Juvenile absence epilepsy

Juvenile abscess epilepsy (SAA) is an idiopathic generalized epilepsy characterized by absences making their debut in puberty, with a high probability of attaching tonic-clonic seizures, and characteristic EEG changes in the form of generalized peak-wave activity with a frequency of 3 Hz and more.

For the first time, Doose et al. in 1965, it was suggested that the group of “children’s picoleptic absenses” was probably heterogeneous. Subsequently, Wolf (1992) proved the nosological independence of the syndrome by proposing to call it adolescent abscess epilepsy.

Frequency. The exact incidence of UAE is not specified. According to the data of K.Yu. Mukhina (2000), the incidence of SAA is 12.3% among idiopathic and cryptogenic forms of epilepsy.

Genetics. Relatives suffering from various forms of epilepsy are detected in 15% of the patients with UAE (K.Yu. Mukhin, AS Petrukhin, 2000). Obeid (1994) found familial cases of epilepsy in relatives of 36% of probands.

Clinic. The disease debuts in the age range of 9-13 years. A characteristic feature is that the first attacks in the clinical picture in 41% of cases are generalized tonic-clonic, they are recorded in 67-85% of patients; then joins absans (for 1-12 months).

Simple abscesses prevail (59%), the duration of attacks ranges from 3 to 30 s, in half of the patients very short abscesses are observed, not exceeding 3-5 s, which are often not noticed by patients and others. Characterized by a low frequency of attacks compared with AED: single attacks during the day, 1 attack in 2-3 days. Tonic-clonic seizures are also rare, their frequency – 1-2 times a year, sometimes less.
Generalized tonic-clonic seizures are clearly associated with a circadian rhythm, occurring more often on waking (56%). The provoking factor is sleep deprivation, hyperventilation is a less significant provoking factor (not more than in 10% of patients).
In the neurological status of focal symptoms is not detected, impaired intelligence is not typical.
EEG. On EEG, the main activity (alpha rhythm) is always preserved. The EEG pattern is a symmetric bilateral synchronous peak-wave activity with a frequency of 3 Hz, sometimes more than 3.5-5 Hz. The absence pattern. Patient P., 15 years. Diagnosis: juvenile absence epilepsy.

Treatment. The effectiveness of treatment is lower than with DAE, therapeutic remission is achieved in 56% of patients (Mukhin K.Yu., Petrukhin A.S., 2000). Moreover, therapeutic efficacy depends on the presence and frequency of tonic-clonic seizures.

Treatment begins with convolux or other valproates, therapeutic doses – up to 30-50 mg / kg / day. Due to the high probability of accession of tonic-clonic generalized seizures, suksilep use impractical.

In the reaction of idiosyncrasy to valproic acid derivatives, lamictal can be used as monotherapy, at a dose of 5-15 mg / kg / day.

In the absence of the effect of monotherapy with valproate in maximum doses, combinations are possible:

  • Valproate + suksilep (initial doses of drugs remain unchanged);
  • valproate + benzodiazepine: clonazepam (antelepsin) 2-8 mg / day or clobazam 5-25 mg / day; the dose of valproate remains 20-30 mg / kg / day;
  • valproate + lamotrigine (lamictal) at a dose of 0.2-5 mg / kg / day; the dose of valproate remains 20-30 mg / kg / day.
    The use of carbamazepine derivatives is ineffective, and in 20% of patients causes a significant increase in absences.

Forecast. Therapeutic remission with adequate therapy is achieved in 65% of cases, the duration of therapy is at least 3 years after the disappearance of the attacks. In 15-25% of cases, recurrence of seizures after a long remission is possible (Loiseau J. et al., 1998).

A neurologist has to deal with forms of absence epilepsy that are not included in the International Classification of Epilepsy and Epileptic Syndromes (New Delhi, 1989). Many of these forms are nosologically highlighted after the adoption of this classification.

local_offerevent_note February 27, 2019

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