Panayiotopoulos Syndrome - History

History

Chrysostomos (Tomis) P. Panayiotopoulos described this syndrome and autonomic status epilepticus particular to childhood through a 30-year prospective study that started in Greece in 1975. Initial publications included patients with EEG occipital paroxysms or occipital spikes that attracted the main attention, but later it became apparent that the same clinical manifestations, and mainly ictal vomiting, could occur in children with EEG extraoccipital spikes or normal EEG.

In Panayiotopoulos’ original study, ictal vomiting occurred in only 24 children out of 900 patients of all ages with epileptic seizures. Twenty-one were otherwise normal children (idiopathic cases constituting what is now considered Panayiotopoulos syndrome), and 3 had symptomatic epilepsies. Half of the seizures were lengthy, lasting for hours (autonomic status epilepticus). The EEG of the 21 idiopathic cases showed great variations: 12 had occipital paroxysms or spikes alone or with extraoccipital spikes; 2 had central spikes and giant somatosensory evoked spikes; 2 had midline spikes; 1 had frontal spikes; 1 had brief generalized discharges; and 3 had consistently normal EEG. Subsequent attention was focused on the predominant group with occipital spikes, which was established as "early onset benign childhood epilepsy with occipital paroxysms". The other group of 9 children with extraoccipital spikes or normal EEGs was reevaluated much later; their clinical manifestations and outcome were similar to those patients with occipital spikes. Based on these results, it has been concluded that these 21 children, despite different EEG manifestations, suffered from the same disease, which is now designated as Panayiotopoulos syndrome to incorporate all cases irrespective of EEG localizations.

However, there was initial scepticism and resistance to these findings, including from influential epileptologists because as explained by Ferrie and Livingston:"(a) ictal vomiting had been considered as extremely rare and hitherto had been mainly described in neurosurgical series of adult patients. In children it was generally not considered as having an epileptic origin; (b) autonomic status epilepticus was not recognised as a diagnostic entity; the proposition that it might be a common occurrence in a benign seizure disorder challenged orthodox concepts of status epilepticus; (c) it implied that paediatricians had been failing to diagnose significant numbers of children with epilepsy, instead erroneously labeling then as having diverse non-epileptic disorders such as encephalitis, syncope, migraine, cyclic vomiting syndrome and gastroenteritis; (d) the characteristic EEG findings suggested alternative diagnoses. Occipital spikes suggested "childhood epilepsy with occipital paroxysms" of Gastaut; multifocal spikes suggested symptomatic epilepsies with poor prognosis.

The veracity of Panayiotopoulos’s initial descriptions has, over the last two decades, been confirmed in large and long term studies from Europe, Japan and South America. The published database on which our knowledge of PS is now based includes over 800 cases of all races; there are few epilepsy syndromes which are better characterised."What emerges are a remarkably uniform clinical picture and a diagnosis which is strikingly useful in helping predict prognosis and dictate management."

Autonomic status epilepticus is the more common type of nonfebrile status epilepticus in otherwise normal children and has been assessed in a consensus statement.