Paediatric Status Epilepticus

Abstract

Status epilepticus (SE) is one of the most common paediatric neurologic emergencies, requiring timely identification and emergency treatment to reduce morbidity and mortality. The evaluation and management of SE are similar in adults and children; however, specific conditions and syndromes with convulsive status epilepticus (CSE) and NCSE occur in children. In NCSE, a correct diagnosis usually needs an EEG. Clinical suspicion of NCSE arises in patients who have disturbance of consciousness with or without minor motor phenomena (e.g., perioral or periorbital low-amplitude jerking). In some cases, only EEG can reveal the epileptiform or rhythmic discharges that lead to diagnosis. The task force recommends describing the EEG correlate of status in a patient using the following descriptors, name of pattern, morphology, location, time-related features, modulation, and effect of intervention, and use the proposed terminology by the American Clinical Neurophysiology Society and the diagnostic “Salzburg EEG criteria for NCSE”. We will describe the electro-clinical features and EEG pattern of the peculiar forms of CSE/NCSE that might be seen in some paediatric epilepsies such as Dravet syndrome; Panayiotopoulos syndrome; Ring chromosome 20-related epilepsy; Angelman syndrome; Rett syndrome; Epilepsy with myoclonic-atonic seizures (Doose syndrome); myoclonic status in nonprogressive encephalopathies; mitochondrial diseases causing SE, absence status, and tonic status in Lennox-Gastaut syndrome; and encephalopathy with electrical status epilepticus in sleep (ESES).