NEUROCARDIOLOGY NEUROCARDIOLOGY

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1 NEUROCARDIOLOGY JON BRILLMAN, M.D CHAIRMAN EMERITUS, DEPARTMENT OF NEUROLOGY ALLEGHENY GENERAL HOSPITAL PROFESSOR OF NEUROLOGY DREXEL UNIVERSITY COLLEGE OF MEDICINE, ALLEGHENY CAMPUS 1

2 SUD SUDDEN DEATH IN MIDDLE AGED MEN SIDS SUDDEN UNEXPLAINED NOCTURNAL DEATH SYNDROME SCARED TO DEATH SUDEP SUDDEN DEATH DURING NATURAL CATASTROPHE SUDDEN DEATH IN DRUG ABUSE SUDDEN DEATH DURING ASTHMA ATTACKS SUDDEN DEATH DURING ALCOHOL WITHDRAWAL SUDDEN DEATH FROM GRIEF SUDDEN DEATH DURING PANIC ATTACKS 2

3 BRAIN-HEART VOODOO DEATH(SUD) CARDIAC STUNNING EKG CHANGES NEUROGENIC ARRYTHMIAS SUDEP PSYCHOSOMATIC ILLNESS AW PERI-PARTUM PARTUM CARDIOMYOPATHY CAN OCCUR UP TO 6 MONTHS POST -PARTUM DILATED CARDIOMYOPATHY WITH LV FAILURE CAUSE UNKNOWN: NUTRITIONAL DEFICIENCIES, SMALL VESSEL CORONARY DISEASE, HORMONAL EFFECTS, TOXEMIA, MYOCARDITIS OR IMMUNOLOGICAL RESPONSE TO FETAL ANTIGEN TREATMENT INCLUDES AGGRESSIVE TX FOR CHF, ANTI-COAGULATION AND IF NO RESPONSE, TRANSPLANTATION RESEMBLES TAKOTSUBO RISK OF RECURRENCE 3

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5 BRAIN-HEART VOODOO DEATH: 600,000/YR. IN U.S. SUDDEN DEATH ASSOCIATED WITH DEATH OF A CLOSE PERSON, DURING ACUTE GRIEF, THREAT OF LOSS OF A CLOSE PERSON, DURING MOURNING OR ON AN ANNIVERSARY, LOSS OF STATUS OR SELF- ESTEEM, PERSONAL DANGER OR THREAT OF INJURY, AFTER DANGER IS OVER, REUNION, TRIUMPH OR HAPPY ENDING. 5

6 NEUROGENIC ARRYTHMIAS AUTONOMIC INFLUENCE SYMPATHETIC OVERWHELMS PARASYMPATHETIC CONSISTANTLY HIGH NOREPINEPHINE LEVELS CONTRACTION BAND NECROSIS(SUBENDOCARDIAL, INVOLVING CONDUCTION SYSTEM) PAC S S AND VPC S ATRIAL FIBRILLATION(CAUSE OR RESULT OF STROKE?) SINUS ARREST TORSADES V-FIB ARREST 6

7 EKG CHANGES T-WAVE INVERSIONS(CEREBRAL T WAVES) U WAVES PRONGATION OF Q-T Q T INTERVAL ARRYTHMIAS REPOLARIZATION CHANGES(RISK OF FATAL ARRHYTHMIA) The neurocardiac lesion: Gross specimen of a patient who died du ring an acute psychological stress shows fresh endocardial hemorrhages (1 of many is shown by the arrow) Samuels, M. A. Circulation 2007;116:77-84 Cardiac contraction band necrosis (also known as coagulative myo cytolysis, myofibrillar degeneration) Samuels, M. A. Circulation 2007;116:

8 Intense mineralization within minutes of the onset of contractio n band necrosis Samuels, M. A. Circulation 2007;116:77-84 NEJM 2005 MYOCARDIAL STUNNING WITTSTEIN ET AL. DESCRIBED 19 PATIENTS WHO PRESENTED WITH LV DYSFUNCTION AFTER SUDDEN EMOTIONAL STRESS. PATIENTS HAD ANGIOGRAPHY AND SERIAL ECHOS, 5 HAD ENDOMYOCARDIAL BIOPSY, 13 HAD CATACHOLAMINE LEVELS AND COMPARED TO PATIENTS WITH MI MYOCARDIAL STUNNING 95% WOMEN CLINICAL PICTURE INCLUDED CHEST PAIN, PULMONARY EDEMA AND CARDIOGENIC SHOCK EKG SHOWED T -WVE INVERSION AND PROLONGED Q -T INTERVAL NO CORONARY ARTERY DISEASE ON ANGIOS SEVERE LV DYSFUNCTION (MEAN EF.20) WITH RAPID RESOLUTION IN IN 2 TO 4 WEEKS TO EF OF.60 BIOPSIES SHOWED CONTRACTION BAND NECROSIS PLASMA CATACHOLAMINE LEVELS WERE DRAMATICALLY HIGHER IN STRESS INDUCED CARDIOMYOPATHY COMPARED TO PATIENTS WITH MI AS WELL AS NEUROPEPTIDE Y TAKOTSUBO TYPE CHANGES IN SOME CASES 8

9 Serial Echocardiographic Assessment of the Ejection Fraction in 19 Patients with Stress Cardiomyopathy 9

10 MECHANISM CATACHOLAMINE INFUSION CAUSES ARRYTHMIA CONTRACTION BAND NECROSIS WITH EARLY CALCIFICATION INCREASE CALCIUM IN CELLS AND NOT IN SR STRESS INDUCED TAKOTSUBO -LIKE CARDIOMYOPATHY IN ELDERLY WOMEN AND PREGNANCY SUDEP Overview Patients with refractory epilepsy face an elevated risk of sudden death, with rates as high as 1% per year This phenomenon, known as sudden unexpected death in epilepsy (SUDEP), is believed to be a seizure-related related occurrence, but the exact underlying mechanisms are uncertain Fp1 F7 F7 SP1 SP1 T7 T7 P7 P7 O1 Fp2 F8 F8 SP2 SP2 T8 T8 P8 P8 O2 SP1 SP2 TP9 TP10 Fp1 F3 F3 C3 C3 P3 P3 O1 Fp2 F4 F4 C4 C4 P4 P4 O2 EKG1 EKG2 200 V A Fp1 F7 F7 SP1 SP1 T7 T7 P7 P7 O1 Fp2 F8 F8 SP2 EKG1 EKG2 200 V P3 O1 Fp2 F4 F4 C4 C4 P4 P4 O2 EKG1 EKG2 200 V B Electroencephalographic and electrocardiographic tracings from a patient with ictal bradycardia (A) and then asystole (B) during a right temporal lobe seizure. Jehi and Najm (2008) 10

11 Cascade of events that lead to neurocardiac damage Samuels, M. A. Circulation 2007;116:77-84 SUMMARY BRAIN LESIONS, INCLUDING STROKE, SAH, SEIZURE DISCHARGES, EMOTIONAL CRISIS MAY PRODUCE ADRENERGIC CATACHOLAMINE STORM WHICH CAUSE ATRIAL AND VENTRICULAR ARRYHMIAS, CONTRACTION BAND NECROSIS OR PULMONARY EDEMA 11

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