Abstract
Stokes-Adams syndrome is defined as a transient loss of consciousness with spontaneous recovery after a decrease in cardiac output that leads to a state of cerebral hypoxia and may cause seizures. It is a rare and poorly documented condition. High-degree atrioventricular block, paroxysmal ventricular arrest, and pulseless ventricular tachycardia have been described as causes, and depending on the case, temporary or permanent pacemaker implantation or cardiac defibrillation may be necessary. We present the case of a 78-year-old male patient with Stokes-Adams syndrome secondary to 2:1 atrioventricular block requiring definitive pacemaker implantation without recurrence of syncopal or convulsive episodes. It is important to consider Stokes-Adams syndrome as a possible cause of syncope or seizures and that the recognition of this disease through an adequate clinical history and interpretation of paraclinical tests can save lives due to the risk of sudden death. There is not much information about this syndrome, since there are few cases reported in the literature.
Plain Language Summary
Stokes-Adams syndrome (SAS) is a rare cardiac disorder characterized by loss of consciousness with or without seizures. For diagnosis, in addition to the above symptoms, an electrocardiogram showing deep bradycardia accompanied by high-grade atrioventricular block, pulseless ventricular tachycardia, and paroxysmal ventricular arrest, among others, is required. We present the case of a 78-year-old male patient with Stokes-Adams syndrome secondary to 2:1 atrioventricular block requiring definitive pacemaker implantation without recurrence of syncopal or seizure episodes. The aim of this report is to remind people of SAS as a possible cause of syncope or seizures, and that recognition of this disease through an adequate clinical history and interpretation of paraclinical tests can be life-saving due to the risk of sudden death.
Background
Stokes-Adams syndrome (SAS) or “Stokes-Adams Attack” is a phenomenon in which there is a transient loss of consciousness with spontaneous recovery secondary to a sudden decrease in cardiac output due to an abrupt reduction in heart rate or rhythm; and when there is a marked reduction in cardiac output, it leads to a state of cerebral hypoxia, creating conditions conducive to seizures. 1
It is a rare phenomenon for which there are no updated epidemiologic data, with few case reports throughout history. 2 There are different causes that induce SAS, most of them are high-degree atrioventricular block, paroxysmal ventricular arrest, and pulseless ventricular tachycardia,3,4 depending on the case, the implantation of a temporary or definitive pacemaker or cardiac defibrillation will be necessary. 5
Case report
This is a 78-year-old male patient with multiple controlled comorbidities such as type 2 diabetes mellitus, chronic arterial hypertension, chronic occlusive arterial disease, and coronary artery disease that required angioplasty with stenting in the right coronary artery years ago. The patient reported clinical symptoms of 15 days of evolution of dizziness and loss of postural tone with loss of consciousness with spontaneous recovery with clonic movements of the four extremities and supraversion of gaze of 1-min duration, with postictal somnolence of 1 min, for which he consulted a first-level health center where he presented a new seizure episode with the same characteristics as the initial one and remitted. On admission, the patient presented with blood pressure (130/70 mmHg) with bradycardia of 40 beats per minute (BPM). Laboratory studies revealed a blood count with 12.110 leukocytes, 57.4% neutrophils, 30.4% lymphocytes, 14.2 g/dL of hemoglobin, and platelets of 281,000. Electrolytes were made at admission, sodium of 139 mmol/L, potassium of 3.9 mmol/L, and electrocardiogram (ECG) with 2:1 atrioventricular block without ischemic changes (Figure 1). In the initial studies, the hemogram showed no changes in cell lines, preserved renal function, and electrolytes within normal limits. A simple cranial tomography was performed and showed evidence of internal carotid artery angiosclerosis in the cavernous segment and age-related changes. The patient had a recent transthoracic echocardiogram with moderate concentric left ventricular hypertrophy, type I diastolic dysfunction, and preserved ejection fraction (left ventricular ejection fraction (LVEF)) of 63%, with mitral and aortic sclerosis without insufficiency, without changes in contractility.
Electrocardiogram in the emergency department on admission of the patient, in sinus rhythm, bradycardia, normal axis, with a single PR interval before the blocked P wave, 2:1 atrioventricular block.
He was evaluated by specialists in internal medicine and emergency medicine, started on pharmacological conciliation with rosuvastatin + ezetimibe 40/10 mg once daily, irbesartan 150 mg once daily, vidagliptin/metformin 50/1000 mg each 12 h daily, glargine insulin 13 UI each night, enoxaparin 40 mg each day, clopidogrel 300 mg as first dose and then 75 mg each day, aspirin 300 mg as first dose and then 100 mg each day, and was transferred to the intensive care unit (ICU) and hemodynamics for further evaluation and treatment.
They gradually started inotropic/chronotropic doses until they were withdrawn. They requested coagulation times, which were prolonged, and an echocardiogram with new findings given by hypokinesia of the apical segment of the inferior wall and a decrease on LVEF of 54%, a 24-h Holter electrocardiographic recording with episodes of second-degree atrioventricular block type Mobitz I, Mobitz II, and third-degree atrioventricular block associated with episodes of intermittent complete bundle branch block. In addition, he underwent arteriography with normal coronary anatomy, with a permeable catheter in the right coronary artery in the proximal segment. Finally, he was evaluated by an electrophysiologist who implanted a permanent pacemaker because of the electrocardiographic findings and the risk of sudden death. Since then, the patient has had a satisfactory clinical course without new syncopal episodes or seizures.
Discussion
SAS is a group of signs and symptoms in which there is a transient loss of consciousness due to a decrease in cardiac output, as evidenced by a decrease in heart rate, rhythm, or conduction; during the episodes, there is cerebral hypoxia, which may lead to seizures, although they are not always present. 1 About epidemiologic data, there is very little information on SAS, but it is known that it occurs more frequently in older age groups, particularly after 50 years of age.3,6
High-degree atrioventricular block, 7 paroxysmal ventricular arrest, and pulseless ventricular tachycardia3,4 have been described as causes. In addition, it is important to take into account other risk factors that may cause rhythm disturbances, as documented by Martínez Sáenz 8 who reported the case of a pregnant patient with a confirmed history of Chagas heart disease causing complete atrioventricular block requiring pacemaker as definitive treatment, or Kumar et al. 9 with a 52-year-old patient with a history of chronic arterial hypertension and obesity, without a history of coronary artery disease, with a normal ECG, who presented with SAS during pharmacological stress echocardiography with dobutamine, among others.10 –14
Paroxysmal ventricular arrest is the rarest cause of SAS and is a rare manifestation in which there is an absence of QRS complexes despite the presence of P waves on an electrocardiogram. 1 It has primary causes such as cardiomyopathies, myocarditis, and acute coronary syndrome; and secondary causes such as hypoxia, hyperkalemia, hypokalemia, massive pulmonary thromboembolism, and drugs such as adenosine. 2 It is often associated with conduction block, although this is not always the case, and treatment is with temporary cardiac pacing, eventually requiring pacemaker implantation. 2
On the other hand, our patient presented an unusual finding demonstrated by Holter, with multiple atrioventricular blocks and healthy coronary arteries. In the literature, there is not enough information to explain such findings; however, Ioakeimidis et al. 15 presented a case of a patient with a history of type 2 diabetes mellitus, with a known left bundle branch block, who debuted with a new complete heart block, with angiography without atherosclerotic lesions as in our patient.
Patients presenting with seizures are often misdiagnosed as having a neurological disorder, so certain clinical features should be noted, such as the initial pallor followed by flushing due to the resumption of normal circulation. 16 A comprehensive clinical history should be obtained from the patient’s admission to determine whether the syncope is of recent onset or has been present for an extended period. In addition, a semiological description of the seizures, if present, is crucial, as they typically have a shorter duration than an unprovoked seizure and do not result in neurological deficits, as observed in our patient. Furthermore, initiating anticonvulsant treatment does not necessarily lead to cessation of the episodes, as noted by Tabuzo et al. 1 If the wrong approach is taken, the patient will never improve. Considering the above, the possible differential diagnosis of syncope should be addressed, from orthostatic hypotension, transient cerebrovascular attack, epilepsy, and aortic stenosis. 3
The purpose of this case is to highlight the importance of obtaining a complete history in the emergency department so that semiology, electrocardiogram, and the patient’s clinical presentation can be used to make an effective diagnosis and ensure the patient’s management. In addition, SAS should be considered in patients over 50 years of age and with comorbidities who present with a seizure episode.
Conclusion
SAS is an infrequent and insufficiently characterized condition resulting from disruptions in rhythm and conduction, as demonstrated in our patient who exhibited a 2:1 atrioventricular block and complete heart block, with rate fluctuations attributed to underlying cardiac, inflammatory, or autoimmune disorders. On the other hand, there is a lack of concise epidemiologic data in the literature, such as incidence or prevalence, making it necessary to investigate and report new clinical cases. The diagnosis is made with an adequate clinical history and supported by tests to rule out differential diseases and to establish the etiology and treatment, which would vary depending on the etiology. In this case, the patient required definitive pacemaker implantation. It should be remembered that SAS is a possible cause of syncope, or seizures and that recognition of this disease can be life-saving due to the risk of sudden death.
