Идиопатични детски фокални епилепсии
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Дата
2017-07-05
Автори
Александрова-Лазарова/Alexandrova-Lazarova, Илияна Антолиева/Iliyana Anatolyeva
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SUMMARY *****
Idiopathic focal epilepsies (IFE) are the most common form of childhood
epilepsy. They are characterized by a variable course and some of
the cases show an atypical evolution that can lead to cognitive decline.
The aims of the study were to analyze the clinical course, the possibility of an atypical evolution and the risk factors for a complicated evolution and to summarize the therapeutic responses and determine the most effective drugs. Materials and methods: We have studied 343 children with rolandic
epilepsy (66 with an atypical evolution), 51 children with idiopathic
ESES, 20 children with symptomatic ESES, 40 children with
Panayiotopoulos syndrome and 13 - with Gastaut syndrome.
Results: Rolandic patients showed the typical for the disease clinical
course: 56,3% had rare seizures (between 2 and 6 for the disease course),
and 66,4% had only nocturnal seizures, permanent seizure remission
was achieved in half of the patients with the first antiepileptic drug. The
atypical rolandic cases showed more aggressive course: a higher percent
of secondary generalized tonic-clonic seizures (82% vs. 69), earlier
mean age of seizures' onset (5 vs. 7), a higher percent of cases with
atypical clinical features (daytime seizures - 50% vs. 30%, postictal paresis
- 33% vs. 10%), cognitive deficit or behavioral changes in 47%. The
analysis of the clinical course of the rolandic patients showed that earlier
age of seizure onset (below 5 years), the multiple and daytime seizures
and the postictal paresis are risk factors for complicated course
and atypical evolution.
Analysis of the treatment responses in typical rolandic epilepsy (TRE)
showed that Valproate (VPA) exceeds Carbamazepine (CBZ) in terms
of its initial and long lasting effects concerning seizure remission. In the
cases of an atypical evolution the most promising were the results with
corticosteroids (CS), Levetiracetam (LEV), Ethosuximide (ESM), Benzodiazepines,
but in general a significant therapeutic resistance was presented.
The lack of an initial seizure remission or reduction with
Oxcarbazepine (OXC), Lamotrigine (LTG) and CBZ and the lack of
permanent seizure remission with VPA, LEV, OXC, CZP è LTG should
be considered as factors for a selection of patients with a risk for an
atypical course.
The patients with rolandic epilepsy (n=32) had normal IQ but the results
were lower than those of healthy controls. The statistical analysis
found a correlation between the lower IQ scores and the earlier age of seizures' onset and the appearance of ESES. No connection between the cognitive deficit and the daytime distribution of seizures and the number of the seizures was established. Patients with rolandic epilepsy
showed memory problems and attention deficit that correlated with the
presence of ESES.
The cases with ESES were characterized by a typical biphasic evolution
and a significant therapeutic resistance. In the idiopathic cases the most
promising were the results with CS, LEV, ESM. The symptomatic cases
were more difficult to treat as seizures and ESES remission was rarely
achieved.
Panayiotopoulos syndrome was characterized by a typical course - mostly
nocturnal (in 67,5% of the cases) and rare (72,5% of the children had
below 6 seizures for the entire disease course) seizures with leading autonomic
symptoms. Occipital epileptiform activity was registered in only
57% of the patients at the time of the diagnosis. The clinical course and
the seizures' semiology were similar despite the localization of the epileptiform
activity. The analysis of the clinical course showed that the
number, the daytime distribution and the duration of the seizures did
not affect the treatment responses. Patients with Gastaut syndrome had
mostly daytime and multiple seizures, VPA and CBZ were the drugs of
choice but the treatment was long lasting because of the significant risk
of seizures' relapse.
Conclusion: IFE are characterized by a favorable course but the possibility
of an atypical evolution should be considered as those patients need close supervision with neuropsychological assessment and EEG
monitoring and appropriate and prompt treatment.
Описание
Дисертационен труд за присъждане на образователна и научна степен "Доктор" /CD/ / Илияна Антолиева Александрова-Лазарова . - София, 2016 . - 181 с. + Автореферат /CD/
Ключови думи
Деца - дисертации; Нервна система - болести - деца - дисертации; Епилепсия, парциална - деца - дисертации , Child - dissertations; Nervous System Diseases - infancy and childhood - dissertations; Epilelepsies, Partial - child - dissertations