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PEMICU 3 KGD

Agustinus Kristantoko
405110090

Cortical Blindness

is the total or partial loss ofvisionin a


normal-appearingeyecaused by
damage to thebrain'soccipital cortex.
Cortical blindness can be acquired or
congenital, and may also be transient in
certain instances.

Acquired cortical blindness is most often


caused by loss of blood flow to the
occipital cortex from either unilateral or
bilateralposterior cerebral
arteryblockage (ischemic stroke) and by
cardiac surgery.
In most cases, the complete loss of vision
is not permanent and the patient may
recover some of their vision (Cortical
visual impairment).

Congenital cortical blindness is most


often caused by perinatal ischemic
stroke, encephalitis, and meningitis.
Rarely, a patient with acquired cortical
blindness may have little or no insight
that they have lost vision, a
phenomenon known asAntonBabinski
syndrome.

Cortical blindness andcortical visual


impairment(CVI), which refers to the partial
loss of vision caused by cortical damage, are
both classified as subsets of neurological visual
impairment (NVI).
NVI and its three subtypescortical
blindness,cortical visual impairment,
anddelayed visual maturationmust be
distinguished from ocularvisual impairmentin
terms of their different etiologies and structural
foci, the brain and the eye respectively.

One diagnostic marker of this distinction


is that the pupils of individuals with
cortical blindness will respond to light
whereas those of individuals with ocular
visual impairment will not.

Causes

The most common cause of cortical blindness is


ischemia (oxygen deprivation) to the occipital
lobes caused by blockage to one or both of the
posterior cerebral arteries. However, other
conditions have also been known to cause
acquired and transient cortical blindness,
including:
Bilateral lesions of the primaryvisual cortex
Side effect of some anti-epilepsy drugs (AEDs)
CreutzfeldtJakob disease, in association with a
rapid onset ofdementia

Infection
Head trauma to the occipital lobe of the
brain
Congenital abnormalities of the occipital
lobe
Eclampsia and, rarely,pre-eclampsia
Hyperammonemia

The most common causes of congenital


cortical blindness are:
Traumatic brain injury (TBI) to the
occipital lobe of the brain
Congenital abnormalities of the occipital
lobe
Perinatal ischemia
Encephalitis
Meningitis

Symptoms

The most common symptoms of acquired and


transient cortical blindness include:
A complete loss of visual sensation and ofvision
Preservation/sparing of the abilities to perceive
light and/or moving, but not static objects (Riddoch
syndrome)
A lack of visual fixation and tracking
Denial of visual loss (AntonBabinski syndrome)
Visual hallucinations
Macular sparing, in which vision in the fovea is
spared from the blindness.

Diagnosis

A patient with cortical blindness has no vision


but the response of his/her pupil to light is
intact (as the reflex does not involve the
cortex).
Therefore, one diagnostic test for cortical
blindness is to first objectively verify the optic
nerves and the non-cortical functions of the
eyes are functioning normally.
This involves confirming that patient can
distinguish light/dark, and that his/her pupils
dilate and contract with light exposure.

Then, the patient is asked to describe


something he/she would be able to
recognize with normal vision. For
example the patient would be asked the
following:
"How many fingers am I holding up?"
"What does that sign (on a custodian's
closet, a restroom door, an exit sign)
say?"
"What kind of vending machine (with a
vivid picture of a well-known brand name

Patients with cortical blindness will not be able


to identify the item being questioned about at
all or will not be able to provide any details
other than color or perhaps general shape.
This indicates that the lack of vision is
neurological rather than ocular.
It specifically indicates that the occipital
cortex is unable to correctly process and
interpret the intact input coming from the
retinas.

Fundoscopyshould be normal in cases of


cortical blindness.
Cortical blindness can be associated with
visualhallucinations, denial of visual loss
(AntonBabinski syndrome), and the
ability to perceive moving but not static
objects. (Riddoch syndrome).

Outcome

The prognosis of a patient with acquired


cortical blindness depends largely on the
original cause of the blindness.
For instance, patients with bilateral occipital
lesions have a much lower chance of recovering
vision than patients who suffered a transient
ischemic attack or women who experienced
complications associated with eclampsia.
In patients with acquired cortical blindness, a
permanent complete loss of vision is rare.

The development of cortical blindness


into the mildercortical visual
impairmentis a more likely outcome.
Furthermore, some patients regain vision
completely, as is the case with transient
cortical blindness associated
witheclampsiaand the side effects of
certain anti-epilepsy drugs.

Recent research by Krystal B. Huxlin and


others on the relearning of complex visual
motion following V1 damage has offered
potentially promising treatments for
individuals with acquired cortical blindness.
These treatments focus on retraining and
retuning certain intact pathways of the
visual cortex which are more or less
preserved in individuals who sustained
damage to V1.

Huxlin and others found that specific


training focused on utilizing the "blind
field" of individuals who had sustained
V1 damage improved the patients'
ability to perceive simple and complex
visual motion.
This sort of 'relearning' therapy may
provide a good workaround for patients
with acquired cortical blindness in order
to better make sense of the visual
environment.

AntonBabinski syndrome

also known as visual anosognosia, is a rare


symptom ofbrain damageoccurring in theoccipital
lobe.
Those who suffer from it are "corticallyblind", but
affirm, often quite adamantly and in the face of
clear evidence of their blindness, that they are
capable of seeing.
Failing to accept being blind, the sufferer dismisses
evidence of his condition and
employsconfabulationto fill in the missing sensory
input. It is named afterGabriel AntonandJoseph
Babinski.

Characteristics

AntonBabinski syndrome is mostly seen following


astroke, but may also be seen afterhead injury.
It is well described by the neurologist Macdonald
Critchley:
The sudden development of bilateral occipital
dysfunction is likely to produce transient physical and
psychical effects in which mental confusion may be
prominent.
It may be some days before the relatives, or the
nursing staff, stumble onto the fact that the patient
has actually become sightless.

This is not only because the patient ordinarily


does not volunteer the information that he has
become blind, but he furthermore misleads his
entourage by behaving and talking as though
he were sighted.
Attention is aroused however when the patient
is found to collide with pieces of furniture, to
fall over objects, and to experience difficulty in
finding his way around.

He may try to walk through a wall or


through a closed door on his way from
one room to another.
Suspicion is still further alerted when he
begins to describe people and objects
around him which, as a matter of fact,
are not there at all.
Thus we have the twin symptoms
ofanosognosia(or lack of awareness of
defect) and confabulation, the latter
affecting both speech and behaviour.

Causes

Why patients with AntonBabinski syndrome deny


their blindness is unknown, although there are
many theories.
One hypothesis is that damage to thevisual
cortexresults in the inability to communicate with
the speech-language areas of the brain.
Visual imagery is received but cannot be
interpreted; the speech centers of the
brainconfabulatea response.
Patients have also reported visual anosognosia after
suffering from ischemic vascular cerebral disease.

A 96 year old man, who was admitted to


an Emergency Room complaining of a
severe headache and sudden loss of
vision, was discovered to have suffered
from a posterior cerebral artery thrombosis
and consequently lost his vision.
He adamantly claimed he was able to see
despite an opthalmologic exam proving
otherwise. An MRI of his brain proved that
his right occipital lobe was ischemic.

Similarly, a 56 year old woman was admitted to


the Emergency Room in a confused state and
with severely handicapped psychomotor skills.
Ocular movements and pupil reflexes were still
intact, but the patient could not name objects
and was not aware of light changes in the
room, and seemed unaware of her visual
deficit.
An MRI revealed ischemic lesions in the left
occipital lobe and a CT angiogram brain scan
unveiled vasculitis of the brain arteries.

CreutzfeldtJakob disease

is adegenerative neurological
disorderthat is incurable and invariably
fatal.
CJD is caused by an agent called aprion.

Prions are misfolded proteins that


replicate by converting their properly
folded counterparts, in their host, to the
same misfolded structure they possess.
The disease leads to rapid
neurodegeneration, causing the brain
tissue to develop holes and take a more
sponge-like texture.

Classification

Types of CJD include:


variant (vCJD):
This is thought to be caused by the
consumption of food contaminated
withprions.
sporadic (sCJD):
This accounts for 85% of cases of CJD.
familial (fCJD):
This accounts for the majority of the other
15% cases of CJD.

iatrogenic:
This form of CJD arises from
contamination with tissue from an
infected person, usually as the result of
a medical procedure.
Medical procedures that are associated
with the spread of this form of CJD
include the use of human-derived
pituitary growth hormones, gonadotropin
hormone therapy,corneal and/or
meningeal transplants.

Eclampsia

is an acute and life-threatening complication


ofpregnancy characterized by the appearance
oftonicclonic seizures(convulsions), usually in
a woman who has developedpreeclampsia.(Pre-eclampsia and eclampsia are
collectively called "hypertensive disorder of
pregnancy" and "toxemia of pregnancy".)
Eclampsia includes convulsions and coma that
happen during pregnancy but are not due to
pre-existing or organic brain disorders.

Signs and symptoms

Typically a woman shows signs ofpregnancyinduced hypertensionandproteinuriabefore the


onset of the hallmark of eclampsia, the eclamptic
convulsion.
Other cerebral signs may precede the convulsion,
such as nausea, vomiting, headaches, andcortical
blindness.
In addition, with the advancement of the
pathophysiological process, other organ symptoms
may be present, including abdominal pain, liver
failure, signs of theHELLP syndrome,pulmonary
edema, andoliguria.

The fetus may already have been compromised


by intrauterine growth retardation, and, with the
toxemic changes occurring during eclampsia, may
sufferfetal distress. Placental bleeding
andplacental abruptionmay also occur.
In some rare cases, there are no convulsions, and
the woman falls directly into a coma. Some
women with eclampsia may experiencetemporary
blindnessupon waking from the coma.
During a convulsion, the fetus may
experiencebradycardia.

Risk factors

Eclampsia, like pre-eclampsia, tends to occur


more commonly in first pregnancies and
young mothers where it is thought that novel
exposure to paternalantigensis involved.
Furthermore, women with pre-existing
vascular diseases (hypertension,diabetes,
andnephropathy) or thrombophilic diseases
such as theantiphospholipid syndromeare at
higher risk to develop pre-eclampsia and
eclampsia.

Having a large placenta (multiple


gestation,hydatidiform mole) also
predisposes women to eclampsia.
In addition, there is a genetic component: a
woman whose mother or sister had the
condition are at higher risk.
Women who have experienced eclampsia
are at increased risk for
preeclampsia/eclampsia in a later
pregnancy.

Pulmonary edemais a rather common


complication of severe eclampsia
affecting approximately 3% of the
people with eclampsia.
Most cases result of aggressive use of
crystalloid solutions for intravascular
volume expansion.

Pathophysiology

While multiple theories have been proposed to


explain preeclampsia and eclampsia, it occurs only
in the presence of aplacentaand is resolved by its
removal.
Placental hypoperfusionis a key feature of the
process.
It is accompanied by increased sensitivity of the
maternal vasculature to pressor agents leading to
vasospasm and hypoperfusion of multiple organs.
Further, an activation of thecoagulationcascade
leads tomicrothrombiformation and aggravates the
perfusion problem.

Loss of plasma from the vascular tree


with the resultingedemaadditionally
compromises the situation.
These events lead to signs and
symptoms of toxemia including
hypertension, renal, pulmonary, and
hepatic dysfunction, andin eclampsia,
specificallycerebral dysfunction.
Preclinical markers of the disease
process are signs of increased platelet
andendothelial activation.

Placental hypoperfusion is linked to


abnormal modelling of the fetalmaternal
interface that may be immunologically
mediated.
The invasion of thetrophoblastappears to
be incomplete.
Adrenomedullin, a potent vasodilator, is
produced in diminished quantities by the
placenta in pre-eclampsia (and thus
eclampsia).

Other vasoactive agents are at play


includingprostacyclin,thromboxaneA2,n
itric oxide, andendothelinsleading to
vasoconstriction.
Many studies have suggested the
importance of a woman'simmunological
toleranceto her baby's father, whose
genes are present in the young fetus and
its placenta and which may pose a
challenge to her immune system.

Eclampsia is seen as a form ofhypertensive


encephalopathyin the context of those
pathological events that lead to pre-eclampsia.
It is thought that cerebralvascular resistanceis
reduced, leading to increased blood flow to the
brain. In addition to abnormal function of
theendothelium, this leads tocerebral edema.
Typically an eclamptic convulsion will not lead to
lasting brain damage; however, intracranial
haemorrhage may occur.

Diagnosis

Seizures during pregnancy that are


unrelated to pre-eclampsia need to be
distinguished from eclampsia.
Such disorders include seizure disorders as
well as brain tumor,aneurysm of the brain,
and medication- or drug-related seizures.
Usually the presence of the signs of severe
pre-eclampsia precede and accompany
eclampsia, facilitating the diagnosis.

Investigations:
CBC,
renal function test(RFT),
Liver function test(LFT),
coagulation screen,
plasma rate concentration,
24 hour urine analysis,
ultrasound

Prevention

Detection and management of preeclampsia is critical to reduce the risk of


eclampsia.
Appropriate management of women with
pre-eclampsia generally involves the use
of magnesium sulphate as an agent to
prevent convulsions, and thus
preventing eclampsia.

Treatment

The treatment of eclampsia requires


prompt intervention and to prevent
further convulsions, control the elevated
blood pressure, and immediately deliver
the baby if possible.
Antiseizure medicatio
Magnesium sulfateis commonly used
and when compared
todiazepam,phenytoinor a combination
ofchlorpromazine,promethazineandpet
hidineit results in better outcomes.

Blood pressure management


Blood pressure management at this stage in
pregnancy may consist ofhydralazineorlabetalol.
Delivery
If the baby has not yet been delivered, steps need
to be taken to stabilize the woman and deliver her
speedily.
This needs to be done even if the baby is
immature, as the eclamptic condition is unsafe for
both baby and mother.

As eclampsia is a manifestation of a multiorgan failure,


other organs (liver, kidney, lungs, cardiovascular
system, and coagulation system) need to be assessed in
preparation for a delivery (often acaesarean section),
unless the woman is already in advanced labor.
Regional anesthesia for caesarean section is
contraindicated when acoagulopathyhas developed.
Invasive haemodynamic monitoring
Invasive haemodynamic monitoring may be useful in an
eclamptic woman with severe cardiac disease, renal
disease, refractory hypertension, pulmonary edema,
andpoor urine output.

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