Stokes-Adams syndrome

Description

Stokes-Adams syndrome is an abrupt, transient loss of consciousness due to a sudden but pronounced decrease in the cardiac output, which is caused by a paroxysmal shift in the mechanism of the heart beat, usually an atrioventricular block.

First descriptions by Gerbezius in 1717 later referenced by Morgagni in 1761 with his two cases. Further description in the English literature by Spens (1792) and Burnett (1824) before the publications of Adams (1827) and Stokes (1846).

Eponymous attribution by Henri Huchard as “la maladie de Stokes-Adams” in 1899.

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 De sedibus, et causis morborum per anatomen indagatis from 1761.

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

…fellow-citizen Anastasio Poggi, a grave and worthy priest. 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

In the same letter, Morgagni refers to the 1717 publication by Slovenian physician Marcus Gerbezius (1658-1718)

But, perhaps, you will suspect, whether the rarity of the pulse be, in fact, a very uncommon symptom, to remain after an epilepsy, in hypochondriac patients, when you shall have compar’d this observation of mine with that of the celebrated Gerbezius (t), which describes the pulse of a strong hypochondriac man, “who was now and then subject to slight epileptic ‘paroxysms’, even when he was in health”, as being “so very slow, that before the subsequent pulsation follow’d that which went before, three pulsations would certainly have pass’d in another healthy person.” 

Morgagni 1761; IX(8): 195

Then in De morbis thoracis (letter LXIV, article 5) regarding the case of a 64 year old merchant from Padua, a full-bodied man, who collapsed following vertigo:

When visiting by way of consultation, I found with such a rarity of the pulse that within the 60th part of an hour the pulsations were only 22 – and this rareness which was perpetual – was perceived to be even more considerable, as often as even two (epileptic) attacks were at hand – so that the physicians were never deceived from the increase of the rareness they foretold a paroxysm to be coming on.

Morgagni 1761; LXIV(5): 505

Death occurred 15 months after the onset of the symptoms. At autopsy, Morgagni observed that the heart was dilated because of the enlargement of ventricles; the aorta was dilated in the same way from the beginning of its curve:

Cor vero amplum valde ob dilatatos ventriculos, non ob parietes factos crassiores. Magna etiam arteria ad curvatura; usque initium aequo latior…spirituum et nervorum vitium.

But the heart is very large because of the dilated ventricles, not because the walls are made thicker. A large artery also has a curvature; until the beginning is equally wider…spiritual and nervous disorders.

Morgagni 1761; LXIV(5): 507

Morgagni remarked that the slow pulse rate could not be connected to aneurisms of the heart and aorta and that the disease had been produced by a disorder of the nerves (“spirituum et nervorum vitium”).

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, however, as far as I could judge, were 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…

Upon visiting him on the morning of the 17th, I found that he had been attacked by several fits during the night. Upon examining his pulse I found that it beat only twenty-three strokes in the minute…an hour after I found it in precisely the same state as before. He was now directed to take some spirits of hartshorn; but, by mistake, it was given him very little diluted, and produced much uneasiness in his mouth and throat. From this cause I found him in great distress at one o’clock; but it seemed to have produced no change in the state of his pulse, which at this time beat twenty-four beats in a minute and was of the same strength and regularity as before.

In the morning of the 18th I was informed that he had been frequently faint…his pulse beat only twenty-six strokes in the minute. About 8 in the evening he had no sooner smelt it [newly toasted bread] than he felt some of the sensations of a beginning fit; and, as soon as he had tasted it, he almost instantly cried out, and fell back senseless, with smart convulsions of all his muscles. He apparently recovered in a few seconds; but hardly any pulse could be felt for a good many seconds. On the morning of the 19th I learnt that…had been attacked with frequent fits attended with violent convulsions…at three in the afternoon I found that it (the pulse) beat only ten strokes a minute, though it still continued equally strong and regular as before…he expired on the 20th.

The day after his death the body was opened by Mr. Fife, and, upon the most careful examination, no morbid appearance of any consequence could be discovered either in the thorax or abdomen.

Spens 1792: 458-465

1824 – Sir William Burnett 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 wrote a 100 page monograph which included descriptions of significant pathological findings from patients with seizures and bradycardia, observed when partial atrioventricular block is approaching completeness and less frequently in established complete heart block.

1846 – William Stokes published his ‘Observations of some cases of permanently slow pulse‘

Associated Persons

• Marcus Gerbezius (1658-1718)
• Giovanni Battista Morgagni (1682-1771)
• Thomas Spens (1763-1842)
• Sir William Burnett (1779-1861)
• Robert Adams (1791-1875)
• William Stokes (1804-1878)

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

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.

Review references

• Pletnew D. Der Morgagni-Adams-Stokessche Symptomenkomplex. In: Kraus F. et al. (eds) Ergebnisse der Inneren Medizin und Kinderheilkunde. Springer 1908: 47-67
• 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.