Abstract
Purpose: Infantile spasms (IS) represent a severe seizure disorder of infancy and early childhood characterized by epileptic spasms along with hypsarrhythmia often accompanied by intellectual disability. According to the current classification and terminology (3) IS can be categorized as known etiology, formerly known as “symptomatic”, when an underlying cause has been observed prior to the onset of spasms, or of “unknown cause” with “unfavorable” and “favorable” outcome (previously referred as “cryptogenic” or “idiopathic”, respectively). Single reports described children with “unknown cause and favorable outcome” (UC/FO) IS who later developed childhood absence epilepsy (CAE). This study aims to determine the prevalence of CAE following IS. Methods: a multicenter retrospective chart review was performed; children with UC/FO IS who subsequently developed CAE during follow-up were identified. Eight Italian pediatric epilepsy centers participated in this study. Results: seven out of 24 (29 %) children (3 males) showing a favorable outcome (UC/FO) IS received a second diagnosis of CAE during follow-up. Mean age at IS presentation was 5.8 months (SD ± 0.9). All achieved seizure control of IS at a mean age of 8.5 months (SD ± 1.3) (3 monotherapy, 4 polytherapy). CAE was diagnosed at a mean age of 8.0 years (SD ± 3.0). Six children achieved sustained remission of CAE with valproic acid, whereas 1 child required dual therapy by adding ethosuximide. Conclusion: although it is not possible to determine whether the association between UC/FO IS and CAE implies a causality relationship, the later occurrence of CAE in patients with UC/FO IS might support a possible role of thalamo-cortical dysfunction.
| Original language | English |
|---|---|
| Pages (from-to) | 77-80 |
| Number of pages | 4 |
| Journal | Seizure |
| Volume | 74 |
| DOIs | |
| Publication status | Published - Jan 2020 |
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Keywords
- Childhood absence epilepsy
- Childhood epilepsy
- Infantile spasms
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
Cite this
Infantile spasms followed by childhood absence epilepsy : A case series. / Giordano, Lucio; Tambucci, Renato; Cocco, Isabella Emanuela; Angriman, Marco; Coppola, Giangennaro; Operto, Francesca Felicia; Farello, Giovanni; Savasta, Salvatore; Belcastro, Vincenzo; Verrotti, Alberto.
In: Seizure, Vol. 74, 01.2020, p. 77-80.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Infantile spasms followed by childhood absence epilepsy
T2 - A case series
AU - Giordano, Lucio
AU - Tambucci, Renato
AU - Cocco, Isabella Emanuela
AU - Angriman, Marco
AU - Coppola, Giangennaro
AU - Operto, Francesca Felicia
AU - Farello, Giovanni
AU - Savasta, Salvatore
AU - Belcastro, Vincenzo
AU - Verrotti, Alberto
PY - 2020/1
Y1 - 2020/1
N2 - Purpose: Infantile spasms (IS) represent a severe seizure disorder of infancy and early childhood characterized by epileptic spasms along with hypsarrhythmia often accompanied by intellectual disability. According to the current classification and terminology (3) IS can be categorized as known etiology, formerly known as “symptomatic”, when an underlying cause has been observed prior to the onset of spasms, or of “unknown cause” with “unfavorable” and “favorable” outcome (previously referred as “cryptogenic” or “idiopathic”, respectively). Single reports described children with “unknown cause and favorable outcome” (UC/FO) IS who later developed childhood absence epilepsy (CAE). This study aims to determine the prevalence of CAE following IS. Methods: a multicenter retrospective chart review was performed; children with UC/FO IS who subsequently developed CAE during follow-up were identified. Eight Italian pediatric epilepsy centers participated in this study. Results: seven out of 24 (29 %) children (3 males) showing a favorable outcome (UC/FO) IS received a second diagnosis of CAE during follow-up. Mean age at IS presentation was 5.8 months (SD ± 0.9). All achieved seizure control of IS at a mean age of 8.5 months (SD ± 1.3) (3 monotherapy, 4 polytherapy). CAE was diagnosed at a mean age of 8.0 years (SD ± 3.0). Six children achieved sustained remission of CAE with valproic acid, whereas 1 child required dual therapy by adding ethosuximide. Conclusion: although it is not possible to determine whether the association between UC/FO IS and CAE implies a causality relationship, the later occurrence of CAE in patients with UC/FO IS might support a possible role of thalamo-cortical dysfunction.
AB - Purpose: Infantile spasms (IS) represent a severe seizure disorder of infancy and early childhood characterized by epileptic spasms along with hypsarrhythmia often accompanied by intellectual disability. According to the current classification and terminology (3) IS can be categorized as known etiology, formerly known as “symptomatic”, when an underlying cause has been observed prior to the onset of spasms, or of “unknown cause” with “unfavorable” and “favorable” outcome (previously referred as “cryptogenic” or “idiopathic”, respectively). Single reports described children with “unknown cause and favorable outcome” (UC/FO) IS who later developed childhood absence epilepsy (CAE). This study aims to determine the prevalence of CAE following IS. Methods: a multicenter retrospective chart review was performed; children with UC/FO IS who subsequently developed CAE during follow-up were identified. Eight Italian pediatric epilepsy centers participated in this study. Results: seven out of 24 (29 %) children (3 males) showing a favorable outcome (UC/FO) IS received a second diagnosis of CAE during follow-up. Mean age at IS presentation was 5.8 months (SD ± 0.9). All achieved seizure control of IS at a mean age of 8.5 months (SD ± 1.3) (3 monotherapy, 4 polytherapy). CAE was diagnosed at a mean age of 8.0 years (SD ± 3.0). Six children achieved sustained remission of CAE with valproic acid, whereas 1 child required dual therapy by adding ethosuximide. Conclusion: although it is not possible to determine whether the association between UC/FO IS and CAE implies a causality relationship, the later occurrence of CAE in patients with UC/FO IS might support a possible role of thalamo-cortical dysfunction.
KW - Childhood absence epilepsy
KW - Childhood epilepsy
KW - Infantile spasms
UR - http://www.scopus.com/inward/record.url?scp=85076243707&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85076243707&partnerID=8YFLogxK
U2 - 10.1016/j.seizure.2019.12.007
DO - 10.1016/j.seizure.2019.12.007
M3 - Article
AN - SCOPUS:85076243707
VL - 74
SP - 77
EP - 80
JO - Seizure : the journal of the British Epilepsy Association
JF - Seizure : the journal of the British Epilepsy Association
SN - 1059-1311
ER -