Among the various manifestations of infantile epilepsy dravet syndrome is a special place and is perhaps the most severe and life-threatening child diseases. This syndrome is manifested already in the first year of baby's life and often leads to serious violation of his / her psychomotor development, and in some cases – and fatal. About this rather rare disease, its main symptoms and treatments we will cover later in the article.
Dravet Syndrome is a rare thing – noted that this disease affected 1 person on 40 thousand newborns (and boys account for about 66% of cases). But this, incidentally, leads to the fact that the correct diagnosis doctors sometimes find it difficult to install and, thus, precious time is lost. And when these syndrome without maintenance therapy the child's condition usually worsens with age.
If you have a kid under the year, frequent bouts with serial or status for (i.e., they follow one another, and in the intervals the patient does not regain consciousness) and also resistant to the action of anticonvulsants, may be suspected dravet syndrome.
The Children often experience multiple seizures throughout the day, and this condition lasts for about a week. Then a couple of weeks followed by a lull, and it's happening again.
Epileptic status with dravet syndrome-a fairly common phenomenon. It may be accompanied by convulsions or non-convulsive be in the form of varying intensity disturbances of consciousness with segmental myoclonias (fast twitch muscle).
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The Main reason for the occurrence of the described syndrome researchers call a genetic predisposition, namely – the presence in the genes of the patient mutations of the sodium channel.
The Provoking factors for the development of the described condition in infants is most frequently a fever during an illness, taking a hot bath, overheating. It can also be severe fatigue or light stimulation (flashing light, the transition from dark rooms to brightly lit, etc.). It should be noted that in the following years, the patient's life will pose a threat to him, causing various strength attacks.
The main manifestations of dravet syndrome can be attributed to focal and generalized seizures. Focal seizures are different in that the area of excitation, causing their appearance is only one part of the brain. In the case of development of pathological neuronal activity in both hemispheres we are talking about generalized seizures.
Seizures in the syndrome dravet are often polymorphic in nature. The children may experience clonic (with the change of muscle tone), tonic (which is a pretty long muscle spasm) and generalized myoclonic seizures.
Often there are attacks in the form of atypical absence seizures – the conditions in which awareness is partially or completely non-responsive to the environment. Baby may at this time, numb, staring at one spot, bend backwards, suddenly to fall or simply drop what you were holding.
Most Often these seizures occur during waking, and awake (in the dream they were only in 3% of patients with this diagnosis).
As a rule, dravet syndrome is characterized by the fact that the symptoms in sequence. Physicians distinguish three main periods of the disease.
As we have said, due to the fact that the dravet syndrome-is a rare disease, experts often have difficulty in its diagnosis. So parents, it is important to provide accurate information about the development of the pathological condition of their child. Named the syndrome can be suspected, if you have the following symptoms:
In addition to the above symptoms for children with dravet syndrome usually characterized by the presence of hyperactivity and attention deficit.
The Forecast of severe myoclonic epilepsy, as a rule, unfavorable. All patients diagnosed with dravet syndrome have delayed mental development, and in half of the cases, it is hard. After four years of age, patients present progressive deterioration, with the development of behavioral abnormalities, including psychosis.
Unfortunately, a fatal outcome in this disease is also very high – up to 18%, and its causes are most likely to be accidents during seizures or epileptic status.
In order to reduce the risk of severe consequences during a seizure the child, parents should be well aware of how it turns out first aid for seizures.
If the child has convulsions in response to the temperature rise (which, as you remember, this disease is one of the main features), observe the following rules:
If the child pronounced the fever, that is, the forehead is hot, and his face reddened, first aid for seizures should be directed at lowering the temperature (cold, wet compress on the forehead, cold to the axillary cavities and groin, wiping the body with water and vinegar in proportion 1:1, antipyretic drugs).
If the kid in the background of high temperature, pale skin, bluish lips and fingernails, fever, cold feet and hands, then wiping and cold packs can not be done. Toddler to keep them warm, give antipyretics as well as tablets “no-Spa” or “Papaverine” at the rate of 1 mg per 1 kg of weight to expand the vessels.
In the case deployed an epileptic seizure with generalized clonic and tonic convulsions, the child should be:
If the seizures take the form of status, be sure to call for an ambulance.
Sick child with this disease is reduced to the slowing of seizures and prevent the development of status form.
When the diagnosis of ‘dravet syndrome” treatment excludes the use of known antiepileptic drugs: "Carbamazepine”, “Finlepsin”, “Phenytoin” and “Lamotrigine", as they only worsen the condition of the patient, aggravating the course of existing forms of attacks.
In addition to the mandatory drug therapy, it is important to remember the prevention of increase in body temperature, as this condition is especially dangerous for the patient. To avoid provoking attacks of light stimulation he offered to wear glasses with blue lenses or one taped to the glass.
When the diagnosis is confirmed start treatment starts with the application of the drug “Topiramate”. It is prescribed a dose of 12.5 mg/day, gradually increasing it up to 3-10 mg/kg/day. (taking your meds twice a day). This tool is especially effective in cases when described children's epilepsy manifested generalized seizures, and epileptic with seizures switching from one half of the body to another (hemiconvulsions).
The Following drugs for monotherapy – derivatives of valproic acid (syrup “Konvuleks”, “Convulsofin” etc.) – is particularly effective in atypical absence seizures, and myoclonus, as well as barbituric acid (“Phenobarbital”) used in generalized seizures, with a tendency to status. By the way, in this case, the marked and high efficiency of the use of bromides.
If necessary, use combination of drugs. The most effective of them is the combination of valproate with “Topiramate”.
Article in other languages:
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HI: https://tostpost.com/hi/health/14266-dravet.html
JA: https://tostpost.com/ja/health/14267-dravet-myoclonic.html
Alin Trodden - author of the article, editor
"Hi, I'm Alin Trodden. I write texts, read books, and look for impressions. And I'm not bad at telling you about it. I am always happy to participate in interesting projects."
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