Pangiotopoulos syndrome: symptoms, diagnosis, dangers, treatment and prevention

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Pangiotopoulos syndrome: symptoms, diagnosis, dangers, treatment and prevention
Pangiotopoulos syndrome: symptoms, diagnosis, dangers, treatment and prevention

Video: Pangiotopoulos syndrome: symptoms, diagnosis, dangers, treatment and prevention

Video: Pangiotopoulos syndrome: symptoms, diagnosis, dangers, treatment and prevention
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Idiopathic childhood epilepsy, which is characterized by vegetative epileptic seizures with impaired consciousness and gaze deviation, is called Panayotopoulos syndrome. This deviation has a favorable outcome, is treatable, but causes considerable discomfort to the patient and those around him. Each attack can occur with varying degrees of severity, you can never predict. To obtain a positive result from therapy, it is recommended to undergo an examination based on EEG.

Treatment includes relief of autonomic epileptic seizures. Be sure to pay attention to preventive measures. It is not recommended to start therapy without first consulting a specialist, as this can lead to negative consequences.

panyotopoulos syndrome in a child
panyotopoulos syndrome in a child

What is this?

In a child, Panagiotopoulos syndrome can be diagnosed between the ages of one and fifteenyears. Parents should carefully monitor the behavior of the baby, so as not to miss the disease and not provoke complications. This occipital epilepsy belongs to a benign idiopathic type and was first described in 1950 by the scientist Panayotopoulos, after whom the disease is named. He has been conducting various research and observations for a long time, which eventually helped to make such an important discovery.

The occipital localization is indicated by the fact that during the paroxysm the patient's gaze is directed to the sides. However, this symptomatology is not observed in every patient. Also, during an EEG examination, a specialist cannot always note epileptic activity in the occipital region, which can complicate the diagnosis.

Panayotopoulos syndrome is accompanied by a variety of symptoms, and there is also a pronounced vegetative character, and because of this, it is almost impossible to make a differential diagnosis. The disease itself does not apply to common pathologies in this age group. But it is most common in children between the ages of three and six.

Views

In modern medicine, there are three types of epilepsy depending on the provoking factors:

  • genetic;
  • structural;
  • metabolic.

To determine exactly what disorder led to the attack, the patient is advised to undergo a thorough examination. You should never engage in self-diagnosis and, especially, treatment, as this can provoke serious and irreversibleconsequences.

In most cases, it is not always possible to determine the form of the violation. It happens that the attack is not associated with epilepsy. For the correct diagnosis, that is, the type of disease, consultation of such highly specialized specialists as an epileptologist, neurologist and radiologist is required. An epileptic seizure occurs due to acute brain damage, impaired blood flow in this organ, hemorrhage, benign and malignant neoplasms, impaired metabolic processes, and also due to prolonged and uncontrolled intake of various drugs. Therefore, before using the drug, you need to consult a doctor and familiarize yourself with the contraindications. Seizures are common in diabetic patients.

Also, experts distinguish pseudoepileptic or psychogenic non-epileptic disorders, which can only be determined in a clinical setting. There are cases when a child has two types of seizures at once.

panyotopoulos syndrome in a child symptoms
panyotopoulos syndrome in a child symptoms

Aura and seizures

Pangiotopoulos syndrome, like other types of epilepsy, is a clinical manifestation of electrical discharges in large quantities, which can attract various parts of the brain. In most cases, before the onset of an attack, the patient has an aura. If everything comes from the back of the brain, then the patient begins to notice colored figures and circles before the eyes. If the focus is located deep in the temporal lobe, then an unpleasant sensation, discomfort occurs in the abdomen, whichgradually rises to the throat.

Seizures in patients with Panagiotopoulos syndrome can be either convulsive or non-convulsive. In this case, the patient can simply freeze in place for a while and not move, and then continue to do their job. And there are situations when a person just stops and starts rubbing his hands. All this comes from the temporal lobe of the brain.

Patients often report some kind of pleasure after an attack. As a result, many refuse treatment because of this. Experts also note such types of seizures as reflex photosensitivity, arising from the frequent flickering of light. Therefore, children are often not allowed to watch various films or cartoons with a high content of special effects.

Absences are also noted in a child with Panagiotopoulos syndrome. This phenomenon is characterized by fading, accompanied by a short blackout of consciousness, while there are no convulsions. In most cases, they are diagnosed in children over the age of four, but may begin earlier if the patient has any other genetic diseases. Their nature can be determined with such examinations as a genetic test, lumbar puncture. Based on the results obtained, treatment is prescribed, which may be based on a ketogenic diet.

Pangiotopoulos syndrome: causes of occurrence

The pioneer scientist believes that children should have a predisposition to epilepsy in the form of a benign epileptiform EEG pattern, which can be only in one child out of 100. This disease is considered hereditary, and the patient himself is predisposed to this deviation.

panyotopoulos syndrome causes
panyotopoulos syndrome causes

Very often, experts associate the causes of Panagiotopoulos syndrome with the period of immaturity of the cerebral cortex, its diffuse increased excitability and excessive epileptic sensitivity of autonomic structures. But so far, medicine has not exactly established provoking pathogenetic mechanisms and triggers. Sometimes seizures, which are confused with epilepsy, can be diagnosed after brain injuries, regardless of the age category, so a neurologist's consultation and an EEG examination are required.

Symptoms of Panagiotopoulos syndrome in a child

A small patient cannot always complain or accurately describe his condition, so parents need to carefully monitor their child with this deviation, even if he is already 10 or 15 years old. This disorder, as already mentioned earlier, is accompanied by epileptic seizures, which can happen at any time and place. Before onset, symptoms such as:

  • not feeling well;
  • nausea;
  • look away, that is, eye deviation (may be short-term, permanent or lasting several hours), sometimes combined with a turn of the head;
  • increased arousal;
  • feeling fear;
  • cephalgia;
  • hyperhidrosis.

In Panagiotopoulos syndrome, vomiting is observed in 25% of patients, which can be one- orrepeated, significantly worsening overall well-being, accompanied by weakness and provokes dehydration. In a child, the skin during an attack is pale, sometimes redden or cyanosis is observed. Loss of consciousness may be accompanied by miosis. There is marked mydriasis. There are cases when the patient's pupils do not react at all to light.

occipital epilepsy
occipital epilepsy

The symptoms of Panayotopoulos syndrome in a child also include tachycardia, impaired breathing, enuresis, encopresis, disorders of the gastrointestinal tract, fever before and after an attack. Less commonly, a patient is diagnosed with profuse salivation, diarrhea. In most children, autonomic paroxysm is accompanied by disorders associated with consciousness. The child is lost in space and time, then fainting follows. The situation worsens gradually, along with an increase in the severity of autonomic symptoms.

Idiopathic occipital epilepsy can also have an atypical form, which is expressed in sudden sleep or falling into a stupor without convulsions, behavior is disturbed, autonomic disorders are noted, but without vomiting and cephalgia.

An epileptic seizure in 25% of children ends with convulsions in only one half of the body. In rare cases, the child has hallucinations, vision becomes worse, and transient blindness may even be diagnosed. Then the patient wants to sleep, and after waking up feels completely he althy.

Epistatus in Panagiotopoulos syndrome - a vegetative epileptic seizure,the duration of which is from half an hour to seven hours. The same baby can be diagnosed with various kinds of disorders at any time of the day.

How often does a child have seizures with Panagiotopoulos syndrome? The frequency is insignificant, for the entire period from five to ten. Between attacks, the neurological status of the patient is without any pronounced features. In development, the child does not lag behind his peers.

What is not epilepsy? Do I need a psychiatric consultation?

Very often this disease is confused with other disorders. In this case, it is better to contact a specialist for qualified help and not engage in self-therapy in order to avoid negative consequences. Epilepsy should not be confused with pathologies such as:

  1. Sleepwalking. Does not apply to this type of disorder, but if there are other pronounced symptoms, then it is worth undergoing an additional examination and clarifying the diagnosis again.
  2. Cephalgia. It does not apply to the symptoms of occipital epilepsy only if the headache is not diagnosed after an attack with fainting.
  3. Tiki. Outwardly different and in no way related to this disease.
  4. Incontinence at night. Not considered a warning sign of pathology.
idiopathic occipital epilepsy
idiopathic occipital epilepsy

With childhood occipital epilepsy, it is not always worth seeking help from psychiatrists. If necessary, a neurologist can refer to this narrow-profile specialist. Consultation is also required if the child's mental function has deteriorated inperiod of epilepsy and, as a result, psychoactive drug therapy was prescribed.

If a small patient has been diagnosed with occipital epilepsy, there is no need to treat it with acupuncture or other non-traditional methods, this will not give any result.

Parents' actions

If a child often twitches at night, his saliva flows, and he walks during sleep, then this is a reason to be wary and seek help. This condition can be observed for no more than one minute, after which the little patient will not remember anything and will continue to sleep on. Some parents do not consider this an attack, but in vain, a thorough examination, an accurate diagnosis, and high-quality therapy are required. A slight twitching of the corners of the mouth, which can spread throughout the body, is also considered a warning sign.

Experts recommend filming nighttime seizures so that later, by appearance, you can determine if a child has occipital epilepsy or not. It happens that the patient wakes up at night and looks at one point for a long period, then vomiting just begins. During the day, the child is no different from his peers, he still moves and communicates. If the attack was the only one, then benign occipital epilepsy is not treated at all. In case of developmental delay or focal symptoms, impaired mobility, loss of skills, neuroimaging, magnetic resonance imaging and complex genetic techniques are necessary.

Diagnosis

Diagnosis should be started at the first sign of a deviation. Viewresearch is appointed exclusively by a specialist after a preliminary examination of the patient and a conversation with his parents. If children have occipital epilepsy at an early age, then a 2-4 hour VEEG monitoring is performed, since the baby has long periods of physiological sleep. For patients of the older age group, this type of examination is recommended only at night.

panyotopoulos syndrome symptoms
panyotopoulos syndrome symptoms

Whatever epilepsy is diagnosed in a child, in addition to this technique, it is recommended to use neuroimaging, which will allow to detect the anatomical substrate of epilepsy. Also, patients with such a serious deviation are prescribed magnetic resonance imaging of the brain, and if calcifications have been detected, then CT is additionally performed. These examinations are serious and have a number of contraindications that should be taken into account without fail. They should not be taken without prior consultation with a specialist, as this can lead to negative and irreversible consequences.

Very often, even the most experienced doctors confuse the symptoms of Panagiotopoulos syndrome with other diseases, such as:

  • acute respiratory viral infection (ARVI);
  • meningitis is an inflammatory process that occurs in the soft membranes of the brain and spinal cord, provoked by various pathogens (bacteria, viruses);
  • intestinal infection;
  • acute type poisoning;
  • liquor-hypertensive crisis;
  • an attack of cephalalgia ormigraines.

Epileptic syndrome cannot be established by a single attack, even if EEG changes are noted. If necessary, the child is registered with a neurologist for a certain period in order to exclude this unpleasant diagnosis. It is worth noting that epileptiform activity may be present on an EEG study during the period of clinical remission, but then disappears closer to adolescence.

Lumbar puncture is recommended for patients only if it is necessary to differentiate this syndrome from an organic lesion, such as a cyst, hematoma, neoplasm in the brain, or a neuroinfection (encephalitis, abscess). It is also necessary to take into account the fact that there will be no disturbances in the cerebral fluid.

Treatment. Preventive measures

Therapy, as mentioned earlier, will depend on the results of the examination and is prescribed exclusively by a specialist. You should not engage in self-diagnosis and, moreover, prescribing treatment, as this can lead to negative consequences, especially in this situation. If you seek qualified help in a timely manner, the result will be positive.

The treatment of epilepsy will depend on its form, the more neglected the situation, the more time and effort will be required to eliminate it. The main method of therapy for this disease is considered to be medication, which will improve the condition in many patients of different age categories. The best option is treatment with one type of drug, if this type wasineffective, then experts prescribe several antiepileptic drugs. It must be remembered that they are not recommended to be taken without a doctor's prescription. Before you start using the drug, carefully read the instructions, pay attention to contraindications and side effects.

The course depends on the general condition of the patient and the neglect of the disease. The arsenal of drugs for children with epilepsy is the same as for adults. In modern practice, there are cases when a child's Panayotopoulos syndrome was cured with the help of Sultiam, which belongs to a specific group of drugs. Also, doctors can prescribe "Carbamazeline", "Oxarbazeline". If the pathology variants are resistant atypical, then the use of Clobazam, Levetiracetam, Valproate is possible. The duration of the course should not exceed two years.

pangiotopoulos syndrome vomiting
pangiotopoulos syndrome vomiting

If an attack in a child with Panagiotopoulos syndrome with organic brain damage lasts from 20 minutes to half an hour, emergency treatment is required. Vegetative status epilepticus can be stopped by rectal or intravenous administration of Phenozepam, Clonazepam, Diazepam.

In addition to drug therapy, a small patient may be prescribed hormonal or immunoglobulin therapy. If the treatment does not give a positive result, then it is possible to use neurosurgery. This method of treatment involves the excision of abnormal areas of the brain or theirisolation. For patients in the younger age group, experts recommend a ketogenic diet.

What is special about this diet?

This approach to nutrition is recommended not only for children with epilepsy, but also for oncological diseases and diabetes. This diet contains not only positive aspects, but also negative ones, which should be taken into account. Without fail, there must be a competent approach and a properly composed balanced menu.

The main feature of the ketogenic diet is the exclusion of carbohydrates from the diet, the use of a significant amount of fats and proteins. Gradually, the level of sugar and insulin in the blood decreases, the liver begins to produce ketone bodies, which affect the oxidation of fatty acids. When the body needs carbohydrates, it begins to burn the layer, and protein helps to reduce appetite.

Dietitians distinguish three types of ketogenic diet:

  1. Standard. Means 5% carbohydrates, 20% protein and 75% fat in the diet.
  2. Target. Before training, the patient is allowed to eat a small amount of fast carbohydrates.
  3. Cyclic. It is allowed to consume carbohydrates several days a week.

Patients are allowed to eat foods such as red meat, chicken, fatty fish and cheese, homemade eggs, cream, butter, avocados, tomatoes, peppers, nuts, olive oil. Unsweetened fruits, dark bitter chocolate, coffee, tea are recommended in small quantities. From the diet you need to completely exclude legumes, sweetgroceries, potatoes, carrots, mayonnaise.

This diet is not always suitable for everyone, so you should first consult with a nutritionist, pediatrician and neurologist.

In this syndrome, due to rare attacks and short duration of pathology, it is recommended to carry out preventive antiepileptic measures. These include:

  • proper and balanced nutrition;
  • consultation with a neurologist;
  • timely treatment and examination;
  • frequent walks in the fresh air;
  • moderate physical and mental activity;
  • quality sleep;
  • exclusion of irritants and stressful situations.

In any situation, at the first warning signs, you should contact a specialist. Even if you heard that Panayotopoulos syndrome in a child was cured by micromovements, this does not mean that you should immediately start experimenting on your child, because each patient requires an individual approach.

Forecast

As for the prognosis, it can be different, it all depends on the nature of the disease. When epilepsy is combined with other progressive serious pathologies, the result will be disappointing, the prognosis is poor, and the therapy is useless. With this syndrome, no matter how dramatic the situation may seem, treatment may not be needed. This is decided only by a specialist after the examination.

In modern practice, there are such cases when even patients of an early age category are injected with hormones, although this is not always appropriate and has a positive result. To exclude negative consequences, you can consult with several doctors and come to a general conclusion, no one ever advises starting treatment, it is better to choose several therapy options and find the right one for your child.

Very often, young patients are diagnosed with benign syndromes that are similar to, but not epileptic seizures. In this case, the child will develop normally. If the pathology is provoked by damage to the brain by a neoplasm, stroke, infectious disease or trauma, then it is recommended to remove the structural anomaly and the seizures will go away on their own and will never disturb again.

You always need to weigh the pros and cons before starting therapy, so as not to make the child a cripple for the rest of his life. It must be understood that epilepsy is a disease that can be treated, even in the presence of structural changes in the brain. The main thing is to find the right technique. At the same time, there is a possibility that the child may lag behind in development due to these disorders. In rare situations, you need to resort to serious actions, such as surgery, it is better to give preference to drug therapy, a ketogenic diet.

Epilepsy and all its forms are a serious disorder that causes considerable discomfort to patients and those around them, but with the right approach, you can find a way out and stabilize the situation.

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