Rolandic epilepsy (RE) – benign partial epilepsy of childhood with centro-temporal adhesions
ER – nosologically independent form of idiopathic partial epilepsy. It is characterized by:
· Debut in 3-13 years;
· Simple partial motor paroxysms with somatosensory aura;
· Secondary-generalized night attacks;
· The presence of specific “rolandic” spikes on the EEG (Ga-staut, 1952);
· Favorable outlook.
The incidence is 15–20% among children with epilepsy 1–15 years old.
Clinic. The peak of the manifestation – 7-8 years. Boys are sick more often (60%). 70 to 80% seizures
are partial, more often they are motorized.
With typical seizures, a child of 5-10 years old comes to parents in full consciousness, shows
his mouth, skewed to one side, from the corner of which oozes saliva. Following a bias of the face, hemifacial jerking usually follows. The entire episode lasts no more than 1-2 minutes. The child says that the attack began with numbness, “pin” injections or “electricity” in the tongue, gums and cheek on one side. This description is important for diagnosis, even without EEG.
The most typical clinical manifestations of rolandic epilepsy are: – somatosensory aura;
unilateral clonic, tonic-clonic convulsions;
speech difficulties (dysarthria);
hypersalivation.
The somatosensory aura usually has one-sided localization and is characterized by paresthesia of the cheek, muscles of the larynx, pharynx, feeling of pinpricks, numbness in the cheek, gums, less often in the tongue. Thus, the main localization of the process is the orofacial region. Localization of the aura on the side opposite to the focus is most typical.
Unilateral epileptic paroxysms manifest as hemi-facies, mainly clonic or tonic-clonic convulsions. Involvement of the lips, tongue, pharyngeal muscles causes a complex of “oropharyngeal” symptoms. The child describes his feelings as “my jaws are pushed to the side,” “my teeth are chattering,” “my tongue is trembling.”
Speech difficulties and hypersalivation lead to gnawing, grunting sounds.
Consciousness is usually not disturbed. Amnesia does not happen. The most characteristic time of attacks is night. Night attacks can be secondary-generalized.
One of the favorable features of rolandic epilepsy is the low incidence of seizures (one attack in 2-12 months). In 13% of patients, according to Lerman, there is only one single attack.
In the neurological status there are no signs of focal lesions of the nervous system.
In a comparative study assessing the intelligence, behavior, and school performance of children with rolandic epilepsy and the control group, no significant differences were found.
On the EEG, in the middle temporal (TZ, T4) and central (СЗ, С4) leads, high-amplitude, diffuse peaks are recorded, followed by a slow wave. Background activity is normal.
Treatment. When working with patients and their parents from the time of diagnosis, an adequate focus on the benign nature of the disease and the prospect of full recovery is necessary.
Since rolandic epilepsy stops after 16 years and has a low risk of return, Panayiotopoulus writes: “I propose to remove the label“ epilepsy ”from patients with rolandic epilepsy.”
NB! Patients with rolandic epilepsy are contraindicated carbamazepines. It has been proven that in patients with RE, these drugs can stimulate negative myoclonus, absences, ESES syndrome (electrical epileptic status in phaso-slow sleep).
The drugs of choice are konvulex, depakin at therapeutic doses, sultiam at a dose of 5 mg / kg / day.
Forecast. The special feature of ER, which distinguishes this form from other epilepsies, is a good prognosis of the disease with spontaneous remission in almost all cases. It is believed that in all patients suffering from ER, after 16 years of age, clinical remission occurs.
Benign occipital epilepsy (DZE) – Benign partial epilepsy with occipital paroxysms
DZE is a form of idiopathic localization-related childhood epilepsy, characterized by simple partial seizures with visual disorders, migraine-like symptoms, and the presence of a specific peak-wave activity in the occipital leads on the EEG. N. Gastaut was described in 1950, detailed by him in 1982.
Frequency. DZE is the second most common form of idiopathic partial epilepsy (IPE) (after rolandic epilepsy).
Genetic data. Aggravated family history of epilepsy is ascertained in patients suffering from ECD in 36.6% of cases. In families, there is also an accumulation of migraine cases – up to 18%. Clinic. Two variants of the disease are distinguished: the Gastaut variant and the Panayiotopoulos variant.
The first type is characterized by onset at an average of 8 years of age. Simple forms are most typical for this form partial sensory paroxysms accompanied by visual disorders: transient visual disturbances -65%, amaz-roses – 52%, elementary visual hallucinations (flickering of luminous objects, flashes of light) -50%, complex, stage-like hallucinations – 14% Gastaut, 1992). These manifestations may be hemi-optical in nature, arising in the equivalent half of the visual fields in both eyes.
Motor attacks are often complex, with the presence of automatism, may be secondary-generalized. Vegetative disorders include epigastric sensations, nausea, vomiting, headache, dizziness. The duration of an attack in ECD varies from a few minutes to several hours. One-third of patients in the post-onset period develop migraine-like symptoms: hemicrania-type headache, nausea, vomiting, dizziness. It is this variant of the DZE that presents the greatest diagnostic difficulties in differentiating with migraine.
The Panayiotopoulos variant is characterized by an early start – an average of 4.9 years. Attacks begin with vomiting, headache, followed by tonic abduction of the eyes and head. Tonic-clonic convulsions follow. The extremely high duration of loss of consciousness up to several hours is dramatic. The typical occurrence of seizures during sleep.
Intellect in children with ECD is normal, focal neurological symptoms are absent.
The EEG of these patients is characterized by normal main activity and the presence of high-amplitude, rhythmically repeated peaks or sharp waves in the occipital or posterior temporal region. Most authors note a significant predominance of involvement of the right hemisphere in ECD.