PSYCHOGENIC NON-EPILEPTIC SEIZURES
L.L. Hryhorczuk, M.D.
September 28, 2013
DEFINITIONS
PAROXYSMAL NONEPILEPTIC EPISODES
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ORGANIC – SYNCOPE,MIGRAINE, TRANSIENT ISCHEMIC ATTACKS (TIAs)
PSYCHOLOGIC-PSYCHOGENIC NON-EPILEPTIC SEIZURES (PNES)
SYNONYMS FOR PSYCHOGENIC NON-EPILEPTIC SEIZURES
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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
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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 30
2. 4 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
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NEUROLOGIST SERVICES
MEDICATION
ELECTROENCEPHALOGRAMS
EXTENDED EEG MONITORING AND VIDEO MONITORING
IMAGING STUDIES
INPATIENT HOSPITAL DAYS
EPIDEMIOLOGY OF PNES
FREQUENCY
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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
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WOMEN 70% OF DIAGNOSED PNES
MEN 30% OF DIAGNOSED PNES
AGE
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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
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RESISTANCE TO ANTIEPILEPTIC DRUGS (AED)
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PRESENCE OF SPECIFIC TRIGGERS LIKE CONFLICT, UPSET OR STRESS
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OTHER TRIGGERS LIKE PAIN, SOUNDS, SPECIFIC MOVEMENTS/ LIGHT
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UNUSUAL CIRCUMSTANCES LIKE ALWAYS IN THE PRESENCE OF AN AUDIENCE
OR IN A DOCTOR’S OFFICE
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USUALLY DO NOT OCCUR DURING SLEEP
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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
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ABRUPT ONSET
LOSS OF AWARENESS
EYE OPENING/WIDENING
TONGUE BITING OR ICTAL CRY SPECIFIC TO GENERALIZED TONIC-CLONIC
SEIZURES
PSYCHOGENIC NON-EPILEPTIC SEIZURE
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PRESERVED AWARENESS
EYE FLUTTER
EPISODES INTENSIFIED OR ALLEVIATED BY OBSERVERS
ABLE TO BE PROVOKED BY AN INDUCTION TECHNIQUE
DIFFERENTIAL DIAGNOSIS
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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