Pseudo -Lennox syndrome is a special type of epilepsy that is relatively rare. The term is derived from the so-called Lennox-Gastaut syndrome, which is similar to the pseudo-Lennox syndrome to a certain extent in terms of the severity of the seizures. In the majority of cases, the disease begins between the ages of 2 and 7 years.
What is Pseudo-Lennox Syndrome?
Basically, the pseudo-Lennox syndrome occurs in numerous cases in childhood. The disease primarily affects those people who already have certain disorders of general development. Sometimes these are only partial functional disorders, which, however, become more pronounced in the course of the pseudo-Lennox syndrome. See whicheverhealth for CD Meanings.
In addition, it is possible that the sick children will continue to develop normally. In addition, the pseudo-Lennox syndrome sometimes appears in patients who suffer from special brain damage.
Current medical research has not yet found a conclusive answer to the question of the causes of pseudo-Lennox syndrome. Instead, the reasons for the development of the condition tend to be idiopathic. An exact cause can therefore not be identified.
In certain cases, there is a connection between the occurrence of pseudo-Lennox syndrome and certain types of brain damage. It is sometimes assumed that genetic causes are also involved in the development of the disease. In addition, medical observations assume that the epileptic seizures that occur in the context of pseudo-Lennox syndrome are related to the maturation of the brain during child development.
With regard to the causes, there is a clear difference between the Lennox-Gastaut syndrome on the one hand and the pseudo-Lennox syndrome on the other. Because in the case of the former, the reasons for the development of the disease have been largely clarified. In addition, it is possible to identify the reasons for the emergence within the framework of diagnostic examination procedures.
In addition, there are no exclusively tonic seizures associated with pseudo-Lennox syndrome. However, it should be emphasized that the individual syndromes cannot always be clearly differentiated, as they may merge into one another. This also applies to the so-called Landau-Kleffner syndrome.
Symptoms, Ailments & Signs
In principle, the characteristic symptoms of pseudo-Lennox syndrome are relatively varied. Above all, various types of epileptic seizures that occur as part of the disease should be mentioned. First and foremost are so-called tonic seizures.
During such a seizure, the muscles in the patient’s body become very stiff. The main reason for this is cramps in the muscles of the skeleton. The length of tonic seizures varies and is usually between a few seconds and a few minutes.
It is also crucial that the affected person stops breathing for a short time during a tonic seizure. As a result, the facial area and the skin on the lips may turn bluish. The eyes often turn upwards and the pupils dilate.
Many patients become unconscious due to the lack of breathing. Because of this, the risk of accidents increases. Head flexion during the tonic seizure is possible. If people clench their jaw too much, they may bite their own tongue. In general, however, tonic seizures occur together with other types of seizures.
In principle, the pseudo-Lennox syndrome is characterized by numerous different forms of epileptic seizures. In addition to the tonic seizures, the so-called Rolando seizures should be mentioned in particular. They occur comparatively often in children. It is also possible that the pseudo-Lennox syndrome arises from normal rolandic epilepsy.
Diagnosis & course of disease
The diagnosis of pseudo-Lennox syndrome is primarily based on the typical changes in the EEG. This leads to strong deviations of a multifocal nature. This phenomenon occurs primarily during the sleep phases. In addition, the pseudo-Lennox syndrome is often accompanied by ESES.
This is a special bioelectrical state that primarily manifests itself during sleep. As a result, significant disruptions in development are possible. Even permanent mental disabilities occur in some cases.
Due to the pseudo-Lennox syndrome, patients suffer from epilepsy and thus from very strong and, above all, painful cramps in the muscles. Usually, an epileptic seizure can also be fatal if it lasts longer or if the sufferer otherwise injures himself during the seizure. Due to the pseudo-Lennox syndrome, the affected person is also supplied with less oxygen, so that the lips sometimes turn blue.
This can also lead to irreversible damage to the brain and internal organs. The patient may then be mentally handicapped. If the convulsion persists, the person affected usually loses consciousness, which can lead to a fall and various injuries. The quality of life is significantly reduced by the pseudo-Lennox syndrome.
An epileptic seizure can quickly lead to death, especially in children. It is not uncommon for patients and their families to need psychological support. Treatment of pseudo-Lennox syndrome is carried out with the help of drugs. Usually there are no complications. However, there is no guarantee that seizures will be completely reduced.
When should you go to the doctor?
Repeated epileptic seizures should be clarified immediately by a doctor. Pseudo-Lennox syndrome can manifest itself in a number of seizure forms, some of which are associated with considerable physical discomfort. Both tonic seizures and myoclonic or atonic-astatic seizures must be evaluated by a doctor. Pseudo-Lennox syndrome is treated by a neurologist. Those affected must be treated in a specialist clinic. With the ESED seizure type, you have to go to a sleep laboratory, where the origin of the suffering is determined using an EEG measurement.
Since a causal therapy is not possible, the countermeasures are limited to a well-coordinated medication and preventive steps. If an epileptic seizure occurs, the emergency services must be called in immediately, especially if status epilepticus occurs, in which the seizure is much longer and more intense. Since this can lead to respiratory arrest or heart failure, first aid measures must be initiated immediately. The patient must then be treated in a specialist clinic and may require physiotherapeutic help.
Treatment & Therapy
It is imperative to treat pseudo-Lennox syndrome appropriately. The therapy of the ESES is of great importance, since otherwise developmental disorders may occur. In principle, however, therapeutic measures are difficult.
For this reason, around 50 percent of sick patients suffer from clearly retarded mental development after late puberty. In addition, anticonvulsants are prescribed to treat pseudo-Lennox syndrome with medication. These are drugs used to treat epileptic seizures.
Patients often receive such drugs as part of a combination therapy in order to increase their effectiveness. Possible drugs here are, for example, sultiam, lamotrigine or various corticosteroids.
Since the exact causes of the development of the pseudo-Lennox syndrome have not yet been fully clarified, no statements can be made about possible ways of preventing the disease. Instead, a doctor should be consulted at the first sign of pseudo-Lennox syndrome.
Pseudo-Lennox syndrome can be preceded by existing brain damage. However, some of the patients do not have such characteristics and were healthy before the onset of the disease. Aftercare is necessary to avoid consequential damage and to enable the person affected to lead a largely normal life. Follow-up care also applies to family members.
They need to be informed about how to appropriately care for the patient in the event of a seizure. In severe cases, the emergency services must be called immediately. Mindfulness and sensitivity in dealing with the syndrome are crucial for follow-up care.
The therapy is done by administering medication. As part of the follow-up care, the frequency and intensity of the epileptic seizures are recorded. These factors provide information on the healing process. The clinical picture is not uniform, there are different manifestations. If the course is mild, the prognosis for healing is favorable.
Follow-up care no longer makes sense if the patient is completely free of symptoms and has no consequential damage. In severe cases, seizures leave permanent damage. Follow-up care accompanies the patient into adulthood. In the case of pronounced cognitive impairments, the affected person is often unable to work and needs individual support in everyday life. It is carried out by relatives, the treating neurologist and in appropriate support facilities.
You can do that yourself
Good compliance is important with this disease: the parents of the young patients must ensure that the medication is taken regularly in accordance with the doctor’s instructions. This is to avoid seizures or at least reduce their number.
As long as the patients are still small, they should not be left alone. Any seizure can be life-threatening, and an attendant can usually prevent more serious accidents. Those affected or their relatives should also ensure that their living environment is safe. For example, the apartment should be barrier-free and not have any tripping hazards such as loose carpets, smooth floors or unsecured cables. Patients with pseudo-Lennox syndrome also benefit from a house emergency call, which can be used to get help quickly in the event of a seizure.