Absence status epilepticus

Introduction
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By C P Panayiotopoulos MD PhD FRCP

Absence status epilepticus is also known as or subsumes Absence status epilepticus in comatose or critically ill patients, Cryptogenic or symptomatic (atypical) absence status epilepticus, Idiopathic (typical) absence status epilepticus, Situation-related absence status epilepticus, and Petit mal status. -ed.

Absence status epilepticus is a prolonged, generalized absence seizure that usually lasts for hours and can even last for days. The cardinal symptom is the altered state of consciousness while the patient is usually fully alert and partly responsive. Absence status epilepticus may be typical, occurring in patients with idiopathic generalized epilepsy, or atypical, occurring in patients with neurocognitive impairment as with epileptic encephalopathies. Absence status epilepticus may also appear de novo, mainly in adults without a previous history of epilepsy. Absence status epilepticus is often misdiagnosed as focal status epilepticus or a confusional nonepileptic condition or epileptic prodrome. Frequently, absence status epilepticus occurs because of ill-advised antiepileptic drug treatment, such as with tiagabine or carbamazepine in patients with idiopathic generalized epilepsy. In this clinical summary, Dr. C P Panayiotopoulos, Consultant Emeritus in Clinical Neurophysiology and Epilepsy at St. Thomas’ Hospital in London, England, details recent developments in the clinical presentation, related epileptic syndromes, pathophysiology, genetics, diagnosis, and optimal pharmacological treatments.

Key Points

  • Absence status epilepticus is a prolonged, generalized absence seizure that usually lasts for hours and can even last for days.
  • The cardinal symptom is the altered content of consciousness while the patient is usually fully alert and partly responsive.
  • Most patients suffer from idiopathic generalized epilepsy, but absence status epilepticus may also appear de novo. Other types of seizure such as absences, myoclonic jerks, and generalized tonic-clonic seizures may predate the first occurrence of absence status epilepticus for many years.
  • Absence status epilepticus is often misdiagnosed as focal status epilepticus, confusional nonepileptic condition, or epileptic prodrome.
  • Frequently, absence status epilepticus occurs because of ill-advised antiepileptic drug treatment such as tiagabine, carbamazepine, gabapentin, oxcarbazepine, pregabalin, or vigabatrin.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Localization
Pathophysiology
Epidemiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
References cited
Contributors