Tonic status epilepticus is a serious, sometimes fatal, condition that may last for days or even months and occurs mainly in children with neurocognitive impairment and severe epilepsy such as the Lennox-Gastaut syndrome. Tonic status epilepticus in these children comprises repetitive frequent tonic seizures. Characteristically, as the status progresses, motor symptoms tend to lessen while autonomic disturbances, including respiratory depression, become more prominent. At final stages, there may be only electrographic evidence of tonic seizures. Video-EEG monitoring is usually mandated for the recognition of autonomic status epilepticus, particularly when motor manifestations are minimal. Treatment is demanding, and there may be some concern about the intravenous administration of benzodiazepines because of a few reports that these may induce tonic status epilepticus; newer antiepileptic drugs such as lacosamide have been reported as effective. Unfortunately, the only systematic studies of tonic status epilepticus are dated 40 years ago. Dr. C P Panayiotopoulos, Consultant Emeritus in Clinical Neurophysiology and Epilepsy at St. Thomas’ Hospital in London, England, reviews the limited information on clinical manifestations, investigative procedures, pathophysiology, and optimal but anecdotal management of patients with tonic status epilepticus.
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