PSYCHOGENIC NON-EPILEPTIC SEIZURES

PSYCHOGENIC NON-EPILEPTIC SEIZURES L.L. Hryhorczuk, M.D. September 28, 2013

DEFINITIONS PAROXYSMAL NONEPILEPTIC EPISODES • ORGANIC – SYNCOPE,MIGRAINE, TRANSIENT ISCHEMIC ATTACKS (TIAs) • PSYCHOLOGIC-PSYCHOGENIC NON-EPILEPTIC SEIZURES (PNES) SYNONYMS FOR PSYCHOGENIC NON-EPILEPTIC SEIZURES • PSEUDOSEIZURES • PSYCHOGENIC SEIZURES • NON-EPILEPTIC SEIZURES • NON-EPILEPTIC EVENTS PREFERRED TERM FOR PATIENTS AND FAMILIES • PSYCHOGENIC NON-EPILEPTIC EVENTS

PSYCHIATRIC DIAGNOSES OF PNES DSM IV-R • SOMATOFORM DISORDERS CONVERSION DISORDER WITH SEIZURES OR CONVULSIONS CRITERIA: • SYMPTOM AFFECTING MOTOR/SENSORY SYSTEM SUGGESTING NEUROLOGIC /MEDICAL CONDITION • PSYCHOLOGICAL FACTORS ASSOCIATED BECAUSE SYMPTOM IS PRECEDED BY CONFLICT/STRESSOR • SYMPTOM IS NOT INTENTIONALLY PRODUCED • SYMPTOM CANNOT BE EXPLAINED BY A MEDICAL CONDITION • SYMPTOM CANNOT BE EXPLAINED BY A SUBSTANCE EFFECT • SYMPTOM CANNOT BE EXPLAINED BY A CULTURAL BEHAVIOR

PSYCHIATRIC DIAGNOSIS OF PNESCONTINUED SOMATIZATION DISORDER CRITERIA: • HISTORY OF MULTIPLE COMPLAINTS BEGINNING BEFORE AGE 30 • 4 PAIN SYMPTOMS • 2 GASTROINTESTINAL SYMPTOMS • 1 SEXUAL SYMPTOM • ONE PSEUDONEUROLOGICAL SYMPTOM SUCH AS SEIZURE • SYMPTOM CANNOT BE EXPLAINED BY A MEDICAL CONDITION OR DIRECT EFFECT OF A SUBSTANCE

PSYCHIATRIC DIAGNOSIS OF PNESCONTINUED • FACTITIOUS DISORDERS WITH PREDOMINANTLY PHYSICAL SIGNS AND SYMPTOMS WITH COMBINED PSYCHOLOGICAL AND PHYSICAL SIGNS AND SYMPTOMS CRITERIA: • INTENTIONAL PRODUCTION OF PHYSICAL/PSYCHOLOGICAL SYMPTOMS • MOTIVATION FOR BEHAVIOR TO ASSUME A SICK ROLE FOR SELF/OTHER • EXTERNAL INCENTIVES FOR BEHAVIOR ARE ABSENT • MALINGERING CRITERIA: • MEDICOLEGAL CONTEXT OF PRESENTATION • MARKED DISCREPANCYCLAIMED DISABILITY AND FINDINGS • LACK OF COOPERATION WITH EVALUATION/TREATMENT • PRESENCE OF ANTISOCIAL PERSONALITY DISORDER

PSYCHIATRIC DIAGNOSIS OF PNESCONTINUED DSM V • SOMATIC SYMPTOM AND RELATED DISORDERS FUNCTIONAL NEUROLOGICAL SYMPTOM DISORDER SOMATIC SYMPTOM DISORDER • FACTITIOUS DISORDERS FACTITIOUS DISORDER IMPOSED ON SELF FACTITIOUS DISORDER IMPOSED ON ANOTHER • MALINGERING

CONSEQUENCES AND COSTS FOR MISDIAGNOSIS OF PNES PATIENT CONSEQUENCES • PATIENTS WITH PNES USUALLY TAKE ANTIEPILEPTIC DRUGS UNNECESSARILY FOR MANY YEARS BEFORE THE DIAGNOSIS IS REVISED. • THIS EXPOSES PATIENTS TO UNTOWARD EFFECTS OF MEDICATION WITH NO BENEFIT TO THEM WHATSOEVER. • A SMALL NUMBER RECEIVE IV MEDICATIONS FOR STATUS EPILEPTICUS THAT MAY HAVE RESULTED IN INTUBATION AND POSSIBLE ADMISSION TO ICU. • THIS LEVEL OF MEDICAL CARE HAS EXPOSED THE PATIENT AND FAMILY TO A HIGH LEVEL OF STRESS WITH NO PROSPECT OF RELIEF FROM THE PROBLEM. UNNECESSARY MEDICAL COSTS • NEUROLOGIST SERVICES • MEDICATION • ELECTROENCEPHALOGRAMS • EXTENDED EEG MONITORING AND VIDEO MONITORING • IMAGING STUDIES • INPATIENT HOSPITAL DAYS

EPIDEMIOLOGY OF PNES FREQUENCY • PREVALENCE IN THE UNITED STATES AND WORLD ARE SIMILAR • 20 TO 30% OF REFERRALS TO EPILEPSY CENTERS ARE PNES • 50 TO 70% BECOME SEIZURE FREE AFTER DIAGNOSIS • 15% ALSO HAVE A COMORBID SEIZURE DISORDER GENDER • WOMEN 70% OF DIAGNOSED PNES • MEN 30% OF DIAGNOSED PNES AGE • TYPICALLY BEGIN IN YOUNG ADULTHOOD • CAN OCCUR IN CHILDREN AND ELDERLY • IN THESE AGE GROUPS NON-EPILEPTIC PHYSIOLOGIC EVENTS ARE MORE COMMON

MISDIAGNOSIS OF PNES • MISDIAGNOSIS OF EPILEPSY IS COMMON • 25% OF PATIENTS WITH A PREVIOUS DIAGNOSIS OF EPILEPSY WHO DO NOT RESPOND TO DRUGS ARE MISDIAGNOSED • PNES ACCOUNTS FOR 90% OF MISDIAGNOSED PATIENTS • OTHER CONDITIONS INCLUDE PAROXYSMAL EVENTS LIKE SYNCOPE • EEGS MISINTERPRETED AS PROVIDING EVIDIENCE FOR EPILEPSY CONTRIBUTE TO THIS PROBLEM • REVERSING A DIAGNOSIS CAN BE VERY DIFFICULT • DIAGNOSIS IS OFTEN PERPETUATED WITHOUT QUESTION • DELAY IN MAKING THE CORRECT DIAGNOSIS OFTEN TAKES 7 TO 10 YEARS

SUGGESTIVE PNES PRESENTATION • RESISTANCE TO ANTIEPILEPTIC DRUGS (AED) • PRESENCE OF SPECIFIC TRIGGERS LIKE CONFLICT, UPSET OR STRESS • OTHER TRIGGERS LIKE PAIN, SOUNDS, SPECIFIC MOVEMENTS/ LIGHT • UNUSUAL CIRCUMSTANCES LIKE ALWAYS IN THE PRESENCE OF AN AUDIENCE OR IN A DOCTOR’S OFFICE • USUALLY DO NOT OCCUR DURING SLEEP • CHARACTERISTICS OF EVENT ARE INCONSISTENT WITH EPILEPSY, SUCH AS SIDE-TO-SIDE HEAD SHAKING, BICYCLING, WEEPING, STUTTERING AND ARCHING OF THE BACK

SUGGESTIVE PNES PRESENTATIONCONTINUED • COMORBID DIAGNOSES LIKE FIBROMYALGIA, CHRONIC PAIN, CHRONIC FATIGUE OR A FLORID REVIEW OF SYSTEMS • PSYCHOSOCIAL HISTORY OF MALADAPTIVE BEHAVIOR OR OTHER PSYCHIATRIC DIAGNOSES • PATIENT’S DEMEANOR OF OVERDRAMATIZATION OR LACK OF CONCERN • HISTORY OF SEXUAL TRAUMA OR PHYSICAL ABUSE WITH EPISODES MORE OFTEN CONVULSIVE THAN LIMP IN PNES

PREDICTABLE DIFFERENCES EPILEPTIC SEIZURE • ABRUPT ONSET • LOSS OF AWARENESS • EYE OPENING/WIDENING • TONGUE BITING OR ICTAL CRY SPECIFIC TO GENERALIZED TONIC-CLONIC SEIZURES PSYCHOGENIC NON-EPILEPTIC SEIZURE • PRESERVED AWARENESS • EYE FLUTTER • EPISODES INTENSIFIED OR ALLEVIATED BY OBSERVERS • ABLE TO BE PROVOKED BY AN INDUCTION TECHNIQUE

DIFFERENTIAL DIAGNOSIS • ABSENCE SEIZURES • BRAINSTEM GLIOMAS • COMPLEX PARTIAL SEIZURES • DIZZINESS, VERTIGO AND IMBALANCE • EPILEPSY IN ADULTS WITH COGNITIVE IMPAIRMENT • EPILEPSY IN CHILDREN WITH COGNITIVE DELAY • EPILEPTIFORM DISCHARGES • FOCAL EEG WAVEFORM ABNORMALITIES • FRONTAL LOBE EPILEPSY • JUVENILE MYOCLONIC EPILEPSY • MYASTHENIA GRAVIS • STATUS EPILEPTICUS

PHYSICAL EXAMINATION PHYSICAL AND NEUROLOGIC EXAMINATIONS USUALLY NORMAL SUGGESTIVE FEATURES • OVERLY DRAMATIC BEHAVIOR • GIVE AWAY WEAKNESS • WEAK VOICE • STUTTERING MENTAL STATUS EXAMINATION SUGGESTIVE FEATURES • ANXIETY • DEPRESSION • INAPPROPRIATE AFFECT • LACK OF CONCERN (LA BELLE INDIFFERENCE)

MEDICAL WORKUP LABORATORY STUDIES • STUDIES TO EXCLUDE METABOLIC/TOXIC CAUSES (HYPONATREMIA, HYPOGLYCEMIA, DRUGS) • PROLACTIN AND CREATINE KINASE LEVELS THAT MAY RISE AFTER GENERALIZED CLONIC-TONIC SEIZURES IMAGING STUDIES • IMAGING STUDIES ARE NORMAL IN PNES • INCIDENTAL FINDINGS SHOULD NOT CONFOUND THE DIAGNOSIS OF PNES

MEDICAL WORKUPCONTINUED EEG AND AMBULATORY EEG • ROUTINE EEG HAS A LOW SENSITIVITY BUT REPEATED NORMAL RESULTS WITH REPEATED ATTACKS AND RESISTANCE TO MEDICATION IS A RED FLAG • AMBULATORY EEG IS USED MORE FREQUENTLY , IS COST EFFECTIVE AND CAN RECORD A HABITUAL EPISODE DOCUMENTING NO EEG CHANGES EEG VIDEO MONITORING • CRITERION STANDARD FOR DIAGNOSIS AND INDICATED FOR PATIENTS WHO HAVE FREQUENT SEIZURES DESPITE MEDICATION • PRINCIPLE IS TO RECORD AN EVENT AND DEMONSTRATE NO EEG CHANGES • EEG HAS LIMITATIONS BECAUSE OF OCCASIONAL FALSE NEGATIVE RESULTS OR MOVEMENTS CAUSING EXCESSIVE ARTIFACT • ANALYSIS OF THE VIDEO (ICTAL SEMIOLOGY) IS AS IMPORTANT AS EEG BECAUSE IT SHOWS BEHAVIORS INCOMPATIBLE WITH EPILEPTIC SEIZURES • USEFUL SIGN IS PRESERVED AWARENESS DURING BILATERAL MOTOR ACTIVITY A SPECIFIC INDICATION OF PNES

MEDICAL WORKUPCONTINUED SHORT TERM OUTPATIENT EEG VIDEO MONITORING WITH ACTIVATION • COST EFFECTIVE WITH SAME SPECIFICITY AS OTHER TESTS AND HIGH SENSITIVITY • TYPICAL EPISODE OBSERVED IN 70 TO 80% OF PATIENTS INDUCTION • PROVOCATIVE TECHNIQUES ARE USEFUL WHEN DIAGNOSIS IS UNCERTAIN AND NO SPONTANEOUS EPISODES OCCUR DURING MONITORING • PRINCIPLE BEHIND INDUCTION IS SUGGESTIBILITY • INTRAVENOUS INJECTION OF SALINE WITH SUGGESTION IS COMMONLY USED

MEDICAL CARE OF PNES PATIENT EDUCATION • MOST IMPORTANT STEP IS DELIVERING THE DIAGNOSIS TO THE PATIENT AND FAMILY • PATIENT’S REACTION WILL BE DISBELIEF AND OFTEN ANGER BECAUSE OF PREVIOUS ORGANIC DIAGNOSIS • MAY COMMENT “ARE YOU ACCUSING ME OF FAKING?” OR “ARE YOU SAYING I’M CRAZY?” WRITTEN INFORMATION • UNLESS PATIENT AND THEIR FAMILY UNDERSTAND THE DIAGNOSIS, THEY WILL NOT FOLLOW THROUGH WITH TREATMENT • HANDOUT “PSYCHOGENIC (NON-EPILEPTIC) SEIZURES: A GUIDE FOR PATIENTS A& FAMILIES”

MEDICAL CARE OF PNESCONTINUED OBSTACLES TO TREATMENT • PHYSICIANS ARE UNCOMFORTABLE WITH THE DIAGNOSIS OF PNES AND MAY GIVE UNCLEAR EXPLANATIONS OR WRITE VAGUE REPORTS • CLINICIANS RECEIVING THESE REPORTS DON’T FIND THEM HELPFUL AND THE PATIENT CONTINUES WITH THE DIAGNOSIS OF SEIZURE DISORDER • DIAGNOSIS SHOULD BE EXPLAINED CLEARLY AS PSYCHOLOGICAL, STRESS INDUCED OR CAUSED BY EMOTIONS • MOST PHYSICIANS ARE TIMID, UNCLEAR AND CONFUSING BECAUSE OF THEIR DISCOMFORT • APPROACH NEEDS TO BE COMPASSIONATE BUT ALSO FIRM AND CONFIDENT

MEDICAL CARE OF PNESCONTINUED • TREATMENT IS PROVIDED BY A MENTAL HEALTH PROFESSIONAL • USE OF PSYCHOTROPIC MEDICATIONS TO TREAT COMORBID ANXIETY AND DEPRESSIVE DISORDERS IS APPROPRIATE • PILOT STUDY USING SELECTIVE SEROTONIN INHIBITORS HAS SHOWN A REDUCTION IN PNES • COGNITIVE BEHAVIORAL THERAPY HAS BEEN HELPFUL IN REDUCING PNES • ACCESS TO MENTAL HEALTH SERVICES MAY BE DIFFICULT PARTICULARILY FOR THE UNINSURED • IF A PSYCHIATRIST IS SKEPTICAL ABOUT THE DIAGNOSIS OF PNES, A CONSULTATION WITH THE NEUROLOGIST TO VIEW THE VIDEO RECORDING MAY BE MORE HELPFUL THAN A WRITTEN REPORT

CONSULTATIONS FOR PNES INPATIENT CONSULTATION • NEUROLOGIST AND A ELECTRONIC VIDEO MONITORING UNIT SHOULD WORK WITH A PSYCHIATRIST WHO UNDERSTANDS PNES • REFERRALS TO PSYCHOLOGISTS, MENTAL HEALTH SOCIAL WORKERS AND MENTAL HEALTH NURSE PRACTITIONERS SHOULD BE MADE AT DISCHARGE FOR SUBSEQUENT PSYCHOTHERAPY OUTPATIENT CONSULTATION • NEUROLOGIST NEEDS TO REMAIN INVOLVED WITH THE 15% OF PNES PATIENTS WHO HAVE A COMORBID DIAGNOSIS OF SEIZURE DISORDER • NEUROLOGIC CONSULTATION MAY BE NEEDED TO DEAL WITH PATIENTS WHO ARE RESISTIVE TO PSYCHIATRIC TREATMENT AND REQUIRE A “BOOSTER SESSION” REVIEWING THEIR FINDINGS AGAIN

ACTIVITY RESTRICTIONS WITH PNES • PATIENTS WITH PNES USUALLY DO NOT REQUIRE LIMITATIONS OF ACTIVITIES • RECOMMENDATIONS REGARDING DRIVING VARY • PRELIMINARY STUDY WITH PNES PATIENTS SHOWED NO INCREASED RISK OF MOTOR VEHICLE ACCIDENTS • RESTRICTIONS ON POTENTIALLY HAZARDOUS ACTIVITIES SUCH AS SWIMMING OR CLIMBING MAY BE APPRORIATE FOR SOME PATIENTS • THE PSYCHIATRIST WISH TO SPEAK WITH THE NEUROLOGIST FOR RECOMMENDATIONS

PROGNOSIS FOR PNES ADULTS • DURATION OF ILLNESS IS THE MOST IMPORTANT PROGNOSTIC FACTOR IN PNES • SYMPTOMS MORE THAN 10 YEARS, MORE THAN 50% CONTINUE WITH SEIZURES AND ARE DEPENDENT ON SOCIAL SECURITY BENEFITS • PATIENTS WITH LIMP OR CATATONIC TYPE EVENTS HAVE A BETTER PROGNOSIS THAN THOSE WITH A CONVULSIVE OR THRASHING TYPE • OUTCOMES IMPROVE WITH PATIENT EDUCATION, FEWER ADDITIONAL SOMATIC COMPLAINTS, NONDRAMATIC PRESENTATIONS, ONSET AND DIAGNOSIS AT A YOUNGER AGE

PROGNOSIS FOR PNESCONTINUED CHILDREN AND ADOLESCENTS • OUTCOMES ARE BETTER THAN WITH ADULTS BECAUSE OF SHORTER DURATION OF THE ILLNESS • PHYSICAL/SEXUAL ABUSE AND SERIOUS MOOD DISORDERS ARE MORE COMMON AND MAY COMPLICATE TREATMENT • PNES MAY LEAD TO SCHOOL REFUSAL AND FAMILY DISCORD THAT REFERENCE: Selim R. Benbadis, M.D., “Psychogenic Nonepileptic Seizures” Medscape Reference Drugs, Diseases and Procedures updated March 19, 2013

PSYCHOGENIC NON-EPILEPTIC SEIZURES