Early treatment with bromides should be considered in EIMFS to control the seizures and status epilepticus and to avoid progressive cognitive impairment. Potassium bromide is an old AED. Plasma concentration monitoring should be considered.

Our patients with typical clinical manifestations of ESES syndrome but with unusual EEG patterns may be variants of this syndrome.

Aim. To present a retrospective study of nine children with Panayiotopoulos syndrome associated with diffuse spikes and waves as the sole EEG manifestation at onset. Methods. Charts of children with typical clinical criteria of Panayiotopoulos syndrome, electroclinically followed between February 2000 and February 2012, were reviewed. Results. Among 150 patients who met the electroclinical criteria of Panayiotopoulos syndrome, we identified nine children who presented with the typical clinical manifestations but who, on EEG, only had diffuse paroxysms at onset that continued along the course of the syndrome. In three, in addition to the diffuse paroxysms, focal spikes appeared later. From a clinical point of view, other features were otherwise unremarkable. Diffuse spike‐and‐wave discharges were observed in all patients when awake and during sleep (100%). Later, three children also had focal spikes during sleep, which were occipital in one, frontal in one, and temporo‐occipital in the remaining patient. Spikes were activated by sleep in all three cases. During disease evolution, no particular electroclinical pattern was observed. Two patients who received clobazam and carbamazepine, respectively, did not respond well to the drugs and valproic acid was added with excellent seizure control. Outcome was good. Conclusions. We present evidence that patients with Panayiotopoulos syndrome may have diffuse discharges at onset as the sole EEG manifestation, which last throughout the course of the syndrome. In some, focal paroxysms developed later. The course was benign. In our group of patients, clinical features and evolution were similar to those of typical cases of Panayiotopoulos syndrome.

Neurofibromatosis type 1 (NF1) is the most common neurocutaneous disease. The NF1 gene is located on chromosome 17q11.2 and codes for a large tumor suppressor protein, called neurofibromin. The prevalence is around 1 in 3,000 individuals, and it is inherited dominantly with a 98% penetrance, but with high phenotypic variability. Neurologic manifestations are mainly tumors, such as optic gliomas, focal areas of high T2-weighted magnetic resonance imaging signals known as unidentified bright objects, mental retardation or learning disabilities, and epilepsy. Other neurologic problems, including attention deficits and headaches, have been described. The prevalence of seizures in patients with NF1 is around 3.8 to 6%. This is considerably higher than the 1 to 2% reported for the general population, or the 4% of children who have experienced a seizure. The majority of classifiable seizures are focal in origin with or without secondary generalization. Most abnormal electroencephalography recordings reveal focal abnormalities. Overall, more than half of the electroencephalography studies are abnormal. The most frequent structural lesions found in patients with NF1 and epilepsy are tumors and malformations of cortical development. Other types of epilepsies and epileptic syndromes have also been described to be associated with NF1, such as West syndrome, Lennox-Gastaut syndrome, myoclonic epilepsy, epilepsy with generalized tonic-clonic seizures, childhood absence epilepsy, and juvenile myoclonic epilepsy. In patients with focal epilepsy, neuroimaging should be performed to exclude neoplastic and other types of focal lesions. In patients with NF1 and seizures, optimal management of antiepileptic drugs and eventually surgery should be considered.