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PEMICU 3 KGDAgustinus Kristantoko
405110090
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Cortical Blindness
is the total or partial loss o "ision in anor#al$appearing e%e &ause' (%'a#age to the (rain)s o&&ipital &orte*+
Corti&al (lin'ness &an (e a&,uire' or&ongenital- an' #a% also (e transient in&ertain instan&es+
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A&,uire' &orti&al (lin'ness is #ost oten&ause' (% loss o (loo' .o/ to theo&&ipital &orte* ro# either unilateral or
(ilateral posterior &ere(ralarter% (lo&kage is&he#i& stroke an' (%&ar'ia& surger%+
In #ost &ases- the &o#plete loss o "isionis not per#anent an' the patient #a%re&o"er so#e o their "ision Corti&al"isual i#pair#ent+
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Congenital &orti&al (lin'ness is #ostoten &ause' (% perinatal is&he#i&stroke- en&ephalitis- an' #eningitis+
2arel%- a patient /ith a&,uire' &orti&al(lin'ness #a% ha"e little or no insightthat the% ha"e lost "ision- a
pheno#enon kno/n as Antona(inskis%n'ro#e+
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Corti&al (lin'ness an' &orti&al "isuali#pair#ent CI- /hi&h reers to the partialloss o "ision &ause' (% &orti&al 'a#age- are(oth &lassi6e' as su(sets o neurologi&al "isuali#pair#ent 7I+
7I an' its three su(t%pes8&orti&al(lin'ness- &orti&al "isual i#pair#ent-an' 'ela%e' "isual #aturation8#ust (e'istinguishe' ro# o&ular "isual i#pair#ent inter#s o their 'ierent etiologies an' stru&turalo&i- the (rain an' the e%e respe&ti"el%+
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:ne 'iagnosti& #arker o this 'istin&tionis that the pupils o in'i"i'uals /ith&orti&al (lin'ness /ill respon' to light
/hereas those o in'i"i'uals /ith o&ular"isual i#pair#ent /ill not+
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Causes
;he #ost &o##on &ause o &orti&al (lin'ness isis&he#ia o*%gen 'epri"ation to the o&&ipitallo(es &ause' (% (lo&kage to one or (oth o theposterior &ere(ral arteries+
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Ine&tion
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;he #ost &o##on &auses o &ongenital&orti&al (lin'ness are=
;rau#ati& (rain inur% ;I to the
o&&ipital lo(e o the (rain Congenital a(nor#alities o the o&&ipital
lo(e
Perinatal is&he#ia En&ephalitis
Meningitis
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>%#pto#s
;he #ost &o##on s%#pto#s o a&,uire' an'transient &orti&al (lin'ness in&lu'e=
A &o#plete loss o "isual sensation an' o "ision
Preser"ationBsparing o the a(ilities to per&ei"elight an'Bor #o"ing- (ut not stati& o(e&ts 2i''o&hs%n'ro#e
A la&k o "isual 6*ation an' tra&king
Denial o "isual loss Antona(inski s%n'ro#e
isual hallu&inations
Ma&ular sparing- in /hi&h "ision in the o"ea isspare' ro# the (lin'ness+
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Diagnosis
A patient /ith &orti&al (lin'ness has no "ision(ut the response o hisBher pupil to light is
inta&t as the re.e* 'oes not in"ol"e the&orte*+
;hereore- one 'iagnosti& test or &orti&al(lin'ness is to 6rst o(e&ti"el% "eri% the opti&ner"es an' the non$&orti&al un&tions o thee%es are un&tioning nor#all%+
;his in"ol"es &on6r#ing that patient &an'istinguish lightB'ark- an' that hisBher pupils
'ilate an' &ontra&t /ith light e*posure+
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;hen- the patient is aske' to 'es&ri(eso#ething heBshe /oul' (e a(le tore&ogni?e /ith nor#al "ision+ or
e*a#ple the patient /oul' (e aske' theollo/ing=
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Patients /ith &orti&al (lin'ness /ill not (e a(leto i'enti% the ite# (eing ,uestione' a(out atall or /ill not (e a(le to pro"i'e an% 'etailsother than &olor or perhaps general shape+
;his in'i&ates that the la&k o "ision isneurologi&al rather than o&ular+
It spe&i6&all% in'i&ates that the o&&ipital
&orte* is una(le to &orre&tl% pro&ess an'interpret the inta&t input &o#ing ro# theretinas+
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un'os&op% shoul' (e nor#al in &ases o&orti&al (lin'ness+
Corti&al (lin'ness &an (e asso&iate' /ith
"isual hallu&inations- 'enial o "isual lossAntona(inski s%n'ro#e- an' thea(ilit% to per&ei"e #o"ing (ut not stati&o(e&ts+ 2i''o&h s%n'ro#e+
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:ut&o#e
;he prognosis o a patient /ith a&,uire'&orti&al (lin'ness 'epen's largel% on the
original &ause o the (lin'ness+
or instan&e- patients /ith (ilateral o&&ipitallesions ha"e a #u&h lo/er &han&e o re&o"ering"ision than patients /ho suere' a transientis&he#i& atta&k or /o#en /ho e*perien&e'&o#pli&ations asso&iate' /ith e&la#psia+
In patients /ith a&,uire' &orti&al (lin'ness- aper#anent &o#plete loss o "ision is rare+
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;he 'e"elop#ent o &orti&al (lin'nessinto the #il'er &orti&al "isuali#pair#ent is a #ore likel% out&o#e+
urther#ore- so#e patients regain "ision&o#pletel%- as is the &ase /ith transient&orti&al (lin'ness asso&iate'/ith e&la#psia an' the si'e ee&ts o&ertain anti$epileps% 'rugs+
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2e&ent resear&h (% Kr%stal +
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AntonBabinski syndrome
also kno/n as "isual anosognosia- is a rares%#pto# o (rain 'a#age o&&urring in the o&&ipitallo(e+
;hose /ho suer ro# it are &orti&all% (lin'- (ut
ar#- oten ,uite a'a#antl% an' in the a&e o&lear e"i'en&e o their (lin'ness- that the% are&apa(le o seeing+
ailing to a&&ept (eing (lin'- the suerer 'is#isses
e"i'en&e o his &on'ition an'e#plo%s &ona(ulation to 6ll in the #issing sensor%input+ It is na#e' ater Ga(riel Anton an' @osepha(inski+
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Chara&teristi&s
Antona(inski s%n'ro#e is #ostl% seen ollo/inga stroke- (ut #a% also (e seen ater hea' inur%+
It is /ell 'es&ri(e' (% the neurologist Ma&'onal'Crit&hle%=
;he su''en 'e"elop#ent o (ilateral o&&ipital'%sun&tion is likel% to pro'u&e transient ph%si&al an'ps%&hi&al ee&ts in /hi&h #ental &onusion #a% (epro#inent+
It #a% (e so#e 'a%s (eore the relati"es- or thenursing sta- stu#(le onto the a&t that the patienthas a&tuall% (e&o#e sightless+
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;his is not onl% (e&ause the patient or'inaril%'oes not "olunteer the inor#ation that he has(e&o#e (lin'- (ut he urther#ore #islea's hisentourage (% (eha"ing an' talking as thoughhe /ere sighte'+
Attention is arouse' ho/e"er /hen the patientis oun' to &olli'e /ith pie&es o urniture- toall o"er o(e&ts- an' to e*perien&e 'i&ult% in6n'ing his /a% aroun'+
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uspi&ion is still urther alerte' /hen he(egins to 'es&ri(e people an' o(e&tsaroun' hi# /hi&h- as a #atter o a&t-are not there at all+
;hus /e ha"e the t/in s%#pto#so anosognosia or la&k o a/areness o'ee&t an' &ona(ulation- the latter
ae&ting (oth spee&h an' (eha"iour+
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Causes
Fh% patients /ith Antona(inski s%n'ro#e 'en%their (lin'ness is unkno/n- although there are#an% theories+
:ne h%pothesis is that 'a#age to the "isual
&orte* results in the ina(ilit% to &o##uni&ate /iththe spee&h$language areas o the (rain+
isual i#ager% is re&ei"e' (ut &annot (einterprete'H the spee&h &enters o the
(rain &ona(ulate a response+ Patients ha"e also reporte' "isual anosognosia ater
suering ro# is&he#i& "as&ular &ere(ral 'isease+
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A 9 %ear ol' #an- /ho /as a'#itte' toan E#ergen&% 2oo# &o#plaining o ase"ere hea'a&he an' su''en loss o
"ision- /as 'is&o"ere' to ha"e suere'ro# a posterior &ere(ral arter% thro#(osisan' &onse,uentl% lost his "ision+
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>i#ilarl%- a 5 %ear ol' /o#an /as a'#itte' tothe E#ergen&% 2oo# in a &onuse' state an'
/ith se"erel% han'i&appe' ps%&ho#otor skills+
:&ular #o"e#ents an' pupil re.e*es /ere stillinta&t- (ut the patient &oul' not na#e o(e&tsan' /as not a/are o light &hanges in theroo#- an' see#e' una/are o her "isual'e6&it+
An M2I re"eale' is&he#i& lesions in the leto&&ipital lo(e an' a C; angiogra# (rain s&an
un"eile' "as&ulitis o the (rain arteries+
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Creut?el't@ako( 'isease
is a 'egenerati"e neurologi&al'isor'er that is in&ura(le an' in"aria(l%atal+
C@D is &ause' (% an agent &alle' a prion+
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Prions are #isol'e' proteins thatrepli&ate (% &on"erting their properl%ol'e' &ounterparts- in their host- to the
sa#e #isol'e' stru&ture the% possess+;he 'isease lea's to rapi'
neuro'egeneration- &ausing the (raintissue to 'e"elop holes an' take a #oresponge$like te*ture+
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Classi6&ation
;%pes o C@D in&lu'e=
"ariant "C@D=
;his is thought to (e &ause' (% the
&onsu#ption o oo' &onta#inate'/ith prions+
spora'i& sC@D=
;his a&&ounts or J5 o &ases o C@D+
a#ilial C@D=
;his a&&ounts or the #aorit% o the other15 &ases o C@D+
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iatrogeni&=
;his or# o C@D arises ro#&onta#ination /ith tissue ro# an
ine&te' person- usuall% as the result oa #e'i&al pro&e'ure+
Me'i&al pro&e'ures that are asso&iate'
/ith the sprea' o this or# o C@Din&lu'e the use o hu#an$'eri"e'pituitar% gro/th hor#ones-gona'otropinhor#one therap%- &orneal an'Bor
#eningeal transplants+
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Eclampsia
is an a&ute an' lie$threatening &o#pli&ationo pregnan&% &hara&teri?e' (% the appearan&e
o toni&&loni& sei?ures &on"ulsions- usuall% ina /o#an /ho has 'e"elope' pre$
e&la#psia+ Pre$e&la#psia an' e&la#psia are&olle&ti"el% &alle' h%pertensi"e 'isor'er o
pregnan&% an' to*e#ia o pregnan&%+
E&la#psia in&lu'es &on"ulsions an' &o#a that
happen 'uring pregnan&% (ut are not 'ue topre$e*isting or organi& (rain 'isor'ers+
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>igns an' s%#pto#s
;%pi&all% a /o#an sho/s signs o pregnan&%$in'u&e' h%pertension an' proteinuria (eore theonset o the hall#ark o e&la#psia- the e&la#pti&&on"ulsion+
:ther &ere(ral signs #a% pre&e'e the &on"ulsion-su&h as nausea- "o#iting- hea'a&hes- an' &orti&al(lin'ness+
In a''ition- /ith the a'"an&e#ent o the
pathoph%siologi&al pro&ess- other organ s%#pto#s#a% (e present- in&lu'ing a('o#inal pain- li"erailure- signs o the
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;he etus #a% alrea'% ha"e (een &o#pro#ise'(% intrauterine gro/th retar'ation- an'- /ith theto*e#i& &hanges o&&urring 'uring e&la#psia- #a%suer etal 'istress+ Pla&ental (lee'ing
an' pla&ental a(ruption #a% also o&&ur+ In so#e rare &ases- there are no &on"ulsions- an'
the /o#an alls 'ire&tl% into a &o#a+ >o#e/o#en /ith e&la#psia #a% e*perien&e te#porar%
(lin'ness upon /aking ro# the &o#a+ During a &on"ulsion- the etus #a%
e*perien&e (ra'%&ar'ia+
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2isk a&tors
E&la#psia- like pre$e&la#psia- ten's to o&&ur#ore &o##onl% in 6rst pregnan&ies an'%oung #others /here it is thought that no"ele*posure to paternal antigens is in"ol"e'+
urther#ore- /o#en /ith pre$e*isting"as&ular 'iseases h%pertension- 'ia(etes-an' nephropath% or thro#(ophili& 'iseases
su&h as the antiphospholipi' s%n'ro#e are athigher risk to 'e"elop pre$e&la#psia an'e&la#psia+
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Pul#onar% e'e#a is a rather &o##on&o#pli&ation o se"ere e&la#psiaae&ting appro*i#atel% 3 o the
people /ith e&la#psia+ Most &ases result o aggressi"e use o
&r%stalloi' solutions or intra"as&ular"olu#e e*pansion+
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Pathoph%siolog%
Fhile #ultiple theories ha"e (een propose' toe*plain pree&la#psia an' e&la#psia- it o&&urs onl%in the presen&e o a pla&enta an' is resol"e' (% itsre#o"al+
Pla&ental h%poperusion is a ke% eature o thepro&ess+
It is a&&o#panie' (% in&rease' sensiti"it% o the#aternal "as&ulature to pressor agents lea'ing to
"asospas# an' h%poperusion o #ultiple organs+ urther- an a&ti"ation o the &oagulation &as&a'e
lea's to #i&rothro#(i or#ation an' aggra"ates theperusion pro(le#+
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Loss o plas#a ro# the "as&ular tree/ith the resulting e'e#a a''itionall%&o#pro#ises the situation+
;hese e"ents lea' to signs an's%#pto#s o to*e#ia in&lu'ingh%pertension- renal- pul#onar%- an'hepati& '%sun&tion- an'8in e&la#psia-spe&i6&all%8&ere(ral '%sun&tion+
Pre&lini&al #arkers o the 'iseasepro&ess are signs o in&rease' platelet
an' en'othelial a&ti"ation+
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Pla&ental h%poperusion is linke' toa(nor#al #o'elling o the etal#aternalintera&e that #a% (e i##unologi&all%
#e'iate'+;he in"asion o the tropho(last appears to
(e in&o#plete+
A'reno#e'ullin- a potent "aso'ilator- is
pro'u&e' in 'i#inishe' ,uantities (% thepla&enta in pre$e&la#psia an' thuse&la#psia+
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:ther "asoa&ti"e agents are at pla%in&lu'ing prosta&%&lin- thro#(o*ane A- nitri& o*i'e- an' en'othelins lea'ing to
"aso&onstri&tion+ Man% stu'ies ha"e suggeste' the
i#portan&e o a /o#an)s i##unologi&altoleran&e to her (a(%)s ather- /hose
genes are present in the %oung etus an'its pla&enta an' /hi&h #a% pose a&hallenge to her i##une s%ste#+
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E&la#psia is seen as a or# o h%pertensi"een&ephalopath% in the &onte*t o thosepathologi&al e"ents that lea' to pre$e&la#psia+It is thought that &ere(ral "as&ular resistan&e is
re'u&e'- lea'ing to in&rease' (loo' .o/ to the(rain+ In a''ition to a(nor#al un&tion othe en'otheliu#- this lea's to &ere(ral e'e#a+
;%pi&all% an e&la#pti& &on"ulsion /ill not lea' to
lasting (rain 'a#ageH ho/e"er- intra&ranialhae#orrhage #a% o&&ur+
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Diagnosis
>ei?ures 'uring pregnan&% that areunrelate' to pre$e&la#psia nee' to (e'istinguishe' ro# e&la#psia+
>u&h 'isor'ers in&lu'e sei?ure 'isor'ers as/ell as (rain tu#or- aneur%s# o the (rain-an' #e'i&ation$ or 'rug$relate' sei?ures+
Usuall% the presen&e o the signs o se"ere
pre$e&la#psia pre&e'e an' a&&o#pan%e&la#psia- a&ilitating the 'iagnosis+
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In"estigations=
CC-
renal un&tion test 2;-
Li"er un&tion test L;-
&oagulation s&reen-
plas#a rate &on&entration-
4 hour urine anal%sis-
ultrasoun'
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Pre"ention
Dete&tion an' #anage#ent o pre$e&la#psia is &riti&al to re'u&e the risk oe&la#psia+
Appropriate #anage#ent o /o#en /ithpre$e&la#psia generall% in"ol"es the useo #agnesiu# sulphate as an agent topre"ent &on"ulsions- an' thuspre"enting e&la#psia+
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;reat#ent
;he treat#ent o e&la#psia re,uirespro#pt inter"ention an' to pre"enturther &on"ulsions- &ontrol the ele"ate'
(loo' pressure- an' i##e'iatel% 'eli"erthe (a(% i possi(le+
Antiseizure medicatio
Magnesiu# sulate is &o##onl% use'an' /hen &o#pare'to 'ia?epa#- phen%toin or a &o#(inationo &hlorpro#a?ine- pro#etha?ine an' pet
hi'ine it results in (etter out&o#es+
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Blood pressure management
loo' pressure #anage#ent at this stage inpregnan&% #a% &onsist o h%'rala?ine or la(etalol+
Delivery
I the (a(% has not %et (een 'eli"ere'- steps nee'to (e taken to sta(ili?e the /o#an an' 'eli"er herspee'il%+
;his nee's to (e 'one e"en i the (a(% is
i##ature- as the e&la#pti& &on'ition is unsae or(oth (a(% an' #other+
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As e&la#psia is a #aniestation o a #ultiorgan ailure-other organs li"er- ki'ne%- lungs- &ar'io"as&ulars%ste#- an' &oagulation s%ste# nee' to (e assesse' inpreparation or a 'eli"er% oten a &aesarean se&tion-unless the /o#an is alrea'% in a'"an&e' la(or+
2egional anesthesia or &aesarean se&tion is&ontrain'i&ate' /hen a &oagulopath% has 'e"elope'+
Invasive haemodynamic monitoring
In"asi"e hae#o'%na#i& #onitoring #a% (e useul in an
e&la#pti& /o#an /ith se"ere &ar'ia& 'isease- renal'isease- rera&tor% h%pertension- pul#onar% e'e#a-an' poor urine output+
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;