Epilepsy syndromes are actually different types of epilepsy, and there are often significant differences between them. They concern, inter alia, the moment at which the onset of the disease occurs, but also the nature of seizures occurring in a given epilepsy syndrome and accompanying other ailments. It should also be emphasized that the determination of the epilepsy syndrome a given patient suffers from is needed to determine his prognosis, but also to plan an appropriate treatment for his epilepsy.

Epilepsy syndromesare distinguished by the causes that led to them and the age at which the patient developed the disease. It should be remembered that epilepsy is a very heterogeneous disease. This disease can appear in people of any age, in addition, the symptoms of two people with epilepsy may be completely different. Epilepsy, however, is not only about seizures - epileptic seizures can also take the form of e.g. loss of consciousness or trembling in some parts of the body.

In addition to epileptic seizures, epilepsy may also be related to problems such as intellectual delay. The causes of epilepsy may differ from one type of epilepsy to another, as well as the age at which the patient developed the disease. Taking into account the nature of epileptic seizures, the age at which the first symptoms of the disease appeared, EEG abnormalities and other problems (apart from epilepsy), it is possible to distinguish between different epilepsy syndromes. Selected epilepsy syndromes are described below, but there are definitely more of them, even several dozen.

Epilepsy syndromes: nocturnal frontal epilepsy

Nocturnal frontal epilepsy typically begins in childhood. The name of the unit comes from the fact that the seizures associated with it occur during sleep, so they may be confused with the patient's experiencing nightmares. In the course of nocturnal frontal epilepsy, the patient experiences motor seizures, such as clenching the hands or bending the knees. In addition to movement disorders, there may also be crying, screaming or groaning. Nocturnal frontal epilepsy is a genetic disorder that is inherited in an autosomal dominant fashion.

Epilepsy syndromes: epilepsyRolanda

Rolandic epilepsy is one of the milder epilepsy syndromes. It typically begins in children between the ages of 3 and 13. In Roland's epilepsy, epileptic seizures are focal, they appear most often at night and usually affect the facial muscles, so patients may experience, for example, drooling. These seizures are usually mild in nature, but there is, unfortunately, the risk that they will become generalized in focal lengths. Most often, however, Roland's epilepsy is mild, and seizures may even tend to subside as the child enters puberty. In addition, Rolandic epilepsy does not always require treatment - sometimes the frequency of its seizures is so rare and they are so low that it is possible to at least postpone the therapy for some time.

Epilepsy syndromes: childhood absences

Another epileptic syndrome that occurs in the youngest patients is childhood absences. Disease typically begins between the ages of 4 and 12. In this syndrome, epileptic seizures consist of recurrent and sudden attacks of unconsciousness lasting several dozen seconds, which may be accompanied, for example, by chewing movements or eye blinking. Childhood absence seizures are an epileptic syndrome with a good prognosis, because the frequency of seizures usually decreases significantly as the child gets older.

Epilepsy - see how to recognize it

Dravet syndrome (severe infantile myoclonic epilepsy)

Dravet syndrome belongs to the group of drug-resistant epilepsy. The disease starts early, as the first symptoms of Dravet syndrome usually occur within the first year of a child's life. The first seizure in this syndrome (usually in the form of a generalized tonic-clonic seizure) occurs in most cases when the child has a fever. Myoclonic epilepsy in infants is severe, incl. due to the fact that the seizures occurring in it tend to be prolonged and become epileptic. As a child grows up, the condition becomes more severe: other types of seizures develop over time, such as absences, focal seizures, and myoclonic seizures. There are numerous other problems associated with Dravet syndrome, besides epileptic seizures, such as:

  • sensory integration disorders
  • increased infection rate
  • Autistic Behavior Spectrum Disorder
  • sleep disturbance
  • growth and nutritional disorders

Epilepsy syndromes: West syndrome

West syndrome develops most often in children aged 3 to 12 months and is associated with three problems: epileptic seizures, intellectual retardation, and a characteristic EEG abnormality called hypsarrhythmia. The disease is classified as quite severe epilepsy due to the fact that it is associated with the tendency to the appearance of epileptic seizures not only in childhood, but also later in life. Another problem is that a patient with West syndrome may transform into another epilepsy syndrome, which is Lennox-Gastaut syndrome.

Epilepsy syndromes: Lennox-Gastaut syndrome

Typically Lennox-Gastaut syndrome begins in children between the ages of two and six. Children may experience many different types of seizures during the course of the disease, usually including tonic and myoclonic seizures. Unfortunately, seizures are not the only problem in the course of this unit - children with Lennox-Gastaut syndrome often suffer from intellectual retardation. The prognosis for Lennox-Gastaut syndrome is rather poor, as the condition is often not responding to treatment with antiepileptic agents.

Epilepsy syndromes: juvenile myoclonic epilepsy

Juvenile myoclonic epilepsy typically develops in the age range of 8 to 20 years. As the name suggests, myoclonic seizures are most typical and typically appear shortly after waking up. Other seizures in juvenile myoclonic epilepsy are absence seizures and tonic-clonic seizures. The condition is associated with a lifelong seizure tendency, but is generally a form of epilepsy in which there is a good chance that pharmacotherapy will prevent symptoms from developing.

Epilepsy syndromes: Ohtahar syndrome

One of the more severe forms of childhood epilepsy is Ohtahar syndrome. Symptoms of the disease appear very early, even in the first few days or the first weeks of a child's life. The seizures in Ohtahar syndrome most often take the form of tonic seizures. The prognosis of patients is quite unfavorable - up to half of children with this unit die before the age of one. In turn, in the remaining patients there is usually a significant intellectual delay, and often cerebral palsy coexists with Ohtahar's syndrome. Another problem with this epilepsy syndrome is that it is not currentlythere are effective methods of treating him.

Epilepsy syndromes: Rasmussen syndrome

Rasmussen's syndrome is quite a mysterious disease, currently treated as one of the autoimmune diseases. Illnesses are observed in children under 10 years of age. Rasmussen's syndrome begins with epileptic seizures, which are usually complex focal seizures, but some patients experience tonic-clonic seizures first. Other abnormalities in children with this condition that appear at different times from the first epileptic seizure are hemiplegia and the ability to detect encephalitis on imaging tests, and cognitive impairment.

Epilepsy syndromes: Panayiotopoulos syndrome

Another form of childhood epilepsy is Panayiotopoulos syndrome. Children between 3 and 6 years of age most often suffer from this unit. In the course of the syndrome, the emerging disorders take the form of autonomic partial seizures, including a quickening of the heartbeat or a sudden paleness. Panayiotopoulos seizures most often occur during sleep. This syndrome is classified as epileptic syndromes with a good prognosis: up to 25% of all patients with this unit experience only one seizure. In other patients, the disease usually resolves with age, usually before the patient is 13 years old.

Epilepsy syndromes: progressive myoclonic epilepsy

Progressive myoclonic epilepsy is not really one epileptic syndrome, but a group of several different diseases. Their characteristic features are myoclonic seizures and accompanying dementia of various kinds. The group of progressive myoclonic epilepsy includes:

  • MERRF team
  • Unverricht-Lundborg disease
  • Lafor's disease

Category:

Neurology