Neurovascula

r disorders
Alexandra NedelcoffGhiranlieff
Erasmus

Content
Aneurysms
Arteriovenous

malformations (AVMs)
Carotid cavernous fistulas
Carotid, vertebral and cerebral arterial
stenoses
Strokes

Aneurysms
1Classification
1.1Saccular aneurysms
1.2Fusiform aneurysms
1.3Microaneurysms
2Signs and symptoms
2.1Subarachnoid
haemorrhage
2.2Microaneurysms
3Risk factors
3.1Genetic association
s
4Pathophysiology
4.1Saccular aneurysms

5Complications
5.1Rupture
5.2Vasospasm
6Diagnosis
7Treatment
7.1Surgical clipping
7.2Endovascular coilin
g
7.3Cerebral bypass sur
gery
8Prognosis
9Epidemiology

An intracranial
aneurysm(also
calledcerebralorbrain
aneurysm) is a
cerebrovasculardisorder
in which weakness in the
wall of acerebralartery
orveincauses a
localizeddilationor
ballooning of the blood
vessel.

Classification
Cerebral aneurysms are classified both by size and shape.
Small aneurysms have a diameter of less than 15mm.
Larger aneurysms include those classified as large (15 to 25mm),
giant (25 to 50mm), and super giant (over 50mm)
Saccular aneurysms
Saccular aneurysms, also known as berry aneurysms = round outpouching
- most common form of cerebral aneurysm.
Fusiform aneurysms
Fusiform dolichoectatic aneurysms = a widening of a segment of an artery around
the entire blood vessel, rather than just arising from a side of an artery's wall.

- usually they do not rupture.

Microaneurysms

Microaneurysms, also known asCharcot-Bouchard aneurysms

- typically occur in smallblood vessels(less than 300micrometre
diameter), most often thelenticulostriatevessels of thebasal ganglia, and are
associated withchronichypertension.
- common cause ofintracranial hemorrhage

Signs and symptoms

A

small, unchanging aneurysm will produce

few, if any, symptoms. Before a larger
aneurysm ruptures:
- a sudden and unusually severe
headache,nausea, vision impairment,vomiting,
andloss of consciousness, or the individual may
be asymptomatic (i.e., experiencing no
symptoms at all)

Subarachnoid
haemorrhage
If an aneurysm ruptures, blood leaks into the space around the brain.
This is called asubarachnoid hemorrhage . Onset is usually sudden
with noprodrome.
- classically presenting as a "thunderclap headache" worse
than previous headaches.
Symptoms of a subarachnoid hemorrhage differ depending on the
site and size of the aneurysm:
a sudden severe headache that can last from several hours to days
nausea and vomiting
drowsiness, confusion and/or loss of consciousness
Visual abnormalities
Meningism

Microaneurysms
A

ruptured microaneurysm may cause an

intracerebral hemorrhage, presenting as a focal
neurological deficit.
Rebleeding,hydrocephalus(the excessive
accumulation ofcerebrospinal fluid),vasospasm
(spasm, or narrowing, of the blood vessels), or
multiple aneurysms may also occur. The risk of
rupture from an unrruptured cerebral aneurysm
varies according to the size of an aneurysm, with
the risk rising as the aneurysm size increases.

Risk factors
Intracranial aneurysms may result from diseases
acquired during life(hypertension,smoking, excess
alcoholconsumption, cocaine use,
trauma,
infections andobesity)
or from genetic conditions:
- autosomal dominant polycystic kidney, disease
,neurofibromatosis type I,Marfan syndrome, multiple
endocrine neoplasiatype
I,
pseudoxanthoma
elasticum,
hereditary hemorrhagic telangiectasia
and Ehlers-Danlos syndrometype II and IV.

Pathophysiology
- Saccular aneurysms

lack oftunica mediaand elastic lamina around

its dilated location (congenital), with wall of sac
made up of thickened hyalinized intima and
adventitia.
~ 25% patients = multiple aneurysms,
predominantly where is familial pattern
affected arteries : Anterior communicating artery,
Posterior communicating artery, Middle cerebral
artery, Internal carotid artery, Tip ofbasilar
artery

Complications
Rupture
Vasospasm

Diagnosis
angiography,
magnetic
CT

resonance imaging,

scans,
cerebrospinal fluid(CSF) analysis (shows
presence of blood)

Treatment
Surgical

clipping
Endovascular coiling
Cerebral bypass surgery

Epidemiology
incidence = 1 per 10,000 persons in the
United States (approximately 27,000
cases a year), with incidence highest in
30-60 year-olds.
occurs more in women, by a ratio of 3 to
2, and are rarely seen in pediatric
populations.

Arteriovenous malformation
(AVMs)
an abnormal connection betweenarteriesandveins,
bypassing thecapillary system.
occurrs in thecentral nervous system, but can appear
in any location. cal problems.
Although many AVMs are asymptomatic, they can
cause intense pain or bleeding or lead to other serious
medical problems.
AVMs are usuallycongenitaland belong to the
RASopathies. The genetic transmission patterns of AVM,
if any, are unknown. AVM is not generally thought to be
an inherited disorder, unless in the context of a specific
hereditarysyndrome.

Diagnosis

Computerized

Tomography (CT),
Magnetic Resonance Imaging (MRI)
MRA (Magnetic Resonance Angiogram)

Treatment
symptomatic,
AVM-specific treatment may also involve
endovascular embolization, neurosurgery or
radiation therapy.
The Spetzler-Martin grading system
developed at the
Barrow Neurological Instituteis utilized by
neurosurgeons to determine operative
versus nonoperative management of AVMs.

Carotid-cavernous fistula
(CCF)
results

from an abnormal
communication between the arterial and
venous systems within thecavernous
sinusin the skull. It is a type of
arteriovenous fistula.
arterial blood under high pressure
enters the cavernous sinus, the normal
venous return to the cavernous sinus is
impeded and this causes engorgement
of the draining veins, manifesting most
dramatically as a sudden engorgement
and redness of the eye of the same side.

Causes
- closed or penetrating head trauma, surgical damage,
rupture of an intracavernous aneurysm, or in association
with connective tissue disorders, vascular diseases and
dural fistulas.

Classification
Type

Description

Fistulous supply from the

internal carotid artery

Supply from the dural

branches of internal carotid
artery

from meningeal branches of

ext carotid artery

combined ICA+ECA

Presentation
bruit(a

humming sound within the skull due to

high blood flow through the arteriovenous
fistula), progressive visual loss, and pulsatile
proptosisor progressive bulging of the eye due
to dilatation of the veins draining the eye. Pain
is the symptom that patients often find the
most difficult to tolerate.
Patients usually present with sudden or
insidious onset of redness in one eye,
associated with progressive proptosis or
bulging.

Diagnosis
CT scans show diffuse enlargement of all the extraocular muscles resulting from
venous engorgement and a characteristically enlarged superior ophthalmic vein.
Selective arteriography is used to evaluate arteriovenous fistulas.

Treatment
endovascular therapy: transarterial or transvenous .
Occasionally: direct transorbital puncture of the cavernous sinus or cannulation of
the draining superior orbital vein are used when conventional approaches are not
possible.
Direct CCF may be treated by occlusion of the affected cavernous sinus (coils,
balloon, liquid agents), or by reconstruction of the damaged internal carotid artery
(stent, coils or liquid agents).
Indirect CCF may be treated by occlusion of the affected cavernous sinus with coils,
liquid agents or a combination of both.

Stroke
neurological deficit of cerebrovascular cause that
persists beyond 24 hours or is interrupted by
death within 24 hours"

Classification
Ischemic
Hemorrhagic

Ischemic
blood supply is decreased, leading to dysfunction of the brain tissue

in that area. There are four reasons why this might happen:
Thrombosis (obstruction of a blood vessel by a blood clot forming
locally)
Embolism (obstruction due to anembolusfrom elsewhere in the
body, see below),
Systemic hypoperfusion (general decrease in blood supply, e.g., in
shock)
Venous thrombosis.

Hemorrhagic

Hemorrhagic
accumulation of blood anywhere within the
skull vault. A distinction is made between
intra-axial hemorrhage(blood inside the
brain) andextra-axial hemorrhage(blood
inside the skull but outside the brain). Intraaxial hemorrhage is due tointraparenchymal
hemorrhageorintraventricular hemorrhage
(blood in the ventricular system).

Signs and symptoms

typically

start suddenly, seconds to minutes, and

in most cases do not progress further.
The symptoms depend on the area of the brain
affected. Some forms of stroke can cause
additional symptoms. For example, in
intracranial hemorrhage, the affected area may
compress other structures. Most forms of stroke
are not associated withheadache, apart from
subarachnoid hemorrhage and cerebral venous
thrombosis and occasionally intracerebral
hemorrhage.

Loss of consciousness, headache, and vomiting

hemiplegiaandmuscle weakness of the face
numbness
reduction in sensory or vibratory sensation
initialflaccidity(hypotonicity), replaced byspasticity
(hypertonicity),hyperreflexia, and obligatory synergies
altered smell, taste, hearing, or vision (total or partial)
drooping of eyelid (ptosis) and weakness of
ocular muscles
decreased reflexes: gag, swallow, pupil reactivity to
light
decreased sensation and muscle weakness of the face
balance problemsandnystagmus
altered breathing and heart rate
weakness insternocleidomastoid musclewith inability
to turn head to one side
weakness in tongue (inability to protrude and/or move
from side to side)

aphasia(difficulty with verbal expression, auditory

comprehension,readingand/orwritingBroca'sor
Wernicke's areatypically involved)
dysarthria(motor speech disorderresulting from
neurological injury)
apraxia(altered voluntary movements)
visual fielddefect
memory deficits (involvement oftemporal lobe)
hemineglect(involvement ofparietal lobe)
disorganized thinking, confusion,hypersexual
gestures (with involvement of frontal lobe)
lack of insightof his or her, usually stroke-related,
disability
altered walkinggait
altered movement coordination
vertigoand or disequilibrium

Causes
Thrombotic

stroke
Embolic stroke
Systemic hypoperfusion
Venous thrombosis
Intracerebral hemorrhage
Silent stroke

Pathophysiology
Micrographshowingcortical
pseudolaminar necrosis, a
finding seen in strokes on
medical imagingand atautopsy
.H&E-LFB stain.

Micrographof the superficial

cerebral cortexshowing neuron
loss andreactive astrocytesin a
person that suffered a stroke.
H&E-LFB stain.

Diagnosis
Physical examination
medical historyof the symptoms and a neurological status,
NIH stroke scale.

Imaging
For diagnosing ischemic stroke in the emergency setting: [42]
CT scans (withoutcontrast enhancements)
sensitivity= 16%specificity= 96%MRI scan
sensitivity= 83%specificity= 98%For diagnosing
hemorrhagic stroke in the emergency setting:
CT scans (withoutcontrast enhancements)
sensitivity= 89%specificity= 100%MRI scan
sensitivity= 81%specificity= 100%

Risk factors
Blood

pressure
Atrial fibrillation
Blood lipids
Diabetes mellitus
Anticoagulation drugs
Surgery
Diet

Management
Definitive

therapy is aimed at removing the

blockage by breaking the clot down (
thrombolysis), or by removing it mechanically
(thrombectomy).
Tight control of blood sugars in the first few
hours does not improve outcomes and may
cause harm.High blood pressure is also not
typically lowered as this has not been found to
be helpful.
Hemicraniectomy