), which
Panic attacks for many years was a standard text in American medical
Cardiopulmonary Myocardial infarction schools. Despite his non-conformist beliefs, he was
Pulmonary embolism appointed professor of medicine at Trinity College in 1849.
Cardiac tamponade In 1854, he published “Diseases of the heart and aorta” in
Mechanical cardiac lesions which, controversially at that time, he stressed the benign
nature of many heart murmurs. This book also contains
the first description of paroxysmal tachycardia and an
mainly as a consultant surgeon in the Richmond and Sir early clinical description
Patrick Dun’s hospitals in Dublin, but had varied medical of pericarditis.6 He was
interests and more than 100 publications to his name. His awarded honorary doct-
publications include “Cases of the diseases of the heart orates by the universities
accompanied with pathological observations”,5 in which he of Oxford, Cambridge,
described a patient experiencing blackouts associated with a and Edinburgh, was
heart rate of 30 beats per minute. Adams also had an president of the British
especial interest in joint pathology and was the first to Medical Association,
describe the synovial cyst arising from the knee joint, which and became president of
was later described by William Morrant Baker (1839–96) in the Royal College of
1877 and named eponymously after him. Adams was a Physicians of Ireland in
distinguished medical teacher and established two medical 1849. A marble statue
schools in Dublin, the first of which was burned down by an of Stokes still graces
angry mob objecting to the practice of body snatching. He the college. He was a
served as president of the Royal College of Surgeons in noted conversationalist,
Ireland on three occasions, and was appointed surgeon to biographer, and anti-
Queen Victoria in 1861.6 quarian and was the first
William Stokes (1804–77) was born in Ballinteer, which practising physician to
is now a suburb of Dublin. His non-conformist religious be elected president
views prevented him attending Trinity College, so instead Figure 2: Robert Adams of the Royal Irish
he first studied medicine under Robert Graves in Dublin’s Courtesy of RCPI. Academy.6

Syndrome and description Investigation Treatment

Vasovagal syncope
The commonest cause of the simple faint.
Collapse results from hypotension resulting
from sudden dilatation of venous capacitance
vessels sometimes associated with vagally
mediated bradycardia.
Orthostatic hypotension
A sudden fall in blood pressure occurring on
standing. It can result from hypovolaemia resulting
from drugs, dehydration, or from vasodilators and
is associated with atherosclerosis, diabetes,
and long-lasting confinement to bed.
Carotid sinus hypersensitivity (three types)
1) Cardioinhibitory, resulting in symptomatic
asystole of longer than 3 s.
2) Vasodepressor, resulting in systolic blood
pressure fall ⭓50 mm Hg.
3) Mixed, a combination of 1) and 2).
Cardiac arrhythmias
Tachyarrhythmias (eg, ventricular tachycardia)
and bradyarrhythmias (eg, sinus arrest and
complete heart block) can cause syncope
through loss of cardiac output.
Frequent causes of neurocardiovascular collapse
Prolonged head-up tilt test done in controlled
surroundings with, if necessary,
pharmacological provocation (isoproterenol,
glyceryl trinitrate).
An active stand under supervised continuous
blood pressure monitoring. A drop of 20 mm Hg
systolic or 10 mm Hg diastolic blood pressure
within 3 min of standing, with reproduction
of symptoms is diagnostic. The value of the
cuff sphygmomanometer in assessments is
questionable.
Carotid sinus massage done by skilled staff
in monitored conditions.
Holter monitoring, external or implantable loop
monitoring, and electrophysiological studies.
Advice on avoidance of situations that trigger
faints, such as standing for a long time in
supermarket queues. If urinary sodium is low,
salt supplements can be given to reduce
hypovolaemia. There may be a role for ␤-blockers,
selective serotonin reuptake inhibitor
antidepressants, fludrocortisone, ␣-agonists,
and, rarely, pacing if there is substantial
associated bradycardia.
Volume replacement and modification of drug
treatment. Compression hosiery reduces
venous pooling in legs. Fludrocortisone increases
sodium retention and reduces hypovolaemia.
Midodrine, an alpha-receptor agonist, increases
blood pressure.
Advice given to avoid trigger factors such as tight
collars. The cardioinhibitory form can be treated
with permanent atrioventricular pacing.
Appropriate antiarrhythmic therapy and insertion
of a pacemaker as necessary.

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For personal use. Only reproduce with permission from The Lancet Publishing Group.
 EPONYM
Conclusion
The term Stokes Adams attack will probably continue to
decline in medical use in favour of more specific
pathophysiological descriptions of cardiovascular syncope.
Should this happen, doubtless William Stokes will still be
remembered as a pioneer cardiologist and medical
educator. He will also be remembered for the periodic
breathing that still bears his name. But what will Robert
Adams, the surgeon who was both an early cardiologist
and rheumatologist, be remembered for? Possibly the
differential diagnosis of deep vein thrombosis of the calf
could in future include “rupture of a Baker Adams cyst”.
We thank Royal College of Physicians of Ireland for permission to use
images of Stokes and Adams, and Robert Mills and Davis Coakley for
their help and advice.
160
For personal use. Only reproduce with permission from The Lancet Publishing Group.
References
1 Sigurd B, Sandoe E. Management of Stokes-Adams syndrome.
Cardiology 1990; 77: 195–208.
2 Stokes W. Observations of some cases of permanently slow pulse.
Dublin Q J Med Sci 1846; 11: 73–85.
3 Getchell WS, Larsen GC, Morris CD, McAnulty JH. Epidemiology
of syncope in hospitalized patients. J Gen Intern Med 1999; 14:
677–87.
4 Kenny RA, O’Shea D, Parry SW. The Newcastle protocols for
head-up tilt table testing in the diagnosis of vasovagal syncope,
carotid sinus hypersensitivity, and related disorder. Heart 2000;
83: 564–69.
5 Adams R. Cases of diseases of the heart accompanied
with pathological observations. Dublin Hosp Rep 1827; 4:
353–453.
6 Coakley D. Irish masters of medicine. Dublin: Town House Books,
1992.
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