Encephalopathy related to status epilepticus during slow sleep (ESES). Pathophysiological insights and nosological considerations.

Encephalopathy related to Status Epilepticus during slow Sleep (ESES) is a childhood epilepsy syndrome characterized by the appearance of cognitive, behavioral, and motor disturbances in conjunction with a striking activation of EEG epileptic abnormalities during non-REM sleep. After more than 50 years since the first description, the pathophysiological mechanisms underlying the appearance of encephalopathy in association with a sleep-related enhancement of epileptic discharges are incompletely elucidated. Recent experimental data support the hypothesis that the development of the ESES encephalopathic picture depends on a spike-induced impairment of the synaptic homeostasis processes occurring during normal sleep and that is particularly pronounced during the developmental age. During sleep, synaptic homeostasis is promoted by synaptic weakening/elimination after the increment of synaptic strength that occurs during wakefulness. The EEG can display modifications in synaptic strength by changes in sleep slow wave activity (SWA). Recent studies during active ESES have failed to show changes in sleep SWA, while these changes occurred again after recovery from ESES, thus supporting a spike-related interference on the normal homeostatic processes of sleep. This impairment, during the developmental period, can lead to disruption of cortical wiring and brain plastic remodeling, which lead to the, often irreversible, neuropsychological compromise typical of ESES. From the nosographic point of view, these pathophysiological data lend support to the maintenance of the term ESES, i.e., "encephalopathy related to status epilepticus during sleep". Indeed, this term conveys the concept that the extreme activation of epileptic discharges during sleep is directly responsible for the encephalopathy, hence the importance of defining this condition as an encephalopathy related to the exaggerated activation of epileptic activity during sleep. In this respect, ESES represents a genuine example of a "pure" epileptic encephalopathy in which sleep-related epileptic activity "per se" has a crucial role in determining the encephalopathic picture. This paper was presented at the 8th London-Innsbruck Colloquium on Status Epilepticus and Acute Seizures held in September 2022.

The expression of interictal, preictal, and postictal facial-wiping behavior in temporal lobe epilepsy: a neuro-ethological analysis and interpretation.

By videotape recordings analysis we investigated the frequencies of interictal, preictal, and postictal wiping or rubbing movements targeting the face region (face wiping, FW) in 17 right and 13 left mesial temporal lobe epilepsy (MTLE) patients. Patients' data were compared with FW frequencies obtained in 22 healthy controls listening to a presentation. Results showed that: (1) FW movements were present in both controls and patients; however, the patient groups showed lower interictal and preictal FW rates relative than controls; (2) right and left temporal lobe seizures were followed by a marked increase in the expression of wiping activities directed to the nose as well as to other face regions with respect to the interictal-preictal period; (3) during the first 5min postictal FW was performed preferentially with the hand ipsilateral to the seizure focus; (4) postictal examination of the patient by an observer, especially if of the opposite sex, resulted in a higher incidence of FW acts. After temporal lobe seizures there is an exaggerated expression of movements targeting the face region, and not exclusively directed to the nose. According to an ethological interpretation of the FW behavior as a motor behavior present throughout the phylogenetic scale, from rodents to primates, we suggest the postictal emergence of an innate action pattern modulated by external emotional-cognitive stimuli.

Telephone-induced seizures: a new type of reflex epilepsy.

PURPOSE: To report a new form of reflex epilepsy in which the seizures are repeatedly and exclusively triggered by answering the telephone. METHODS: Three patients with a history of telephone-induced seizures were studied in detail by means of clinical, EEG, and neuroradiologic investigations. Intensive video-EEG monitoring to record the reflex seizures also was performed in all cases. RESULTS: The patients (two men, one woman, aged 21 to 30 years) had the onset during early adulthood of complex partial and secondarily generalized seizures exclusively triggered by answering the telephone. The seizures were stereotyped, with subjective auditory or vertiginous auras and inability to speak or understand the spoken voices. In one patient, a telephone-induced seizure arising from the dominant temporal lobe was recorded by means of video-EEG technique. In the interictal EEGs, temporal abnormalities were detected in all cases. The patients had a normal neurologic examination and normal magnetic resonance imaging or computed tomography scans. CONCLUSIONS: We suggest that telephone epilepsy is a previously unrecognized form of reflex epilepsy induced by a complex auditory stimulus involving the lateral temporal areas.