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• W1972534723 abstract "To the Editor: The recent “proposed diagnostic scheme for people with epileptic seizures and with epilepsy of the International League Against Epilepsy (ILAE) Task Force on classification”1 makes a significant contribution by recognizing Panayiotopoulos syndrome (PS) 2 among the idiopathic focal epilepsies of childhood in addition to the “benign childhood epilepsy with centrotemporal spikes” and the “late-onset childhood occipital epilepsy (Gastaut type).” They proposed a descriptive name “early-onset benign childhood occipital epilepsy” attached to an eponym “Panayiotopoulos type (syndrome)”1. I wish to draw attention that the descriptive nomenclature of PS as “occipital epilepsy,” also previously described as “with occipital paroxysms,” may be misleading. Occipital paroxysms in their classic form with fixation-off sensitivity is a rare finding in PS and certainly nonspecific 3. Interictal EEG in PS mainly manifests with multifocal spikes at various locations, although occipital spikes often (70%) predominate 2, 4, 5. EEGs may be normal or without occipital spikes (30%) 2, 4, 5. In the original study of Panayiotopoulos 4 of 21 otherwise normal children with ictal vomiting; occipital spikes occurred in 12 (57%); the others had extraoccipital spikes (five), infrequent brief generalized discharges (one), or consistently normal EEG (three) 4. Subsequent attention was focused on the predominant group of occipital spikes and occipital paroxysms, but this is now corrected to include the group of “extraoccipital spikes or normal EEG.” The clinical manifestations of PS are the same irrespective of EEG localizations. Occipital epilepsy also is incorrect for the following good reasons: (a) onset of seizures is mainly with autonomic symptoms and particularly emesis (80%) 5. Of occipital symptoms, only deviation of the eyes may originate from the occipital regions, but this rarely occurs at onset. Visual symptoms are exceptional and not consistent in recurrent seizures; (b) interictal occipital spikes may never occur, (c) even ictal EEG has documented anterior onset 6. Characterizing PS as “epilepsy” also is controversial and, in my opinion, unsatisfactory. One third of children with PS have a single seizure, which by the operational definition of epilepsy (more than two seizures) is not epilepsy. Further, PS is not “a chronic neurological condition characterized by recurrent epileptic seizures” (the current definition of the ILAE glossary) 7. All these point out that PS should be classified among “conditions with epileptic seizures that do not require a diagnosis of epilepsy,” which is a new concept of the ILAE proposal to incorporate febrile, benign neonatal, single seizures, isolated clusters of seizures, and rarely repeated seizures (oligoepilepsy) 1. PS is a common clinical phenotype of the benign childhood seizure susceptibility syndrome 2, 5. It manifests with autonomic seizures and autonomic status epilepticus that in one third are singular events. Prognosis is excellent even for those (∼10%) who may initially have many seizures. Interictal EEG shows significant variability, even for the same child. Predominantly PS affects children aged 3–7 years, probably as the result of susceptible emetic and autonomic centers of this age group 5. PS is a significant missing land in pediatric epileptology with immense clinical and management implications. Prospective studies are needed to clarify the many typical and atypical clinical presentations of PS, their autonomic components, and the EEG variations." @default.
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• W1972534723 date "2002-10-11" @default.
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• W1972534723 title "Panayiotopoulos Syndrome or Early-onset Benign Childhood Occipital Epilepsy" @default.
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• W1972534723 doi "https://doi.org/10.1046/j.1528-1157.2002.10102_2.x" @default.
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