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Block VII : The Nervous System & Psychiatry

Module : Seizure
Course Period : Academic Year !" # !"$
%
th
Semester
Name : Stude&t
'uida&ce
(aculty o) Medici&e
Bra*i+aya ,&iversity
!"$
M-.,/ S0I1,20 !"$
ST,.0NT ',I.ANC0
Course Period : %
th
Semester
M-.,/0 : N02V-,S SYST0M & PSYC3IAT2Y
S,BM-.,/0: N02V-,S SYST0M
T-PIC : S0I1,20
"4 S,B5T-PICS :
1. Introducton
2. Neurophysoogy of Cerebra Cortex
3. Pathophysoogy of Sezure
4. Dfferenta dagnoss of Sezure
5. Investgaton n Epeptc Sezure
6. Sezure Management
4 C-NT2IB,T-2S
1. Nuru H, Department of Anatomy Hstoogy
2. Dan Hasanah, Department of Physoogy
3. Machus Husna, Department of Neuroogy
4. Nurdana, Department of Pharmacoogy
$4 C-MP0T0NCY A20A
Ths modue s a part of the eaboraton of
1. The area of competence 2 e. The Cnca Sk
2. The area of competence 3 e. The Scentfc-Base of Medca Scences .
3. The area of competence 4 e. The Management of Heath Probems
4. The area of competence 7 e. The Professonasm.
%4 C-MP0T0NCY C-MP-N0NT
1. The Cnca Sk : Neuroogca examnaton
2. The Scentfc-Base of Medca Scences : To appy the concepts and prncpe
of Bomedca Scences, Cnca Scences and Pubc Heath n appropate wth
Prmary Heath Care.
3. The area of competence 4 .e. Management of Heath Probems: To manage
the dseases, ness and patents probem as a ndvdua person, a part of
famy and communty and To prevent dseases and ness
4. The Professonasm: to have professona atttude
64 C/INICA/ C-MP0T0NC0
In a vgnette of a patent wth sezure, the student be abe to:
1. make a cnca dagnoss of sezure, to make smpe ads and addtona
nvestgaton requested by student hmsef ( such as smpe aboratory or
EEG).
2. Make a |udgement that an nta treatment s requred before beng referred
and descrbe to carry out an nta treatment and mmedatey refer to the
reevant specast (emergency cases).
74 /0A2NIN' -B80CTIV0S
At the end of the Teachng-Learnng Process of ths topc, n a vgnette of a
patent wth a sezure the student shoud be abe to:
1. Descrbe the anatomca structure that take part n sezure
2. Descrbe mechansm of sezure
3. Descrbe mechansm of epeptogeness
4. Descrbe the defnton sezure and epeptc sezure
5. Descrbe the cassfcaton of sezure and epeptc sezure
6. Descrbe the pathophysoogy of epeptc sezure
7. Descrbe the sgns and symptoms of epepsy
8. Descrbe the outne a practca approach to dagnoss and nta management
of sezure
9. Dfferentate between true sezure and psychogenc sezure
10.Demonstrate the neuroogca examnaton of sezure
11. Descrbe antepeptc drugs
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Ths topc s a part of Modue of The Nervous System ntegratedy desgned for
medca student of the 4
th
semester through Teachng-Learnng Process n the 7
th
Bock both n Lecture and Sma Group Dscusson. Ths part of Modue w
factate the student to have an understandng and approach to the patent wth
sezure.
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I4 I&troductio&
A4 .e)i&itio&s
A sezure (from the Latn sacire-to take possesson of) s the cnca manfestaton
of an abnorma, excessve, hypersynchronous dscharge of a popuaton of cortca
neurons. Epepsy s a dsorder of the centra nervous system characterzed by
recurrent sezures unprovoked by an acute systemc or neuroogc nsut.
Epeptogeness s the sequence of events that turns a norma neurona network nto
a hyperexctabenetwork. Recognzng the dstncton between sezures and
epepsy s essenta. Epepsy may requre chronc treatment (wth antepeptc
medcaton and, n some cases, surgery) whereas therapy for an soated sezure s
drected toward the underyng cause and may not requre antepeptc drugs
(AEDs). Furthermore, epepsy often has profound psychosoca ramfcatons for the
patent, and s thus a dagnoss to be assgned wth care.
B4 -vervie*
In order to understand the concepts of sezures, epepsy and epeptogeness, we
w frst consder some of the basc anatomc and eectrophysoogc propertes of
the cerebra cortex, and the factors that determne the eve of neura actvty at the
ceuar and ce network eve. We w then dscuss the physoogc bass of the
eectroencephaogram (EEG), routney used n assessng patents wth sezures and
other neuroogca dsorders. Fnay, we w address some of the man features of
the abnorma physoogca actvty that occurs wthn a sezure focus, and present a
few of the proposed mechansms that may undere certan sezure types.
II4 Neuro:hysiolo;y o) the Cere<ral Corte=
A4 Basic A&atomy o) Corte=
The human cerebra cortex conssts of 3 to 6 ayers of neurons. The phyogenetcay
odest part of the cortex (archpaum) has 3 dstnct neurona ayers, and s
exempfed by the hppocampus, whch s found n the meda tempora obe. The
ma|orty of the cortex (neocortex or neopaum) has 6 dstnct ce ayers and covers
most of the surface of the cerebra hemspheres. A partcuary mportant cortca
structure n the pathophysoogy of one of the more common epepsy syndromes s
the hppocampus. Ths structure s common n tempora obe epepsy. The
hppocampus conssts of three ma|or regons: subcuum, hppocampus proper
(Ammons horn) and dentate gyrus. The hppocampus and dentate gyrus have a
three ayered cortex. The subcuum s the transton zone from the three to the sx
ayered cortex. Important regons of the hppocampus proper ncude CA1, CA 2 ,
CA3. The cortex ncudes two genera casses of neurons. The pro|ecton, or
prncpa, neurons (e.g.,pyramda neurons) are ces that "pro|ect" or send
nformaton to neurons ocated n dstant areas of the bran. Interneurons (e.g.,
basket ces) are generay consdered to be oca-crcut ces whch nfuence the
actvty of nearby neurons. Most prncpa neurons form exctatory synapses on post-
synaptc neurons, whe most nterneurons form nhbtory synapses on prncpa
ces or other nhbtory neurons. Recurrent nhbton can occur when a prncpa
neuron forms synapses on an nhbtory neuron, whch n turn forms synapses back
on the prncpa ces to acheve a negatve feedback oop. (ustrates an exampe of
a type of nterneuron-granue ce and ts roe n a negatve feedback oop n the
hppocampus.)
Recent work suggests that some nterneurons appear to have rather extensve
axona pro|ectons,rather than the oca, confned axona structures prevousy
suggested. In some cases, such nterneurons may provde a very strong
synchronzaton or pacer actvty to arge groups of neurons.
B4 Basic Neuro:hysiolo;y a&d Neurochemistry 'over&i&;
0=cita<ility
Gven that the basc mechansm of neurona exctabty s the acton potenta, a
hyperexctabe state can resut from ncreased exctatory synaptc
neurotransmsson, decreased nhbtory neurotransmsson, an ateraton n votage-
gated on channes, or an ateraton of ntra- or extra-ceuar on concentratons n
favor of membrane depoarzaton. A hyperexctabe state can aso resut when
severa synchronous subthreshod exctatory stmu occur, aowng ther tempora
summaton n the post synaptc neurons. Acton potentas occur due to
depoarzaton of the neurona membrane, wth membrane depoarzaton
propagatng down the axon to nduce neurotransmtter reease at the axon termna.
The acton potenta occurs n an a-or-none fashon as a resut of oca changes n
membrane potenta brought about by net postve nward on fuxes. Membrane
potenta thus vares wth actvaton of gand- gated channes, whose conductance
s affected by bndng to neurotransmtters; or wth actvaton of votage-gated
channes, whose conductance s affected by changes n transmembrane potenta;
or wth changes n ntraceuar on compartmentazaton.
Neurotransmtters are substances that are reeased by the presynaptc nerve
termna at a synapse and subsequenty bnd to specfc postsynaptc receptors for
that gand. Lgand bndng resuts n channeactvaton and passage of ons nto or
out of the ces. The ma|or neurotransmtters n the bran aregutamate, gamma-
amno-butyrc acd (GABA), acetychone (ACh), norepnephrne, dopamne,
serotonn,and hstamne. Other moecues, such as neuropeptdes and hormones,
pay moduatory roes that modfy neurotransmsson over onger tme perods.
The ma|or exctatory neurotransmtter s the amno acd gutamate. There are
severa subtypes of gutamate receptors. Gutamate receptors can be found
postsynaptcay on exctatory prncpa ces as we as on nhbtory nterneurons,
and have been demonstrated on certan types of ga ces. The onotropc
subcasses are the apha-amno-2,3-dhydro-5-methy-3-oxo-4-soxazoepropanoc
acd (AMPA), kanatereceptors, and N-methy-D-aspartate (NMDA); these aow on
nfux upon actvaton by gutamate . They are dfferentated from one another by
caton permeabty as we as dfferenta senstvty to pharmacoogca
agonsts/antagonsts. A onotropc gutamate receptors are permeabe to Na+ and
K+, and t s the nfux of Na+ and outfow of K+ through these channes that
contrbute to membrane depoarzaton and generaton of the acton potenta. The
NMDA receptor aso has a Ca++ channe that sbocked by Mg++ ons n the restng
state, but under condtons of oca membrane depoarzaton, Mg++ s dspaced
and the channe becomes permeabe to Ca++; nfux of Ca++ tends to further
depoarze the ce, and s thought aso to contrbute to Ca++ medated neurona
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n|ury under condtons of excessve neurona actvaton (such as status epeptcus
and schema), potentay eadng to ce death, a process termed exctotoxcty. The
other ma|or type of gutamate receptor s the metabotropc receptor, whch
functons by means of receptor-actvated sgna transducton nvovng membrane-
assocated G-protens . There are at east 3 subtypes of metabotropc receptors,
based on dfferenta agonst potency, mechansm of sgna transducton, and pre-
versus post-synaptc ocazaton.
Expermenta studes usng anma epepsy modes have shown that NMDA, AMPA
and kante agonsts nduce sezure actvty, whereas ther antagonsts suppress
sezure actvty. Metabotropc agonsts appear to have varabe effects key
dependent upon ther dfferent ocaton and mechansms of sgna transducton. The
ma|or nhbtory neurotransmtter, GABA, nteracts wth 2 ma|or subtypes of
receptor: GABAA and GABAB receptors. GABAA receptors are found
postsynaptcay, whe GABAB receptors are found presynaptcay, and can thereby
moduate synaptc reease. In the adut bran, GABAA receptors are permeabe to C-
ons; upon actvaton C- nfux hyperpoarzes the membrane and nhbts acton
potentas. Therefore, substances whch are GABAA receptor agonsts, such as
barbturates and benzodazepnes, are we known to suppress sezure actvty.
GABAB receptors are assocated wth second messenger systems rather than C-
channes, and ead to attenuaton of transmtter reease due to ther presynaptc
ocaton. The second messenger systems often resut n openng of K+ channes,
eadng to a hyperpoarzng current. Certan GABAB agonsts, such as bacofen,
have been reported to exacerbate hyperexctabty and sezures.
Reevant to epepsy, gutamate and GABA both requre actve reuptake to be
ceared from the synaptc ceft. Transporters for both gutamate and GABA exst on
both neurons and ga (prmary astrocytes). Interference wth transporter functon
has aso been shown to actvate or suppress epeptform actvty n anma modes,
dependng on whch transporter s beng bocked.
C4 (actors 'over&i&; 0=cita<ility o) I&dividual Neuro&s
The compexty of neurona actvty s party due to varous mechansms controng
the eve of eectrca actvaton n one or more ceuar regons. These mechansms
may act nsde the neuron or n the ceuar envronment, ncudng other ces (e.g.,
neghborng neurons, ga, and vascuar endothea ces) as we as the extraceuar
space, to modfy neurona exctabty. The former may be termed "neurona" or
"ntrnsc," and the atter "extra-neurona" or "extrnsc."
"4 0=am:les o) &euro&al >i&tri&sic? )actors i&clude:
The type, number and distribution of voltage- and ligand-gated channels. Such
channes determne the drecton, degree, and rate of changes n the
transmembrane potenta, whch n turn determne whether an acton potenta
occurs. Votage-gated sodum channes, for exampe, form the bass of the rapd
depoarzaton consttutng the acton potenta. Among gand-gated channe THE
GABA receptor compex medates nfow of chorde ons whch hyperpoarze the
ce, formng the bass of neurona nhbton, as descrbed prevousy.
Biochemical modification of receptors. For exampe, phosphoryaton of the NMDA
receptor ncreases permeabty to Ca++, resutng n ncreased exctabty.
Activation of second-messenger systems. Bndng of norepnephrne to ts apha
receptor, for exampe, actvates cycc GMP, n turn actvatng G-protens whch open
K+ channes, thereby decreasng exctabty.
Modulating gene expression, as by RNA editing. For exampe, edtng a snge base
par of mRNA encodng a specfc gutamate receptor subunt can change the on
seectvty of the assembed channe.
4 0=am:les o) e=tra5&euro&al >e=tri&sic? )actors i&clude:
hanges in extracellular ion concentration due to variations in the volume of the
extracellular space. For exampe, decreased extraceuar voume eads to ncreased
extraceuar K+concentraton,resstng the outward movement of K+ ons needed
to repoarze the ce, thereby effectvey ncreasng exctabty.
Remodeling of synaptic contacts. For exampe, movement of an afferent axon
termna coser to the target ce body ncreases the kehood that nward onc
currents at the synapse w brng thetarget neuron to threshod. The coupng
between the pre- and post-synaptc eements can be made more effcent by
shortenng of the spne neck. In addton, prevous synaptc experence such as a
bref burst of hgh frequency stmuaton (e.g., ong-term potentaton-LTP) aso
ncreases the effcacy of such synapses, ncreasng ther exctabty.
Modulating transmitter metabolism by glial cells. Exctabty ncreases, for
exampe, f ga
metabosm or uptake of exctatory transmtters such as gutamate or ACh
decreases.
.4 3o* Net*ork -r;a&izatio& I&)lue&ces Neuro&al 0=cita<ility
Neurons are connected together n eaborate arrays that provde addtona eves of
contro of
neurona exctabty. An exampe of a very basc neurona network s the we-
studed dentate gyrus and hppocampus. In the dentate gyrus, afferent connectons
to the network can drecty actvate the pro|ecton ce (e.g., granue ces). The nput
can aso drecty actvate oca nterneurons (bpoar and basket ces), and these
may nhbt pro|ecton ces n the vcnty (feed-forward nhbton). Aso, the
pro|ecton neuron may n turn actvate the nterneurons whch n turn act on the
pro|ecton neurons (feedback nhbton). Thus, changes n the functon of one or
more ces wthn a crcut can sgnfcanty affect both neghborng and dstant
neurons. For exampe, sproutng of exctatory axons to make more numerous
connectons can ncrease exctabty of the network of connected neurons.
Aternatvey, oss of nhbtory neurons w aso ncrease the exctabty of the
network. Inhbtory functon can aso be reduced by a oss of exctatory neurons that
actvate or "drve" the nhbtory neurons.
III4 Patho:hysiolo;y o) Seizures: A& Alteratio& i& the
Normal Bala&ce o) I&hi<itio& a&d 0=citatio&
A4 Basic Mecha&isms o) (ocal Seizure I&itiatio& a&d Pro:a;atio&
The hypersynchronous dscharges that occur durng a sezure may begn n a very
dscrete regon of cortex and then spread to neghborng regons. Sezure ntaton
s characterzed by two concurrent events:
1) hgh-frequency bursts of acton potentas, and 2) hypersynchronzaton of a
neurona popuaton. The synchronzed bursts from a suffcent number of neurons
resut n a so-caed spke dscharge on the EEG. At the eve of snge neurons,
epeptform actvty conssts of sustaned neurona depoarzaton resutng n a
burst of acton potentas, a pateau-ke depoarzaton assocated wth competon
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of the acton potenta burst, and then a rapd repoarzaton foowed by
hyperpoarzaton. Ths sequence s caed the paroxysma
depoarzng shft. The burstng actvty resutng from the reatvey proonged
depoarzaton of the neurona membrane s due to nfux of extraceuar Ca++,
whch eads to the openng of votage-dependent Na+ channes, nfux of Na+, and
generaton of repettve acton potentas. The subsequent hyperpoarzng
afterpotenta s medated by GABA receptors and C- nfux, or by K+ effux,
dependng on the ce type.
Sezure propagaton, the process by whch a parta sezure spreads wthn the bran,
occurs when there s suffcent actvaton to recrut surroundng neurons. Ths eads
to a oss of surround nhbton and spread of sezure actvty nto contguous areas
va oca cortca connectons, and to more dstant areas va ong assocaton
pathways such as the corpus caosum.
The propagaton of burstng actvty s normay prevented by ntact
hyperpoarzaton and a regon of surroundng nhbton created by nhbtory
neurons. Wth suffcent actvaton there s a recrutment of surroundng neurons va
a number of mechansms. Repettve dscharges ead to:
1) an ncrease n extraceuar
K+, whch bunts the extent of hyperpoarzng outward K+ currents, tendng to
depoarze neghborngneurons;
2) accumuaton of Ca++ n presynaptc termnas, eadng to enhanced
neurotransmtter reease; and
3) depoarzaton-nduced actvaton of the NMDA subtype of the exctatory amno
acd receptor, whch causes more Ca++ nfux and neurona actvaton. Of equa
nterest, but ess we understood, s the process by whch sezures typcay end,
usuay after seconds or mnutes, and what underes the faure of ths
spontaneous sezure termnaton n the fe-threatenng condton known as status
epeptcus.
B4 Curre&t Theories as to 3o* I&hi<itio& a&d 0=citatio& Ca& Be
Altered at the Net*ork /evel
Our understandng of the CNS abnormates causng patents to have recurrent
sezures remans mted. It s mportant to understand that sezures and epepsy
can resut from many dfferent pathoogc processes that upset the baance between
exctaton and nhbton. Epepsy can resut from processes whch dsturb
extraceuar on homeostass, ater energy metabosm, change receptor functon,
or ater transmtter uptake. Despte ma|or dfferences n etoogy, the outcome of
synchronous burstng of cortca neurons may superfcay appear to have a smar
phenotype. Sezure phenotype may be modfed more by the ocaton and functon
of the neurona network recruted nto the synchronous burstng than by the
underyng pathophysoogy. Because of the we organzed and reatvey smpe
crcuts wthn the entorhna-dentate-hppocampa oop, the mbc system has been
ntensvey studed n expermenta modes of epepsy. These nvestgatons have
ed to two theores regardng the ceuar network changes whch cause the
hppocampus, among the most common stes of orgn of parta sezures, to become
hyperexctabe. The frst proposes that a seectve oss of nterneurons decreases
the norma feed-forward and feedback nhbton of the dentate granue ces, an
mportant group of prncpa neurons. The other theory suggests that synaptc
reorganzaton foows n|ury and creates recurrent exctatory connectons, va
axona "sproutng," between neghborng dentate granue ces. More recenty, t has
been proposed that the oss, rather than beng of GABAergc nhbtory neurons, s
actuay of exctatory neurons whch normay stmuate the nhbtory nterneurons
to, n turn, nhbt the dentate granue ces. These mechansms of hyperexctabty
of the neurona network are not mutuay excusve, coud act synergstcay, and
may coexst n the human epeptc bran. Sezures may aso appear to arse from
wdespread cortca areas vrtuay smutaneousy. The mechansms
underyng such generazed sezures are uncertan. One type of generazed sezure,
the absence sezure, (aso caed pett ma) s a generazed sezure consstng
cncay of a bref starng spe n con|uncton wth a characterstc burst of spke-
wave compexes on the EEG. Generazed spkewave dscharges n absence sezures
may resut from aberratons of oscatory rhythms that are normay generated
durng seep by crcuts connectng the cortex and thaamus. Ths oscatory
behavor nvoves an nteracton between GABAB receptors, Ca++ channes and K+
channes ocated wthn the thaamus. Pharmacoogc moduaton of these receptors
and channes can nduce absence sezures, and there s specuaton that genetc
forms of absence epepsy may be assocated wth mutatons of components of ths
system.
C4 0:ile:to;e&esis: The Tra&s)ormatio& o) a Normal Net*ork I&to a
3y:ere=cita<le Net*ork
Cnca observatons suggest that certan forms of epepsy are caused by partcuar
events. For exampe, approxmatey 50% of patents who suffer a severe head n|ury
w deveop a sezure dsorder. However, n a sgnfcant number of these patents,
the sezures w not become cncay evdent for months or years. Ths "sent
perod" after the nta n|ury ndcates that n some cases the epeptogenc process
nvoves a gradua transformaton of the neura network over tme. Changes
occurrng durng ths perod coud ncude deayed necross of nhbtory
nterneurons (or the exctatory nterneurons drvng them), or sproutng of axona
coateras eadng to reverberatng, or sef-renforcng, crcuts. In the future,
patents at rsk for deveopng epepsy due to an acqured eson may beneft from
treatment wth "ant-epeptogenc" compounds that coud prevent these network
changes.
An mportant expermenta mode of epeptogeness s kndng, dscovered by
Goddard and coworkers n the 1960s. Day, subconvusve stmuaton (eectrca or
chemca) of certan bran regons such as the hppocampus or amygdaa resut n
eectrca afterdscharges, eventuay eadng to stmuatonnducedcnca sezures,
and n some nstances, spontaneous sezures. Ths change n exctabty s
permanent and presumaby nvoves ong-astng bochemca and/or structura
changes n the CNS. A varety of changes have been measured n kndng modes,
ncudng ateratons n gutamate channe propertes, seectve oss of neurons, and
axona reorganzaton. However, the exact mechansms underyng kndng, and ts
appcabty to human epeptogeness, reman unknown
IV4 .i))ere&tial dia;&osis o) e:ile:sy i& adults a&d childre&
"4 .i))ere&tial dia;&osis o) e:ile:sy i& adults
Epeptc sezures need to be consdered n the dfferenta dagnoss of a range of
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cnca presentatons. These are prncpay:
Loss of awareness
Generased convusve movements
Drop attacks
Transent foca motor attacks
Transent foca sensory attacks
Faca musce and eye movements
Psychc experences
Aggressve or voca outbursts
Epsodc phenomena n seep
Proonged confusona or fugue states
The prncpa dfferenta dagnoses for each presentng cnca scenaro foow wth
bref expanatory text the key dagnostc features of each dagnoss. It s not
uncommon for a patent to come to medca attenton after a dramatc event, but
not to do so after mnor epsodes. Understandng the occurrence and nature of
mnor events s cruca to makng an accurate dagnoss. A checkst of symptoms to
specfcay enqure for s gven n Tabe 1.
Have there been any spontaneous and otherwse unexpaned paroxysma symptoms?
In partcuar:
Sudden fas
Invountary |erky movements of mbs whst awake
Bank spes
Unexpaned ncontnence of urne wth oss of awareness, or n seep
Odd events occurrng n seep, e.g. fa from bed, |erky movements, automatsms
Epsodes of confused behavour wth mpared awareness, recoecton
Possbe smpe parta sezures
Epgastrc rsng sensaton
D| vu
Premonton
Fear
Eaton, Depresson
De-personazaton, dereazaton
Inabty to understand or express anguage (wrtten or spoken)
Loss of memory, dsorentaton
Ofactory, gustatory, vsua, audtory haucnaton
Foca motor or Somatosensory defct, or postve symptoms (|erkng, tngng).
Ta<le " Checklist o) :ossi<le seizure5related sym:toms to e&Buire )or *he&
co&sideri&; a :ossi<le dia;&osis o) e:ile:sy
1.1 Principal differential diagnoses
"4"4" /oss o) a*are&ess
Whatever the cause the patent may have amnesa for both the event and ts exact
crcumstances. The three man causes are: syncope, epepsy, and cardac
arrhythmas. Transent cerebra schaema due to vascuar abnormates s ess
common. Mcroseeps (very short daytme naps) may occur wth any cause of severe
seep deprvaton or dsrupton. Other causes of dagnostc confuson are much rarer
and ncude: hypogycaema or other ntermttent metaboc dsorders, structura
anomaes of the sku base affectng the branstem, or esons affectng the CSF
crcuaton.
Syncope
Syncope s the commonest cause of epsodes of oss of awareness. Smpe
fants or vasovaga syncopa attacks can usuay be reated to dentfabe
precptants. Most often they occur on gettng up qucky, or standng for
proonged perods, partcuary f assocated wth perphera vasodaton (e.g.
durng hot, stuffy weather, crowded trans or rooms, or are reated to drug or
acoho use). Frghtenng, emotona or unpeasant scenes, and panfu stmu
may aso be trggers, due to ncreased vaga actvty. There are varous other
causes of syncopa attacks, and cassfcaton depends on termnoogy. Cough
and mcturton syncope are we recognsed. Changes n ntrathoracc
pressure (cough syncope), mpared baroreceptors due to atheroma
of the carotd (carotd snus syncope), cardac arrhythmas, or autonomc
dsturbances may aso ead to cerebra hypoperfuson and fantng. As these
may not be due to vasovaga refex changes, the typca aura of a vasovaga
syncope may not be present.
Epepsy
Severa types of sezure may present wth oss of awareness as the soe
reported feature. These ncude absences, compex parta, tonc or atonc
sezures. Typca absences nvove arrest of actvty, reduced or ost
awareness, eyed bnkng or twtchng, and sometmes sma myoconc faca
or mb |erks, or bref faca automatsms such as p smackng or chewng.
Typca absences are usuay bref but often occur many tmes per day. There
may aso be soated myoconc |erks. Atonc sezures usuay gve rse to drop
attacks but may appear to cause bank spes f the patent s sat or ayng
down and so cannot fa. Compex parta sezures may cause oss of
awareness wth few f any other features. Detaed enqury must aways be
made for any assocated psychc or motor phenomena that may rase the
possbty of a sezure dsorder.
Cardac dsorders
There are often prodroma features smar to smpe syncope, as we as
paptatons, chest pan, shortness of breath or other features of
cardovascuar nsuffcency. Attacks due to transent compete heart bock
are abrupt and short wth rapd oss of conscousness. Lack of cardac output
may be due to short epsodes of ventrcuar tachycarda or fbraton.
Proongaton of the OT nterva may ead to such events. Attacks may be
preceded by paptatons, extreme fatgue or presyncopa features. Mtra
vave proapse and aortc stenoss may present wth epsodc oss of
awareness due to fuctuatng cardac output or assocated arrhythmas. Aortc
stenoss and hypertrophc cardomyopathy s especay prone to present wth
epsodes of sudden coapse wth oss of awareness durng exercse.
Mcroseeps
Any cause of seep deprvaton may ead to bref day-tme naps, sometmes
astng for ony a few seconds. Impared quaty of seep may aso be a factor.
The most mportant s obstructve seep apnoea. Narcoepsy can present wth
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short perods of suddeny fang aseep durng the day.
Panc attacks
Panc attacks usuay present wth feengs of fear and anxety, assocated
wth autonomc changes and hyperventaton. Ths eads to dzzness or
ghtheadedness, orofaca and/or perphera paraesthesa (whch may be
asymmetrc), carpopeda spasm, twtchng of the perpheres, burred vson,
or nausea. Occasonay these preudes may be forgotten, and attacks present
wth oss of awareness. Often, but not aways, there s a cear precptant,
such as a partcuar stuaton. None of these features are consstent, however,
and dfferentaton from epepsy can be dffcut.
Hypogycaema
Hypogycaemc attacks causng oss of conscousness are extremey rare
except n patents wth treated dabetes metus. Very occasona cases may
be seen due to nsun secretng tumours. In such cases there may be a
hstory of a mssed mea pror to the attack.
Other neuroogca dsorders
If a head n|ury causes oss of conscousness, there s amnesa. In accdenta
head n|ury, partcuary road traffc accdents, t may be dffcut to
dstngush amnesa caused by the n|ury from cases n whch there was a oss
of conscousness that caused the accdent. Isoated epsodes of oss of
awareness may aso be caused by abuse of psychotropc drugs or other
substances. Occasonay, structura CNS abnormates may present wth
epsodes of oss of awareness.
Non-epeptc attack dsorder (NEAD)
Non-epeptc attack dsorder, prevousy known as pseudosezures typcay
gves rse epsodes of two broad types:
(a) attacks nvovng motor phenomena
(b) attacks of yng motoness.
The atter are often proonged, contnung for severa mnutes or sometmes hours.
Such behavour s very rare n epeptc sezures: there w neary aways be other
postve phenomena n epeptc attacks that ast for more than a few mnutes. In
addton, attacks are often trggered by externa events or stress. Patents wth
NEAD often have a hstory of abnorma ness behavour. Non-epeptc attack
dsorder s much commoner n femaes than maes, and usuay commences n
adoescence or eary aduthood (see Tabe 2).
Epeptc attack NEAD
Precptatng cause Rare Common, emotona & stress reated
When aone or aseep Common May be reported
Onset Usuay short May be short or over severa mnutes
Aura Varous, usuay stereotyped Fear, panc, atered menta state
Speech Cry, grunt at onset; mutterng, words
n automatsms Sem-vountary, often unntegbe
Movement Atonc, tonc; f conc, Asynchronous fang of mbs; pevc
synchronoussma amptude |erks thrustng; opsthotonous
In|ury Tongue btng, fa; drected voence May bte tongue, cheeks, p, hands,
Rare throw sef to ground. Drected
voence not uncommon
Conscousness Compete oss n generazed tonc Varabe, often nconsstent
wth
conc; may be ncompete n compexs sezure type
parta
Response to stmuatonNone n generazed tonc-conc; may Often reacts and ths may termnate
respond n compex parta and post epsode
ctay
Incontnence Common Sometmes
Duraton Few mnutes Few mnutes, may be proonged.
Recovery Depends on sezure type. Few May be rapd or very proonged
mnutes and more proonged
confuson
Ta<le .i))ere&tiatio& o) e:ile:tic seizures a&d &o&5e:ile:tic attack disorder
>N0A.?
"4"4'e&eralised co&vulsive moveme&ts
Epepsy
A generased convuson s generay the most ready dagnosed epeptc
casscay, there s a cry, generased stffenng of body and mbs, foowed
by rhythmc |erkng of a four mbs, assocated wth oss of awareness,eyes
starng banky, tongue btng and urnary ncontnence. The generazed
convusve movements usuay ast for a mnute or so, and as the attack
proceeds the |erkng sows n frequency and ncreases n amptude. There s
often cyanoss, and afterwards rreguar breathng foowed by confuson,
headache and seepness.
Syncope wth secondary |erkng movements
Peope who fant often have sma, bref myoconc twtches of the
extremtes. Wth proonged cerebra hypoperfuson these may be more
promnent, and be reported as "a convuson". The myoconc |erkng s
usuay rreguar and short ved.
Prmary cardac or respratory abnormates presentng wth secondary
anoxc sezures
Epsodes of compete heart bock may have syncopa features foowed by
coapse and secondary anoxc sezures. Usuay the attacks ast for ess than
one mnute.
Invountary movement dsorders and other neuroogca condtons
There s no ateraton n conscousness. The best known s paroxysma
knesogenc choreoathetoss. Attacks are usuay precptated by sudden
specfc movements. They ast a few seconds to mnutes. Paroxysma
dystona presents wth attacks astng for mnutes to hours. Patents wth
known nvountary movement dsorders such as dopathc torson dystona
may show severe acute exacerbatons mmckng convusve movements.
Patents wth earnng dsabtes often have stereotyped or repettve
movements, whch may ncude head bangng or body rockng, and more
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subte movements whch may be dffcut to dfferentate from compex parta
sezures.
Hyperekpexa
Attacks are charactersed by excessve starte, may cause stffenng, and
coapse wth a sudden |erk of a four mbs. Attacks are provoked by sudden
unexpected stmu, usuay audtory. Hyperekpexa needs to be dstngushed
from sezures nduced by starte.
Non-epeptc attack dsorder (NEAD)
Non-epeptc attacks nvovng promnent motor phenomena are commoner
than those wth arrest of actvty. Movements are vared but often nvove
sem purposefu thrashng of a four mbs, waxng and wanng over many
mnutes, dstractbty or nteracton wth the envronment, promnent pevc
movements and back archng (Tabe 2). Non epeptc attacks may be dffcut
to dfferentate from compex parta sezures of fronta obe orgn, whch can
present wth very bzarre motor attack
"4"4$ .ro: attacks
Any cause of oss of awareness may proceed to a sudden coapse or drop attack.
Epepsy, syncope and other cardovascuar dsorders are the commoner causes of
drop attacks.
Epepses
Sudden drop attacks are common n patents wth earnng dsabtes and
secondary generased epepses. The fas may be tonc or atonc.
Cardovascuar
If cerebra hypoperfuson s suffcent to cause sudden coapse there s
usuay oss of awareness (see above). Syncope and cardac abnormaty are
rare causes of a presentaton wth drop attacks.
Movement dsorders
Most movement dsorders that cause drop attacks have other more
promnent features whch make the dagnoss cear (e.g. Parknson's dsease).
Paroxysma knesogenc choreoathetoss may cause drop attacks f there s
ower mb nvovement.
Branstem, spna or ower mb abnormates
There are usuay fxed neuroogca sgns. Tumours of the thrd ventrce may
present wth sudden epsodes of coapse. Spna cord vascuar abnormates
may present wth ower mb weakness eadng to fas wthout mparment of
awareness.
Catapexy
Catapexy usuay occurs n assocaton wth narcoepsy, athough t may be
the presentng cnca feature. There s no oss of conscousness wth attacks.
Attacks may be precptated by emoton, especay aughter. Often
there s ony oss of tone n the neck musces, wth sumpng of the head
rather than compete fas.
Metaboc dsorders
Perodc parayss due to sudden changes n serum potassum s rare. The
condton may be fama or assocated wth other endocrne dsorders or
drugs. Usuay there s a gradua onset, and the attacks ast for hours.
Idopathc drop attacks
These attacks are most common n mdde aged femaes. They take the form
of a sudden fa wthout oss of conscousness. Characterstcay the patents
remember fang and httng the ground. Recovery s nstantaneous, but
n|ury may occur.
Vertebrobasar schaema
Ths condton s over dagnosed and probaby accounts for very few drop
attacks. Typcay, the attacks occur n the edery, wth evdence of vascuar
dsease and cervca spondyoss. The attacks may be precptated by head
turnng or neck extenson resutng n dstorton of the vertebra arteres and
are of sudden onset, wth features of branstem schaema such as dpopa,
vertgo, and batera faca and mb sensory and motor defcts
"4"4% Tra&sie&t )ocal motor attacks
The commonest cause of transent foca motor attacks s epepsy. Tcs may deveop
n adoescence. Paroxysma movement dsorders are rare, athough unatera
paroxysma knesogenc choreoathetoss may mmc motor sezures. Transent
cerebra schaema usuay presents wth negatve phenomena. Tonc spasms of
mutpe sceross are usuay seen once other features of the ness have become
apparent, but may be apresentng feature.
Foca motor sezures
Foca motor sezures may nvove |erkng and posturng of one extremty, or
refect the spread of epeptc actvty aong the prmary motor cortex. There
s often assocated paraesthesa. There may be ocased transent weakness
foowng the attack for seconds or mnutes, sometmes onger. Sezures
arsng n many dfferent bran regons may cause dystonc posturng.
Epepsa partas contnua s a rare form of epepsy that often causes
dagnostc confuson. There s very frequent foca motor actvty such as
|erkng of the hand. Ths can persst for hours or days, contnue nto seep,
and may go on for years. The movements often become sow and penduous,
wth some assocated dystonc posturng.
Tcs
Tcs usuay present wth stereotyped movements n chdhood or
adoescence, sometmes restrcted to one partcuar acton (e.g. eye bnk)
but may be mutpe n nature. Tcs may be confused wth myoconc |erks.
They can be suppressed vountary, athough to do so eads to a rse n
psychoogca tenson and anxety that s then reeved by the patent aowng
the tcs to occur. Repettve tcs and stereotypes are partcuary common n
those wth nteectua dsabty.
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Transent cerebra schaema
Transent schaemc attacks (TIAs) usuay present wth negatve phenomena,
.e. oss of use of a mb, hempega or other defcts, athough postve
phenomena such as paraesthesae may occur. Transent schaemc attacks
may ast for a few mnutes, but may persst for up to 24 hours. TIAs are not
usuay stereotyped or repeated wth the frequency of epeptc sezures, and
there are usuay assocated features to suggest vascuar dsease.
Tonc spasms of mutpe sceross
These spasms usuay occur n the settng of known mutpe sceross, but
may be the presentng feature, athough other evdence of mutpe sceross
may be found on examnaton and nvestgaton. The spasms may ast for
severa seconds, sometmes onger than one mnute.
Paroxysma movement dsorders
Paroxysma knesogenc choreoathetoss may present wth foca motor
attacks that are very smar to epeptc events. Tremor may occur n a
varety of movement dsorders and s usuay suffcenty persstent to
eucdate the nonepeptc nature, but may be dffcut to dstngush from
certan forms of epepsa partas contnua. Myoconus of subcortca orgn
may be suspected from the dstrbuton of nvoved musces (e.g. spna
myoconus may be restrcted to specfc segments, ether unatera or
batera). Perphera nerve entrapment usuay presents wth weakness but
occasonay can present wth epsodc |erks or twtches.
"4"46 Tra&sie&t )ocal se&sory attacks
Somatosensory attacks
Epeptc sezures nvovng the prmary sensory cortex are ess common than
motor sezures, and may cause spreadng paraesthesa. Sezures nvovng
the second sensory areas or mesa fronta cortex may cause sensory
usons. There are usuay other epeptc features due to nvovement of
ad|acent or reated bran structures. Transent sensory phenomena may aso
be seen n perphera nerve compresson or other abnormates of the
ascendng sensory pathways, hyperventaton or panc attacks and n TIAs.
TIAs are not usuay stereotyped or repeated wth the frequency of epeptc
sezures, and there are usuay assocated features to suggest vascuar
dsease.
Lesons of sensory pathways cause persstent symptoms, but dagnostc
confuson may arse n the eary natura hstory, when compants are
ntermttent, or f they are posture reated. Hyperventaton may be
assocated wth ocased areas of paraesthesa (e.g. one arm). Intermttent
sensory usons may be experenced n reaton to amputated or anaesthetc
mbs. Mgranous epsodes may aso cause ocased areas of paraesthesa,
but usuay have the dstncton of a gradua evouton of sensory phenomena,
both postve and negatve, and assocated features of mgrane.
Transent vestbuar symptoms
Acute attacks of vertgo may occasonay be due to a sezure n pareta or
tempora obes. In these cases there are generay assocated features that
pont to cerebra nvovement, such as a foca somatosensory symptoms, de|a
vu or dsordered percepton. Perphera vestbuar dsease s a much more
common cause and may gve rse to paroxysma rotatona vertgo and
percepton of near moton and there are often aso other symptoms of
audtory and vestbuar dsease such as: deafness, tnntus, pressure n the
ear and reaton to head poston.
Vsua symptoms
Mgrane s a common cause of epsodc vsua phenomena. The evouton s
usuay gradua, over severa mnutes, wth fortfcaton spectra, and
assocated photophoba, nausea and headache. Epeptc phenomena are
usuay much shorter, evovng over seconds, and the vsua haucnatons are
more commony of cooured bobs, rather than |agged nes.
"4"47 (acial muscle a&d eye moveme&ts
Changes n faca movements may occur n varous neuroogca condtons ncudng
foca motor sezures, compex parta sezures wth automatsms, tcs, dystonas or
other
paroxysma movement dsorders, especay drug nduced dysknesas and
hemfaca spasm, as we as psychoogca dsorders.
Parta sezures
Bengn roandc epepsy usuay presents wth sezures n chdhood affectng
the
face, often wth unatera grmacng, hemcorporea sensory and motor
phenomena, or secondary generased sezures occurrng n seep. Foca
motor
sezures may cause twtchng of one sde of the face that may be restrcted to
specfc areas. Eye devaton may be seen wth sezures arsng n fronta,
pareta or occpta cortex.
Compex parta sezures may cause automatsms wth p smackng, chewng,
swaowng, snffng or grmacng, wth amnesa and mpared awareness. If
these
features are due to sezure actvty the attacks are usuay reatvey
nfrequent, whereas wth dystona or other movement dsorders epsodes are
key to occur many tmes per day.
Movement dsorders
Hemfaca spasm typcay presents n the edery or mdde aged wth
custers of
attacks that ntay nvove the eye but subsequenty spread to the rest of
that sde of the face. Faca weakness may deveop that perssts between
attacks.
Bruxsm may occur ether durng the day or n seep, especay n chdren
wth earnng dsabty. Epsodes are usuay more proonged than wth the
automatsms of compex parta sezures, and there are no assocated
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features to
suggest an epeptc bass.
As wth dystona and other movement dsorders affectng the face there may
be evdence of nvovement esewhere, and attacks are usuay more frequent
than s seen wth soated sezures.
Other neuroogca dsorders
Defects of eye movement contro are common n patents wth a wde range
of neuroogca dsorders. There are usuay assocated features that ndcate
a nonepeptc bass. Bzarre eye movements aso occur n bndness and may
be mstaken for epeptc actvty. Carefu examnaton s requred to ascertan
the precse features of the eye movement dsorder, and n partcuar any
precptatng factors or features of cerebear or branstem dsease.
"4"49 Psychic e=:erie&ces
Intermttent psychc phenomena can be seen n parta sezures (especay of
tempora obe orgn), mgrane, panc attacks, transent cerebra schaema, drug
nduced fashbacks, or wth usons assocated wth oss of a sensory modaty as
we as psychotc nesses.
Epepsy
Parta sezures of tempora obe orgn are especay key to be manfest by
auras nvovng psychc phenomena. The most common are fear, de|a vu,
memory fashbacks, vsua, ofactory or audtory haucnatons. Other
manfestatons ncude atered percepton of the envronment wth a
dstancng
from reaty or change n sze or shape of ob|ects; atered anguage functon;
emotons such as sadness, eaton, and sexua arousa.
Psychc experences may have some reaton to past experences. They are
usuay recaed as bref scenes, sometmes strung together. They usuay ack
carty, for exampe a patent may descrbe an uson of someone standng n
front of them who they know, but they cannot name them or descrbe them n
deta. A rsng epgastrc sensaton may occur aone or n assocaton wth
such experences. Eementa vsua phenomena, such as fashng ghts, are
more often seen n occpta obe epepsy. Atered thought patterns may be
seen n both tempora and fronta obe sezures.
Mgrane
Mgranous psychc phenomena may nvove an nta heghtenng of
awareness. The prncpa features are usuay vsua usons that may be
eementa or compex. They rarey have the same ntense emotona
components of tempora obe usons or haucnatons. The tme course s
usuay more proonged than wth parta sezures, and there are assocated
features of a poundng headache, photophoba and nausea or vomtng. There
may be recognzed precptants, and there s often a reevant famy hstory.
Panc attacks
These are usuay assocated wth feengs of fear and anxety.
Hyperventaton may ead to dzzness and ght-headedness. There are often
unpeasant abdomna sensatons smar to the epgastrc aura of parta
sezures. The evouton, assocated ncreases n heart rate and respraton,
onger tme course and hstory of precptatng factors generay make the
dagnoss cear.
Drug nduced fashbacks
These share many of the quates of psychc tempora obe sezures. They are
ndvduased haucnatons usuay reated to the crcumstances of the drug
abuse, often wth emotona content of fear or anxety. A carefu hstory
shoud be taken for substance abuse, especay LSD and mescane.
Haucnatons or usons caused by oss of a prmary sense
Haucnatons and usons of an absent mb are we recognsed n
amputees. Smary, peope who ose sght ether n the whoe or part fed
may experence vsua haucnatons or usons n the bnd fed. Such
phenomena can be eementa or compex and ncude evovng scenes.
Smar experences can occur wth deafness.
Such experences due to the oss of a prmary sense present partcuar
dagnostc dffcuty when they occur n the settng of a structura eson whch
coud resut n both phenomena. An occpta nfarcton, for exampe, coud
cause vsua oss and coud aso gve rse to epeptc sezures. Often the
haucnatons due t sensory oss are more proonged, astng for mnutes or
hours, but can be bref.
Psychotc haucnatons and deusons
Haucnatons and deusons are the hamark of psychotc nesses. The
foowng features woud suggest a psychatrc rather than epeptc bass:
compex nature wth an evovng or argued theme, audtory nature nvovng
nstructons or thrd person anguage, paranod content or assocated thought
dsorder. Psychotc epsodes are usuay more ong astng than soated
epeptc sezures, athough ntermttent psychoss may have a smar tme
course to nonconvusve status.
Persstent mood changes may be a hepfu gude, but even short tempora
obe sezures may be foowed by mood changes astng for hours or days.
Furthermore, furres of epeptc attacks may themseves cause an organc
psychoss astng for severa days. Rumnatons and pseudo-haucnatons, n
whch the patent retans some nsght, may occur n affectve dsorders.
Non-epeptc attack dsorder (NEAD)
Non-epeptc attack dsorder may be assocated wth reports of haucnatons
and usons. These often evove out of prevous questonng by members of
the medca professon. Intay the symptoms may seem pausbe, but
shoud be suspected f they are ford and mutpe n type (e.g. audtory,
ofactory and vsua at dfferent tmes) wth evovng stores or patterns of
expresson.
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"4"4@ A;;ressive or vocal out<ursts
These are rarey epeptc n nature f they occur n soaton. They are especay
common n aduts and chdren wth earnng dsabtes. In ths settng there s
organc bran dsease whch coud ower the overa sezure threshod. Most cases do
not have ther bass n epepsy.
A not nfrequent forensc ssue s the occurrence of voent, or other, crmes n
patents wth epepsy, t whch t s a defence cam that the crme was commtted n
a state of automatsm. Certan features are strong evdence aganst an epeptc
bass to the attack.
Absence of a pror hstory of epepsy wth automatsms
Premedtaton and evdence of pannng or preparaton
Drected voence
Evdence of compcated and organzed actvty durng the epsode
Reca of events durng the epsode
Wtness accounts not ndcatve of a dsturbance of conscousness
Subsequent attempts at escape or conceament of evdence.
"4"4A 0:isodic :he&ome&a i& slee:
Attacks occurrng durng seep present partcuar dagnostc dffcutes because they
are
often poory wtnessed, and the patent may have tte, f any, reca of the event or
the precedng crcumstances.
Norma physoogca movements
Whoe body |erks commony occur n norma sub|ects on fang aseep.
Fragmentary physoogca myoconus usuay nvoves the perpheres or the
face, and occurs durng stages 1 and 2 and REM seep. Perodc movements of
seep may be an age reated phenomenon, beng seen n ess than 1% of
young aduts, but occurrng wth ncreasng frequency durng mdde and od
age such that they are present n perhaps haf the edery popuaton.
Typcay these movements occur at reguar ntervas of 10-60 seconds and
may occur n custers over many mnutes.
Fronta obe epepsy
Fronta obe sezures may dspay specfc seep reated characterstcs
causng
dagnostc confuson. Such attacks are often frequent, bref, bzarre and
maybe
restrcted to seep. Attacks may ncude apnoea, dystonc, myoconc, or
choreform movements that may be unatera or batera, and some
retenton of awareness. The attacks are scattered throughout the nght, and
usuay arse from non-REM seep. Frequency s hghy varabe, but some
patents have more than 20 attacks n a nght. An mportant cue to the
dagnoss s the occurrence of
addtona secondary generased sezures and sezures occurrng n
wakefuness.
Other epepses
Sezures arsng n other bran regons may present wth nocturna attacks.
Patents may be aroused by an aura, athough often ths s not recaed when
attacks arse from seep. Compex automatsms, n whch patents get out of
bed and wander around may cause confuson wth parasomnas. Wth
nocturna sezures of any type the partner s frequenty awoken by partcuar
components, such as vocasaton and does not wtness the onset.
Generased tonc-conc sezures not uncommony occur on or shorty
after awakenng.
Pathoogca fragmentary myoconus
Excessve fragmentary myoconus persstng nto seep stages 3 and 4 may
be seen wth any cause of dsrupted nocturna seep.
Restess eg syndrome
The restess eg syndrome s charactersed by an urge to move the egs,
especay n the evenng when yng or sttng. It may be assocated wth
varous unpeasant paraesthesae. A patents wth restess egs have perodc
movements of seep. These may be severe and can aso occur durng. In
addton there may be a varety of bref daytme dysknesas.
Non REM parasomnas
These nvove nght terrors or seep wakng. They usuay present n
chdhood or adoescence, and are often fama. The attacks arse from sow
wave seep, typcay at east thrty mnutes, but not more than four hours,
after gong to seep and the tmng s often stereotyped. Attacks may be
spaced out by months or years and rarey occur more than once per week,
and usuay no more than one attack occurs n a snge nght. They are more
key after stressfu events, or when seepng n a strange bed.
Nght terrors nvove ntense autonomc features (sweatng, fushng,
paptatons) and a ook of fear. Patents may reca a frghtenng scene or
experence, but do not usuay recount a vvd dream pror to the attacks.
They may be dffcut to arouse, and confused for severa mnutes.
Vocasatons are common. Seep wakng may nvove gettng out of bed and
performng compex tasks. Sometmes t s possbe to ead the patent back
to bed wthout awakenng. They may respond f spoken to, but ther speech s
usuay sow or monosyabc. Bref, abortve epsodes are commoner,
nvovng sttng up n bed wth fdgetng and shuffng (mmckng a compex
parta sezure). Non-REM parasomnas may cause sef n|ury but rarey
drected aggresson. They are assocated wth enuress.
REM parasomnas
REM parasomnas usuay occur n mdde age or the edery, and show a
marked mae predomnance. They more often occur n the ater porton of
seep. Durng REM seep patents may have an ncrease n the frequency or
severty of fragmentary myoconus, thrash about, ca out, dspay drected
voence, or appear to enact vvd dreams. Attacks may ast from seconds to
mnutes. If awoken patents may reca part of these dreams. Athough REM
seep behavor dsorders may occur n heathy edery sub|ects they are aso
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seen n assocaton wth drugs (e.g. trcyccs) or acoho, or centra nervous
system dseases such as mutsystem atrophy. The possbty of REM seep
dsorders needs to be consdered both at nta presentaton, and aso n
patents known to have centra nervous system dsorders.
Seep apnoea
Patents wth seep apnoea usuay present wth day-tme hypersomnoence.
However, the apnoec epsodes may cause epsodc gruntng, fang about or
other restess actvty that appears to mmc nocturna epepsy. Occasonay
the resutant hypoxa eads tsef to secondary sezures.
Other movements n seep
Nocturna body rockng may occur n patents wth earnng dsabty, or
foowng head n|ures. In patents wth many dfferent forms of day-tme
dysknesas, smar movements may occasonay occur durng overnght
seep, usuay n the settng of bref arousas.
"4"4"! Prolo&;ed co&)usio&al or )u;ue states
Epeptc sezures usuay ast for seconds or mnutes. After generased convusons
(or ess often compex parta sezures) there may be confuson astng for many
mnutes, but rarey more than an hour. Such epsodes ony present dagnostc
dffcuty f the nta sezure s unwtnessed or forgotten. Nevertheess, epeptc
states can ast for onger perods of tme, as can other types of cerebra dsorder
and the dfferenta dagnoss of proonged epeptc confusona states (non-
convusve status) shoud ncude: acute encephaopathy, nonconvusve status
epeptcus, transent goba amnesa, ntermttent psychoss, hysterca fugue
(NEAD).
Acute encephaopathy
Vrtuay any severe metaboc dsturbance may cause an acute
encephaopathy (e.g. dabetc ketoacdoss, hypogycaema, respratory, rena
or hepatc faure, drug ngeston, hyperpyrexa, sepss). Transent metaboc
dsturbances are most often seen n treated dabetes metus due to nsun
nduced hypogycaema. Occasonay metaboc dsorders may present wth
exacerbatons wth symptomatoogy astng for hours or days that gve the
appearance of an epsodc condton. These ncude: porphyra and urea cyce
enzyme defects. Acute neuroogca condtons aso need to be consdered,
partcuary: encephats, menngts, other ntracrana nfecton, head n|ury,
cerebra nfarcton or haemorrhage. Drug abuse may cause soated epsodes
or recurrent bouts, reated to ntoxcatons.
Nonconvusve status epeptcus
Patents wth compex parta sezures, typca or atypca absences may
present wth proonged confusona states due to compex parta epeptcus
or absence status. Such attacks may be the frst manfestaton of the sezure
dsorder, or occur n the settng of known epepsy.
Intermttent psychoss
Athough usuay more sustaned, psychatrc dsorders may present wth
epsodes of deusons, haucnatons or apparent confuson, astng for hours
or days.
Transent goba amnesa
These epsodes typcay commence acutey, and ast for mnutes or hours
and nvove both retrograde and anterograde amnesa. Patents may
perform compexactvtes, but afterwards have no reca of them. There s a
ack of other neuroogca features to the attacks, and conscousness appears
to be preserved. The attacks may nvove batera meda tempora
dysfuncton whch n some patents may be on the bass of schaema, whst
some may have an epeptc bass.
Hysterca fugue
A fugue state may arse wthout an organc physca cause, as a converson
symptom. These epsodes may be bref or very proonged, astng for days or
even weeks. If seen at the tme of an epsode nconsstences are often found
on examnaton of the menta state. In some cases, the queston of
mangerng arses, most commony n a stuaton n whch the person's state
prevents questonng by Law Offcers. The dagnoss s more dffcut to
dentfy f the patent s ony seen subsequenty. The matchng of wtness
accounts and the apparent sequence of events s essenta, but t may reman
dffcut to come to a frm concuson. In ths stuaton, there s sometmes a
forensc aspect, typcay when the person concerned s aeged to have
commtted a crme and they profess to have no memory of the events. A
checkst of possbe sezure-reated symptoms to enqure for when
consderng a possbe dagnoss of epepsy can be found n Tabe 1.
4 .i))ere&tial dia;&osis o) e:ile:sy i& childre&
There are a range of possbtes n the dfferenta dagnoss when consderng the
chd wth a funny turn. The consderatons w vary dependng on certan key
features of the event, as we as the age of the chd. The hstory s key to any
dagnoss, and s key to be far more usefu than any nvestgaton that can be
requested. Aways seek the nta event - a trgger or warnng, however young the
chd. There may be a typca behavor pror to any event or a sghty oder chd
may be abe to reate a feeng pror to or durng the event. A descrpton of each
change n the chd shoud be sought, and the evdence of oss or not of awareness.
Other mportant aspects of the hstory are medcatons taken, deveopmenta
hstory and past medca hstory but nformaton from these w ony be
suppementary to the hstory of the events themseves. There are a number of
possbtes wthn the dfferenta dagnoss of epepsy n chdhood; however a ack
of awareness of such aternatves remans the ma|or reason for error and the
premature, possbe msdagnoss, of epepsy. The foowng s a cassfcaton of
possbtes, athough not exhaustve.
Sy&co:e & related disorders
Dsorders of orthostatc contro Refex syncope
Respratory syncope Refex & expratory apnoec syncope
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Fantng ark
Upper arway obstructon
Cardac syncope Arrhythmas
Compete heart bock
Wof parknson whte
Brugada syndrome
Branstem syncope Tumour
Bran stem hernaton or compresson
Other Hyperekpexa
Anoxc epeptc sezures
Neurolo;ical Tcs
Myoconus
Paroxysma dystona
Sandfers syndrome
Paroxysma dysknesas
Catapexy
Bengn paroxysma vertgo/tortcos
Mgrane
Aternatng hempega
Eye movement dsorders
Overfow movements
BehaviouralC:sychiatric Daydreams
Dssocatve states
Sef gratfcaton behavour
Hyperventaton
Panc/anxety
Non epeptc attack dsorder
Fabrcated attacks
Pseudosyncope
Stereotypes/rtuastc behavor
Parasom&ias Seep myoconus
Headbangng
Confusona arousa
REM seep dsorder/nght terrors
Ta<le $ Classi)icatio& o) :ossi<ilities i& childhood e:ile:sy
2.1 The child under the age of 12 months
In a chd of ths age t may be ncredby dffcut to determne the true nature of an
event
when takng the hstory for the frst tme. There s key to be consderabe anxety
on the part of carers, and f there s any doubt, referra s advsed
Is the event reated to any partcuar actvty?
!or example, feeding may suggest reflux, history of medication ingestion may
suggest dystonic reaction, sleep may suggest benign sleep myoclonus.
What s the ma|or motor component?
Is the ma|or motor component apparent hypertona, hypotona or dystona?
Is ths movement repettve or sustaned?
"ither repetitive short duration over short period or #ith greater duration bet#een.
Repetitive $er%s may imply sei&ures, although only from sleep may be sleep
myoclonus. Repetitive spasms #ill be stereotyped, in clusters. 'udden sustained
hypertonia may give a clue to hypere%plexia or brainstem syncope
Are sem-purposefu movements seen at any tme? This may give a clue as to the
state of a#areness
What parts of the body are nvoved n the movements? !or example, #hole body,
eyes vs limb, all limbs vs single(multiple
Is there a behavour change?
Does the chd stare unresponsvey?
)s eye movement seen in association #ith this* an they be distracted*
Is the chd dstressed?
)f so intermittently or continuously* +istress may suggest a#areness, particularly in
bet#een spasms during clusters
Is there apparent fear?
,f course that may be manifest by distress, and therefore an apparent a#areness
of #hat is happening
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Is the event charactersed by other phenomena?
Is there a coour change?
-allor or cyanosis may be seen in cardiac arrythmias. cyanotic attac%s are
commonly associated #ith reflux. !lushing may be seen #ith repetitive
movement in self gratification behavior
Is there a change n breathng pattern?
+eep(stertorous breathing #ith loss of a#areness may be seen in generalised
sei&ures, compared #ith retained a#areness in self gratification behaviours.
Can the event be nterrupted?
)f so, then the event is probably a partial sei&ure or N"A+. )f the event cannot
be interrupted, then possibly a generalised sei&ure.
2.2 In the toddler (aged 1 to 3 years)
Is the event reated to any partcuar actvty?
'leep or #a%ing state. parasomnias are very common at this age and
in some instances mimic sei&ures. Night terrors usually occur at a
specific time each night, and during #hich the child may appear
unreachable. onfusional arousals may also cause alarm as the child is
inaccessible
Adverse(noxious events. if the event is al#ays triggered by this
consider
breath holding(reflex anoxic sei&ures
!ever. as in febrile sei&ures
Movement / as in paroxysmal dys%inesias
!eeding / as in 'andifers syndrome
"xcitement / as in overflo# movements
Note stereotypic movements(ritualistic behaviours may be seen in
children
#ith developmental delay(communication disorders at any time, but
xcitement(stress or boredom
"motion / as in cataplexy
What s the ma|or motor component?
- Is a fa the ma|or component of the event?
- Is the ma|or motor component apparent hypertona, hypotona or dystona?
o 0ypertonia consider reflex(epiratory syncope, tonic(tonic clonic
sei&ure, hypere%plexia
o 0ypotonia consider syncope, cataplexy, 1a%inetic2 drop attac% although
the latter unli%ely to occur in the absence of other sei&ure types
o +ystonia consider dys%inesia, benign paroxysmal torticollis 3B-T4
o 5nsteadiness consider benign paroxysmal vertigo 3B-64, intermittent
ataxia
- Is ths movement repettve or sustaned?
o "ither repetitive short duration over short period or #ith greater
duration bet#een. Repetitive $er%s may imply sei&ures. Although
repetitive short movements predominantly of the face may be tics
- Are sem-purposefu movements seen at any tme?
- This may give a clue as to the state of a#areness
- What parts of the body are nvoved n the movements?
- !or example, #hole body, eyes vs limb, all limbs vs single(multiple
Is there a behavour change?
- Does the chd stare unresponsvey?
o )s eye movement seen in association #ith this* an they be
distracted*
- Is the chd dstressed?
o )f so intermittently or continuously*
- Is there apparent fear?
o ,f course that may be manifest by distress, and therefore an apparent
a#areness of #hat is happening.
Is the event charactersed by other phenomena?
- Is there a coour change?
o -allor or cyanosis may be seen in cardiac arrythmias. cyanotic attac%s
are commonly associated #ith expiratory apnoeic 3breath holding4
attac%s. !lushing may be seen #ith self gratification behaviour.
- Is there a change n breathng pattern?
o "ither an increase or decrease may give a clue to the above.
- Can the event be nterrupted?
2.3 In the older child (aged 3 to 12 years)
Is the event reated to any partcuar actvty?
- 'leep or #a%ing state. parasomnias are very common at this age and in
some instances mimic sei&ures.
- Movement / as in paroxysmal dys%inesias
- "xcitement / as in overflo# movements
- Note stereotypic movements(ritualistic behaviours may be seen in children
#ith developmental delay(communication disorders at any time, but
particularly excitement(stress or boredom.
- "motion / as in cataplexy
What s the ma|or motor component?
- Is a fa the ma|or component of the event?
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- Is the ma|or motor component apparent hypertona, hypotona or dystona?
o 0ypertonia consider reflex(epiratory syncope, tonic(tonic clonic
sei&ure, hypere%plexia
o 0ypotonia consider syncope, cataplexy, 1a%inetic2 drop attac% although
the
latter unli%ely to occur in the absence of other sei&ure types
o +ystonia consider dys%inesia
o 5nsteadiness consider intermittent ataxia
- Is ths movement repettve or sustaned?
o "ither repetitive short duration over short period or #ith greater
duration bet#een. Repetitive $er%s may imply sei&ures. Although
repetitive short movements predominantly of the face may be tics
- Are sem-purposefu movements seen at any tme?
o This may give a clue as to the state of a#areness although older child
li%ely to be able to relay information about event
- What parts of the body are nvoved n the movements?
o !or example, #hole body, eyes vs limb, all limbs vs single(multiple
Is there a behavour change?
- Does the chd stare unresponsvey?
o )s eye movement seen in association #ith this* an they be distracted
/ by touch rather than voice alone* The most common cause of 1blan%
spells2 remains daydreaming. Typical absence sei&ures occur relatively
fre7uently,at any time and are of short duration 38-9: seconds4
- Is there apparent fear?
- Is there aggressve/destructve behavour?
o True 1rage2 attac%s in isolation are rarely, if ever epileptiform in origin.
Typically they are seen in children #ith learning difficulties, but are not
unseen in children #ith very specific learning problems that have not
yet been recognised. The children may become aggressive and
destructive, have little, if any recollection of the event
- Is the event charactersed by other phenomena?
- Is there a coour change?
o -allor or cyanosis may be seen in cardiac arrythmias. -allor is li%ely to
be seen in association #ith any syncope. !lushing may be seen #ith
self gratification behavior
- Is there a change n breathng pattern?
o "ither an increase or decrease may give a clue to the above
- Is there vsua symptomatoogy?
o 6isual disturbance, if so at #hat stage of the event / may suggest
primary
or secondary phenomenon. ;hat is the character of such 3repeated4
stereotyped colours or formed ob$ect li%ely to be sei&ure. late distorted
vision* secondary.
- Are there sensory symptoms?
o )f so, then maybe partial sei&ure, or paraesthesias due to other causes
- Is there vocasaton?
o Audible #ords or nonsense* At #hat stage of the event* +irected
speech(vocal outbursts are unli%ely to be epileptic.
- Can the event be nterrupted?
o )f so unli%ely to be a sei&ure.
V4 INV0STI'ATI-NS IN 0PI/0PTIC S0I1,20
Informaton shoud be provded to chdren, young peope and aduts and fames
and/or carers as approprate on the reasons for tests, ther resuts and meanng, the
requrements of specfc nvestgatons, and the ogstcs of obtanng them. A
nvestgatons for chdren shoud be performed n a chd-centred envronment.
0lectroe&ce:halo;ram >00'?
Chdren, young peope and aduts requrng an EEG shoud have the test performed
soon after t has been requested. An EEG shoud be performed ony to support a
dagnoss of epepsy n aduts n whom the cnca hstory suggests that the sezure
s key to be epeptc n orgn. The EEG shoud not be used to excude a dagnoss
of epepsy n a chd, young person or adut n whom the cnca presentaton
supports a dagnoss of a non-epeptc event. An EEG may be used to hep
determne sezure type and epepsy syndrome n chdren, young peope and aduts
n whom epepsy s suspected. Ths enabes them to be gven the correct prognoss.
For chdren, young peope and aduts n whom epepsy s suspected, but who
present dagnostc dffcutes, specast nvestgatons shoud beavaabe.
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Neuroima;i&;
Neuromagng shoud be used to dentfy structura abnormates that cause certan
epepses. MRI shoud be the magng nvestgaton of choce n chdren, young
peope and aduts wth epepsy. MRI s partcuary mportant n those: who deveop
epepsy before the age of 2 years or n aduthood who have any suggeston of a
foca onset on hstory, examnaton or EEG (uness cear evdence of bengn foca
epepsy) n whom sezures contnue n spte of frst-ne medcaton. Chdren, young
peope and aduts requrng MRI shoud have the test performed soon.
Neuromagng shoud not be routney requested when a dagnoss of dopathc
generased epepsy has been made. CT shoud be used to dentfy underyng gross
pathoogy f MRI s not avaabe or s contrandcated, and for chdren or young
peope n whom a genera anaesthetc or sedaton woud be requred for MRI but not
CT. In an acute stuaton, CT may be used to determne whether a sezure has been
caused by an acute neuroogca eson or ness.
-ther tests
In aduts, approprate bood tests (for exampe, pasma eectroytes, gucose,
cacum) to dentfy potenta causes and/or to dentfy any sgnfcant co- morbdty
shoud be consdered. In chdren and young peope, other nvestgatons, ncudng
bood and urne bochemstry, shoud be undertaken at the dscreton of the
specast to excude other dagnoses, and to determne an underyng cause of the
epepsy.
Neuro:sycholo;ical assessme&t
Neuropsychoogca assessment shoud be consdered n chdren, young peope and
aduts n whom t s mportant to evauate earnng dsabtes and cogntve
dysfuncton, partcuary n regard to anguage and memory. Referra for a
neuropsychoogca assessment s ndcated: when a chd, young person or adut
wth epepsy s havng educatona or occupatona dffcutes when an MRI has
dentfed abnormates n cogntvey mportant bran regons when a chd, young
person or adut compans of memory or other cogntve defcts and/or cogntve
decne.
VI4 '0N02A/ P2INCIP/0S -( M0.ICA/ MANA'0M0NT
Aims of treatment
The primary goal of treatment is to ensure the best possible quality of life that is compatible with
the nature of the patients seizure disorder and with any associated mental or physical dis-
abilities. To achieve this general goal, various objectives need to be addressed whenever relevant
or feasible.
'e&eral Treatme&t Ma&a;eme&t
"4 Com:lete seizure co&trol
If excepton s made for the contro of ongong sezures and status epeptcus, the
treatment of epepsy s prmary prophyactc (e.g. amed at preventng sezure
recurrence). In genera terms, therefore, the prmary ob|ectve of treatment shoud
be compete sezure contro where ths s feasbe. Ths, however, shoud not be
acheved at a costs. Antepeptc drugs can produce severe adverse effects,
partcuary when they are admnstered at hgh dosages or n combnaton, and the
stuaton shoud never arse where a patent s made to suffer more from the
adverse effects of treatment than from the symptoms of the dsease. Whenever
compete sezure freedom proves to be a non-reastc goa, optma treatment
shoud resut from the best compromse between the desre to mnmze sezure
frequency and the need to mantan adverse effects wthn acceptabe mts.
4 2eductio& o) seizure severity
Athough most outcome studes n epepsy have focused on sezure frequency,
sezure severty, partcuary wth respect to occurrence of potentay n|urous cta
manfestatons, s by tsef an mportant determnant of quaty of fe. In patents
whose sezures cannot be controed competey, t makes sense to am at
suppressng preferentay those sezures whch are most ds- abng. Assessng the
most ds- abng sezure types can requre assstance from an externa observer, but
the patents perceptons are more mportant. A sezure component that may
appear trva or neggbe to an observer may be perceved as very dstressng by
the patent.
$4 Avoida&ce o) adverse e))ects
The prescrpton of antepeptc medcaton entas a sgnfcant rsk of adverse
effects. Whe many patents wth recenty dagnosed epepsy can be controed at
ow dosages, whch produce ony modest detectabe toxcty, patents wth severe
epepses may have to pay a sgnfcant prce n terms of adverse effects to avod or
mnmze sezure recurrence.
%4 Su::ressio& o) su<cli&ical e:ile:tic activity
Antepeptc drug therapy shoud be amed prmary at suppressng the cnca
manfestatons of sezures, and normazaton of the eectroencephaogram (EEG)
generay s nether a ma|or nor an attanabe ob|ectve, nor n some cases even
desrabe. In certan stuatons, however, suppresson of epeptform EEG
abnormates s a |ustfabe therapeutc goa. Ths s the case when there s a cose
correate between cnca sezures and EEG paroxysms, and the sezures are not
easy quantfabe cncay, as n chdhood absence epepsy or some
photosenstve epepses. The EEG may aso be mportant n gudng treatment n
nfants and chdren wth severe epeptform EEG abnormates co-exstng wth
bran dysfuncton.
64 2eductio& o) seizure5related mortality a&d mor<idity
In some cases where sezures are trggered by a treatabe cause, such as a bran
tumour, remova of the atter s essenta to reduce any reated morbdty and
mortaty. In recent years, however, evdence has accumuated that sezures per se
are aso assocated wth an ncreased mortaty, party n reaton to accdents whch
may occur durng a sezure and party n reaton to the rsk of sudden unexpected
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death n epepsy (SUDEP).
74 Addressi&; co5mor<idities
Many symptomatc epepses are aetoogcay reated to maformatve, vascuar,
neopastc, degeneratve, nfammatory or meta- boc dsorders whch affect the
centra nervous system, and approprate management of these condtons must be
part of the comprehensve care of these patents.
Neuropsychatrc dsorders are aso reatvey common n patents wth epepsy .
Athough these co-morbdtes have been tradtonay regarded as a consequence of
the physca and psychosoca mtatons assocated wth sezures, and the adverse
effects of ant- epeptc drugs, there s ncreasng evdence that other factors are
aso nvoved. In partcuar, pre-exstng depresson or a hstory of sucde attempt
have been dentfed as separate rsk factors for ncdent unprovoked sezures.
Many physcans treatng epepsy do not routney screen for psychatrc dsorders,
whch s regrettabe because these co- morbdtes may mpact on quaty of fe
more than epepsy and sezures themseves, and therefore need to be dentfed
and treated as approprate. In a recent study from the USA, the severty of
depressve symptoms and the adverse effects of drugs n patents wth epepsy
correated ndependenty wth sub|ec- tve heath status, and these factors
expaned 72% of the varance
94 Avoida&ce o) adverse dru; i&teractio&s
Patents recevng therapy wth a combnaton of drugs are at rsk for adverse drug
nteractons, at ether the pharmacoknetc or pharmacodynamc eve. Drug
nteractons are not restrcted to those resutng from combnatons of antepeptc
drugs, but aso nvove medcatons taken for other ndcatons . Physcans shoud
be aware of ths, and shoud take a necessary steps to mnmze any adverse
consequences.
@4 Avoida&ce o) o<structio& to :atie&tDs li)e
Therapeutc outcome may be nfuenced by the patents abty to dentfy and
avod stuatons whch coud affect susceptbty to sezures, such as excessve
seep deprvaton, or - n some photo- senstve epepses - exposure to ntermttent
fashng ghts or certan vdeo games. Whe these rsk factors need to be dscussed
and approprate counseng gven, t s equay mportant to avod undue restrctons
on the patents festye. For exampe, acoho abuse shoud be actvey
dscouraged, but there s no reason to prohbt one gass of beer or wne at mea
tmes. In genera, patents shoud be encouraged to ve a norma fe, whe avod-
ng extreme devatons from what woud be consdered a reguar festye.
Prescrpton of medcaton shoud aso be amed at mnmzng nterference wth
day actvtes. Antepeptc drugs whch can be gven once or twce day are ess
key to obstruct day rou- tnes and to cause psychosoca embarrassment, and
they are assocated wth a better compance. For drugs whch can be gven once or
twce day but do not have a ong haf-fe, a twce-day schedue may be preferabe
because t mnmzes the adverse consequences of mssng one dose. In genera,
once-day dosng does not enta better compance than twce-day dosng, but t
may have psychoogca advantages, partcuary n patents who are sezure free
and perceve each act of p-takng as the ony unpeasant remnder of ther
dsease.
A4 Preve&tio& o) e:ile:to;e&esis
Experments n anma modes suggest that some antepeptc drugs not ony exert
a symptomatc effect by rasng sezure threshod, but mght aso antagonze
epeptogenc processes, .e. the mechansms through whch an epeptc condton
becomes estabshed. The suggeston has been made that recurrent cnca sezures
may aso ead to rreversbe neuroanatomca changes whch may render the
dsease more dffcut to contro, but evdence for ths s controversa. If
uncontroed sezure actvty eads to the chronczaton of the dsorder, a case
coud be made for eary and aggressve treatment, and for preferenta use of drugs
whch putatvey antagonze epeptogenc processes. Avaabe studes, however,
suggest that n most epepsy syn- dromes antepeptc drugs exert merey a
symptomatc effect and do not affect the natura course of the dsease .
Ehe& should treatme&t <e startedF
Antepeptc drugs (AEDs) are the manstay of the treatment of epepsy, and
athough ther number has expanded exponentay, current prncpes governng
drug therapy are n many ways smar to those estabshed a century ago. Because
AED therapy s typcay mantaned for severa years and often for fe,
partcuarynaduts,a decson to ntate treatment has far-reachng consequences
and needs to be based on carefu rsk-beneft anayses.
The predcted effcacy of AEDs has to be weghed aganst potenta adverse effects,
whe at the same tme consderng the rsks assocated wth wthhodng of
treatment. These rsks shoud be assessed n the broadest sense, takng nto
account the patents perspectve n reaton not ony to the rsk of further sezures
but aso the rsk of sezure-reated morbdty and mortaty, as we as AED toxcty.
Ideay, assessment of the potenta benefts of treatment shoud nvove an
understandng of the natura course of untreated epepsy and ts consequences.
The rsk of recurrence after a frst unprovoked sezure has been anaysed n severa
studes.A meta-anayss concuded that the average rsk of havng a further sezure
was 46%, but varaton across studes was substanta. The rsk of subsequent
sezures was much hgher after a second sezure (more than 70%), even n a cohort
n whch most patents were on treatment after ther second sezure.On the bass of
ths ncreased rsk, there has been a consensus that treatment s ndcated after at
east two unprovoked sezures.
From a practca vewpont, a key fndng s that the rsk of recurrence after a frst
sezure vares consderaby n reaton to the patents characterstcs. In ndvduas
wth rsk factors (eg, epeptform eectroencephaogram |EEG| dscharges, an
abnorma neuroogca examnaton, or other evdence of a structura CNS
abnormaty presumed to be responsbe for the sezure), the rsk of recurrence after
a frst sezure mght be smar to the average rsk after two sezures.Ths has been
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taken nto account n the newy proposed Internatona League Aganst Epepsy
(ILAE) defnton of epepsy as "a dsorder of the bran characterzed by an endurng
predsposton to generate epeptc sezures" and requrng "occurrence of at east
one unprovoked sezure".
The key to optmum epepsy management s to adapt treatment decsons to the
characterstcs of the ndvdua. In genera, AED monotherapy s ndcated after two
sezures, but n hgh-rsk patents ntaton of treatment after one sezure mght be
|ustfabe. AED choce s determned by sezure type, adverse-effect profe, and
patent-specfc features, ncudng age, sex (wth speca reference to chdbearng
potenta), and comorbdtes. Dose ttraton and dosng regmens aso need to be
carefuy ndvduased. Faure to acheve sezure freedom shoud prompt
dagnostc reassessment and eary consderaton of the feasbty of epepsy
surgery. Combnaton therapy can be benefca n patents who dd not respond to
two or three sequenta monotherapes, athough n some cases earer nsttuton of
poytherapy mght be |ustfed. Sezure freedom s aways the utmate goa, but t
shoud not be pursued at a costs and overtreatment shoud be carefuy avoded. In
patents who do acheve sezure contro, dscontnuaton of AEDs after at east 2-4
years of sezure freedom shoud be consdered, after carefu assessment of
potenta benefts versus the rsk of reapse and reated mpcatons.
A4 M-.,/0 TASG
Fnd out your answers to the foowng tasks by yoursef after dscuss t wth your
group or after readng the suggested references beow.
1. What are the anatomca structures that take part n sezure ?
2. Expan the mechansm of sezure n hypergycema !
3. Expan the mechansm of epeptogeness !
4. What s the defnton of sezure and epeptc sezure ?
5. Descrbe the cassfcaton of sezure and epeptc sezure !
6. Descrbe the pathophysoogy of epeptc sezure !
7. Descrbe the sgns and symptoms of epepsy !
8. How to manage a frst tme sezure ?
9. How to dfferentate between true sezure and psychogenc sezure ?
10.What are ndcatons to perform bran magng n sezure?
11.Whch AED s most approprate for genera epeptc sezure?
12.Make schematc agorthm to dfferentate symptoms of sezure n chdren!
13.Make schematc agorthm to dfferentate symptoms of sezure n adut !
"!4 T0AC3IN'5/0A2NIN' P2-C0SS
1. Each student shoud work the tasks n a Student-Worksheet (hand wrtten
n A4-sze book) pror to Sma Group Dscusson (home work).
2. The Student Worksheet w be coected before Sma Group Dscusson .
3. Student shoud dscuss the tasks n Sma Group Dscusson. The concuson
of the dscusson w be coected at the end of the dscusson.
4. The factators make notes on thngs the student dscuss whch need to
carfy at the end of each sesson or n ecture to acheve the earnng
ob|ectves and coect the report of dscusson.
""4 T30 ASS0SSM0NT
1. The Formatve Evauaton w be assessed through Observaton Sheets
eaboratng the Learnng Sk and Content Mastery
2. The Summatve Evauaton w be assesed together wth the other modues
n Mdde Semester Test and or End Semester Test schedued.
"4 20(020NC0S
Amercan Epepsy Socety. Basc Mechansm of Sezures and Epepsy. 2009.
Care of the Patent wth Sezures. 2
nd
ed. AANN Cnca Practce Gudene Seres. 2009
Enge |, et a (eds). Epepsy : Goba Issue for The Practcng Neuroogst. New York : Demos Medca
Pubshng. 2005
Dagnoss and Management of Epepsy n Aduts. Scottsh Intercoegate Gudenes Network. 2003
Stafstrom CE. Hypergycema Lowers Sezure Threshod. "pilepsy urr., 2003; 3(4): 148-149.
Grant C and Warow C. Foca epepsy n dabetc non-ketotc hypergycaema. BM<, 1985; 290: 1204-
1205.
Perucca E, Tomson T, The pharmacoogca treatment of epepsy n aduts. =ancet Neurol 2011; 10:
446-56
THE EPILEPSIES : The Dagnoss And Management of Epepsy n Adut and Chdren In Prmary and
Secondary Care. NICE Cnca Gudne.2012
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