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The Contribution of the EEG Technologists in the Diagnosis of Panayiotopoulos Syndrome (Susceptibility to Early Onset Benign Childhood Autonomic Seizures)

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The Contribution of the EEG Technologists in the Diagnosis of Panayiotopoulos Syndrome (Susceptibility to Early Onset Benign Childhood Autonomic Seizures) Seizure (2004) 13, 565—573 View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector The contribution of the EEG technologists in the diagnosis of Panayiotopoulos syndrome (susceptibility to early onset benign childhood autonomic seizures) Sue Sanders a, Shaun Rowlinson a, Ioannis Manidakis a, Colin D. Ferrie b, Michael Koutroumanidis a,* a Department of Clinical Neurophysiology and Epilepsies, Lambeth Wing, St Thomas’ Hospital, London SE1 7EH, UK b Department of Paediatric Neurology, Leeds General Infirmary, Leeds, UK KEYWORDS Summary Purpose: To assess the contribution of the EEG technologists in the diagno- Epilepsy diagnosis; sis of children with epileptic seizures. Methods: We analysed the clinical information EEG technologists; obtained by the EEG technologists from children with epileptic seizures and their par- Panayiotopoulos ents, and assessed its value for the generation of a clinically useful EEG report and a plausible electroclinical diagnosis. Interviews were based on a qualitative question- syndrome naire, and were videotaped. We focused on Panayiotopoulos syndrome (PS) because it has a high rate of misdiagnosis, usually for encephalitis or other severe cerebral insults. Results: Between 1998 and 2001, 424 EEG were performed in 308 children aged 1—14 years, of whom 228 (74%) had one or more epilepticseizures. We diag- nosed PS in 14 children (6.1%), mainly based on clinical information. Three other had symptomatic ictal vomiting. In 9 of the 14 children with PS, diagnosis was achieved by the information collected by the EEG technologist. Five of these children were being treated for encephalitis, and management was altered accordingly. In a further three children the diagnosis of PS was confirmed. Conclusion: These findings demon- strate that the contribution of the EEG technologists to the diagnosis of people with epilepsies can expand well beyond their established role of recording and describ- ing an EEG. We propose that technologists should be actively involved in prospective electroclinical studies if carefully designed protocols are used. © 2004 BEA Trading Ltd. Published by Elsevier Ltd. All rights reserved. Introduction The EEG is the most important investigation in the diagnosis and management of epilepsies especially when it is performed by competent technicians, *Corresponding author. Tel.: +44-20-7922-8221; and carefully studied and interpreted in the con- fax: +44-20-7922-8263. E-mail address: [email protected] text of a well-described clinical setting by physi- (M. Koutroumanidis). cians with clinical experience in epilepsies. Even 1059-1311/$30 — see front matter © 2004 BEA Trading Ltd. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.seizure.2004.01.006 566 S. Sanders et al. under these conditions, however, there are factors manifestations: in PS mild and brief ictal autonomic that can limit the diagnostic yield of the EEG. Lack symptoms in the presence of clear consciousness of obtainable clinical information is the most im- would suggest trivial non-epileptic conditions such portant. Up to 70—80% of the EEG referrals are for as atypical migraine, gastroenteritis or syncope, epileptic disorders, and commonly come from gen- while prolonged and severe attacks may simulate eral paediatricians or general physicians who may life threatening insults such as encephalitis, for not be familiar with the electroclinical expression which many of these children are treated.11 It may of the various epilepticsyndromes. 1 In addition, ev- not be surprising that in the absence of adequate eryday practice in most EEG laboratories shows that clinical information some clinical neurophysiolo- a frequently significant and always frustrating pro- gists, particularly if not experienced in paediatric portion of request forms may be inadequately com- electroencephalography and epilepsies, may inter- pleted, and that clinical information is often poor pret an EEG with multifocal spikes as suggestive or missing. of multifocal epilepsy with poor outcome,12 thus Recently, there has been a promising tendency for contributing to misdiagnosis. non-medical health professionals, such as epilepsy The purpose of this report is to demonstrate the nurses and social workers, to become more involved contribution of our EEG technologists in the diag- in the management, including diagnosis of people nosis of PS. with epilepsies.2,3 The principal role of the EEG technologists is to obtain a high quality recording and an astute factual report. An EEG technologist Methods spends 15—20 min preparing a patient for recording; this time may be valuably used to obtain additional EEG methodology clinical information, such as on the possibility of minor seizures, precipitating factors, circadian dis- All EEG recordings are performed with digital EEG tribution and other aspects of the patient’s symp- machines (Nicolet-Voyageur) with montage refor- tomatology that will allow tailoring of the recording matting capabilities. Electrodes are positioned to the clinical needs and later, optimal EEG inter- according to the international 10—20 system. A pretation. standard EEG is a 15- to 20-min recording during An example of how the contribution of the EEG alert wakefulness with sessions of overbreathing technologists may be upgraded to better assist and photicstimulation. The EEG technologisthow- the diagnosticyield of the EEG in patients with ever is expected to tailor the test according to the epilepsies is illustrated by our approach to diag- clinical hints that he or she might derive either nosing benign focal epilepsies of childhood, and from the referral form or the information acquired in particular Panayiotopoulos syndrome (PS). PS is during patient’s preparation for the test. The first a newly recognised condition that lies within the pages of the EEG may also provide clues. In children spectrum of benign childhood seizure susceptibility with centro-temporal spikes from the outset of the syndrome.4 It is characterised by mainly autonomic recording, or in those with a history suggestive of focal seizures and functional spikes of multiple to- Rolandicseizures, for example, we placeadditional pography. Autonomicsymptoms and signs (mainly C5 and C6 electrodes halfway between C3 and T3, vomiting) occur from the onset in 80% of seizures, and C4 and T4 respectively,13 while we ask those half of which last more than 30 min amounting to with generalised discharges to count their breaths status epilepticus. Other ictal autonomic changes aloud during hyperventilation to detect possible ic- may include pallor, mydriasis, cardiorespiratory, tal cognitive impairment on video-EEG.14 We often gastrointestinal and thermoregulatory alterations, recommend sleep recordings when routine EEGs incontinence, and hypersalivation. Syncope-like are inconclusive, especially on patients with strong events may also occur during the ictal sequence. clinical evidence of epilepsy. However, not infre- More conventional ictal manifestations include eye quently in such cases the responsible technologist deviation, staring, and hemi or generalised convul- decides to proceed with recording during natu- sions. Speech arrest, hemifacial spasms, visual hal- ral sleep by allowing–—if possible–—the patient to lucinations and oropharyngolaryngeal movements dose. For sleep EEGs, patients are partially sleep occur less frequently, suggesting a maturation re- deprived, and recordings are taken early in the lated continuum with Rolandic epilepsy.5—10 afternoon during natural sleep. If idiopathicgener- The diagnosis of PS may be easily missed. Most alised epilepsy is suggested either clinically or by general practitioners and paediatricians are not generalised spike-wave during sleep, overbreath- familiar with the notion that prominent autonomic ing with breath counting and photic stimulation are symptoms and signs may occur as epileptic seizure performed on awakening. EEG technologists and epilepsy diagnosis 567 Collection of clinical information by the EEG months (range 21—162), and the total number of technologists seizures for each child ranged from 1 to 12 (mean 4). Brain MRI was performed in seven children (1—3 This is part of an on-going prospective study that and 6—9) and was normal in all. Three other chil- started in January 1998, and includes all children dren (1.3% of all children with seizures, or 21% of aged 1—14 years referred for EEG to our depart- those with autonomicseizures) had symptomatic ment, irrespective of the reason for request and the or cryptogenic focal seizures with predominantly provisional clinical diagnosis. All children and their autonomic ictal features including vomiting. Eigh- carers are interrogated by the EEG technologist teen children (8% of those with seizures) had benign through a qualitative questionnaire (Appendix A) Rolandicepilepsy. with particular emphasis on the circumstances of the attacks and the initial ictal symptoms includ- EEG ing emeticand other autonomicsymptoms and signs. Information is provided by witnesses, usu- Interictal EEG in those diagnosed with PS showed ally parents. When PS is suspected, the interview remarkable variation from normal to severely with parents and children is videotaped during the epileptiform (Table 2 and Figs. 1 and 2). The com- EEG session. Additional information–—if needed–—is monest abnormality consisted of multifocal inde- obtained at a later stage, usually during follow-up pendent spikes at various locations,
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