Adams–Stokes syndrome

Stokes–Adams syndrome (alternative eponyms include Gerbec-Morgagni-Adams-Stokes syndrome)^[1] refers to a sudden, transient episode of syncope, occasionally featuring seizures. Named after two Irish physicians, Robert Adams (1791–1875)^[2] and William Stokes (1804–1877)^[3], the first description of the syndrome was published in 1717 by Slovene physician Marcus Gerbezius which was 44 years after its publication quoted by Giovanni Battista Morgagni.

Signs and symptoms

Prior to an attack, a patient may become pale, their heart rhythm experiences a temporary pause, and collapse may follow. Normal periods of unconsciousness last approximately thirty seconds; if seizures are present, they will consist of twitching after 15–20 seconds. Breathing continues normally throughout the attack, and so on recovery the patient becomes flushed as the heart rapidly pumps the oxygenated blood from the pulmonary beds into a systemic circulation which has become dilated due to hypoxia.^[4]

As with any syncopal episode that results from a cardiac dysrhythmia, the faints do not depend on the patient's position. If they occur during sleep, the presenting symptom may simply be feeling hot and flushed on waking.^[4]

Diagnosis

Stokes-Adams attacks may be diagnosed from the history, with paleness prior to the attack and flushing after it particularly characteristic. The ECG will show asystole, an AV block, or ventricular fibrillation during the attacks.

Causes

The attacks are caused by lack of cardiac output due to cardiac asystole, heart block, Lev's disease or ventricular fibrillation. The resulting lack of blood flow to the brain is responsible for the faint.

Treatment

Initial treatment can be medical, involving the use of drugs like isoproterenol (Isuprel) and epinephrine (adrenaline). Definitive treatment is surgical, involving the insertion of a pacemaker – most likely one with sequential pacing such as a DDI mode as opposed to the older VVI mechanisms,^[4] and the doctor may arrange the patient to undergo Electrocardiography to confirm this type of treatment.^[5]

Prognosis

If undiagnosed (or untreated), Stokes–Adams attacks have a 50% mortality within a year of the first episode. The prognosis following treatment is very good.

References