Rolandic epilepsy
| Classification according to ICD-10 | |
|---|---|
| G40.08 | Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with focal onset seizures - benign epilepsy in childhood with centrotemporal spikes in the EEG |
| ICD-10 online (WHO version 2019) | |
The Rolando epilepsy , rolandic epilepsy or benign childhood epilepsy with centrotemporal Spikes (abbreviated BCECTS from English benign childhood epilepsy with centrotemporal spikes ) is the most common form of epilepsy in childhood.
clinic
Rolando epilepsy manifests itself in clonic or myoclonic seizures with somatosensory components. It often starts with tingling or numbness of the tongue, lips, gums or the inside of the cheek on one side of the face. This is more often followed by slight cramps and usually also twitching in the same regions, including the facial muscles on one side (hemifacial cloni or myoclonus). When the swallowing and chewing muscles are involved, swallowing disorders and increased salivation lead to gurgling or grunting noises or gnashing of teeth. A disturbance of consciousness is usually only faked by a speech disorder (speech arrest) that persists during the rest of the seizure. Younger children in particular show more frequent seizures with muscle twitching (clonies) of an arm, a whole side of the body or secondary generalized tonic-clonic seizures.
Regardless of the time of day, three-quarters of all those affected only have the seizures while they are asleep, in around 15% they occur both in the sleeping and waking states and in the remaining 10% only in the waking state. In the case of distribution during the night, there is a clear preference for the early morning hours. The duration of the seizures is between a few seconds and a maximum of a few minutes, with nocturnal seizures being stronger and longer lasting than those occurring during the day. If they spread, they can exceptionally last longer than 10 minutes and be followed by paralysis of the parts of the body involved. Generalized tonic-clonic seizures practically never occur during the day.
Seizures usually only occur at longer intervals of several weeks to months and only very rarely several times in a row. On the other hand, about every fifth child has frequent seizures, possibly several times within 24 hours. Even then, there are almost always long seizure-free times. The predominantly rare and usually relatively mild seizures are also the explanation for the fact that this form of epilepsy is often not recognized or only recognized with delay. This is often the case when the children happen to sleep with their parents (like on vacation) and they wake up to a loud gurgle or "grunt" from the children. Fatigue and sleep promote seizures.
The classic description of a seizure by parents is that their children come to them at night, appear awake but cannot speak and point to their mouth, which is twisted to one side and running out of saliva. Occasionally, muscle twitching can also be observed. Only after the seizure can the children report that they woke up with a “numb” or “electrifying” feeling in the mouth area. In rare, unusual, or atypical types of seizures, some children initially complain of abdominal pain, visual disturbances including blindness and flashing lights, and dizziness. A combination with absenteeism, myoclonic or astatic seizures appears to be possible, and a status of Rolando seizures has been described in individual cases.
Diagnosis
In addition to the clinical diagnosis based on the typical type of seizure, the electroencephalogram (EEG) is used for diagnosis. It shows the typical spikes ("Rolando spikes") or sharp waves in the centrotemporal area of the brain.
The characteristic EEG changes are foci with highly tense epileptogenic or "spasmodic" activity over the middle temporal lobe , the rolando or central region or over the back of the head, often spreading to the opposite side. Usually there are high peaks with deflections in two directions ( biphasic spikes ), which are followed by slow waves. Remarkably, there are often changes of page and location as well as strong fluctuations in intensity. There is no relationship between the extent of the EEG changes and the frequency and severity of seizures. In addition, even impressive EEG changes do not have to be accompanied by clinically recognizable seizures, which is shown, among other things, with recordings from symptom-free siblings. The EEG changes increase with sleepiness and with increasing depth of sleep. Because they can only be observed during sleep in around one in three children, it makes sense to take a sleep EEG in cases of doubt.
causes
It is probably a genetic disorder with an unexplained inheritance. 90% of the gene carriers are healthy, but show abnormalities in the EEG.
Rolando epilepsy is an example of a foci-shaped partial epilepsy that is suspected of circumscribed damage to the brain, but for which no cause can be found with the methods available today and for which a hereditary influence can be assumed. Apparently there are some changes in some of the nerve cells of the respective children and adolescents, which in a certain age segment temporarily lead to increased excitability and thus to the possibility of seizures.
Rolando epilepsies usually begin between the ages of three and 13, with a peak between four and eight years. The first attacks are more often triggered by febrile infections.
Almost half of the children have other seizures or epilepsies in the family. In about a third of the siblings, the activity described above can be detected in the EEG above the typical areas, even if they do not have any seizures. This suggests that hereditary factors play a role in Rolando epilepsies, although no further details are known.
therapy
Treatment for rolandic epilepsy is only necessary if seizures are frequent or severe. Sultiam is used as the drug of first choice in German-speaking countries , but other anticonvulsants such as clobazam or valproic acid are also effective. Although carbamazepine is also widely used, there have been reports of worsening of the EEG and the seizure situation with carbamazepine.
forecast
Rolando epilepsy has a good prognosis. The seizures usually go away during puberty .
History
Martin Ruland the Elder is considered to be the first to describe the clinical symptoms of Rolando epilepsy (1597). Despite the similarity of names, the clinical picture is not according to Ruland d. Ä., But named after the Italian anatomist Luigi Rolando (1773–1831). Rolando gave its name to the central furrow , also known as the Rolando furrow or fissure (out of date). The brain tissue around the central furrow is considered to be the place of origin of the electrical discharges in the brain in Rolando epilepsy.
The comprehensive understanding of clinical symptoms, EEG appearances and prognosis developed from the 1950s and led to the definition of the syndrome in the late 1960s.
Individual evidence
- ^ S. Shinnar, C. O'Dell, AT Berg: Distribution of epilepsy syndromes in a cohort of children prospectively monitored from the time of their first unprovoked seizure. In: Epilepsia , 1999, 40, pp. 1378-1383. PMID 10528932
- ^ G. Wohlrab: Epilepsy treatment in children and adolescents: Continuity and change. In: Epileptologie , 2003, 20, pp. 25-30.
- ↑ M. Holtkamp: Prokonvulsive Effects of Anticonvulsant Substances. In: Akt Neurol. , 2002, 29, pp. 6-7.
- ↑ AC van Huffelen: A tribute to Martinus Rulandus. A 16th-century description of benign focal epilepsy of childhood. Arch Neurol . 1989; 46, pp. 445-447. PMID 2495786
- ↑ Martin Ruland: Curationum empiricarum et historicarum in certis locis & notis hominibus optime riteque probatarum & expertarum, centuria nona. Volume 9. Henricpetri, Basel 1597.
- ↑ CT Lombroso: Sylvian seizures and midtemporal spike foci in children. In: Arch Neurol. , 1967, 17, pp. 52-59. PMID 6026172
