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Dr. Vinayak Hiremath
SAH = extravasation of blood into the
CLASSIFICATION OF STROKE
(20% of Strokes)
(80% of Strokes)
Subarachnoid hemorrhage (SAH) is a pathologic
condition that exists when blood enters the
The most common cause of SAH is trauma
The most common cause of spontaneous SAH is an
aneurysmal bleed (65-80%)
Anatomy of Subarachnoid space
In the central nervous system, the subarachnoid
cavity (subarachnoid space) is the interval between
the arachnoid membrane and pia mater.
It is occupied by spongy tissue consisting of trabeculae
(delicate connective tissue filaments that extend from
the arachnoid mater and blend into the pia mater) and
intercommunicating channels in which the
cerebrospinal fluid is contained.
This cavity is small on the surface of the hemispheres
of the brain. On the summit of each gyrus the pia
mater and the arachnoid are in close contact, but in
the sulci between the gyri, triangular spaces are left, in
which the subarachnoid trabecular tissue is found.
At certain parts of the base of the brain, the arachnoid
is separated from the pia mater by wide intervals,
which communicate freely with each other and are
named subarachnoid cisternae; in these the
subarachnoid tissue is less abundant.
The subarachnoid space is the location of the interface
between the vascular tissue and the cerebrospinal
fluid and is active in the blood brain barrier.
When blood vessels of the brain are weak, abnormal, or under unusual pressure, a
hemorrhagic stroke can occur. In hemorrhagic strokes, bleeding may occur within the
brain, as an intracerebral hemorrhage. Or bleeding may occur between the inner and
middle layer of tissue covering the brain (in the subarachnoid space), as a subarachnoid
Incidence about 9-10/100,000/yr
Higher in Japan (3 times) and Finland
Mean age of onset 51 years (6th decade)
Facts and figures
Men predominate until age 40, then more
women (55%); some studies – 3:2 ratio
Seasonal (winter/spring), diurnal (late morning)
and day (Sunday)
30 % rupture during sleep
About 50 % of patients with an aneurysm have
warning prior to SAH (6-20 days)
The Overall case fatality varied from 32-67%
Population-based study in England with essentially
complete case ascertainment
24 hour mortality: 21%
7 days: 37%
30 days: 44%
Pobereskin JNNP 2001;70:340-3
Highest mortality occurs immediately after the
hemorrhage and then decreases rapidly
Rebleeding is estimated to occur in 50% of
ruptured aneurysms within 6 months of the
first hemorrhage, and afterwards at 3% per year
50-60% of patients die after rebleeding and 25%
are left disabled
Probably the best natural history data come from S
Pakarinen: (Incidence, aetiology, and prognosis of primary subarachnoid
hemorrhage: a study based on 589 cases diagnosed in a defined urban population
during a defined period. Neurol Scand (Suppl) 1967; 29:1-128)
Unselected series, involving the entire city of Helsinki,
from 1954 to 1961; minimal surgical withdrawals; all
sudden deaths were autopsied
Mortality at 1 day was 32%
Mortality at 1 week was 43%
Mortality at 1 month was 56%
Mortality at 6 months was 60%
Alcohol consumption (debatable)
Diurnal variations in blood pressure
Pregnancy and parturition
Slight increased risk during lumbar puncture
and/or cerebral angiography in patient with
Slight increased risk with advancing age
Following cocaine abuse
3rd trimester of
>excessive intake of
foods high in fats and
Bleeding into the brain tissue
and subarachnoid space
Blood Clots in the
Epileptic Seizure :
BLOCKAGE OF THE
CEREBRAL BLOOD FLOW/VOLUME/METABOLISM
CBF is globally decreased after SAH
Mean CBF decreases with time after SAH;
it reaches a nadir in 10 to 14 days, after which it slowly
increases toward normal, In patients with poor grades, CBF
and cerebral metabolism may remain depressed for weeks ;
Cerebral blood volume is markedly increased in patients
with severe neurological deficits
INTRACRANIAL PRESSURE RESPONSES
Mean ICP was 10 mm Hg in patients with clinical grades
1 and 2, 18 mm Hg in patients with clinical grades 2 and
3, and 29 mm Hg in those with clinical grades 3 to 5.
Vasospasm, which was more common in patients with a
poor clinical grade and larger SAH, was associated with a
significant rise in ICP from a mean of 16 mm Hg in those
without vasospasm to 29 mm Hg in those with
An aneurysm is an abnormal widening or
ballooning of a portion of an artery due to
weakness in the wall of the blood vessel
Mechanism of formation of aneurysms
One theory is – that they result from a developmental defect
in the media & elastica
Other theory says that aneurysmal process is initiated by the
focal destruction of internal elastic membrane caused by
hemodynamic forces acting at bifurcations & branching of
As a result of local weakness in vessel wall intima bulges out
covered only by adventetia
The sac gradually enlarges and subsequently rupture
For patients of SAH who arrive alive at hospital, the
mortality rate over the next month is about 45%. Of those
who survive, more than 50% are left with major
neurologic deficits as a result of
Cerebral vasospasm with infarction, or
If the patient survives but the aneurysm is not
obliterated, the rate of rebleeding is about
20% in the first 2 weeks,
30% in the first month, and about
3% per year afterwards
The annual risk of rupture for aneurysms <10 mm in
size is 0.1%, and for aneurysms >10 mm in size is 0.5–
the surgical morbidity rate far exceeds these
GIANT ANEURYSMS, those >2.5 cm in diameter
account for 5% of cases.
The three most common locations are
-The terminal internal carotid artery,
-Middle cerebral artery (MCA) bifurcation, and
-Top of the basilar artery.
Their risk of rupture is ~6% in the first year after
identification and may remain high indefinitely.
They often cause symptoms by compressing the adjacent
brain or cranial nerves.
Saccular aneurysms occur at the bifurcations of the large to
medium-sized intracranial arteries.
Rupture is into the
in the basal cisterns and often into
the parenchyma of the adjacent brain.
Approximately 85% of aneurysms occur in the anterior circulation,
mostly on the circle of Willis.
About 20% of patients have multiple aneurysms,
- - many at mirror sites bilaterally.
Aneurysms are seldom seen in childern on
autopsy and so SAH in childern is rare
Beyond childhood they gradually increase in size & the
frequency of rupture increases between 35-65 yrs of age
There is increased incidence of
fibromuscular dysplasia of extracranial arteries,
moyamoya, AV malformations &
coarctation of aorta
Numerous studies have documented familial occurrence of
saccular aneurysms, lending support to the idea that
genetic factors may play a role in their development 35
As an aneurysm develops, it typically forms a neck with a
At the site of rupture (most often the dome) the wall thins,
and the tear that allows bleeding is often 0.5 mm long.
Aneurysm size and site are important in
predicting risk of rupture.
Those >7 mm in diameter and
Those at the top of the basilar artery and
At the origin of the posterior
communicating artery are at greater risk of
The main symptom is a severe headache that starts suddenly
(often called thunderclap headache). It is often worse near the
back of the head. Many persons often describe it as the "worst
headache ever" and unlike any other type of headache pain.
The headache may start after a popping or snapping feeling in
Eye discomfort in bright
Mood and personality changes, including and
Nausea and vomiting
often with neck stiffness, and
vomiting is common
Pain at other sites
Occipital and posterior cervical pain may signal a
posterior inferior cerebellar artery (PICA) or anterior
inferior cerebellar artery aneurysm.
Pain in or behind the eye and in the low temple can
occur with an expanding MCA aneurysm
Loss of consciousness
At the moment of aneurysmal rupture with major
SAH, the ICP suddenly rises.
This may account for the sudden transient loss of
consciousness that occurs in nearly half of patients
which may be preceded by headache.
In 10% of cases, aneurysmal bleeding is severe
enough to cause loss of consciousness for several
What causes symptoms & signs?
Blood leaking from the
Local pressure effects of the
CLINICAL MANIFESTATIONS- cont.
FOCAL NEUROLOGICAL DEFICITS
Although sudden headache in the absence of focal
neurologic symptoms is the hallmark of aneurysmal rupture,
FOCAL NEUROLOGIC DEFICITS MAY OCCUR.
Anterior communicating artery or MCA bifurcation
aneurysms may rupture into the adjacent brain or subdural
space and form a hematoma large enough to produce mass
The common deficits that result include hemiparesis,
aphasia, and abulia (progressive drowsiness or slow
Anatomic – clinical corellation of
focal neurological Deficits
Third nerve palsy (ptosis, diplopia,dilatation of pupil &
divergent squint) indicates an aneurysm at the junction of
post. Comunicating &post. Cerebral arteries
A sixth nerve palsy may indicate an aneurysm in the
cavernous sinus .but uni or bilateral palsy could be because
of raised ICP
Unilateral blindness indicates aneurysm lying
anteromedially in the circle of willis usually at the origin of
Anatomic – clinical corellation of
focal neurological Deficits- cont
Transient paresis of one or both lower limbs indicates ant
comunicating A. aneurysm that has interfered with
circulation in ant. Cerebral arteries.
Hemiperesis or aphasia suggests aneurysm at the first
bifurcation of middle cerebral artery
The side of aneurysmal rupture may be
unilateral preponderace of headache
hemorrhage (Terson Syndrome)
Other conditions which may present as severe
headache need to be differentiated, like:-
Cerebral venous thrombosis,
Diffuse vasospasm (call-flemming syndrome),
Intracranial or extracranial arterial dissection
CSF examination assumes great importance in
differentiating these conditions from SAH 52
D/D- SAH to be differentiated from
THUNDERCLAP HEADACHE is a variant of
migraine that simulates a SAH.
Headache of explosive onset may also be
caused by ingestion of sympathomimetic
drugs or ingestion of tyramine containg foods
in a pt. who is taking MAO inhibitors
Sudden severe headache may also be a
symptom of phaeochromocytoma
Before concluding that a patient with sudden, severe
headache has thunderclap migraine or any other type
of severe headache,
a definitive workup for aneurysm is always required in
the form of CT scan, Lumbar puncture, and if the
diagnosis still remains doubtful, then cerebral
DELAYED NEUROLOGIC DEFICITS
There are four major causes of delayed
Acute hydrocephalus can cause stupor and
coma and can be mitigated by placement of
an external ventricular drain.
More often, subacute hydrocephalus may
develop over a few days or weeks and causes
progressive drowsiness or slowed mentation
(abulia) with incontinence.. It may clear
spontaneously or require temporary
Chronic hydrocephalus may develop
weeks to months after SAH and present
as normal pressure hydrocephalus
(NPH) manifested by gait difficulty,
incontinence, or impaired mentation.
Subtle signs may be a lack of initiative
in conversation or a failure to recover
Causes symptomatic ischemia and infarction in ~30% of
patients and is the major cause of delayed morbidity and
Signs of ischemia appear 4–14 days after the hemorrhage,
most often at 7 days.
The severity and distribution of vasospasm determine
whether infarction will occur.
Vasospasm is believed to result from direct effects of
clotted blood and its breakdown products on the arteries
within the subarachnoid space.
Spasm of major arteries produces focal symptoms
referable to the appropriate vascular territory.
All of these focal symptoms may present abruptly,
fluctuate, or develop over a few days.
In most cases, focal spasm is preceded by a decline in
Patient may develop fluctuating hemiperisis or aphasia
Severe cerebral edema in patients with infarction from
vasospasm may increase the ICP enough to reduce
cerebral perfusion pressure.
Hyponatremia. Hyponatremia may be profound and can develop
quickly in the first 2 weeks following SAH. There is both
natriuresis and volume depletion with SAH, so that patients
become both hyponatremic and hypovolemic.
Both atrial natriuretic peptide (ANP)and brain
natriuretic peptide (BNP) have a role in
producing this "cerebral salt-wasting syndrome.“
There may some role of antidiuretic hormone also, causing water
Typically it clears over the course of 1–2 weeks and, in the setting
of SAH, should not be treated with free-water restriction as this
may increase the risk of stroke. 60
SYSTEMIC CHANGES ASSOCIATED WITH SAH ECG changes suggestive of subendicardial or myocardial ischemia
Elevation of troponis/CPK (MB) levels
In some patients cardiac dysfunction may be severe enough to
cause fall in EF % and heart failure
There is evidence that structural myocardial lesions produced by
circulating catecholamines and excessive discharge of sympathetic
neurons may occur after SAH, causing these ECG changes and a
reversible cardiomyopathy sufficient to cause shock or congestive
NON CONTRAST CT SCAN.
CT Scan is Initial investigative procedure
The extent and location of subarachnoid blood on
noncontrast CT scan help
locate the underlying aneurysm,
identify the cause of any neurologic deficit, and
predict delayed vasospasm.
A high incidence of symptomatic vasospasm in the MCA
and ACA has been found when early CT scans show
subarachnoid clots >5 x 3 mm in the basal cisterns or layers
of blood >1 mm thick in the cerebral fissures.
CT scans less reliably predict vasospasm in the vertebral,
basilar, or posterior cerebral arteries. 63
More than 95% of cases have enough blood to be
visualized on a high-quality NONCONTRAST CT SCAN
obtained within 72 h.
CT scan reveals subarachnoid hemorrhage in the right sylvian fissure; no evidence of
hydrocephalus is apparent.
(A)Noncontrast CT demonstrates subarachnoid hemorrhage (arrows). (B)3-D
reconstruction image from a CT angiogram demonstrates an aneurysm (arrow) from the
anterior communicating artery as the cause of the bleed.
Exact site of offending aneurysm can be inferred from the
location of main clot in CT scan
Collection of blood in the anterior inter-hemispheric fissure
indicates rupture of an ant. Communicating artery
Blood in sylvian fissure – middle cerebral artery
Blood in ant. Perimesencephalic cistern- post.
Communicating artery or distal basilar artery aneurysm
MRI can also detect blood in the proton density sequence
If the scan fails to establish the diagnosis of SAH and no
mass lesion or obstructive hydrocephalus is found,
Then a lumbar puncture should be performed to establish
the presence of subarachnoid blood.
Usually CSF becomes grossly bloody within 30 minutes of
bleed with RBC counts upto 1 million/c mm or more
Lysis of the red blood cells and subsequent conversion of
hemoglobin to bilirubin stains the spinal fluid yellow within
This xanthochromic spinal fluid peaks in intensity at 48 h
and lasts for 1–4 weeks, depending on the amount of
subarachnoid blood 68
Once the diagnosis of hemorrhage from a ruptured
saccular aneurysm is suspected, four-vessel
conventional x-ray angiography (both carotids and both
vertebrals) is generally performed
to localize and define the anatomic details of the
offending aneurysm and
to determine if other unruptured aneurysms exist).
CT angiography is an alternative method for locating
the aneurysm and may be sufficient to plan definitive
in 5-10% pts with aneurysmal bleed , there may not be
any evidence of aneurysmal rupture on angiography.
It may be because
in some cases obliteration of lesion occurs
In some lesions were somewhat more benign
Pts without evidence of an offending aneurysm or an
arteriovenous malformationhave a better prognosis
Perimesencephalic hemorrhage also caries a better
In this cisterns surrounding midbrain & upper pons
gets filled up with blood
Mild headache, no vasospasm
Usually no aneurysm is found at the expected site i.e.
at the top of basillary artery
Lab Inv. – cont.
Close monitoring (daily or twice daily) of electrolytes
is important because hyponatremia can occur
precipitously during the first 2 weeks following SAH
An asymptomatic troponin elevation is common.
The electrocardiogram (ECG) frequently shows ST-
segment and T-wave changes similar to those
associated with cardiac ischemia.
Prolonged QRS complex, increased QT interval, and
prominent "peaked" or deeply inverted symmetric T
waves are usually secondary to the intracranial
hemorrhage ( cerebral T waves).
Serious ventricular dysrhythmias are unusual
ECHO Echocardiography reveals a pattern of regional wall
motion abnormalities that follow the distribution of
sympathetic nerves rather than the major coronary
The sympathetic nerves themselves appear to be injured
by direct toxicity from the excessive catecholamine
The medical management of SAH focuses on
Protecting the airway,
Managing blood pressure
Preventing rebleeding prior to treatment,
Treating hyponatremia, and
Preventing pulmonary embolus.
Because rebleeding is common, all patients who are not candidates for
early aneurysm repair are put on bed rest in a quiet room and are given
stool softeners to prevent straining.
If Headache or neck pain is severe, mild sedation and analgesia are
Extreme sedation is avoided because it can obscure changes in
Adequate hydration is necessary to avoid a decrease in blood volume
predisposing to brain ischemia.
So adequate amount of fluid is administered so as to maintain above
normal circulating blood volume & central venous pressure
Managing raised ICP
Intracranial hypertension following aneurysmal rupture
occurs secondary to
acute hydrocephalus, or
loss of vascular autoregulation.
Perilesional edema if infarct occurs
Managing raised ICP- cont.
Patients who are stuporous should undergo emergent
ventriculostomy to measure ICP and to treat high ICP
in order to prevent cerebral ischemia.
Medical therapies designed to combat raised ICP e.g.,
mild hyperventilation, mannitol, and sedation can also
be used as needed. (Mannitol dose-25-100gm
High ICP refractory to treatment is a poor prognostic
Prior to definitive treatment of the ruptured aneurysm,
care is required to maintain adequate cerebral perfusion
while avoiding excessive elevation/fall of arterial pressure.
If the patient is alert, it is reasonable to lower the blood
pressure to <150 systolic using nicardipine, labetolol, or
If the patient has a depressed level of consciousness, then
ICP(Intracranial pressure) should be measured and the
cerebral perfusion pressure targeted to 60–70 mmHg.
Managing Seizure Activity
Seizures are uncommon at the onset of aneurysmal
The quivering, jerking, and extensor posturing that often
accompany loss of consciousness with SAH are probably
the sharp rise in ICP or, perhaps,
acute generalized vasospasm rather than seizure.
Use of anti-seizure drugs is controvercial.
However, phenytoin is often given as prophylactic therapy
since a seizure may promote rebleeding.
MANAGING VASOSPASM Vasospasm remains the leading cause of morbidity
and mortality following aneurysmal SAH.
Treatment with the calcium channel antagonist
nimodipine (60 mg PO every 4 h) improves outcome,
perhaps by preventing ischemic injury
Nimodipine can cause significant hypotension in
some patients, which may worsen cerebral ischemia in
patients with vasospasm
Managing Hydrocephalus Acute hydrocephalus can cause stupor or coma.
It may clear spontaneously or require temporary
external ventricular drainage.
When chronic hydrocephalus develops, permanent
ventricular shunting is the treatment of choice.
Role of Steroids
Glucocorticoids may help reduce the head and neck
ache caused by the irritative effect of the subarachnoid
There is no good evidence that they reduce cerebral
edema, are neuroprotective, or reduce vascular injury,
Their routine use therefore is not recommended
Adjunctive Therapies and Measures
Keep the patient's core body temperature at 37.2°C
Consider antiemetics for nausea or vomiting.
Elevate the head of the bed 30° to facilitate intracranial
venous drainage. Emergent ventricular drainage by the
neurosurgeon may be necessary.
Maintain the patient's serum glucose level at 80-120
mg/dL; use sliding or continuous infusion of insulin if
Fluids and hydration
Do not over hydrate patients because of the risks of
Patients with subarachnoid hemorrhage (SAH) may also
have hyponatremia from cerebral salt wasting.
Early aneurysm repair prevents
allows the safe application of techniques
to improve blood flow (e.g., induced
hypertension and hypervolemia)
An aneurysm can be "clipped" by a
neurosurgeon or "coiled" by an
Hunt - Hess scale
is helpful in assessing the patient
before deciding for intervntional
Mild headache, normal mental status, no cranial nerve or
motor findings –(GCS* score 15, no motor deficits)
Severe headache, normal mental status, may have cranial nerve
deficit –(GCS score 13–14, no motor deficits)
Somnolent, confused, may have cranial nerve or mild motor
DEFICIT- (GCS SCORE 13–14, WITH MOTOR DEFICITS)
GRADE 4 :
Stupor, moderate to severe motor deficit, may have intermittent
reflex posturing- (GCS score 7–12, with or without motor
Coma, reflex posturing or flaccid (GCS score 3–6, with or
without motor deficits)
Management based on HH scaleGrades 1 & 2
Current approach is to go for intervention within 24
This will prevent rebleed and will allow measures to be
taken to improve cerebral circulation
Grade 3 patients- if their condition allows , they too
would be benefitted by the procedure
Grade4- the outcome is generally poor but some times by
putting a ventricular drain and improving the grade, pt89
•Involves placing a metal clip across the aneurysm
neck, thereby immediately eliminating the risk of
•This approach requires craniotomy and brain
retraction, which is associated with neurologic
involve placing platinum
coils, or other embolic
material, within the
aneurysm via a catheter that
is passed from the femoral
. Endovascular techniques-CONT
The aneurysm is packed tightly to enhance thrombosis
and over time is walled-off from the circulation
The benefit of endovascular therapy is durable.
However, some aneurysms have a morphology that is
not amenable to endovascular treatment.
Thus, surgery remains an important treatment option
But for aneurysms which are not approachable by
surgery, endovascular technique would become the
ANEURYSM TRIAL (ISAT)
The only prospective randomized trial of surgery
versus endovascular treatment for ruptured aneurysm,
the International Subarachnoid Aneurysm Trial
(ISAT), was terminated early when 24% of patients
treated with endovascular therapy were dead or
dependent at 1 year compared to 31% treated with
surgery, a significant 23% relative reduction.
After 5 years, risk of death was lower in the coiling
Although the proportion of survivors who were
independent was the same in both groups
Risk of rebleeding was low, but more common in the
GRADE CLINICAL PRESENTATION
3 DROWSY WITH DEFICIT
4 MAJOR NEURODEFICIT
BOTTERELL ET AL
patient will need to have someone with for the next several
days to watch for worsening of symptoms (see below) and to
allow to rest.
Start with light activity around the house for the first 3 days
Gradually increase activity starting with short walks 1-2 times
Avoid contact sports, skating, bike riding, or other such
activities for 6 weeks.
Encourage pt to do passive range of motion
Instruct the relative to feed pt on time with proper food low in
Low in cholesterol low in fat and give citrus fruits ,moderate
in fluid intake and increase fiber diet to improve health..
Take medications as prescribed and
gradually decrease pain medications as pain
Instruct pt and their relative to follow
Educate and instruct the patient and her
family to monitor BP and PR before giving
Follow up with primary care physician for
all medical issues.
Genetics of Intracranial
Ref. Neurosurgery clinics of North America, July 1998, pp 485-493
Observations to suggest genetic involvement:
Association of various heritable disorders with ICA
The familial aggregation of ICA in the absence of known systemic
Heritable Disorders associated with ICA:
Heritable CT disorder account for at least 5% of the cases of ICA
True frequency may be higher due to variability in phenotypic
expression and negative family history due to new mutation
The most important ones are:
Ehlers-Danlos type IV
Ehlers-Danlos Syndrome Type IV
Defeciency of collagen type 3
Mutation in the gene encoding for pro 1(III) chain of type III collagen
Joint hypermobility, hyperelastic/fragile skin, abnormal scaring
Type IV is least common and the most lethal
Vascular catastrophes account for the great majority of deaths
Clues to the diagnosis
Facial appearance: thin nasal bridge, thin lips, lobeless ears,
prematurely aged appearance
Bruising tendency, MVP, spontaneous pneumothorax, abnormal
scarring, varicose veins
Prevalence?, 14 in 202 patient
Saccular or fusiform, located in the cavernous sinus
Thin walled, difficult to repair
Mutation in gene encoding fibrillin-1 (glycoprotein, major
component of microfbrils in ECM and elastic tissues)
Abnormalities of the skeleton, cardiovascular system, eyes,
Variability in the phenotypic expression
Saccular or fusiform, dissecting aneurysms
Propensity to the proximal internal carotid artery
Fragility of the connective tissue is not a major problem
Ectasia and tortuosity of the vessels
Neurofibromatosis type 1
Mutation in NF1 gene encoding neurofibromin which
may have regulatory role in the development of
Café-au-lait spot, lish nodule, neurofibroma
Vascular complication; stenosis rupture, aneurysm,
Saccular or fusiform
Vascular fragility, distorted anatomy
Association with intracranial arterial occlusive disease
Mutation in two genes PKD1 and PKD2
PKD1 linked disease is more severe
Polycystin encoded by PKD1 is an integral membrane protein, play
a role in the integrity of ECM of CT
Most common monogenetic disorder
Cysts in kidney, liver, spleen, pancreas, ovaries
Cardiovascular abnormalities: MVP, aortic and cervicocephalic
dissection, coronary and AAA
Saccular, fusiform, dissecting
Ruputre occurs in earlier age, increased risk of developing new ICA
ICA in 25% of patient with ADPKD at autopsy, 10% on MRA
Family screening with MRA yields ICA in 20-25%
Cause of death in one-fifth
ADPKD account for 2-7% of all ICA
First described by Chamber et al. In 1954
With the exception of ADPKD, Ehlers-Danlos IV,
Pompe disease, Idiopathic nonarteriosclerotic
cerebral calcification there is no association with
heritable CT disease
7-20% of first or second-degree relatives of patient
with SAH have ICA
First-degree relatives of patient with SAH have twice
to fivefold increase risk of having ICA
First-degree relatives have threefold increased risk
compared to second-degree relatives
Inheritance pattern is unknown, most likely
multifactorial, or AD
Rupture at younger age (5 year earlier), 71% has
ruptured by age of 50 Vs 42% in nonfamilial ICA
Anterior communicating artery aneurysms are
underrepresented, MCA is overrepresented
Rupture at smaller size
Increase female preponderance
Siblings are more likely to harbor aneurysms at the
same side at mirror sites
Severity of hemorrhage is similar to nonfamilial ones
Screening for familial ICA
Poor prognosis once ruptured
Low surgical risk for the nonruptured (5% morbidity, 2% mortality)
Caveat: aneurysms may develop over short period of time
Indication for surgery for asymptomatic ICA is still unclear (critical size)
Families with two or more affected members
Restricted to first-degree relatives (yield 9-29%)
Screening between the ages of 35-65
Youngest patient with familial ICA is 6 years old
De novo aneurysms occur at a rate of 2% per year
Repeat screening at 6-month to 5-year intervals
Screening for familial ICA …/cont’d
MRA is the most widely used
Critical size for detection 3-5 mm
Helical CT angiography
Screen the human genome for ICA gene by testing a
large number of distinct highly pleomorphic genetic
Analyze variations in the sharing of marker alleles
among affected sibling pairs
Candidate gene sequence analysis e.g., PKD1 or COL3A1
Heritable Disorders Associated with
Locus Gene Gene Product
Achondroplasia AD 4p16.3 FGFR3 Fibroblast growth
factor receptor 3
Alkaptonuria AR 3q2 AKU ?
ADPKD AD 16p13.3 PKD1 Polycystin
Cohen syndrome AR 8q22 CHS1 ?
Syndrome type I
AD 9q COL5A1 Ollagen type V
Syndrome type IV
AD 2q31 COL3A1 Collagen type III
Fabry disease AR XL-R Xq22.1 -galactosidase A
Kahn syndrome AR ? ? ?
Marfan’s syndrome AD 15q21.1 FBN1 Fibrillin-1
NF1 AD 17q11.2 NF1 Neurofibromin
Noonan syndrome AD 12q22 NS1 ?
Heritable Disorders Associated with
Intracranial Aneurysms …/cont’d
Locus Gene Gene Product
AD 9q34.1 HHT1 Endoglin
imperfecta type 1
AD 17q22.1 COL1A1 Collagen type 1
Pompe disease AR 17q23 GAA -Glucosidase
AD & AR ? ? ?
AR ? ? ?
Tuberous sclerosis AD 9q34/16p13.3 TSC1/TSC2 ?/Tuberin
Wermer syndrome AD 11q13 MEN1 ?
3M syndrome AR ? ? ?
AcoD 14q32.1 P1 1-Antitrypsin
How well a patient with subarachnoid
hemorrhage does depends on a number of
different factors, including:
Location and amount of bleeding
Older age and more severe symptoms can lead
to a poorer outcome.
People can recover completely after treatment.
But some people die even with treatment.
SAH is a neurolgical emergency
It is less common but an important cause of stroke(5%)
saccular/berry aneurysmal rupture is the commonest cause of
Most common presenttion is an excruciating headache which
needs to be differentiated from other causes of severe headache
including thunderclap migrane
CT scan is very helpful in making a dianosis and differentiating it
from other headaches of severe intensity
If CT is negative but there s strong suspecion of SAH then one
should go for CSF examination
If othe CT scan & CSF are inconclusive then cerebral
angiography is the last resort
Complications include rerupture, hydrocephalus, vasospasm
Management icludes – bed rest, maintaing a clear airway,
managing BP, giving adequate amount of fluids,
monitoring hyponatremia preventing vasospasm and
instituting measures to lower ICP
one third of patients are prone to develop rebleed which
can be effectively taken care of by interventional modalities
i.e. surgical (clipping)/ endovascular technique (coiling)
Randomzed trial (ISAT) has shown coiling to superior to
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