PI reflex trait in JME is related to seizures without preferential circadian occurrence and reduced response to antiepileptic drugs.

ECS is related to a worse outcome regarding control of all seizure types, persistence of EM, and higher frequency of limb myoclonia, as well as the total number of TCS and/or MTCS.

Despite normal physiology of blinking, EM can manifest as jerk, flicker or flutter, with or without EEG abnormalities and independently of IPS, suggesting that eye closure sensitivity seems to include both, a motor and a visual component.

JME patients with a combination of praxis induction and eye-closure/photosensitivity had greater executive dysfunction, revealing an association between reflex ictogenic mechanisms and cognitive performance.

Epileptic spasms were defined by the International League Against Epilepsy Task Force on Classification and Terminology in 2001 as a specific seizure type. Epileptic spasms without hypsarrhythmia have been described in some series of patients, occurring either in infancy or childhood. More prolonged epileptic spasms without hypsarrhythmia were previously defined as a different seizure type, and referred to as "tonic spasm seizures". Here, we present a 5-year-old boy who started having epileptic spasms without hypsarrhythmia at 8 months of age, effectively treated with oxcarbazepine. With the withdrawal of medication, epileptic spasms returned. Video-EEG monitoring revealed high-voltage slow waves superimposed by low-voltage fast activity, followed by an electrodecremental phase and a burst of asymmetric fast activity, time-locked to clinical tonic spasm seizures. Brain MRI showed left temporal atrophy with temporal pole grey/white matter junction blurring and ictal PET-CT showed left basal frontal hypermetabolism. Seizures were refractory to several AEDs and vigabatrin was introduced with seizure cessation. Despite efforts to classify epileptic spasms, these are still considered as part of the group of unknown seizure types. In some cases, a focal origin has been suggested, leading to the term "periodic spasms" and "focal spasms". In this case, epileptic spasms without hypsarrhythmia, associated with tonic spasms, may be a variant of focal spasms and might be considered as an epileptic syndrome. [Published with video sequence]A cluster of three tonic spasms, characterized by sudden flexion of the head and proximal muscles, lasting 5, 4.3 and 1 second, respectively. The spasms were accompanied simultaneously by upward deviation of the eyes and oral automatisms at the end.Key words for the video research on www.epilepticdisorders.com

Gastaut type idiopathic childhood occipital epilepsy is an uncommon epileptic syndrome characterised by frequent seizures, most commonly presenting as elementary visual hallucinations or blindness. Other occipital (non‐visual) symptoms may also occur. Interictal EEG typically shows occipital paroxysms, often with fixation‐off sensitivity. Ictal EEG is usually characterised by interruption by paroxysms and sudden appearance of low‐voltage, occipital, fast rhythm and/or spikes. Despite well described clinical and EEG patterns, to our knowledge, there are very few reports in the literature with video‐EEG recording of either seizure semiology or fixation‐off phenomena. We present a video‐EEG recording of a 12‐year‐old girl with Gastaut type epilepsy, illustrating the interictal and ictal aspects of this syndrome. Our aim was to demonstrate the clinical and neurophysiological pattern of a typical seizure of Gastaut type epilepsy, as well as the fixation‐off phenomena, in order to further clarify the typical presentation of this syndrome. [Published with video sequences]

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