Epilepsy – colloquially called seizure disorder – (synonyms: epilepsia; epilepsy; epileptic seizure; grand mal; petit mal; cerebral seizure; ICD-10-GM G40.-: Epilepsy), the condition is described when seizures occur repeatedly due to an underlying chronic process. It involves dysfunction of the central nervous system. According to the ILAE Working Group, epilepsy is a disease of the brain when:
- At least two unprovoked seizures (or reflex seizures) have occurred more than 24 hours apart
- One provoked seizure (or reflex seizure) and a probability of another seizure in the range of the risk of relapse after two unprovoked seizures (at least 60%) for the next 10 years
- Diagnosis of an epilepsy syndrome (such as Rolando’s epilepsy).
According to the new DGN guidelines, epilepsy may be present even after an unprovoked seizure. The prerequisite is that the risk of another unprovoked seizure during the next ten years must be at least 60%, which is comparable to the general risk of relapse after two unprovoked seizures,
An epileptic seizure occurs when there are excessive or even synchronous discharges of sufficiently large groups of nerve cells in the brain. We speak of an “occasional seizure” when an epileptic seizure occurs due to a one-time provocation, due to internal or external influences such as metabolic disorders or lack of sleep. Classification according to seizure types:
- Focal (local) seizures*
- Generalized (affecting the whole body) seizures* .
- Unknown (non-classifiable epileptic seizures).
* See “Symptoms – Complaints” below for description. To assess long-term recurrence risk, it is important to differentiate into acutely symptomatic versus unprovoked seizure after a first epileptic seizure. For a detailed list of seizure types, see “Classification” below. In early childhood, the generalized forms of epilepsy are predominant. A seizure usually lasts no longer than 2 minutes. A seizure lasting more than 5 minutes is defined as “status epilepticus”. In approximately 15% of patients, status epilepticus represents the initial manifestation of epilepsy in adulthood. Approximately 12% of all epileptic seizures occur during sleep. Frequency peak: The disease occurs predominantly in childhood and has a second frequency peak in older age (> 50 years). Approximately one-third of epilepsies first occur at age > 60 years.Doose syndrome (rare form of childhood epilepsy) usually begins at age 1-5 years. The prevalence (disease incidence) is 0.7-0.8%. 70 million people have regular epileptic seizures (worldwide). Lifetime prevalence (disease frequency throughout life) for epilepsy is >5%. The probability of a single, isolated epileptic seizure is > 10%. In this case, one speaks of an “occasional seizure”. The cause is a single provocation by internal or external influences such as metabolic disturbances or sleep deprivation. The incidence (frequency of new cases) in children is about 60 cases per 100,000 inhabitants per year. In adults, the incidence is 30-50 diseases per 100,000 inhabitants per year (in Germany). In older age, it increases to 140 diseases per 100,000 inhabitants per year.The index of Doose syndrome is 10 diseases per 100,000 inhabitants per year. Course and prognosis: In most cases, the epileptic seizure is followed by a postictal phase that can last up to 24 hours. During this time, memory impairment, speech disorders, paresis (paralysis), depressive moods, or aggressive states may occur.At least one third of patients experience further seizures within the following three years after the first epileptic seizure. In Doose’s syndrome, signs include muscles suddenly cramping or going limp, falling seizures, and pauses in consciousness. The prognosis depends primarily on the etiology (cause). In idiopathic (cause not identifiable) or even cryptogenic form (cryptein = to hide), prognosis is difficult to establish. If the cause is known and adequate therapy is given, the epileptic seizures disappear permanently (in approx. 60-80% of cases).The mortality rate (number of deaths in a given period, relative to the number of the population in question) is 2.5%. Causes of premature death are pneumonia (pneumonia), cerebrovascular (affecting blood vessels of the brain) and neoplastic (neoplasm) diseases. The most common cause of death is SUDEP: Sudden Unexpected Death in Epilepsy. This is a vegetative accompanying reaction of the epileptic seizure, which manifests itself in cardiac arrhythmias (heart rhythm disturbances), respiratory insufficiency (breathing weakness) as well as electrolyte shifts. In rare cases, seizure-related fatalities can also occur (e.g., drowning). Myoclonic epilepsies of early childhood with encephalopathic character have the worst prognosis. In this form, life expectancy is also reduced. A long-term British-Swedish study showed that people with epilepsy were 11 times more likely to die before their 56th birthday (compared with the general population). In particular, patients who also have a mental illness or use alcohol or other drugs are at increased risk. A common cause of death among those with epilepsy is suicide. Partly responsible for this high excess mortality in the first two years of the disease are sometimes fatal cancers (primary brain tumor, brain metastases) that triggered epileptic seizures. This was particularly true in patients younger than 60 years of age compared with older patients (15% versus 1%). In the younger deceased, external causes (e.g., accidents) were also significant (12.8% versus 1.4%). Vital threatening causes are generalized tonic-clonic seizures (“grand mal”) in status epilepticus. Epilepsy is considered overcome when patients have an age-related epilepsy syndrome and are beyond that age (e.g., Rolando’s epilepsy); further, when they have remained seizure-free for 10 years and have not received antiepileptic drugs in the past 5 years. Comorbidities: Epilepsy is increasingly associated with psychiatric comorbidities such as depression and generalized anxiety disorder. Depression and substance abuse are associated with an increased risk of mortality (death rate) (nine-year observation period; 8.8% of epilepsy patients (vs. 0.7% of controls) died at a mean age of 34.5 years. Children have attention-deficit/hyperactivity disorder (ADHD) in 15-35% of cases.
