Fits, Faints and Funny Turns. Dr Aidan Neligan PhD MRCP Consultant Neurologist HUH and NHNN, Queen Square

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1 Fits, Faints and Funny Turns Dr Aidan Neligan PhD MRCP Consultant Neurologist HUH and NHNN, Queen Square

2 Moya et al., 2009

3 What is referred to a First Seizure Clinic? Prospective study of 200 consecutive adult patients attending a 1 st Seizure clinic over a 16 month period 69.5% GP referrals 30.5% Hospital/A+E 28% given a new diagnosis of epilepsy, 13% a diagnosis of a single unprovoked seizure What are the remainder? Is it similar in children? McFadyen, 2004

4 Paediatric 1 st Seizure Clinic Referrals Referrals to a paediatric 1 st Seizure in Canada between 01/ /08/ referrals Hamiwka et al., 2007

5 Paediatric 1 st Seizure Clinic Referrals II Of the 94 children diagnosed with an epileptic event, 36 (38%) had suffered at least one previous. In all cases, the referral letter did not comment on previous events Unrecognised events included absence (2), myoclonic (5) and complex partial seizures (8) and more subtle seizures Hamiwka et al., 2007

6 1 st Seizure Clinic Episodes of TLOC In the 1 st Seizure Clinic, the main differential lies between convulsive syncopal episodes and epileptic events History Eye witness accounts

7 Epileptic (GTCSz) vs Syncope? Posture Prodrome Relation to exercise Sleep Stimulus Predisposing factors stress, SD, heat, dehydration, diarrhoea Nausea, sweating, pallor, pupillary dilation Chest pain, palpitations, blurred vision, lightheadedness

8 Epileptic (GTCSz) vs Syncope II? Description of Fall Keeling over, stiff - Tonic phase epilepsy, rarely syncope Flaccid collapse Syncope (all variants) Movements Beginning before the fall GTCS Beginning after the fall GTCS, Syncope Symmetrical, synchronous GTCS Asynchronous Syncope, functional TLOC, may be GTCS

9 Epileptic (GTCSz) vs Syncope III? Movements (c) Beginning at onset of unconsciousness GTCS Beginning after onset of consciousness - Syncope Lasting less than 15s GTCS more likely than GTCS Lasting for 30s to minutes GTCS Restricted to one limb or one side GTCS Pelvic thrusting Functional GTCS (PNES) Waxing and waning Functional GTCS (PNES)

10 Epileptic (GTCSz) vs Syncope IV? Other Aspects? Ictal cry GTCS Automatisms (lip smacking, chewing, blinking) GTCS Cynanotic face GTCS, Cardiac Syncope Eyes open GTCS as likely as Syncope Eyes closed Functional TLOC (PNES) Lateral tongue bite GTCS Head consistently turned to one side GTCS more likely than syncope

11 Epileptic (GTCSz) vs Syncope V? Other Aspects (c)? Frothing at the mouth GTCS Incontinence (urinary, faecal) GTCS as likely as Syncope Brainstem dysfunction Steal syndrome, vertebrobasilar TIA (vertigo, diplopia, dysarthria, ataxia etc) Co-morbidities DM, IPD, IHD, Post-ictal/recovery Eye- witness account is of critical importance!!!

12 Accuracy of Eye-Witness Accounts Decreases the more complicated the semiology (CPS vs GTCS) Lateralisation Duration In a study of the accuracy of eye-witness accounts, 73 patients (42 F) admitted for diagnostic V-EEG, description of seizures were collobrated with V-EEG. Rugg-Gunn et al., 2001

13 Rugg-Gunn et al., 2001

14 Rugg-Gunn et al., 2001

15 Assessing the accuracy of Eye- Witness Accounts IV 43/73 had seizures video-record Rugg-Gunn et al., 2001

16 How long do GTCS typically last? Video- analysis of 120 GTCS in 47 patients

17 How long do GTCS typically last II? Theodore et al., 1994

18 How long do GTCS typically last III? Theodore et al., 1994

19 How long do GTCS typically last IV? GTCS duration was seconds (mean 62.2s) 83% of GTCS in this series were preceded by a partial seizure No GTCS lasted >2 minutes Theodore et al., 1994

20 How common is Convulsive Syncope? Myoclonus Asynchronous Jerks of the limbs lasting a few seconds reported incidence 12-45% (Lin et al., 1982, Newman & Graves, 2001) Significantly higher in individuals performing the mess trick (Lempert et al., 1994)

21 Features of Convulsive Syncope? 83% fell backward, the remainder fell to the side or forward Myoclonus occurred in 38 (90%) of 42 syncopal episodes Never preceded the fall, starting 2.6±0.9 seconds after LOC (range seconds)

22 Features of Convulsive Syncope II The typical duration was 6.6 ± 3.5 seconds (range seconds) It outlasted partial responsiveness 25% and verbal responsiveness in 10% These individual usually remembered twitching when asked afterwards Myoclonus activity was mostly continuous with pauses never exceeding 1 second. Lempert et al., 1994

23 At what Age does Syncope occur?

24 What do you do if you think someone has had a 1 st seizure? Single, multiple? Provoked, unprovoked Witnessed? ECG Imaging? EEG? Treatment? Driving?

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27 What do you do if you think someone has had a Seizure? 4 questions - 1) Was the TLOC consistent with a seizure? - 2) If this was a seizure, was it an epileptic seizure? - 3) If this was an epileptic seizure, what is the seizure semiology (type SPS, CPS, PGS, SGS) - 4) If this is an epileptic seizure, what is the epileptic syndrome?

28 What is PNES? Seizures can be subdivided into three categories: epileptic seizures, PNES, and physiologic nonepileptic events PNES present as paroxysmal time-limited, alterations in motor, sensory, autonomic, and/or cognitive signs and symptoms, but unlike epilepsy, PNES are not caused by ictal epileptiform activity

29 What is PNES II? In contrast to ES, which are a manifestation of excessive and hypersynchronous discharges in the brain, PNES have psychologic underpinnings and causes. Physiologic NEEs are neither epileptic nor psychogenic, rather they are events associated with systemic alterations that produce an ictus (e.g., convulsive syncope or hypoglycaemic seizures).

30 How common is PNES? Average delay in making a diagnosis of PNES is 7-8 years Gold-standard for diagnosis is Video-EEG, which is a limited resource Many patients undergo multiple VEEG evaluations as they may be searching for confirmation of a diagnosis or they may be referred to various centres for second opinions by physicians, who are either unaware of the previous evaluations or diagnoses, or who are dubious of the diagnosis of PNES

31 The Incidence of PNES? Only two true epidemiological studies of the incidence of PNES performed to date 1 st study was performed in Iceland all patients with new-onset seizures were considered for VEEG, which was performed in the only available laboratory The authors identified 14 patients aged years with definite PNES The majority were women (78.6%) The incidence was calculated at 1.4 per year per

32 The Incidence of PNES II? The highest incidence of PNES was noted in the year age group (3.4 per ) with no patients diagnosed over the age of 55 Highest in female patients aged years (5.9 per ) The authors estimated that patients with PNES constitute about 5% of all patients with new-onset seizures

33 The Incidence of PNES III? The 2 nd study was performed in Hamilton County, Ohio. Found a mean incidence of PNES to be about 3.03 per Incidence was about two times higher than the Icelandic study The highest incidence was in the year group (4.38 per ) 73% were women

34 The Prevalence of PNES? There are many reports that indicate that the prevalence of PNES to be between 10-20% in children and 10-58% of adults who are referred to epilepsy Centres with suspected seizures The most frequently quoted figures are 20-30% The estimated prevalence of PNES is between 1/50000 and 1/3000 or 2 to 33 per Of note estimated that 20-30% of those referred to an Epilepsy Centre with refractory epilepsy have in fact PNES

35 The Prevalence of comorbid Epilepsy and PNES? The reported prevalence of comorbid epilepsy and PNES varies considerably but reported to be as low as 9% and as high as 63% In the Icelandic study, 50% of people diagnosed with PNES had comorbid epilepsy In the Ohioan study, only 16/177 (9%) diagnosed with comorbid epilepsy and PNES In a review, coexistence of PNES and epilepsy in 9.4% Current accepted estimate is that about 10% of patients with PNES have comorbid epilepsy

36 Clinical Features of PNES LaFrance et al., 2013a

37 Clinical Features of PNES II LaFrance et al., 2013a

38 Diagnosis of PNES LaFrance et al., 2013a

39 Diagnosis of PNES II the rule of 2s At least two normal EEG studies, with at least two seizures per week, resistance to two or more AEDs 85% positive predictive value for PNES LaFrance et al., 2013a

40 Management of PNES The take home message is that establishing the diagnosis of PNES, as securely as possible, is the first step in treatment of patients with PNES LaFrance et al., 2013b

41 Management of PNES II La

42 Management of PNES II LaFrance et al., 2013b

43 Websites

44 Thank you Questions??

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