PANAYIOTOPOULOS SYNDROME - A BENIGN CHILDHOOD SUSCEPTIBILITY TO AUTONOMIC RATHER THAN OCCIPITAL SEIZURES
Abstract number :
2.380
Submission category :
Year :
2003
Submission ID :
540
Source :
www.aesnet.org
Presentation date :
12/6/2003 12:00:00 AM
Published date :
Dec 1, 2003, 06:00 AM
Authors :
Colin D. Ferrie, Sue Sanders, Shaun Rowlinson, Thalia Valeta Department of Paediatric Neurology, Leeds General Infirmary, Leeds, West Yorkshire, United Kingdom; Department of Clinical Neurophysiology and Epilepsies, St. Thomas[apos]s Hospital, London, Uni
Panayiotopoulos syndrome is recognised by the ILAE as an idiopathic occipital lobe epilepsy of young children. This reflects its initial recognition based upon the frequent occurrence in it of interictal occipital epileptiform abnormalities. However, the most charactreritic ictal manifestation -emesis- is not a feature of other occipital epilepsies, but is a typical autonomic symptom. We sought to further characterise the clinical and EEG features of Panayiotopoulos syndrome, and in particular determine whether it should be classified as an epilepsy syndrome with autonomic seizures rather than as an occipital epilepsy.
The study was conducted prospectively between 1998 and 2001 and involved all children aged 1-14 years referred for an EEG to St Thomas[rsquo]s Hospital, London. Clinical data, including detailed interrogation about ictal symptoms, was collected using a standard proforma on all such children irrespective of diagnosis at referral. Where appropriate, other data was collected from old medical records. Standard EEG recordings (with additional C5 [amp] 6 electrodes) was supplemented, if necessary, with sleep and video-EEG recordings. In children with features compatible with Panayiotopoulos syndrome fuller video-taped histories were obtained. On the basis of clinical and EEG features children were classified as [lsquo]not Panayiotopoulos syndrome[rsquo], [lsquo]typical Panayiotopoulos syndrome[rsquo] and [lsquo]possible Panayiotopoulos syndrome[rsquo]. The latter designation was given if clinical or EEG features were compatible but not typical or the information was inadequate to make a definite diagnosis.
424 EEGs were performed on 308 children. 228 children had had one or more definite epileptic seizure(s). 14 children (6.1%) were diagnosed with typical Panayiotopoulos syndrome (median age 4.5 years, range 2-14) and 11 (5%) had possible Panayiotopoulos syndrome. Of the 203 children who did not have Panayiotopoulos syndrome 3 had ictal vomiting of symptomatic cause. In one child with a dysembryoplatic neuroepithelial tumour seizures were typical of those of Panayiotopoulos syndrome. Ictal symptoms were predominanlty autonomic and included emesis (with or without vomiting), syncopal-like episodes, cardiorespiratory and thermoregulatory irregularities, skin colour changes and pupillary abnormalities. More conventional non-autonomic symptoms often ensued. The commonest interictal EEG abnormality consisted of multifocal spikes, mainly occipital (68%) and frontal (34%). Cloned-like repetitive multifocal spike-wave complexes were seen in 19%.
Panayiotopoulos syndrome is one of the most common epilepsy syndromes in children referred for EEG. Neither its ictal clinical features or its interictal EEG manifestations suggest an occipital lobe origin. It should be considered an epilepsy characterised by autonomic seizures.