Status epilepticus: what is it, symptoms, treatment, drugs, prognosis

What is status epilepticus?

Status epilepticus (or epistatus) Is a neurological emergency. Left untreated (or insufficiently treated), prolonged seizures can lead to irreversible neurological injury or death. Prompt treatment must be started. If the initial agents fail, it may be necessary to induce an iatrogenic coma. In any case, the person with status epilepticus must be closely monitored and often a continuous EEG is required to confirm that the seizures have stopped not only clinically but also electrically.

Signs and symptoms

Epilepsy Is a group of diseases characterized by electrical discharges in the brain. There are no established factors that cause epileptic seizure that are common to all patients. However, people with epilepsy often refer to visual phenomena such as flickering light or sunbeams that precede the seizure. In some patients, the likelihood of an attack increases with stress, fatigue, insufficient food intake and / or refusal to take prescribed medications.

Status epilepticus is classified as follows:

• Generalized convulsive status epilepticus (GSES):
  • explicit (70 percent of HSES): consists of continuous tonic and / or clonic activity with impairment or loss of consciousness;
  • invisible (30% of HSES): less obvious than the overt type, consisting of facial twitching, nystagmoid eye movements, or slight twitching of the limbs.
• Non-convulsive status epilepticus (NECE):
  • complex partial status epilepticus: this type occurs in patients with a history of partial seizures, but can also occur as a result of acute trauma (for example, new stroke). This type of status epilepticus can cause focal motor manifestations (for example, nystagmus), but can also cause more subtle phenomena such as confusion, personality changes, or psychosis;
  • non-convulsive status epilepticus: occurs in patients with idiopathic generalized epilepsy. As with complex partial status epilepticus, clinical symptoms may be subtle, including confusion or personality changes.

Causes

The exact cause of epilepsy is unknown. Hereditary factors have been suggested as a possible cause of essential epilepsy. Some types of epilepsy occur as a symptom of other conditions, while others are thought to be caused by head injuries. Some families may have a genetic predisposition for epilepsy, but scientists do not understand the hereditary factors that can make a person vulnerable to seizures.

The most common causes of recurrent seizures in infants include:

• genetic congenital metabolic disorders;
• other metabolic disorders;
• malformations of the brain;
• injuries sustained several months before delivery or several weeks after delivery (perinatal);
• acute lack of oxygen (hypoxia).

In children, typical causes of new-onset epileptic seizures may include:

• inflammation of the membranes surrounding the brain and spinal cord (meningitis);
• inflammation of the brain (encephalitis);
• brain abscesses;
• brain cancer;
• exposure to poisons or toxins;
• diseases that affect the blood vessel system (vascular disease);
• degenerative brain diseases;
• head injury.

Epileptic seizures that occur in infants or children as a result of an abnormally high temperature (febrile seizures) usually do not recur. In adults, the onset of epileptic seizures can sometimes be associated with a brain tumor, head trauma, stroke, cerebrovascular disease, and / or degenerative brain disease. However, in many cases, the cause cannot be determined.

Affected populations

The estimated incidence of epilepsy is 50-70 per 100 thousand people, the prevalence is 5-10 per thousand (0.5-1%). 5% of the population suffer at least one seizure during their life, in 20-30% of patients the disorder is lifelong. The incidence of epilepsy is more common in older people, i.e. in children and the elderly. About 15 percent of people with epilepsy have status epilepticus. Altogether, about 1.5 million Russians suffer from epilepsy, but most patients do not develop epilepsy due to effective medications.

Symptomatic disorders

Symptoms of the following disorders may be similar to those of status epilepticus. Comparisons can be useful for differential diagnosis:

• Wilson-Konovalov disease - a rare hereditary disease that affects liver, eyes and neuromuscular system. Symptoms develop due to excessive accumulation of copper in body tissues, especially in the liver, brain, and eyes. Early diagnosis and treatment of Wilson-Konovalov disease can prevent serious chronic disability. Neuromuscular symptoms of Wilson-Konovalov disease usually appear between the ages of 12 and 32. These symptoms may include drooling, joint pain (dysarthria), speech impairment (dysphasia), poor muscle coordination, tremor, involuntary muscle twitching, muscle stiffness and double vision. Other late symptoms of Wilson-Konovalov disease may include cognitive decline, behavior changes, stones in the kidneys, depression and other mental disorders.
• Coca disease (hyperexlexia) is a very rare hereditary disease of the neurological system. People with Coca disease have an excessive startle response to sudden and / or unexpected noise, movement, or touch. When a person with Coca disease is frightened, the head may bend back and muscle twitching (myoclonic twitching) may occur. A frightened person may also fall to the ground while still. Some people with Coca's disease also experience epilepsy.
• Myoclonus - a neurological movement disorder in which the skeletal muscle experiences sudden involuntary contractions, leading to sudden movements. There are 3 types of myoclonus: intentional, rhythmic, and arrhythmic. Intentional myoclonus is characterized by episodes of involuntary muscle contractions that are triggered by voluntary movements, such as a purposeful action. With arrhythmic myoclonus, muscle cramps are arrhythmic and sudden. Muscle twitching can be limited to one muscle or affect all skeletal muscles on one or both sides of the body. The stimulus to start an episode can be sensory (visual, auditory, and / or tactile), or it can be fatigue, stress, or anxiety. A violent startle reaction may also be present. Rhythmic (segmental) myoclonus is characterized by very rapid and frequent muscle twitching. Unlike arrhythmic myoclonus, this type of disease is not relieved by sleep or caused by sudden stimuli or voluntary movements. Myoclonic muscle twitching can sometimes be confused with the muscle twitching and stiffness that occurs with some forms of epilepsy.
• Narcolepsy- a rare neurological sleep disorder characterized by extreme unnatural sleepiness during the day, sudden loss of voluntary muscle tone (cataplexy), hallucinations, sleep paralysis and / or sleep disturbance at night. Symptoms usually appear between the ages of 10 and 20. The development and severity of symptoms varies greatly from patient to patient. The first symptom is usually severe daytime sleepiness. A person with narcolepsy may describe feelings of drowsiness, tiredness, lack of energy, "sleep attacks" and / or an inability to resist sleep. People with narcolepsy and cataplexy may fall asleep so suddenly that they seem to be falling to the floor unconscious. In sleep paralysis, the person with narcolepsy wants to move but cannot.

Standard treatments

Status epilepticus is treated with anticonvulsants, which try to prevent and control seizures. Medicines currently in use include:

• phenytoin;
• valproic acid;
• carbamazepine;
• phenobarbital;
• clonazepam;
• ethosuximide (zarontin);
• primidone;
• acetazolamide;
• paraldehyde;
• trimethadione;
• corticotropin;
• corticosteroids.

In the case of epilepsy caused by a brain tumor or drug-resistant temporal lobe epilepsy, you can try brain surgery after medication has failed to stop seizures. Typically, surgery is not done until other treatments have been unsuccessful. The success rate of such operations is approximately 55–70 percent.

It is very important to protect the person with epilepsy from self-harm during a seizure. Protective measures should include clearing the area of ​​any hard or sharp objects, loosening tight clothing, and placing a flat, soft object under the head. The patient should be turned on their side and, if possible, something soft and flat (such as a pillow or towel) should be placed between the teeth. Containment is not recommended. The introduction of artificial respiration should be attempted only if respiration does not begin after the seizure has stopped. When the seizure is over, the patient should be allowed to sleep or helped to return home if he seems confused. If the patient wants to sleep, the head and shoulders should be raised.

It is possible that some people with epilepsy who have not had seizures for a long period of time (several years) may reduce or stop taking anticonvulsants under close supervision doctor.

The Food and Drug Administration (FDA) has approved the anticonvulsant drug Felbamat for use in intractable epilepsy. One of the main advantages of this drug is that it is not a sedative and therefore does not make the user drowsy or lethargic.

The FDA has approved Lamotrigine (Lamictal) for the treatment of intractable partial seizures. The drugs phosphenytoin sodium, topiramate, and gabapentin have also been approved by the FDA for the treatment of epilepsy.

Forecast

10 to 30% of people with status epilepticus die within 30 days. The vast majority of these people have an underlying brain disease that causes their seizure status, such as a brain tumor, brain infection, brain injury, or stroke. However, patients with diagnosed epilepsy who have status epilepsy also have an increased risk of death if their condition is not stabilizes quickly, their medications and sleep patterns are not adapted and adhered to, and levels of stress and other stimulants (seizure triggers) are not controlled. However, with optimal neurological care, adherence to medication, and a good prognosis (no other major uncontrolled brain diseases or other organic diseases) a person - even patients who have been diagnosed with epilepsy - in otherwise in good health, can survive with minimal or no brain damage, and reduce the risk of death and even avoid seizures seizures in the future.