Pseudo-Lennox syndrome is a special type of epilepsy that is comparatively rare. The term is derived from the so-called Lennox-Gastaut syndrome, which pseudo-Lennox syndrome resembles to some extent in terms of the severity of the seizures. In the majority of cases, the disorder takes onset between the ages of 2 and 7 years.
What is pseudo-Lennox syndrome?
Basically, pseudo-Lennox syndrome appears in numerous cases already in childhood. In this context, the disease primarily affects those individuals who have previously exhibited certain disturbances in general development. Sometimes these are only partial disturbances of functions, which, however, become more pronounced in the course of the pseudo-Lennox syndrome. In addition, it is possible that the affected children develop normally. In addition, in some cases, pseudo-Lennox syndrome is seen in patients suffering from specific brain damage.
Causes
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 disorder tend to be idiopathic. Thus, a precise cause cannot be identified. In certain cases, there is a connection between the occurrence of pseudo-Lennox syndrome and certain damage to the brain. Sometimes it is suspected that genetic causes are also involved in the development of the disease. In addition, medical observations suggest that the epileptic seizures that occur in pseudo-Lennox syndrome are related to the maturation of the brain during child development. In terms of causes, a clear difference is found between Lennox-Gastaut syndrome on the one hand and pseudo-Lennox syndrome on the other. This is because in the case of the former, the reasons for the development of the disease have been clarified for the most part. Moreover, it is possible to identify the reasons for the development within the framework of diagnostic examination procedures. Moreover, seizures that are exclusively tonic do not occur in association with pseudo-Lennox syndrome. It should be emphasized, however, that the individual syndromes cannot be clearly delineated in every case, as they may merge into one another. This also applies to the so-called Landau-Kleffner syndrome.
Symptoms, complaints, and signs
Basically, the characteristic symptoms of pseudo-Lennox syndrome are relatively varied. First and foremost are various types of epileptic seizures that occur in the course of the disease. In the first place are so-called tonic seizures. During such a seizure, the muscles in the patient’s organism stiffen strongly. This is mainly caused by cramps in the muscles of the skeleton. The length of tonic seizures varies and usually ranges from a few seconds to several minutes. Another decisive factor is that the affected person’s breathing stops 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 upward and there is dilation of the pupils. Due to the intermittent breathing, many patients become unconscious. For this reason, the risk of accidents increases. Flexion of the head during the tonic seizure is possible. If the affected persons tense the jaw too much, they bite their own tongue in some cases. Basically, however, tonic seizures occur together with other types of seizures. In principle, pseudo-Lennox syndrome is characterized by numerous different forms of epileptic seizures. In addition to tonic seizures, the so-called Rolando seizures should be mentioned in particular. They occur comparatively often in infantile patients. It is also possible that pseudo-Lennox syndrome arises from normal Rolando epilepsy.
Diagnosis and course of the disease
The diagnosis of pseudo-Lennox syndrome is made primarily on the basis of the typical change in the EEG. This involves severe deviations of a multifocal nature. This phenomenon occurs mainly during sleep phases. In addition, pseudo-Lennox syndrome is often accompanied by ESES.This is a special bioelectric condition that is primarily manifested during sleep. As a result, significant disturbances in development are possible. Even permanent mental disabilities occur in some cases.
Complications
Due to pseudo-Lennox syndrome, patients suffer from epilepsy and thus very severe and especially painful convulsions in the muscles. Usually, an epileptic seizure can also be fatal if it is prolonged or if the affected person is otherwise injured during the seizure. Due to pseudo-Lennox syndrome, the affected person is also supplied with less oxygen, so that the lips sometimes turn blue. This can also cause irreversible damage to the brain and internal organs. It is possible that the patient is mentally disabled afterwards. In the case of a persistent convulsion, the affected person usually loses consciousness, so that a fall and various injuries may also occur in the process. The quality of life is considerably reduced by the pseudo-Lennox syndrome. Especially in children, an epileptic seizure can quickly lead to death. It is not uncommon for the patients and their relatives to require psychological support. Pseudo-Lennox syndrome is treated with the help of medication. As a rule, there are no complications. However, there is no guarantee that seizures will be completely limited.
When should you see a doctor?
Repeated epileptic seizures should be promptly evaluated by a doctor. Pseudo-Lennox syndrome can be manifested by a number of seizure types, some of which are associated with significant physical discomfort. Tonic seizures as well as myoclonic or atonic-astatic seizures must be clarified by a physician. Pseudo-Lennox syndrome is treated by a neurologist. Affected individuals must be treated in a specialized clinic. In the case of the seizure form ESES, one must visit a sleep laboratory where the origin of the condition is determined by means of an EEG measurement. Since causal therapy is not possible, countermeasures are limited to well-tailored medication and preventive steps. If an epileptic seizure occurs, the emergency medical 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 cardiac failure, first aid measures must be initiated immediately. The patient must then seek treatment at a specialized clinic and may require physiotherapeutic assistance.
Treatment and therapy
It is imperative to treat pseudo-Lennox syndrome appropriately. In this context, the therapy of ESES is of great importance, because otherwise developmental disorders may occur. In principle, however, measures for therapy are difficult. For this reason, about 50 percent of affected patients suffer from significantly retarded mental development from late puberty onwards. In addition, anticonvulsants are prescribed to treat pseudo-Lennox syndrome with medication. These are drugs that are used to treat epileptic seizures. Patients often receive such drugs as part of a combination therapy to increase efficacy. Possible drugs here include sultiam, lamotrigine, or various corticosteroids.
Prevention
Because the exact causes for the development of pseudo-Lennox syndrome have not yet been fully elucidated, no statements can be made about possible ways to prevent the disease. Instead, a physician should be consulted at the first signs of pseudo-Lennox syndrome.
Follow-up
Pseudo-Lennox syndrome may be preceded by preexisting damage to the brain. However, some patients do not have such features and were healthy before the onset of the disease. Follow-up care is necessary to prevent secondary damage and to allow the affected person to lead a largely normal life. Follow-up care also relates to the relatives. They must be informed about how to care for the patient appropriately during an attack. In the event of a severe episode, the emergency medical services must be called immediately. Attentiveness and sensitivity in dealing with the syndrome are crucial for aftercare. Therapy is by means of medication administration. The frequency and intensity of epileptic seizures are recorded during follow-up.These factors provide information about the healing process. The course of the disease is not uniform, there are different manifestations. In mild cases, the prognosis for healing is favorable. Follow-up care is no longer advisable if the patient is completely symptom-free without secondary damage. In severe cases, the seizures leave permanent damage. Follow-up care accompanies the patient into adulthood. In cases of pronounced cognitive impairment, the patient is often unable to work and requires individual support in everyday life. This is provided by relatives, the treating neurologist, and in appropriate support facilities.
What you can do yourself
Good compliance is important in this disorder: parents of young patients must ensure that medication is taken regularly according to the doctor’s instructions. This should prevent seizures or at least reduce their number. While patients are still young, they should not be left alone. Any seizure can be life-threatening and an accompanying person can usually prevent worse accidents. Patients or their relatives should also make sure that their living environment is safe. For example, the home should be barrier-free and free of tripping hazards such as loose carpets, slippery floors or unsecured cables. Patients with pseudo-Lennox syndrome also benefit from a home emergency call, which can be used to summon help quickly in the event of an attack. Being dependent on constant monitoring and assistance can be very stressful for patients and their families. Here it would be advisable to seek psychotherapeutic treatment. Relaxation techniques (for example, Jacobson’s progressive muscle relaxation, tai chi, qigong or yoga) have also proven beneficial, especially for relatives. There are many places on the Internet where people can get in touch with others affected, such as the Epilepsy Network (www.epilepsie-netz.de) with its forum (www.forum.epilepsie-netz.de). Also “Rehakids, the forum for special children” has already addressed the pseudo-Lennox syndrome (www.rehakids.de).
