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De novo Absence Status Epilepticus in a pediatric cohort: Electroclinical pattern in a multicenter Italian patients
C Pepi1, E Cesaroni2, P Striano3
1Child Neurology Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital Research Institute, Rome, Italy; Child Neurology and Psychiatry Unit, System Medicine Unit, Tor Vergata University, Rome, Italy.
Insights
Absence Status Epilepticus (AS) in children can be a distinct epilepsy syndrome. Early antiepileptic drug treatment is crucial to prevent recurrent seizures and ensure a good prognosis.
Area of Science:
- Neurology
- Pediatric Epilepsy
- Genetics
Background:
- Absence Status Epilepticus (AS) is a prolonged, generalized, non-convulsive seizure affecting consciousness.
- It can be the sole manifestation of epilepsy in children with normal development.
Purpose of the Study:
- To describe pediatric patients with recurrent, unprovoked de novo AS.
- To evaluate the response to antiepileptic drugs (AEDs) in these patients.
Main Methods:
- Retrospective review of 13 pediatric patients (2005-2019).
- Inclusion criteria: normal development, AS as only manifestation, normal tests, EEG, and follow-up.
- Genetic testing (aCGH, karyotype, NGS, WES) performed in 10 patients.
Main Results:
- 13 patients (7 female, 6 male), mean age at onset 9.3 years.
- Excellent response to Valproic Acid (VPA) or Ethosuximide (ETS).
- 5 patients with delayed treatment experienced recurrent AS; 10 underwent genetic testing.
Conclusions:
- De novo AS may represent a distinct, age-related, self-limited epilepsy syndrome.
- Prognosis is good with AEDs, but relapse risk is high if untreated.
- Early and adequate AED therapy is essential.
Purpose:
Absence Status epilepticus (AS) is a form of Non Convulsive Status Epilepticus defined as a prolonged, generalized and non-convulsive seizure, with an altered content of consciousness. We aim to describe a group of healthy children, who presented recurrent and unprovoked de novo AS as the only manifestation of their epilepsy, with an excellent response to antiepileptic drugs.
Method:
We retrospectively reviewed the electroclinical and genetic features of 13 pediatric patients, referring to our epilepsy centers from 2005 to 2019, on the following criteria: (1) regular psychomotor development, (2) one or more unprovoked AS as the only epileptic manifestation, (3) normal blood testing, (4) normal neuroimaging, (5) EEG recording, (6) available follow-up (1-14 years).
Results:
Patients are 7 females and 6 males, aged 7-22, with a mean age at AS onset of 9,3 years. All of them started an antiepileptic therapy, with an excellent response to Valproic Acid (VPA) or Ethosuximide (ETS). 5 patients did not start the therapy immediately after the first AS and they presented recurrent AS (from 2 to 4 episodes). 10 of them performed aCGH, karyotype, NGS panel or Whole Exome Sequencing.
Conclusions:
We suggest that de novo AS may be a well-defined age-related and self-limited epilepsy syndrome, with a good prognosis and excellent response to therapy, but it comes with a high risk of relapsing if not adequately treated with antiepileptic drugs.
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