Table Of ContentProgress in
ns
ier
sd
esor
r
gis Epileptic Disorders
oD
r
P
c
i
t
p
e
l
i
p
E
Seizures and Syndromes Seizures and Syndromes
e
of onset in the f
Li of onset in the Two First
Solomon L. Moshé
Two First Years of Life J. Helen Cross of
s Years of Life
Julitta de Bellescize r
a
e
Linda de Vries Y
T Douglas Nordli t
s
his book focuses on the seizures and syndromes r
Federico Vigevano i
occurring in the newborn and the little child. F
During this period of life, there is a very high o
incidence of seizures, reflecting multiple etiologies w
depending on the circumstances under which T
seizures occur. These seizures may have a benign
cause but for others they may lead to more devas- e
h
tating consequences.
t
This work provides new insights on how it is best to approach seizures n
and epilepsy in the first two years of life, to systematically create a i
blueprint upon which diagnostic and treatment decisions can be based. t Solomon L. Moshé
e
The data are highly reflecting the state of the art and also individualize s
n J. Helen Cross
for the particular milieu of the patient is in taking into account both
o
nature (i.e. genetics), and nurture (i.e. events that may interfere with
Julitta de Bellescize
normal development) and result in seizures and epilepsy. f
o
Linda de Vries
s
e
m
Douglas Nordli
o
r Federico Vigevano
d
n
y
S
d
n
a
s
e
r
u
z
i
e
S
ISBN: 978-2-7420-1397-5
Progress in
ns
ier
sd
esor
r
gis Epileptic Disorders
oD
r
P
c
i
t
p
e
l
i
p
E
Seizures and Syndromes Seizures and Syndromes
e
of onset in the f
Li of onset in the Two First
Solomon L. Moshé
Two First Years of Life J. Helen Cross of
s Years of Life
Julitta de Bellescize r
a
e
Linda de Vries Y
T Douglas Nordli t
s
his book focuses on the seizures and syndromes r
Federico Vigevano i
occurring in the newborn and the little child. F
During this period of life, there is a very high o
incidence of seizures, reflecting multiple etiologies w
depending on the circumstances under which T
seizures occur. These seizures may have a benign
cause but for others they may lead to more devas- e
h
tating consequences.
t
This work provides new insights on how it is best to approach seizures n
and epilepsy in the first two years of life, to systematically create a i
blueprint upon which diagnostic and treatment decisions can be based. t Solomon L. Moshé
e
The data are highly reflecting the state of the art and also individualize s
n J. Helen Cross
for the particular milieu of the patient is in taking into account both
o
nature (i.e. genetics), and nurture (i.e. events that may interfere with
Julitta de Bellescize
normal development) and result in seizures and epilepsy. f
o
Linda de Vries
s
e
m
Douglas Nordli
o
r Federico Vigevano
d
n
y
S
d
n
a
s
e
r
u
z
i
e
S
Progress in Epileptic Disorders
Volume 13
Seizures and Syndromes
of onset in the
Two First Years of Life
Progress in Epileptic Disorders series
International Advisory Board
AlexisArzimanoglou–Lyon,France
JeanAicardi–Paris,France
IngmarBlümcke–Erlangen,Germany
FrançoisDubeau–Montreal,Canada
MichaelDuchowny–Miami,USA
AristeaGalanopoulou–NewYork,USA
AlexanderHammers–London,UK
YushiInoue–Shizuoka,Japan
PhilippeKahane–Grenoble,France
MichaelKerr–Cardiff,UK
SilviaKochen–BuenosAires,Argentina
CarlaMarini–Florence,Italy
DougNordli–Chicago,USA
GraemeSills–Liverpool,UK
PierreThomas–Nice,France
TorbjörnThomson–Stockholm,Sweden
SarahWilson–Melbourne,Australia
Progress in Epileptic Disorders
Volume 13
Seizures and Syndromes
of onset in the
Two First Years of Life
SolomonL.Moshé
J.HelenCross
JulittadeBellescize
LindadeVries
DouglasNordli
FedericoVigevano
Disclosures and Acknowledgements
Proceedings of the international Progress in Epileptic Disorders Workshop on Seizures and
Syndromes of onset in the first two years of life that took place at Grottaferrata, at the
outskirts of Rome, Italy, in November 14th to 17th, 2013.
The workshop was organized by the journal EPILEPTIC DISORDERS, Educational Journal
oftheILAE.ItwassupportedbyanunrestrictededucationalgrantfromUCB.Theresearch
association“AssociationESEFNP”independentlymanagedthegrant.Allparticipantswere
selected by the scientific committee on the basis of their expertise in the field and their
competence in contributing to a highly interactive, collaborative and forward-looking
workshop.
UCBwasnotinvolvedinthecontentofthesymposiumorpreparationoftheproceedings;
an honorarium was not provided to speakers, chairs, authors or other participating experts.
All organizational issues were under the responsibility of ANT CONGRES.
The John Libbey Eurotext editions independently supported the present publication.
ANTCONGRES,theJohnLibbeyEurotexteditionsandUCBareacknowledgedfortheir
contribution to the success of the workshop and the present publication.
ISBN: 978-2-7420-1397-5
ISSN: 1777-4284
Vol.13
Published by
Éditions John Libbey Eurotext
127, avenue de la République, 92120 Montrouge, France
Tel.: +33 (0)1 46 73 06 60
Website: www.jle.com
John Libbey Eurotext
42-46 High Street, Esther, Surrey, KT10 9KY
United Kingdom
© 2015, John Libbey Eurotext. All rights reserved.
Unauthorizedduplicationcontravenesapplicablelaws.
ItisprohibitedtoreproducethisworkoranypartofitwithoutauthorisationofthepublisheroroftheCentreFrançais
d’ExploitationduDroitdeCopie(CFC),20,ruedesGrands-Augustins,75006Paris.
Contents
Preface............................................................................................................... VII
Workshop participants..................................................................................... IX
List of authors................................................................................................... XI
SEIZURES AND SYNDROMES IN NEONATES
Foundationsofneonatalepileptology:classificationofseizuresandepilepsies
in the neonate and their etiology, electroencephalography, prognosis and
pathophysiology
EliM.Mizrahi,RonitM.Pressler................................................................... 1
EEG monitoring in neonatal seizures: what is done, what could be done?
SylvieNguyenTheTich.................................................................................. 17
Neuro-imaging in neonatal seizures
LaurenC.Weeke,LindaG.vanRooij,MonaC.Toet,FlorisGroenendaal,
LindaS.deVries............................................................................................ 27
Clinical and electrographical neonatal seizures: to treat or not to treat
LenaHellström-Westas.................................................................................. 39
Genetics of neonatal epilepsies and the forgotten epilepsy phenotypes
MariaRobertaCilio,MichaelS.Oldham......................................................... 49
Genetic basis of epileptic encephalopathy
LuciaFusco,DomenicoSerino,GiuliaBarcia,RimaNabbout......................... 61
Treatment of neonatal seizures with antiepileptic drugs
ChrysanthyIkonomidou................................................................................. 71
Neonatal seizures in hypoxic-ischaemic encephalopathy:
the impact of therapeutic hypothermia
EvonneLow,GeraldineB.Boylan.................................................................. 81
VI SeizuresandSyndromesofonsetintheTwoFirstYearsofLife
From conventional genetic analysis to next generation sequencing
in diagnostics of epilepsies
JohannesR.Lemke......................................................................................... 95
Treatable newborn seizures due to inborn errors of metabolism
JaumeCampistol,BarbaraPlecko................................................................... 105
Determining outcome from neonatal seizures
FrancescoPisani,CarlottaSpagnoli................................................................. 123
SEIZURES AND SYNDROMES IN INFANTS
The epidemiology of infancy onset seizures
ChristinEltze................................................................................................. 139
Concept of epileptic encephalopathy: is it relevant?
FedericoVigevano,NicolaSpecchio................................................................. 155
The role of EEG in the management of seizures in infancy
ThomasBast.................................................................................................. 163
Optimizing imaging in aiding management
WilliamDavisGaillard,MatthewWhitehead,JonathanMurnick..................... 171
“Benign” epilepsies in infants: are they always benign?
JulittadeBellescize,NicolaSpecchio,AlexisArzimanoglou.............................. 185
Fever-susceptibility syndromes. Predicting outcome
IngridE.Scheffer,RosemaryBurgess,ChristopherReid................................... 205
Infantile spasms semiology and pathophysiology: have we made progress?
DouglasR.NordliJr....................................................................................... 213
Trials for the treatment of infant seizures: theory, practice, ethics
and potential future trends
AndrewLux,FinbarJ.K.O’Callaghan.......................................................... 223
The role of ketogenic diet
GiangennaroCoppola..................................................................................... 235
Optimizing treatment for neonatal and infantile seizures –
Do guidelines have a role in management?
JoM.Wilmshurst,J.HelenCross................................................................... 249
Preface
Thereisaveryhighincidenceofseizuresduringthefirsttwoyearsoflife.Thismayreflect
multipleetiologiesdependingonthecircumstancesunderwhichseizuresoccur;sometimes
but not always seizures may lead to or herald epilepsy. In the immediate neonatal period,
seizures can occur in the presence of provoked insults such as hypoxia ischemia, vascular
events, traumatic injury changes in the extracellular milieu including toxic metabolic
causes and drug withdrawal, as well as infections. Genetics may play an important role in
the expression of acute seizures but also in the development of epilepsy later on. Genetic
abnormalities may also give rise to brain dysplasias.
Withinthefirsttwoyearsoflifethereisthefrequentonsetofdifficulttocontrolepilepsy
with often devastating consequences (i.e. West syndrome or Dravet syndrome), although
on other occasions the epileptic syndromes may have a more benign cause (i.e. benign
familial neonatal seizures).
Ongoing efforts are to understand how seizures may occur in the developing brain, their
consequences, the development of biomarkers, and effective treatments to promptly stop
ongoing seizures and alter the course of epileptic encephalopathies. The simplistic view
that seizures in the immature brain occur because of increased excitation and decreased
inhibition is no longer pertinent as multiple age-specific (and also sex-specific) factors
are intertwined, including changes in the conformation or function of channels, regional
variability in the expression of various neurotransmitters and patterns of connectivity
among these regions.
The topics presented in this volume address the questions raised above, and provide new
insights on how it is best to approach seizures and epilepsy in the first two years of life,
to systematically create a blueprint upon which diagnostic and treatment decisions can
be based. The data are highly reflecting the state of the art and also individualize for the
particular milieu of the patient in taking into account both nature (i.e. genetics), and
nurture (i.e. events that may interfere with normal development) and result in seizures
and epilepsy.
Wewouldliketothanktheauthorsfortheirkeenobservations,whichhelpmoveforward
the field.
Solomon L. Moshé and Alexis Arzimanoglou
