Benign rolandic epilepsy is a genetically determined focal epilepsy associated with age—related hyperexcitability of the cortex of the central temporal region of the brain. Disease is manifested by rare, occurring mainly at night, convulsive seizures in one half of the face, tongue and pharynx; in some cases, generalized seizures. The diagnosis is established on the basis of the clinical features of the disease and EEG data, if necessary, polysomnography and MRI of the brain are performed. Pathology does not lead to violations of the psycho-physical development of the child and passes without a trace by the end of adolescence.
G40.0 Localized (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures with focal onset
In pediatrics, benign rolandic epilepsy (BRE) is the most common pathology from the group of benign focal epilepsies. It accounts for 15% of all cases of epilepsy in children under the age of 15 and about 20% of cases of convulsive syndrome in children. Disease occurs with a frequency of 21 cases per 100 thousand children. Benign rolandic epilepsy can occur at the age of 2 to 14 years, but in 85% of cases it manifests in the age period from 4 to 10 years. The benign course of classical rolandic epilepsy is characterized by the absence of psycho-neurological changes and the complete disappearance of epiprimes by the age of 15-18.
The exact causes of BRE have not yet been established. In some cases (according to various data, 20-60%), the hereditary nature of the disease is traced, but direct autosomal recessive or autosomal dominant inheritance is not observed. This served as the basis for the polygenic theory of inheritance of benign rolandic epilepsy, according to which the disease is determined by two different genes.
In modern neurology and epileptology, the dominant idea is that BRE occurs due to impaired maturation of the cerebral cortex in the central temporal region. Benign rolandic epilepsy is an age-dependent disease and is probably associated with increased excitability of the brain in children. The latter is due to a number of factors: functional and structural features of epileptogenic regions (neocortex, hippocampus, amygdala), the prevalence of excitatory neurotransmitters, low GABA content, immaturity of GABA receptors, an increased number of excitatory recurrent synapses. As the child’s brain matures, its excitability decreases and the epileptogenicity of the foci of BRE gradually disappears. As a result, there is a decrease in the number of epileptic seizures and a complete recovery of patients when they reach puberty.
The most typical for classical BRE are simple partial seizures that occur without loss of consciousness. As a rule, the attack is preceded by a sensory aura in the form of a unilateral feeling of tingling, tingling or numbness on the face, lips, gums, tongue and throat. Then there is a motor paroxysm, which can be represented by both tonic and clonic muscle contractions, as well as their combination. Depending on the localization of seizures, seizures of benign rolandic epilepsy can be hemifacial and pharyngoral. A hemifacial attack occurs with convulsions in the muscles of half of the face. Pharyngoral attack is characterized by unilateral convulsions in the muscles of the tongue, lips, pharynx and larynx. It is accompanied by hypersalivation and speech impairment. Convulsive contractions of the laryngeal muscles leads to the appearance of characteristic throat sounds resembling gurgling, gargling or grunting. As benign rolandic epilepsy develops, convulsive seizures can “change” the side.
80% of the seizures that accompany BRE occur at night and are closely associated with periods of falling asleep and waking up. Much less often there is a combination of nocturnal seizures with seizures occurring during the daytime. In 20% of cases, benign rolandic epilepsy occurs with brachiofacial seizures, during which facial convulsions spread to the homolateral arm. In 8% of cases, the involvement of the homolateral lower limb is observed. In some cases, benign rolandic epilepsy is accompanied by complex partial seizures, for which short-term loss of consciousness is typical. Approximately 20% of patients have secondary generalized epiprimes, during which convulsions seize all the muscles of the body. Such attacks occur with complete loss of consciousness.
Benign rolandic epilepsy is characterized by a short duration of epiprimes (up to 2-3 minutes) and their low frequency. Seizures are isolated or observed several times a year. Sometimes at the beginning of the disease, seizures may be more frequent, but as the child grows older, their decrease is observed.
Unlike the epilepsy described by Jackson, classical benign rolandic epilepsy is not accompanied by a delay in the psycho-motor development of the child and does not lead to oligophrenia. However, it is impossible to say about the absolute absence of neurological disorders, since about 3% of patients have transient hemiparesis after seizures.
The main way to diagnose benign rolandic epilepsy is EEG. Since in some patients EEG changes are observed only during sleep, with normal daytime EEG, nighttime EEG video monitoring or polysomnography is additionally necessary.
A pathognomonic sign of benign rolandic epilepsy is the detection of high-amplitude acute waves or peaks located in the central temporal leads against the background of normal basic activity. Slow waves often follow the peak, together with the peak they make up the so-called “Rolandic complex” with a duration of up to 30ms. Visually, such complexes resemble QRST complexes on an electrocardiogram. Usually, “rolandic complexes” are localized on the side opposite to convulsive seizures, but they can also have a bilateral character. The peculiarities of EEG patterns of benign rolandic epilepsy include their variability from one EEG record to another.
First of all, benign rolandic epilepsy needs to be differentiated from symptomatic epilepsies that occur with intracerebral tumors, traumatic brain injuries, inflammatory brain lesions (abscess, encephalitis, purulent meningitis). Rolandic epilepsy is confirmed by the absence of pathological changes in the neurological status and behavior disorders, the preservation of intelligence, anamnestic data, normal basic activity on the EEG. In some cases, an MRI of the brain is performed to clarify the diagnosis.
The differential diagnosis of benign rolandic epilepsy and nocturnal epilepsy with complex partial epilepsy presents great difficulties. This is due to the fact that speech disorders typical of an attack of benign rolandic epilepsy can be interpreted as disorders of consciousness. On the other hand, it is quite difficult to adequately diagnose nocturnal disorders of consciousness. In such cases, EEG data play a crucial role in the diagnosis, which in frontal and temporal epilepsy demonstrate focal changes in brain activity in the corresponding leads.
Certain difficulties are caused by the differentiation of classical BRE from pseudolennox syndrome, which is observed in 5% of patients with symptoms of rolandic epilepsy. The combination of typical Rolandic epiprimes with atypical absences, myoclonic and asthenic seizures, intellectual and mnestic disorders, as well as the detection on the EEG of diffuse peak activity or slow complexes characteristic of the Lennox—Gastaut syndrome, indicate in favor of the pseudolennox syndrome.
For pediatric neurologists and epileptologists, the question of the expediency of treating benign rolandic epilepsy is very debatable. Since even without treatment, benign rolandic epilepsy ends in recovery, then, according to some authors, it is not an indication for the appointment of antiepileptic therapy. Other neurologists, pointing out the likelihood of a diagnostic error when making the diagnosis of “benign rolandic epilepsy” and considering the possibility of its transition to pseudolennox syndrome, recommend antiepileptic therapy for repeated epiprimes.
In the treatment of children with BRE, only 1 antiepileptic drug (monotherapy) is always used. Treatment usually begins with the appointment of one of the drugs valproic acid. If it is intolerant or ineffective, as a rule, they switch to taking topiramate or levetiracetam. In children over 7 years of age, carbomazepine may be used, but it should be remembered that in some cases it can lead to the phenomenon of aggravation, i.e., to an increase in seizures.