VASCULAR LESIONS OF THE BRAIN

CLASSIFICATION
Congenital Acquired Idiopathic
1. AVM & fistula 1. Traumatic 1. Moyamoya
2. Cavernous § Some arteriovenous fistula 2. Some arteriovenous
malformation § Traumatic aneurysm fistula
3. Telangiectasis 2. Degenerative
4. Venous anomaly § Atherosclerotic
(angioma) § Berry aneurysm
§ Arterial dissection
§ Spontaneous intracerebral h”ge
3. Infectious
§ Mycotic aneurysm

ARTERIOVENOUS MALFORMATIONS (AVM)

Pathophysiology § In the early embryonic stage, the circulatory system has vascular channels
around 200µm in diameter that will mature to form capillary channels
between the arterial and venous sides
§ Focal failure of maturation will lead to a nidus of persistent embryonic,
low-resistance vessels connecting the arteries and veins
§ Increased blood flow leads to enlargement of nidus, afferent arteries &
efferent veins predisposes to degeneration & aneurysm formation
§ Leads to intracerebral or subarachnoid haemorrhage
§ Brain tissue around the area can become an epileptic focus or undergo
ischaemia
Epidemiology § Most dangerous congenital vascular malformation
§ Cause intracranial bleeds and epilepsy in many cases
§ 0.1% of population
§ 90% - supratentorial; 10% - posterior fossa
§ 90% - single lesion; 9% - multiple
§ 1-2% - causes of all stroke; 3% young stroke, 9% subarachnoid bleed
Clinical § Presents between age 10-40 years old
presentation § Depends on: Age, size, location & vascular features
§ Symptoms:
o >50% present w brain haemorrhage
o 20-25% w seizures
o Localized headache
o 15% may have difficulty w movement, speech & vision
Brain § Abnormal & “weakened” blood vessels over time eventually burst from
haemorrhage high pressure of blood flow from the arteries
§ 1-3% chance per year of bleeding
§ Risk of bleeding = 105 – age (in years)
Diagnosis Typically identified on:
§ Cross-sectional imaging CT or MRI
§ Angiography – required to plan therapy
 Combination of MRI & angiography often used to assess likely success & risk of
surgical, endovascular or radiosurgical therapy

Angiography: Gold standard for diagnosis, treatment planning & follow up

after treatment
§ Anatomical & physiological info such as nidus & configuration
§ Its relationship to surrounding vessels
§ Localization of draining or efferent portion of brain AVM
Contrast transit time provides additional info regarding flow state of lesion
that is critical for endovascular treatment planning

The AVM is seen as a dense collection of

vessels (the nidus) that connect the arteries
directly to the veins without an intervening
capillary system.
Next is a picture depicting approximate
location of this AVM. Red arteries bring
oxygenated, high pressure blood to the AVM
nidus. Blue veins drain blood from the AVM.
Arrows indicate direction of the blood flow.
Treatment § Surgery is the mainstay of the treatment
§ Radiosurgery is a useful option in lesions at high risk for surgical therapy,
small AVM & lesion that has not bleed
§ Endovascular embolization can be useful adjunct to radiosurgery
o <5% cured by this approach alone
o Effective adjunct to radiosurgery & surgery
o Reduce nidus size of large brain AVM
o Reduce blood loss & to occlude vessels that may be difficult to control
during surgery
o A/w relatively low risk of disabling complications
§ Surgical excision – open microsurgical excision used as a definitive
treatment of selected patients w AVMs
o Immediate cure in patients considered to be at high risk of h”ge
o Complicated & often requires detailed planning w review of imaging
§ Stereotactic radiosurgery (Gamma knife)
o Indication: Small, difficult to reach by surgery
o Mechanism: Produce direct damage to vessels that will cause scar &
allow AVM to “clot off”
o Takes 2 years to cure AVM
Complications § Radiation necrosis; can produce new neurologic deficits & seizures
of radiosurgery § Cranial nerve deficits, headache & cyst formation
§ Incidence of complications related to location & volume treated
CAVERNOUS MALFORMATION (CMs)
Introduction § Also referred to as cavernous angiomas, cavernous hemangiomas, or
cavernomas
§ Collection of dilated blood vessels form a lesion
§ May occur sporadically or in a familial pattern
Gross § “Mulberry” appearance w engorged purplish clusters
§ Vary from 2mm to several cm in diameter
Microscopic Composed of dilated, thin walled capillaries w a simple endothelial lining and
a thin fibrous adventitia
§ Elastic fibers & smooth muscle not present in vessel walls
§ Classic description of CM = there is no intervening brain tissue b/w
channels of lesion. However, this may not be an essential criterion of CM
Location § Most common location: Cerebrum (70-90%)
§ Can occur throughout supratentorial compartment – most commonly
subcortical & predispose to rolandic & temporal areas
§ Posterior fossa lesiosn comprise pprox.. 25%; majority in pons & cerebellar
hemispheres
§ Spinal cord CM is reported
Clinical § M:F = 1:1
presentation § Mean age: 30-40y
§ Presentation specific to location
§ Supratentorial CMs = h”ge, siezures & progressive neurologic deficits
§ Infratentorial CMs = h”ge & progressive neurologic deficits
§ Lesions in brainstem = clinical neuropathies & long tract sign that cause ->
progressive neurologic decline dt high vol. of critical nuclei & fiber tracts
Imaging CT:
§ Look for haemorrhage
§ Usually demonstrates a non-specific irreg, hyperdense mass w variable
degrees of calcification
§ A faint perilesional blush w contrast administration is a variable &
non=specific finding

MRI:
§ Center containing small, high intensity signal foci surrounded by a null
signal corona, dark on T1 & T2 weighted images

Angiography:
§ A capillary blush or early draining vein; similar to angiographic
appearance of meningiomas
§ Digital subtraction angiography appears to be much more sensitive than
MRI for detecting presence of CM-assoc. atypical venous drainage
Treatment Asymptomatic: Observe, irrespective of location
 Surgical resection indication: Progressive neurologic deficit, intractable
epilepsy & recurrent haemorrhage
Fig:
a) CT scan brain plain showing
well-defined lesion in left
temporoparietal region with
evidence of hemorrhage

b) Follow-up CT scan showing

complete excision

BERRY ANEURYSM
Introduction § Most subarachnoid hemorrhages (SAH) are caused by ruptured
intracranial saccular (berry) aneurysms.
Epidemiology § Prevalence 3.2% in a population
§ Mean age of 50 years, 1:1 gender ratio
§ 20 to 30% have multiple cerebral aneurysms
§ Rupture of an intracranial aneurysm account for 0.4 to 0.6% of all deaths.
§ Approximately 10 percent of patients die prior to reaching the hospital,
and only one-third has a "good result" after treatment.
Location § Most intracranial aneurysms ~85 percent% are located in the anterior
circulation, predominantly on the circle of Willis.
§ Common sites include:
o Junction of the anterior communicating artery w anterior cerebral a.
o Junction of the posterior communicating artery w internal carotid a.
o Bifurcation of the middle cerebral artery
§ Posterior circulation sites include:
§ Top of basilar artery
§ Junction of basilar artery and superior or anterior inferior cerebellar a.
§ Junction of vertebral artery and the posterior inferior cerebellar a.

Risk factors § Hereditary syndromes:

o Connective tissue diseases (Ehlers-Danlos syndrome,
pseudoxanthoma elasticum polycystic kidney disease)
o Familial aneurysm
§ Hypertension
§ Cigarette smoking
§ Alcohol consumption
§ Estrogen deficiency
§ Coarctation of aorta
Pathogenesis § Aneurysms form in relation to defects in smooth muscle containing tunica
media, commonly at branch points or at the edge of atheroma
§ Tunica intima then stretches outward, fragmenting the internal elastic
lamina and carrying the connective tissue of adventitia outward with it
§ Laplace law: at any given pressure, the stretching force on the wall
increases as the diameter increases
Clinical § Asymptomatic unless rupture
manifestation Rupture:
§ SAH, sudden onset headache w meningism, usually no focal neurologic
deficit, comatose
§ Acute hydrocephalus
§ Vasospasm
Some unruptured aneurysm:
§ Mass effect: Headache, visual acuity loss, cranial neuropathies (CNIII
palsy), pyramidal tract dysfunction & facial pain
§ Emboli: Ischaemia
Investigation § CT brain, CTA: Blood in the subarachnoid space
§ Lumbar puncture: blood in CSF
§ Cerebral angiogram
Treatment § Open craniotomy & clipping of aneurysm
§ Endovascular occlusion
§ Segmental occlusion +/- bypass using graft

SPONTANEOUS INTRACEREBRAL HAEMORRHAGE

Introduction § 2nd most common cause of stroke, 10% of all stroke
Etiologies § Older patients: degenerative changes associated w chronic hypertension
§ Younger patients: vascular malformation, aneurysm or drug abuse
§ Other causes: cerebral infarction transformation, cerebral amyloid
angiopathy, coagulopathy, tumours
Pathogenesis of § Penetrator vessels in chronic hypertension develop intimal hyperplasia
hypertensive with hyalinosis in the vessel wall à predisposes to focal necrosis à
vasculopathy causing breaks in the wall of the vessel
§ These microscopic "pseudoaneurysms" have been associated with small
amounts of blood outside their walls.
§ Massive hemorrhage can occur when the clotting system is unable to
compensate for the disruption in the vessel wall.
Clinical § Vary according to location & size of haemorrhage
presentation § Onset & progression: Neurologic s&s usually increase gradually over min
or few hours. However, some are obtunded or comatose when first
discovered or upon arrival to ED
§ Headache, vomiting & decreased level of consciousness:
o Headache dt traction on meningeal pain fibers, ↑ ICP, blood in CSF
o Stiff neck & meningism, if there is intraventricular blood
Investigations Non-contrast head CT:
§ Evaluate for presence of acute ICH; define size & location, extension into
ventricular sys, surrounding edema, & shifts in brain contents (herniation).
No further investigation needed unless:
§ Atypical haematoma location or appearance, presence of SAH, younger
patients, history of neoplasm, blood dyscrasias, bacterial endocarditis
§ MRI, MRA, Cerebral angiogram
Treatment § Most do not require surgery
§ Ventricular drainage to relieve hydrocephalus
§ Surgery for evacuation of haematoma
CEREBRAL AMYLOID ANGIOPATHY (CAA)
§ Deposition of congophilic material in small- to medium-sized blood
vessels of the brain and leptomeninges
§ This weakens structure of vessel walls and makes them prone to bleeding
§ CAA usually manifests with spontaneous lobar haemorrhage
OTHER CAUSES OF NON-TRAUMATIC ICH
Other causes § AVM § Moyamoya disease
§ Dural arteriovenous fistula § Vasculitis
§ Hemorrhagic infarction (including § Cerebral hyperperfusion
cerebral venous thrombosis) syndrome
§ Septic embolism, mycotic aneurysm § Reversible cerebral
§ Brain tumor vasoconstriction syndromes
§ Bleeding disorders, liver disease, (RCVS)
thrombolytic therapy § Drugs
§ Central nervous system infection (eg,
herpes simplex encephalitis)