History

Fact Explanation
Feeling light headed/ faintness/ dizzy Is due to reduction in cardiac out put as a result of sudden changes in heart rate or rhythm[1][2][4]
Loss of consciousness Manifests when blood flow to brain is reduced due to sudden but pronounced decrease in the cardiac output, which is caused by a sudden change in the heart rate or rhythm. Partial or complete heart block is the usually seen in these patients but other arrhythmias such as ventricular tachycardia or ventricular fibrillation may also cause syncope. These attacks are usually transient lasting only for seconds, not related to posture and can occur several times during a day[1][2][4]
Seizure When there is prolonged reduction in cerebral perfusion due to reduced cardiac out put as described above, the patient develops seizures. Absence of an aura helps to differentiate Stokes-Adams syndrome from seizures originating due to primary brain pathology[1][2][3]
History of congenital heart disease There are instances where the heart block is congenital in origin with or without associated structural defects[1]
History of diphtheria infection This is known to cause myocarditis and conduction defects, such as complete heart block and may present with Stokes-Adams episodes[1]
History of acute rheumatic fever Acute rheumatic carditis can rarely cause acquired complete atrio-ventricular block and can present with Stokes-Adams attacks. It should therefore be excluded, particularly in pediatric patients[1][5]
History of cardiac surgery/ cardiac catheterisation Direct injury to conduction tissue can occur during cardiac surgery or cardiac catheterization that can lead to acquired complete heart block and Stokes-Adams syndrome[1][5]
History of neuro-muscular disease Heart block with Stokes-Adams syndrome can occur in neuro-muscular disorders such as acute inflammatory demyelinating polyradiculoneuropathy, myotonic dystrophy etc.[1][6][7]
Patient presenting with acute myocardial infarction In 2-7% of patients with acute myocardial infarction, is complicated with complete heart block[1]
History of neoplastic disease Primary or secondary neoplasms infiltrating the myocardium and the conducting system can also produce complete heart block[1]
History of sarcoidosis A cause for acquired complete heart block in young adults. Can present with rhythm abnormalities, Stokes-Adams attacks and sudden cardiac death[1]
History of taking medications such as digoxin and certain anti arrhythmic agents Digoxin toxicity can produce arrhythmias such as atrial fibrillation and complete heart block. Antiarrhythmic agents such as quinidine, procaineamide, lidocaine etc. can depress cardiac conduction[1]
References
  1. O'ROURKE RA. Clinical Cardiology: The Stokes-Adams Syndrome--Definition and Etiology; Mechanisms and Treatment Calif Med [online] 1972 Jul, 117(1):96-99 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1518479
  2. ABDON NJ, JOHANSSON BW, LESSEM J. Predictive use of routine 24-hour electrocardiography in suspected Adams-Stokes syndrome. Comparison with cardiac rhythm during symptoms. Br Heart J [online] 1982 Jun, 47(6):553-558 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC481181
  3. DíAZ-CASTRO O, ORIZAOLA P, VáZQUEZ S, GONZáLEZ-RíOS C, PARDO M, FERNáNDEZ-LOPEZ JA, ESCRICHE D. Images in cardiovascular medicine. "Stokes-adams epilepsy": sometimes we need the electroencephalogram. Circulation [online] 2005 Aug 23, 112(8):e101-2 [viewed 03 July 2014] Available from: doi:10.1161/CIRCULATIONAHA.104.503144
  4. SIGURD B, JENSEN G, MEIBOM J, SANDOE E. Adams-Stokes syndrome caused by sinoatrial block. Br Heart J [online] 1973 Oct, 35(10):1002-8 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/4759460
  5. CARANO N, BO I, TCHANA B, VECCHIONE E, FANTONI S, AGNETTI A. Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature. Ital J Pediatr [online] 2012 Oct 30:61 [viewed 03 July 2014] Available from: doi:10.1186/1824-7288-38-61
  6. WANI BA, MISRA M, SHAH M, MUFTI S. Acute inflammatory demyelinating polyradiculoneuropathy presenting as complete heart block and Stoke-Adams attacks. Postgrad Med J [online] 1989 Feb, 65(760):103-4 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/2780457
  7. NOEL C, GAGNON RM. Cardiac conduction abnormalities and Stokes-Adams attacks in myotonic dystrophy. Can Med Assoc J [online] 1978 Jun 10, 118(11):1402-4 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/657033

Examination

Fact Explanation
Pallor followed by flushing There is initial pallor during the attack followed by facial flushing due to reactive hyperemia with the resumption of normal circulation[1]
Bradycardia Slow pulse can be detected during an attack if was caused by a bradyarrhythmia such as heart block[1][2]
Tachycardia Rapid pulse may be detected during an attack if it was caused by a tachyarrhythmia[1][2]
Variable blood pressure Blood pressure is the manifestation of cardiac output and systemic peripheral resistance. Cardiac output is a manifestation of stroke volume and pulse rate. Stroke volume is affected by the pulse rate. e.g. during tachycardia the diastole shortens and ventricular filling is affected, so that the end diastolic volume reduces. This leads to a low stroke volume. In these patients blood pressure may vary depending on variations in the pulse rate[1]
References
  1. O'ROURKE RA. Clinical Cardiology: The Stokes-Adams Syndrome--Definition and Etiology; Mechanisms and Treatment Calif Med [online] 1972 Jul, 117(1):96-99 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1518479
  2. ABDON NJ, JOHANSSON BW, LESSEM J. Predictive use of routine 24-hour electrocardiography in suspected Adams-Stokes syndrome. Comparison with cardiac rhythm during symptoms. Br Heart J [online] 1982 Jun, 47(6):553-558 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC481181

Differential Diagnoses

Fact Explanation
Epilepsy Patients with Stokes-Adams syndrome may present with recurrent seizures. Patients with epilepsy also presents with recurrent seizures. Therefore it is important to differentiate between these two conditions[1]
Vasovagal syncope Syncope can occur in patients with exaggerated autonomic reflexes causing reflex bradycardia and hypotension that occur in response to a wide variety of stimuli (e.g. prolonged sitting or standing, pain, anxiety, sight of blood etc.) which leads to a transient reduction in cardiac out put[2]
Carotid sinus hypersensitivity This is a condition associated with syncope, unexplained falls, and drop attacks in older people. The characteristic finding is hypotension or triggering of symptoms in response to carotid sinus massage [3]
Orthostatic hypotension This is a condition in which there is a sudden drop in blood pressure with upright posture which cause syncope. Orthostatic hypotension develops secondary to autonomic nerve damage and hypofunction of the autonomic nervous system[2]
Transient ischemic attack (TIA) TIA is brief episode of focal loss of brain function which lasts less than 24-hour duration and arise due to focal cerebral ischemia. Dizziness and impaired consciousness can occur during an attack but not very common[4]
References
  1. DíAZ-CASTRO O, ORIZAOLA P, VáZQUEZ S, GONZáLEZ-RíOS C, PARDO M, FERNáNDEZ-LOPEZ JA, ESCRICHE D. Images in cardiovascular medicine. "Stokes-adams epilepsy": sometimes we need the electroencephalogram. Circulation [online] 2005 Aug 23, 112(8):e101-2 [viewed 03 July 2014] Available from: doi:10.1161/CIRCULATIONAHA.104.503144
  2. RAJ SR, COFFIN ST. Medical therapy and physical maneuvers in the treatment of the vasovagal syncope and orthostatic hypotension. Prog Cardiovasc Dis [online] 2013 Jan-Feb, 55(4):425-33 [viewed 03 July 2014] Available from: doi:10.1016/j.pcad.2012.11.004
  3. TAN MP, CHADWICK TJ, KERR SR, PARRY SW. Symptomatic presentation of carotid sinus hypersensitivity is associated with impaired cerebral autoregulation. J Am Heart Assoc [online] 2014 Jun 19 [viewed 03 July 2014] Available from: doi:10.1161/JAHA.113.000514
  4. SORENSEN AG, AY H. Transient ischemic attack: definition, diagnosis, and risk stratification. Neuroimaging Clin N Am [online] 2011 May, 21(2):303-13, x [viewed 03 July 2014] Available from: doi:10.1016/j.nic.2011.0

Investigations - for Diagnosis

Fact Explanation
Electrocardiography (ECG) Arrhythmias that can give rise to Stokes-Adams syndrome are divided into several groups according to their electrocardiographic manifestations. They are as follows, 1) The transient period of asystole that occurs when there is sudden interruption to A-V impulse transmissions, before the junctional or ventricular pacemaker takes over the heart rate as seen when sinus rhythm or incomplete heart block converts to complete heart block 2) Ventricular asystole resulting from failure of the junctional pacemaker to assume heart rate when sinoatrial node impulse generation ceases (atrial stand still) as seen in patients with inferior wall myocardial ischemia 3) Asystole in the presence of established heart block 4) Paroxysmal ventricular tachycardia or ventricular fibrillation in the presence of complete heart block. These occur when ventricular ectopic foci generate impulses that cause ventricular contraction 5) Paroxysmal ventricular tachycardia ventricular fibrillation with normal A-V conduction seen commonly after acute myocardial infarction 6) Supraventricular tachycardias and bradycardias 7) Sinus bradycardia, sinoatrial block, sinoatrial arrest in patients with pre-existing heart disease who are unable to increase stroke volume, to maintain sufficient cerebral perfusion 8) Combined forms, in which paroxysmal tachyarrhythmias are followed by a period of asystole due to a delay in automaticity of pacemakers that were suppressed during the tachycardia. Different electrocardiographic changes may produce Stokes-Adams attacks in the same patient at different instances. In case of initial negative ECG but presence of evidence to suggest Stokes-Adams syndrome 24 hour ECG monitoring can be carried out[1][2][4][5]
Electroencephalogram (EEG) Can be considered for patients presenting with seizures but have normal ECG, in order to rule out other causes for seizures[3]
References
  1. ABDON NJ, JOHANSSON BW, LESSEM J. Predictive use of routine 24-hour electrocardiography in suspected Adams-Stokes syndrome. Comparison with cardiac rhythm during symptoms. Br Heart J [online] 1982 Jun, 47(6):553-558 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC481181
  2. O'ROURKE RA. Clinical Cardiology: The Stokes-Adams Syndrome--Definition and Etiology; Mechanisms and Treatment Calif Med [online] 1972 Jul, 117(1):96-99 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1518479
  3. DíAZ-CASTRO O, ORIZAOLA P, VáZQUEZ S, GONZáLEZ-RíOS C, PARDO M, FERNáNDEZ-LOPEZ JA, ESCRICHE D. Images in cardiovascular medicine. "Stokes-adams epilepsy": sometimes we need the electroencephalogram. Circulation [online] 2005 Aug 23, 112(8):e101-2 [viewed 03 July 2014] Available from: doi:10.1161/CIRCULATIONAHA.104.503144
  4. SIGURD B, JENSEN G, MEIBOM J, SANDOE E. Adams-Stokes syndrome caused by sinoatrial block. Br Heart J [online] 1973 Oct, 35(10):1002-8 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/4759460
  5. JENSEN G, SIGURD B, MEIBOM J, SANDOE E. Adams-Stokes syndrome caused by paroxysmal third-degree atrioventricular block. Br Heart J [online] 1973 May, 35(5):516-520 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC458647

Management - Specific Treatments

Fact Explanation
Anticholinergic medication Blocks the parasympathetic activity on the heart and increases the heart rate and cardiac output. Indicated in instances such as complete heart block complicating recent inferior wall myocardial infarction, treatment and prevention of Stokes-Adams syndrome precipitated by sinus bradycardia, sinoatrial block and bradycardia-tachycardia syndrome etc.[1]
Adrenergic agonist Medication Act by increasing heart rate and contractility . Can abolish some forms of atrial and ventricular extrasystoles. Also improve coronary artery blood flow. These are indicated when ventricular asystole or bradyeardia occurs in complete heart block and intracardiac pacing is unavailable[1][4]
Pacemaker Insertion of a permanent pacemaker is the treatment when the results of drug therapy is inconsistent or frequent complications arise. It is also useful in patients with congenital heart block. The general indications for implantable pacemaker include complete heart block associated with heart failure, complete heart block with Stokes-Adams syndrome, complete heart block following acute anterior or inferior wall myocardial infarction, second degree A-V block complicating anterior wall myocardial infarction and complete heart block following cardiac surgery[1][2]
Cardioversion Can be considered in hemodynamically unstable patients, for rapid restoration of sinus rhythm[3]
References
  1. O'ROURKE RA. Clinical Cardiology: The Stokes-Adams Syndrome--Definition and Etiology; Mechanisms and Treatment Calif Med [online] 1972 Jul, 117(1):96-99 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1518479
  2. VARDAS PE, SIMANTIRAKIS EN, KANOUPAKIS EM. New developments in cardiac pacemakers. Circulation [online] 2013 Jun 11, 127(23):2343-50 [viewed 02 July 2014] Available from: doi:10.1161/CIRCULATIONAHA.112.000086
  3. SUCU M, DAVUTOGLU V, OZER O. Electrical cardioversion Ann Saudi Med [online] 2009, 29(3):201-206 [viewed 02 July 2014] Available from: doi:10.4103/0256-4947.51775
  4. REDWOOD D. Intravenous isoprenaline and orciprenaline as a guide to the drug treatment of Stokes-Adams attacks. Br Med J [online] 1968 Feb 17, 1(5589):419-421 [viewed 03 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1985067