Lennox-Gastaut syndrome

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Classification according to ICD-10
G40.4 Other generalized epilepsy and epileptic syndromes
Lennox syndrome
ICD-10 online (WHO version 2019)

The Lennox-Gastaut syndrome (LGS), also known under the synonym Lennox-Gastaut syndrome is known, a most difficult to treat form of epilepsy that in children usually begins in the period between the second and sixth years, with frequent and different seizure types goes hand in hand and the cause of which is a causal multiple damage to the brain, which occurred either prenatally ( prenatal ), during birth (perinatal) or postnatally (postnatal).

history

The syndrome was named after the American neurologist and epileptologist William G. Lennox (Boston / USA) and the French neuroanatomist, neurologist, clinical neurophysiologist and epileptologist Henri Gastaut ( Marseille / France) who developed this form of epilepsy in the 1950s and 1960s Years and years for the first time described in detail from a scientific point of view and dealt intensively with their research and delimitation from other forms of epilepsy. Gastaut relied on the doctoral thesis of his colleague Charlotte Dravet.

Frequency of occurrence

An estimated 5 out of 100 children with epilepsy have LGS, with boys being more likely to be affected than girls in percentage terms . While some of the children showed no abnormalities before the onset of the seizures, the other part already had some form of epilepsy, which then passed into the LGS. On average, one in five children with LGS had been diagnosed with West syndrome (symptomatic) before it appeared, which is characterized by BNS cramps and often turns into LGS within the second year of life.

root cause

The cause of this form of epilepsy must be considered on a case-by-case basis, as there is no uniform cause: LGS develops from West syndrome in one in five children. Otherwise, information about neonatal cramps or focal and generalized seizures can be found in the anamnesis .

In up to two thirds of children with LGS, epilepsy occurs as a consequence or symptom of brain damage ( encephalopathy ) or of another disease or developmental disorder. Common causes are, for example, tuberous sclerosis (Bourneville syndrome), metabolic diseases , encephalitis such as B. encephalitis , meningitis or toxoplasmosis , profound organic brain disorders such. B. due to lack of oxygen during the birth or due to a premature birth as well as various kinds of traumatic brain injuries . If such or a similar cause can be proven, one speaks of a symptomatic Lennox-Gastaut syndrome , since the seizures are an accompanying symptom or feature (Symptom) of something else.

In up to a third of the cases, no underlying disease can be proven that apparently led to the occurrence of LGS. In such cases one speaks of a cryptogenic or idiopathic Lennox-Gastaut syndrome .

features

As a rule, epileptic seizures in the context of LGS occur for the first time between the ages of two and six, although in exceptional cases the time of first manifestation can also be within the second year of life or after the eighth year of life. The appearance shows clear parallels to the West Syndrome , so that a relationship is likely.

The occurrence of seizures several times a day is typical for LGS. What is also striking is the wide range of seizure types that occur in this variety in no other epilepsy syndrome: the most common are tonic seizures of varying severity that often occur during sleep (in around nine out of ten children), the second most common are myoclonic seizures to observe, which occur more frequently when tired.

Atonic seizures, atypical absences, focal and sometimes generalized tonic-clonic (grand-mal) seizures can also occur. In addition, one in two children develops an epileptic status ( status epilepticus ), usually in the form of a nonconvulsive status, which is characterized by confusion, apathy and a lack of reactions. In particular, the atypical absences can occur with status-like accumulation. The attacks often lead to sudden falls (with tonic, atonic and myoclonic cramps) or noticeable loss of posture, which is why some affected children wear a helmet to protect their head.

Children with LGS often show a significant delay in overall physical development, a cognitive disability and behavioral problems.

diagnosis

As a clinical picture, LGS is often difficult to narrow down or clearly differentiate from other clinical pictures, as there are diverse and sometimes fluid transitions to similar syndromes . As already mentioned, there is no uniform cause that would simplify the diagnosis.

This form of epilepsy is clearly visible through frequent and varied seizures. When measuring the brain waves by means of an EEG , a generally slowed down basic rhythm with always slow spike-wave patterns or multifocal and generalizing sharp-slow-wave discharges , which occur with a frequency between 1.5 and 2.5 times per Second can be registered. Changes to the pattern are possible to the extent that there may be side differences and focal peculiarities in the derivation. Tonic patterns, i.e. series of rapid spikes, can often be registered during sleep.

In particular, the differential diagnosis to pseudo-Lennox syndrome must be clarified, which differs from LGS in that no tonic seizures occur. Because of this, the diagnosis must be made by EEG during sleep (sleep EEG), since the tonic seizures typical of LGS usually occur during sleep.

An imaging examination of the brain using magnetic resonance tomography (MRT) is possible to check the presence of a cerebral organic feature . If the movement patterns in the tonic seizures, in particular, are lateral, this suggests, for example, brain damage on the corresponding side, which should be investigated.

During the general medical examination, most children with LGS notice peculiarities in the physical area, in particular a significant delay in development can be seen. In addition, there are often cognitive weaknesses, a corresponding reduction in performance and behavioral problems. The peculiarities can already have appeared before the first occurrence of the epileptic seizures, but also appear only up to two years after the manifestation of the LGS and are often attributable to the underlying disease that led to the seizures.

therapy

LGS is a form of epilepsy that is comparatively difficult to treat, even though it is e.g. B. is often easier to treat than the attacks that occur in West Syndrome . A diagnosis as early as possible is important, but cannot guarantee that the therapy will be successful.

If a treatable organic cerebral peculiarity is the cause of the seizures, in some cases, after carefully weighing the advantages and disadvantages, an operative correction by epilepsy surgery is possible, and by eliminating the cause, the seizures can be reduced or disappear. If (falls) seizures occur very frequently and cannot be stopped with medication, vagus nerve stimulation and partial cutting of the bar connecting the two halves of the cerebrum (callosotomy) can be considered.

A consistent ketogenic diet , which is also used in therapy-resistant epilepsy, can apparently help in about one out of three cases .

However, the therapy of LGS is mostly based on the administration of medication, whereby the medication treatment is comparatively difficult and, despite all efforts, it is often impossible to achieve freedom from seizures. In some cases, therapy resistance develops or therapy resistance is present from the start.

If therapy with just one drug ( monotherapy ) is unsuccessful, several, but usually not more than three, drugs are administered at the same time ( combination therapy ). The drugs valproate , levetiracetam , lamotrigine , topiramate , benzodiazepines and felbamate are often used in the treatment of LGS . With the anti- epileptic rufinamide , a new option is available for the additional therapy of LGS in patients from the age of four; there may be a significant decrease in the frequency and severity of the seizures. The drug Epidiolex, which consists of 99.9% cannabidiol of plant origin, has been approved for treatment in the USA since 2018.

The course and drug treatability are usually much more favorable in pseudo-Lennox syndrome .

forecast

In general, LGS (or the damage to the brain on which epilepsy syndrome is often based) is characterized by a comparatively unfavorable prognosis - both in terms of medical treatability and quality of life and child development, and in some cases also life . Regardless of the extent and impact of the underlying disease, early onset of LGS and frequent tonic seizures are seen as features of an unfavorable overall prognosis. In some cases, in the course of the LGS, the epileptic seizures become secondary, while progressive cognitive and physical impairments as well as behavioral disorders often come to the fore.

Statistically speaking, five out of 100 children do not survive the first five years of their life, and they do not die from epileptic seizures but from the underlying illness that triggered the seizures or the complications resulting from it.

Statistically, little more than half of the children with LGS can be treated comparatively satisfactorily with medication, with only 5 to 10 children remaining free of seizures in the long term and only 15 out of 100 children developing cognitively and motor correctly and leading an independent and largely symptom-free life in adulthood . The latter are usually those in whom there were no abnormalities before the first appearance of LGS and no underlying disease (brain damage) is the cause of the attacks.

An increase in developmental abnormalities can usually be observed in those children who had disorders of cognitive and psychomotor development even before the first manifestation of LGS. However, this course is often not due to the epileptic seizures, but to the underlying disease, e.g. B. on a progressive dysfunction of the brain. But also an unfavorable course of the epileptic seizures, especially if the convulsions occur in a status-like manner, can cause additional permanent damage.

Many children are clearly physically and cognitively impaired even after the seizures have ceased, although this cannot usually be attributed primarily to the epileptic seizures, but to their cause (cerebral peculiarity or its severity). Learning disorders, language disorders and movement disorders as well as impairment of cognitive performance and cerebral palsy are also common in adulthood .

See also

literature

  • Ulrich Altrup, Christian E. Elger : Epilepsy. Information in texts and images for those affected, relatives and interested parties. Novartis Pharma-Verlag, Nuremberg 2000, ISBN 3-933185-49-1 .
  • Laura Doermer: Moritz my son. Bertelsmann, Munich 1990, ISBN 3-570-08222-9 (experience report).
  • Günter Krämer : Diagnosis of epilepsy. Trias, Stuttgart 2003, ISBN 3-8304-3077-9 .
  • Günter Krämer, Ritva A. Sälke-Kellermann (ed.): The Lennox-Gastaut syndrome (= epilepsy reports. Volume 5). Blackwell Wissenschafts-Verlag, Berlin et al. 1998, ISBN 3-89412-341-9 .
  • Heiko Puckhaber: Childhood Epilepsy. An interdisciplinary task. 5th, unchanged edition. Klotz, Eschborn near Frankfurt am Main 2000, ISBN 3-88074-240-5 .
  • Hansjörg Schneble: Epilepsy in Children. How your family learns to live with it. Trias, Stuttgart 1999, ISBN 3-89373-528-3 .
  • Ulrich Stephani: The Lennox-Gastaut Syndrome. Diagnosis, treatment and support in everyday life. Trias, Stuttgart 2008, ISBN 978-3-8304-3467-2 .

Individual evidence

  1. ^ WG Lennox, JP Davis: Clinical correlates of the fast and the slow spike-wave electroencephalogram. In: Pediatrics . tape 5 , 1950, pp. 626-644 .
  2. ^ H. Gastaut, H. Régis: On the subject of Lennox '"akinetic" petit mal. In memory of WG Lennox. In: Epilepsia . tape 2 , 1961, p. 298-305 .
  3. H. Gastaut, J. Roger, R. Soulayrol et al.: Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as ' petit mal variant') or Lennox syndrome. In: Epilepsia . tape 7 , 1966, pp. 139-179 .
  4. C. Dravet: Encéphalopathie Épileptique de l'Enfant avec Pointe-onde lente diffuse (“petit mal variant”). Thesis. Marseille 1965.