De novo Absence Status Epilepticus in a pediatric cohort: Electroclinical pattern in a multicenter Italian patients cohort

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 manif...

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Bibliographic Details
Published in:Seizure (London, England) England), 2019-12, Vol.73, p.79-82
Main Authors: Pepi, C., Cesaroni, E., Striano, P., Maiorani, D., Pruna, D., Cossu, S., Di Capua, M., Vigevano, F., Specchio, N., Cusmai, R.
Format: Article
Language:English
Subjects:
Absence
Adolescent
Anticonvulsants - therapeutic use
Child
Electroencephalography
Epilepsy - drug therapy
Epilepsy - physiopathology
Ethosuximide - therapeutic use
Female
Genetic epilepsy
Humans
Italy
Male
NCSE
Retrospective Studies
Status Epilepticus - drug therapy
Status Epilepticus - physiopathology
Valproic Acid - therapeutic use
Young Adult
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Summary: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. 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). 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. 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.
ISSN:1059-1311
1532-2688
DOI:10.1016/j.seizure.2019.10.021