PNES(FUNCTIONAL SEIZURES)

Pseudoseizure diagnosis and management

1. Psychogenic nonepileptic seizures DR RAGHU NANDHINI

2. OVER VIEW DEFINITION EPIDEMIOLOGY ETIOLOGY CLINICAL FEATURES DIFFERENTIAL DIAGNOSIS DIAGNOSIS TREATMENT PROGNOSIS

3. DEFINITION PNES are characterized by sudden and time-limited disturbances of motor, sensory, autonomic, cognitive , and/or emotional functions that can mimic epileptic seizures. Historical terms for PNES, including pseudoseizures and hysterical seizures, are now discouraged Psychogenic nonepileptic seizures - UpToDate

4. EPIDEMIOLOGY Incidence rates of PNES in the general population are not well established. estimated incidence rate ranges from 1.5 to 5 per 100,000 persons per year The prevalence of PNES has been estimated to be between 2 to 33 per 100,000 persons Sigurdardottir KR, Olafsson E. Incidence of psychogenic seizures in adults: a population-based study in Iceland. Epilepsia

5. EPIDEMIOLOGY most commonly - third decade of life female predominance young children and older adults can be affected Race, marital status, and years of education does not influence the prevalence of PNES

6. ETIOLOGY

7. PNES/DS causes Physical symptoms caused by psychological causes can fall under 3 categories: -Somatoform disorder -Factitious disorder -Malingering

8. Somatoform disorder Unconscious production of physical symptoms due to psychological factors 2 somatoform disorders relevant to PNES are -conversion disorder -somatization disorder Majority of patients with PNES have conversion disorder DSM-IV added a new subcategory conversion disorder with seizures

9. Factitious disorder and malingering Patient is purposely deceiving the physician, i.e. faking the symptoms Malingering - the reason for the deception is tangible and rationally understandable Factitious disorder- the motivation is a pathologic need for the sick role

10. Who has dissociative seizures? Can happen to anyone, at any age Some factors make DS more likely -Women (>70%) -Young adults -History of injury or disease -Severe emotional upset or stressful life events -People with other psychiatric conditions depression personality disorders anxiety sel f- h a rm

11. What causes dissociative seizures? Often caused by traumatic events such as: -accidents -severe emotional upset -psychological stress (such as a divorce) -difficult relationships -physical or sexual abuse -being bullied

12. Dissociative seizures Sudden r emembe r i n g traumatic experience Too difficult to cope with Person splits off Emotional reaction causes a physical effect Seizures unconscious reaction unco n tr o ll ed

13. Clues which should raise the suspicion Resistance to antiepileptic drugs (AED) is usually the 1st clue Presence of specific triggers that are unusual for epilepsy Emotional triggers - stress, pain, certain movements, sounds, and seeing of lights Circumstances in which attacks occur Presence of audience, sleep

14. A history of sexual or physical abuse is reported in one-third to one-half of patients Individuals with a hx of sex abuse may be more like to have clinical events that are more severe and more likely to resemble epileptic seizures They are more likely to exhibit self-harming behaviors and other medically unexplained symptoms Paras ML, Murad MH, Chen LP, et al. Sexual abuse and lifetime diagnosis of somatic disorders: a systematic review and meta-analysis. JAMA 2009 ;

15. CLINICAL FEATURES Recognizing PNES can be challenging even for experienced observers, in part because of the broad diversity of presentations. Nonetheless , clues that raise suspicion for this diagnosis are often apparent from the clinical history

16. Precipitants and setting- PNES Witnesses present- Most episodes of PNES occur in front of witnesses the occurrence of an episode in the doctor's waiting or examination room was estimated to have a 75 percent predictive value for PNES in a study of patients undergoing electroencephalography (EEG) monitoring, episodes that occurred at the time of electrode placement were found to be PNES, not epileptic seizures Woollacott IO, Scott C, Fish DR, et al. When do psychogenic nonepileptic seizures occur on a video/EEG telemetry unit? Epilepsy Behav 2010;

17. Relationship to sleep- PNES tend not to occur during sleep b y contrast, epileptic seizures can occur during sleep Patients with PNES may appear to be asleep just before seizure onset, but the EEG in these cases demonstrates wakefulness

18. Stress- PNES would be more likely to be associated with stressful situations , stress is also commonly cited as a seizure precipitant in patients with epilepsy Menstrual cycle- Increased seizure frequency during the perimenstrual time period suggests epileptic seizures. In one series, perimenstrual exacerbation was associated with 13 of 27 patients with epileptic seizures versus 1 of 38 patients with PNES Ettinger AB, Weisbrot DM, Devinsky O. Patient reporting of seizure exacerbation near the time of menses helps distinguish epileptic from nonepileptic seizures. J Epilepsy 1998;

19. Ictal features

20. Motor activity- movements in PNES are more often asynchronous, variable, and may wax and wane over the course of the ictus Specific movements such as writhing, thrashing, pelvic thrusts, opisthotonus (arched back), and jactitation (rolling from side to side) suggest PNES, but these are not always present , particularly in children

21. Tongue-biting and self-injury- Classic symptoms of epileptic seizures such as tongue-biting, incontinence , and self-injury are more common in epileptic seizures, but they can occur in a third or more of patients with PNES A tongue bitten on the side (versus the tip) and severe tongue-biting (with laceration) are more specific for epileptic seizure . Seizure related burn injuries are also highly specific for epileptic seizures

22. Level of awareness- Incomplete loss of consciousness during the episode, suggested either by responsiveness to stimuli or by later recall of events during segments of ictal unresponsiveness , supports PNES . The presence of an alpha rhythm on the EEG ( ie , neurophysiologic evidence of wakefulness) during an episode in which the patient is clinically altered or amnestic also supports a nonepileptic process. Bell WL, Park YD, Thompson EA, Radtke RA. Ictal cognitive assessment of partial seizures and pseudoseizures . Arch Neurol 1998; 55:1456.

23. Vocalizations - Ictal features of emotional overlay, such as weeping, stuttering, and vocalizations with affective content , are relatively uncommon in epileptic seizures and suggest PNES Auras – A seizure aura is frequently reported in PNES (25 to 60 percent ) and may be a more common symptom than in epilepsy Autonomic signs – Autonomic manifestations during an ictus ( eg , tachycardia, cyanosis) suggest epileptic seizure, and their absence , particularly during a major convulsion, suggests PNES Opherk C, Hirsch LJ. Ictal heart rate differentiates epileptic from non-epileptic seizures. Neurology 2002; 58:636.

24. Eye closure – Eyes are usually open during the ictus of a convulsive epileptic seizure .Forced eye closure in particular suggests PNES Atonia – Ictal atonia is not a common PNES manifestation. However, when prolonged events of atonia occur, they almost always represent PNES rather than epileptic seizure Duration – While the ictus of an epileptic seizure is typically very brief, often less than one minute , PNES are rarely less than one minute and are usually much longer Frequency – Patients with PNES generally report a higher seizure frequency than patients with epilepsy

25. Postictal symptoms

26. Return to baseline- Rapid alerting and reorientation are common after PNES but uncommon with epileptic seizures, except for certain seizure types, such as absence or frontal lobe seizures Respiratory changes - The postictal period after GTCS -deep and prolonged inspiratory and expiratory phases ( stertorous breathing pattern) , compared with shallow, rapid respirations in patients after a PNES . Epileptic seizures arising from the frontal lobe , however, were associated with a postictal breathing pattern similar to PNES

27. Response to treatment — Most patients with PNES have seizures for many years prior to diagnosis,and most are treated unsuccessfully with antiseizure drugs . A failure to make even small improvements in seizure frequency despite vigorous antiseizure drug trials suggests the diagnosis of PNES . Similarly , patients who present with prolonged PNES are often treated with drug protocols for status epilepticus and fail to respond

28. DIFFERENTIAL DIAGNOSIS

29. DIAGNOSIS Clinical features of events are often not sufficiently sensitive or specific to definitively distinguish seizures from PNES, and confirmatory video-EEG testing is usually required to supplement the history Levels of diagnostic certainty — Video-EEG is the gold-standard test for the diagnosis of PNES and should be performed in all patients in whom this diagnosis is suspected

30. How are P NES diagnosed? Try and rule out possible physical causes first, including epilepsy Taking a personal history Neurological history Psychological development and mental health family history What happens during the seizure What situations ? Any warning ? What happens during seizure or a witness ? How long the seizures last ? What you remember ? How you feel afterwards and recovery ?

31. How are P NES diagnosed? Laboratory Studies Blood tests - excluding metabolic or toxic causes of seizures (e.g. hyponatremia, hypoglycemia, drugs/toxins) Level of AED in Pt’s blood, whether AED is being taken? Proper dose? Imaging Studies Should be obtained to exclude physical cause Normal in psychogenic nonepileptic seizures Electroencephalogram Records the electrical activity of the brain Often used to see if seizures are caused by disrupted brain activity

32. Epilepsy & prolactin level elevation > 2-3 fold prolactin elevation measured within 10 – 20 mins of seizure suggests presence of epileptic seizure The lack of such an elevation makes it unlikely that an ICTAL event was epileptic if the event was a tonic-clonic seizure Limitations :- -Cannot be used to differentiate simple partial seizures or absence seizures from nonepileptic seizures -Prolactin levels may increase during syncope - Complex partial seizures that do not arise from the temporal lobe do not lead to prolactin elevation - 10% to 20% of patients with tonic-clonic seizures may not show a postictal prolactin rise -Level rises predictably only after a single seizure, patients having > 2 seizures in 12 hours have progressively smaller elevations, presumably because stored prolactin from pituitary lactotrophs is exhausted

33. The diagnosis of PNES can be challenging, with delays as long as 10 to 15 years in some case series . This is due in part to the broad diversity of PNES presentations and the lack of one single unifying presenting symptom. Other sources of misdiagnosis include an inadequate history, Co-occurrence of PNES and epilepsy in the same patient, poor clinician-patient rapport, reliance upon clinical observation of the event discomfort in making a psychiatric diagnosis, and reluctance to obtain a psychiatric evaluation before the clinician feels confident about the diagnosis-

34. How are PNES diagnosed? Routine EEG is not helpful in confirming diagnosis of PNES Repeatedly normal EEG findings Frequent attacks Resistance to medications EEG video monitoring Standard for diagnosis Indicated in all patients having frequent seizures despite AED May be PNES

35. EEG video monitoring Principle is to record an episode and demonstrate that no change in the EEG occurs during the clinical event Inductions - Provocative techniques - intravenous injection of saline - principle is suggestibility

36. Treatment Medical Care Most important step is delivering the diagnosis to patients and their families Obstacle to effective treatment- Physicians are uncomfortable with the diagnosis of PNES They may write, "no EEG change during the episode, no evidence for epilepsy," or "seizures were nonepileptic.“

37. Treatment Role of the Neurologists Determine whether organic disease exists Once the symptoms are shown to be psychogenic, the exact psychiatric diagnosis and its treatment are best handled by the psychiatrist

38. Treatment Role of the Psychiatrist Psychotherapy Treatment coexisting anxiety or depression Patient education Family members education Patient & Family members education Thorough patient education is the first step in treatment Patients and their families must understand about the disease Necessity to comply with the recommendations of the psychiatric caregiver

39. Prognosis Duration of illness is probably most important prognostic factor in PNES Early & definite diagnosis of PNES is critical Generally better in children and adolescents than in adults -duration of illness is shorter -psychopathology or stressors are different in pediatric patients than in adults Prognosis depends on -Pt’s motivation -Treatment of underlying psychological illness -Good medical help

40. Activity Patients with PNES usually do not require any limitation of activities Nevertheless, restrictions on potentially hazardous activities may be appropriate in some cases

41. Take home message Everything which moves is not seizure Rule out other possible physical cause Take proper history Most common cause of non epileptic seizures is PNES Susceptible person Presence of stress Frequent attacks Repeatedly normal EEG Not responding to AED Video EEG showing no abnormal electrical discharges during attack confirms the diagnosis

42. Take home message Early diagnosis is essential Best to be managed by a psychiatrist Delivering the diagnosis is usually the first step Explain the disease to patient as well family members Treatment of co morbid psychiatric illness is necessary Psychotherapy is given Advised to follow up with Psychiatrist