Stokes-Adams syndrome

These syncopal attacks are typically abrupt in onset and recovery, often accompanied by pallor, slow pulse, and, occasionally, seizure-like motor activity due to cerebral hypoperfusion. While consciousness is typically regained spontaneously as cardiac output is restored, the condition poses significant morbidity and mortality risks due to prolonged asystole.

The syndrome is classically associated with advanced atrioventricular (AV) block but may also be observed in other bradyarrhythmias or pauses due to sick sinus syndrome. Modern management includes pacemaker implantation to prevent recurrence.

The eponym commemorates Robert Adams (1791–1871) and William Stokes (1804–1878), two Irish physicians who described the clinical features of sudden syncope associated with slow or irregular cardiac rhythms. Though Giovanni Battista Morgagni also documented similar cases in the 18th century, the triad of bradycardia, syncope, and convulsion came to be most strongly associated with the writings of Adams and Stokes in the mid-19th century.

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History of Stokes-Adams syndrome

1717 – Marcus Gerbezius described two cases, presenting with extremely slow pulse rate and seizures. Both patients suffered from slow but regular pulses, dizziness, syncope, and occasional epileptic seizures. His careful observations of the bradyarrhythmia and seizures were recorded in detail.

1761 – Giovanni Battista Morgagni described Stokes-Adams attacks in at least 2 sections of in De Sedibus et Causis Morborum, described several cases of sudden death and collapse linked to cardiac disease “a slow, unequal pulse preceded the sudden cessation of life.”

For example in De Morbis Capitis (letter IX, article 7); he details the case of the priest Anastasio Poggi:

He was in his sixty-eighth year, of a habit moderately fat, and of a florid complexion, when he was first seized with the epilepsy, which left behind it the greatest slowness of pulse, and in like manner, a coldness of the body.

Morgagni 1761; IX(8): 192

1792 – Thomas Spens (1763-1842) published the first account in the English literature of Stokes-Adams syndrome with seizures secondary to heart block.

He described the case of a 54 year old male with a slow, regular pulse; random faints with loss of consciousness; an undisturbed pulse-rate with a static character, even under duress distress; and convulsions, which if prolonged, resulted in hardly any pulse being felt.

On the 16th of May 1792, about 9 o’clock in the evening, I was sent for to see T. R, a man in the 54th year of his age, a common labouring mechanic…I was much surprised, upon examining the state of his pulse, to find that it beat only twenty-four strokes to the minute. These strokes…at perfectly equal intervals, and of the natural strength of the pulse of a man in good health. He informed me that about three o’clock in the afternoon he had been suddenly taken ill whilst standing in the street; that he had fallen to the ground senseless; and that, according to the accounts given him by those who were present, he had continued in that state for about five minutes…

Spens 1792: 458-465

1824 – Sir William Burnett (1779-1861) recounted the case of a 46 year old officer of the Navy suffering from bouts of epilepsy with a remarkable slowness of the pulse. Burnett related his case to the two previously described cases of Morgagni.

During the paroxysms he exhibited all the usual symptoms of epilepsy; yet these were of very short duration, sometimes lasting only a few minutes, and never being followed by a disposition to sleep…the countenance was very sallow, tongue white, and pulse 24 in the minute.

Burnett 1824: 205

1827 – Robert Adams (1791-1875) provided the first clinical description of a patient suffering bradycardia, syncopal attacks, and signs of cerebral hypoperfusion. His 1827 case described a 68-year-old man with “remarkable slowness of the pulse” and apoplectic attacks, leading him to suggest the heart as the primary source of cerebral symptoms.

In May 1819, I saw this gentleman: he was just then recovering from the effects of an apoplectic attack, which had suddenly seized him three days before…What most attracted my attention was, the irregularity of his breathing, and remarkable slowness of the pulse, which generally ranged at the rate of 30 in a minute.

Where the heart is slow in transmitting the blood it receives… [this is] a means of accounting for the lethargy, loss of memory, and vertigo which attends these cases… apoplexy must be considered less a disease in itself than symptomatic of one, the organic seat of which was in the hear.

Adams 1827

1846 – William Stokes (1804-1878) published Observations of some cases of permanently slow pulse in the Dublin Quarterly Journal of Medical Science. He reviewed five cases of sudden fainting spells with very slow (often regular) pulse rates (sometimes as low as 10–25/min); occasional convulsions; and recovery, often with flushing as heart rhythm resumes. Stokes explicitly acknowledged Adams’s earlier work and framed his own as building on that foundation

Mr Adams has recorded a case of permanently slow pulse, in which the patient suffered from repeated cerebral attacks of an apoplectic nature, though not followed by paralysis The attention of subsequent writers on diseases of the heart, has not been sufficiently directed to this case, which is an example of a very curious and, as there is reason to believe, special combination of symptoms The following cases will still further elucidate a subject on which there is but little information extant

The observations are published with the view of drawing the attention of the Profession to a combination of cerebral and cardiac phenomena, of which our knowledge is still imperfect

Stokes 1846

1899 – Eponymous attribution by Henri Huchard as “la maladie de Stokes-Adams“

1911 – Sir Thomas Lewis (1881–1941) revisited the Adams-Stokes syndrome with detailed electrocardiographic studies, linking the condition to complete heart block and introducing objective measures of arrhythmia.

The syndrome of Adams and Stokes may be described as a clinical condition in which a persistently slow pulse is associated with syncopal or epileptic attacks.

In discussing certain pathological aspects of this syndrome it should be clearly understood at the outset that the definition includes several separate pathological entities. The majority of the typical cases are unquestionably the result of heart-block.

Lewis 1911

1952 – S. de Boer published On the Origin and Essence of the Morgagni-Adams-Stokes Syndrome, highlighting the historical attributions and reinforcing the key diagnostic features.

Late 20th century – Implantable cardiac pacemakers became the standard of care, dramatically improving outcomes in patients with high-grade AV block and recurrent Stokes-Adams attacks.

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Associated Persons

• Marcus Gerbezius (1658-1718)
• Giovanni Battista Morgagni (1682-1771) – Described collapse and death associated with slow pulse.
• Thomas Spens (1763-1842)
• Sir William Burnett (1779-1861)
• Robert Adams (1791-1875) – First detailed clinical correlation of syncope with bradycardia
• William Stokes (1804-1878) – Extended Adams’ work; observed bradycardia and convulsions.
• Thomas Lewis (1881–1941): Introduced electrocardiographic confirmation

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Alternative names

• Stokes-Adams syndrome; la maladie de Stokes-Adams (1899)
• Adams-Stokes syndrome
• Morgagni-Adams-Stokes syndrome (1908)
• Gerbezius-Morgagni-Spens-Adams-Stokes syndrome
• Gerbezius-Morgagni-Adams-Stokes syndrome or Syndrome GMAS

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References

Historical references

• Gerbezii M. Constitutio; anni 1717 a Dn. D. Marco Gerbezio Labaco 10 Decemb. descripta. Academiae Caesareo-Leopoldinae Naturae Curiosorum Ephemerides, 1719; [Appendix: 22–24]
• Morgagni GB. De Morbis Capitis (letter IX, article 7). De sedibus, et causis morborum per anatomen indagatis libri quinque. 1765 [English translation: Diseases of the Head (letter IX, article 7). The seats, and the causes of diseases investigated by anatomy 1761; 192-195]
• Morgagni GB. De morbis thoracis (letter LXIV, article 5). De sedibus, et causis morborum per anatomen indagatis libri quinque. 1765 [English translation: Diseases of the thorax (letter LXIV, article 5). 1761; 505-508]
• Spens T. History of a case in which there took place a remarkable Slowness of the Pulse. Medical commentaries. 1792: 458-465
• Burnett W. Case of Epilepsy, attended with remarkable Slowness of the Pulse. Med Chir Trans. 1827;13(Pt 1):202-211.
• Adams R. Cases of diseases of the heart accompanied with pathological observations. Dublin Hospital Reports. 1827; 4: 353–453.
• Stokes W. Observations of some cases of permanently slow pulse. Dublin Quarterly Journal of Medical Science. 1846;2(1):73–85. Reprinted in Medical Classics 1939; 3: 727-738.
• Pletnew D. Der Morgagni-Adams-Stokessche Symptomenkomplex. In: Kraus F. et al. (eds) Ergebnisse der Inneren Medizin und Kinderheilkunde. Springer 1908: 47-67

Review references

• Cremer M. Über die direkte Ableitung der Aktionströme des menschlichen Herzens vom Oesophagus und über das Elektrokardiogramm des Fötus. Münch Med Wochenschr. 1906;53:811
• Lewis T. The so-called “Adams-Stokes’ syndrome. in: The mechanism of the heart beat with especial reference to its clinical pathology. London, Shaw. 1911
• De Boer S. On the origin and essence of the Morgagni-Adams-Stokes syndrome. Ann Intern Med. 1952; 37(1): 48-64.
• Harbison J, Newton JL, Seifer C, Kenny RA. Stokes Adams attacks and cardiovascular syncope. Lancet. 2002; 359(9301): 158-160.

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