PMID- 33248400
OWN - NLM
STAT- MEDLINE
DCOM- 20210414
LR  - 20210414
IS  - 1525-5069 (Electronic)
IS  - 1525-5050 (Linking)
VI  - 113
DP  - 2020 Dec
TI  - Three different scenarios for epileptic spasms.
PG  - 107531
LID - S1525-5050(20)30711-3 [pii]
LID - 10.1016/j.yebeh.2020.107531 [doi]
AB  - Epileptic Spasms (ES) is a type of seizure usually occurring in the context of a 
      severe childhood epileptic syndrome associated to significant 
      Electroencephalogram (EEG) abnormalities. There are three scenarios in which ES 
      may occur. The first one is represented by West Syndrome (WS): ES occur in a 
      previously non encephalopathic infant in association with the development of a 
      hypsarrhythmic EEG pattern. In most cases, standard treatment with 
      Adrenocorticotropic Hormone (ACTH), steroids or vigabatrin leads to a reversal of 
      the electroclinical picture. The second scenario is represented by Developmental 
      and Epileptic Encephalopathies (DEEs): ES are documented, often along other 
      seizures types, in an infant who often shows developmental delay since birth; the 
      EEG pattern is pathological both in wakefulness and in sleep, without typical 
      features of hypsarrhythmia; therapies (with the exception of few potentially 
      treatable syndromes) are poorly effective. The last scenario is represented by ES 
      in the context of Focal Epilepsies (FEs): ES, sometimes showing focal signs or 
      closely related to focal seizures, are associated with focal brain lesions. 
      Treatment with ACTH, steroids or vigabatrin may not be effective as well as 
      antiepileptic drugs for focal epilepsies. In drug-resistant patients, surgery 
      should be considered. Although there are some gaps in our current scientific 
      knowledge concerning the peculiar electroclinical and physiopathological features 
      of ES, we nowadays possess the necessary tools to correctly frame this unique 
      seizure type into one of these scenarios and therefore properly manage the 
      diagnostic and therapeutic workup.
CI  - Copyright (c) 2020 Elsevier Inc. All rights reserved.
FAU - Fusco, Lucia
AU  - Fusco L
AD  - Intensive Neurological Diagnostic Unit, Neuroscience Department, Bambino Gesu 
      Children's Hospital, Rome, Italy. Electronic address: lucia.fusco@opbg.net.
FAU - Serino, Domenico
AU  - Serino D
AD  - Paediatric Neurology Department, Royal Aberdeen Children's Hospital, Aberdeen, 
      UK.
FAU - Santarone, Marta Elena
AU  - Santarone ME
AD  - Intensive Neurological Diagnostic Unit, Neuroscience Department, Bambino Gesu 
      Children's Hospital, Rome, Italy.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20201125
PL  - United States
TA  - Epilepsy Behav
JT  - Epilepsy & behavior : E&B
JID - 100892858
RN  - GR120KRT6K (Vigabatrin)
SB  - IM
MH  - Child
MH  - Electroencephalography
MH  - *Epilepsy/complications/drug therapy
MH  - Humans
MH  - Infant
MH  - Spasm
MH  - *Spasms, Infantile/drug therapy
MH  - Vigabatrin/therapeutic use
OTO - NOTNLM
OT  - Developmental Epileptic Encephalopathy
OT  - Epilepsy Surgery
OT  - Epileptic Spasms
OT  - Infantile Spasms
OT  - West Syndrome
COIS- Declaration of Competing Interest None of the authors have any conflicts of 
      interest to declare.
EDAT- 2020/11/29 06:00
MHDA- 2021/04/15 06:00
CRDT- 2020/11/28 20:10
PHST- 2020/09/04 00:00 [received]
PHST- 2020/10/14 00:00 [revised]
PHST- 2020/10/14 00:00 [accepted]
PHST- 2020/11/29 06:00 [pubmed]
PHST- 2021/04/15 06:00 [medline]
PHST- 2020/11/28 20:10 [entrez]
AID - S1525-5050(20)30711-3 [pii]
AID - 10.1016/j.yebeh.2020.107531 [doi]
PST - ppublish
SO  - Epilepsy Behav. 2020 Dec;113:107531. doi: 10.1016/j.yebeh.2020.107531. Epub 2020 
      Nov 25.