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  • Updated 10.05.2021
  • Released 09.29.1994
  • Expires For CME 10.05.2024

Generalized tonic-clonic status epilepticus

Introduction

Overview

Status epilepticus is a serious medical condition with annual incidence up to 10 to 20 per 100,000 cases in the United States with a relatively high mortality. Generalized tonic-clonic status epilepticus is the most life-threatening form of status epilepticus. Increased awareness of this condition and the evolving treatment paradigms is essential in the management of patients with this condition. This article discusses the definition, clinical presentation, diagnostic workup, and current management of generalized tonic-clonic status epilepticus. A literature review of the epidemiology, etiology, pathophysiology, and treatment of acute status epilepticus and refractory status epilepticus is also provided.

Key points

• Intravenous antiseizure medications have clear roles in the treatment of status epilepticus.

• Autoimmune causes of status epilepticus are increasingly recognized and may direct treatment.


• Research indicates changes in synaptic properties, including internalization of receptors in pathophysiology of status epilepticus that may complicate treatment of refractory status epilepticus.


• Continuous video-EEG monitoring is essential in management, diagnosis, and treatment of refractory status epilepticus.

Historical note and terminology

The first description of what we recognize today as generalized convulsive status epilepticus (GCSE) goes back to the first millennium B.C. in Babylonian cuneiform stone tablets (54). Louis Calmeil, in his doctoral thesis for University of Paris, was the first who used the term état de mal" (state of disease) in medical literature to describe status epilepticus and mentioned that the term was in fact in common usage by patients at the Salpêtrière hospital in Paris (06). In his work he first pointed out the distinctive characteristic of status epilepticus in that there is no recovery of consciousness between the seizures as opposed to a cluster of seizures when consciousness is regained in between. In addition, he noticed the severe and often fatal outcome of this condition. Subsequently, further advancement in characterization, description, and treatment of this condition was made by other physicians in 19th and early 20th century, such as Désiré-Magloire Bourneville (1840-1909), Armand Trousseau (1801-1867), Jean-Martin Charcot (1825-1893), William Gowers (1845-1915), L Pierce Clark (1870-1933), and Thomas Prout (1862-1939). With the introduction of EEG to clinical practice, major advances in the understanding of status epilepticus were made in the 1950s and 1960s. In 1962, at the 10th European electroencephalography meeting in Marseilles led by Gastaut, status epilepticus was proposed to be defined as a seizure [that] persists for a sufficient length of time, or is repeated frequently enough to produce a fixed and enduring epileptic condition (16). The definition was broad and nonspecific and could include the full spectrum of seizure types. As International League Against Epilepsy (ILAE) international classification of seizure types emerged, parallels between classification of status epilepticus and this new seizure classification were drawn. In a 1981 revision, the ILAE defined status epilepticus as a seizure [that] persists for a sufficient length of time or is repeated frequently enough that recovery between attacks does not occur. In the subsequent 20 years, new definitions included specific and minimum amount of time as a criterion for status epilepticus, ranging between 30 to 60 minutes, mostly based on some animal models that showed neuronal injury in prolonged seizures.

In 1999, Lowenstein and colleagues suggested an operational definition of status epilepticus to prompt the start of management of this condition well in advance of reaching the 30 minutes or longer duration suggested in its definition (33). They suggested that generalized convulsive status epilepticus be defined as a continuous seizure longer than 5 minutes or 2 or more discreate seizures with incomplete recovery of consciousness in between. This operational definition was widely adopted. These recommendations were mostly based on the data that observed seizures longer than 5 minutes are less likely to cease on their own or longer seizures are more likely to become resistant to medications.

In 2015, an ILAE task force proposed a conceptual definition of status epilepticus as follows: Status epilepticus is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which lead to abnormally, prolonged seizures (after time point t1). It is a condition, which can have long-term consequences (after time point t2), including neuronal death, neuronal injury, and alteration of neuronal networks, depending on the type and duration of seizures (48). For generalized convulsive status epilepticus, the task force considered 5 minutes and 30 minutes as duration of t1 and t2, respectively.

For a more detailed review of the history of status epilepticus, see Neligan and Shorvon (37).

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