Panayiotopoulos Syndrome

Panayiotopoulos syndrome (susceptibility to early onset benign childhood seizures with occipital and extra-occipital spikes) documented in large-scale independent studies from around the world of over 600 patients has only recently been officially recognized in the new Classification Scheme of the ILAE. Panayiotopoulos syndrome affects 6 per cent of children aged 1-15 years with seizures or 13 per cent in the age group 3-6. The clinical manifestations consist of mainly autonomic seizures and autonomic status epilepticus with ictal emesis as a cardinal symptom. Interictal EEG usually shows multifocal spikes that predominate in the posterior regions. Prognosis is invariably excellent and one-third of patients have a single seizure.

Despite high prevalence and striking clinical and EEG features, Panayiotopoulos syndrome is practically unknown and conspicuously absent from relevant 'epilepsy' books and paediatric journals. The result is avoidable morbidity, erroneous treatment and costly hospital admissions. Panayiotopoulos syndrome is often disguised as non-epileptic disorders such as migraine, gastroenteritis or syncope. Seizures often start and relentlessly progress in a manner suggestive of an acute and serious cerebral insult such as encephalitis for which many of these children are erroneously treated.

This book details all aspects of Panayiotopoulos syndrome based on a critical analysis of the accumulating literature and the prospective studies of the author over the last 30 years. Data are meaningfully tabulated and there are numerous examples of typical and atypical cases. Interictal and ictal EEG are finely illustrated in over forty figures. The differential diagnosis from non-epileptic and other epileptic syndromes is superb. Particularly useful are the guidelines for diagnosis, diagnostic procedures and management. Equal importantly, the author provides means of finding the true dimensions of Panayiotopoulos syndrome in any paediatric department through retrospective studies and audits.

Panayiotopoulos syndrome affects children in the intermediate age between febrile seizures and Rolandic epilepsy. It is the missing land of a significant part of paediatric clinical and EEG epileptology. Practising paediatric epileptology may be problematic without thorough knowledge of the many aspects of Panayiotopoulos syndrome.

Multidisciplinary researchers of the autonomic nervous system and emesis who now have the advantage to examine common ictal-seizure autonomic manifestations that were not previously considered. That these are specific to childhood is a significant challenge for investigation.