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: 1.SYMPTOM AFFECTING MOTOR/SENSORY SYSTEM SUGGESTING NEUROLOGIC /MEDICAL CONDITION 2.PSYCHOLOGICAL FACTORS ASSOCIATED BECAUSE SYMPTOM IS PRECEDED BY CONFLICT/STRESSOR 3.SYMPTOM IS NOT INTENTIONALLY PRODUCED 4. SYMPTOM CANNOT BE EXPLAINED BY A MEDICAL CONDITION 5. SYMPTOM CANNOT BE EXPLAINED BY A SUBSTANCE EFFECT 6. SYMPTOM CANNOT BE EXPLAINED BY A CULTURAL BEHAVIOR

PSYCHIATRIC DIAGNOSIS OF PNES CONTINUED SOMATIZATION DISORDER CRITERIA: 1.HISTORY OF MULTIPLE COMPLAINTS BEGINNING BEFORE AGE PAIN SYMPTOMS 3.2 GASTROINTESTINAL SYMPTOMS 4.1 SEXUAL SYMPTOM 5.ONE PSEUDONEUROLOGICAL SYMPTOM SUCH AS SEIZURE 6.SYMPTOM CANNOT BE EXPLAINED BY A MEDICAL CONDITION OR DIRECT EFFECT OF A SUBSTANCE

PSYCHIATRIC DIAGNOSIS OF PNES CONTINUED FACTITIOUS DISORDERS WITH PREDOMINANTLY PHYSICAL SIGNS AND SYMPTOMS WITH COMBINED PSYCHOLOGICAL AND PHYSICAL SIGNS AND SYMPTOMS CRITERIA: 1.INTENTIONAL PRODUCTION OF PHYSICAL/PSYCHOLOGICAL SYMPTOMS 2.MOTIVATION FOR BEHAVIOR TO ASSUME A SICK ROLE FOR SELF/OTHER 3.EXTERNAL INCENTIVES FOR BEHAVIOR ARE ABSENT MALINGERING CRITERIA: 1.MEDICOLEGAL CONTEXT OF PRESENTATION 2.MARKED DISCREPANCYCLAIMED DISABILITY AND FINDINGS 3.LACK OF COOPERATION WITH EVALUATION/TREATMENT 4.PRESENCE OF ANTISOCIAL PERSONALITY DISORDER

PSYCHIATRIC DIAGNOSIS OF PNES CONTINUED 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 PRESENTATION CONTINUED 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 WORKUP CONTINUED 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 WORKUP CONTINUED 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 PNES CONTINUED 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 PNES CONTINUED 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 PNES CONTINUED 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