STROKE AND CEREBROVASCULAR DISEASE

Stroke is the 2nd common cause of death (9%) and a major cause of disability. The
annual death rate is about 200 per 100 000 (12% of all deaths). Rates are higher in
Asian and black African populations than in Caucasians. Stroke is uncommon below
the age of 40 and commoner in males. The death rate following stroke is 20-25%.
Hypertension is the most treatable stroke risk factor; stroke is decreasing in the 40-60
age groups because hypertension is treated. Thromboembolic infarction (80%),
cerebral and cerebellar hemorrhage (10%) and subarachnoid hemorrhage (SAH)
(about 5%) are the main causes; arterial dissection and arteriovenous malformations
also contribute.
Definitions
 Stroke. Is defined as a syndrome of rapid onset of cerebral deficit (usually
focal) lasting >24 hours or leading to death, with no cause apparent other than
a vascular one.
 Completed stroke : the deficit has become maximal, usually within 6 hours.
 Stroke-in-evolution describes progression during the first 24 hours.
 Minor stroke. Patients recover without significant deficit, usually within a
week.
 Transient ischemic attack (TIA) means a sudden focal deficit lasting from
seconds to 24 hours with complete recovery). TIAs have a tendency to recur,
and may herald thromboembolic stroke.

Pathophysiology
Completed stroke
This is caused by:

 Arterial embolism from a distant site.

 Arterial thrombosis.
 Hemorrhage (intracranial or SAH).

Less commonly, other processes cause stroke:

 venous infarction
 carotid or vertebral artery dissection
 polycythemia and hyperviscosity syndromes
 fat and air embolism.
 multiple sclerosis.
 Mass lesions.

Transient ischemic attacks

TIAs are usually the result of microemboli. TIAs may be caused by a fall in cerebral
perfusion (e.g. a cardiac dysrhythmia, postural hypotension or decreased flow
through atheromatous arteries). Infarction is usually averted by autoregulation (p.
1127). Rarely, tumors and subdural hematomas cause episodes indistinguishable
from thromboembolic TIAs: a clinical TIA is thus not a reliable indicator of
thromboembolism. Principal sources of emboli are cardiac thrombi and
atheromatous plaques/thrombi within the great vessels, carotid and vertebral systems.
Cardiac thrombi (mural and valvular) follow atrial fibrillation, often secondary to
valvular disease, or myocardial infarction. Polycythemia is also a cause.
Thromboembolism from sources outside the brain generates 80% of TIAs and also
70% of strokes.
Carotid and vertebral artery dissection
Dissection accounts for around 20% of strokes below age 40 and is sometimes a
sequel of head or neck trauma. Stroke, TIA or sudden headache/migraine-like
symptoms occur, sometimes with neck pain at the site of dissection.
Risk factors and prevention
Low-dose aspirin is used in primary prevention in men and women with a 10-year risk
of coronary heart disease greater than 10%.
For brain hemorrhage, hypertension, bleeding disorders, anticoagulants and
antiplatelet drugs, pre-existing cerebral aneurysms and AVMs are risk factors.

Table 21-25. Factors reducing stroke risk

    Reduction in stroke risk

Risk factor Action Infarction Haemorrhage SAH

Hypertension Treat and monitor ++ ++ Probable

Smoking Stop ++ + Probable

Lifestyle More active + 0 0

Alcohol Moderate intake + 0 0

High cholesterol Statins, diet + 0 0

Raised hematocrit Reduce + 0 0

Atrial fibrillation Anticoagulate + Increases risk 0

slightly

Obesity Weight reduction Probable Probable 0

Diabetes Good control Probable 0 0

Severe carotid Surgery ++ 0 0

stenosis
 Sleep apnea Treat + 0 0

++: Major correlation with reduced stroke risk, +: moderate correlation,

SAH: subarachnoid hemorrhage.

Rarer risk factors and other causes of stroke

1. Thrombocythemia and thrombophilia (protein C deficiency, factor V Leiden)
predispose to cerebral venous thrombosis.
2. Anti-cardiolipin and lupus anticoagulant antibodies (antiphospholipid
syndrome predispose to arterial thrombotic strokes in young patients.
3. Endocarditis.
4. Low-dose estrogen-containing OCPs do not increase stroke risk significantly
in healthy women but probably do so with other risk factors, e.g. uncontrolled
hypertension or smoking.
5. Migraine.
6. Vasculitis (polyarteritis, (SLE), GCA, and granulomatous CNS angiitis).
7. Amyloidosis can present as recurrent cerebral hemorrhage.
8. Hyperhomocysteinemia predisposes to thrombotic strokes. Folic acid therapy
does not reduce the incidence.
9. Neurosyphilis, mitochondrial disease.
10. Drugs of abuse e.g. cocaine, cold remedies containing vasoconstrictors.
11. Cox-2 inhibitors

Vascular anatomy
The circle of Willis is supplied by the two internal carotid arteries and by the basilar.
Stenosis and plaques proximal to the circle of Willis are seen typically at four
extracranial sites :

1. origin of common carotid artery (1)

2. origin of internal carotid artery (2)
3. origin of vertebral artery (3)
4. Subclavian artery (4) and carotid artery syphon - within the cavernous sinus.

Autoregulation
Usually, constant cerebral blood flow (CBF) is maintained at mean arterial blood
pressures between 60 and 120 mmHg, smooth muscle in small arteries responding
directly to changes in pressure. CBF is normally independent of perfusion pressure,
i.e. there is autoregulation.
In disease, CBF autoregulation can fail. Contributory causes are:

 severe hypotension with S BP <75 mmHg

 severe hypertension with S BP >180 mmHg
 increase in blood viscosity, e.g. polycythemia
 raised ICP
 Increase in arterial PCO2 and/or fall in arterial PO2.
 CLINICAL SYNDROMES
Transient ischemic attacks (TIAs)
The most common features are Hemiparesis and aphasia, others are:
Amaurosis fugax
This is a sudden transient loss of vision in one eye. When due to the passage of
emboli through the retinal arteries it is often the first clinical evidence of internal
carotid artery stenosis - and forerunner of a hemiparesis. Amaurosis fugax also occurs
as a benign event in migraine.
Transient global amnesia
Episodes of amnesia/confusion lasting several hours, occurring principally in people
over 65 and followed by complete recovery, are presumed to be due to posterior
circulation ischemia. The exact cause is unknown. Episodes rarely recur and there is
little evidence that they are actually thromboembolic.
Clinical findings in TIA
Table 21-26. Features of transient ischaemic attacks
Anterior circulation Posterior circulation
Carotid system Vertebrobasilar system
Amaurosis fugax Diplopia, vertigo, vomiting
Aphasia Choking and dysarthria
Hemiparesis Ataxia
Hemisensory loss Hemisensory loss
Hemianopic visual Hemianopic visual loss
loss
  Bilateral visual loss
  Tetraparesis
  Loss of consciousness (rare)
  Transient global amnesia (possibly)
. Consciousness is usually preserved in TIA. There may be clinical evidence of a
source of embolus, e.g.:

 carotid arterial bruit (stenosis)

 atrial fibrillation or other dysrhythmia
 valvular heart disease/endocarditis
 recent myocardial infarction
 Difference between right and left brachial BP.

An underlying condition may be evident:

 atheroma
 Hypertension, postural hypotension.
 bradycardia or low cardiac output
 diabetes mellitus
 rarely, arteritis, polycythaemia, neurosyphilis, HIV
 Antiphospholipid syndrome.

Differential diagnosis
TIAs can be distinguished from other transient episodes:
 Mass lesions: occasionally give identical features to TIAs.
  Focal epilepsy is usually recognized by its positive features (e.g. limb jerking
and loss of consciousness) and progression over minutes. In a TIA,
involuntary limb movements do occur occasionally; deficit is usually
instantaneous.
 A focal prodrome in migraine sometimes causes diagnostic difficulty.
Headache, common but not invariable in migraine, is rare in TIA. Typical
migrainous visual disturbances are not seen in TIA.
Prognosis
Prospective studies show that 5 years after a single thromboembolic TIA:

 30% have had a stroke, a third of these in the first year

 15% have suffered a myocardial infarct.

TIAs in the anterior cerebral circulation carry a more serious prognosis than those in
the posterior circulation.
Cerebral infarction
Following vessel occlusion brain ischaemia occurs, followed by infarction. The
infarcted region is surrounded by a swollen area which does not function but is
structurally intact. This is the ischaemic penumbra, which is detected on MRI and can
regain function with neurological recovery.
Within the ischaemic area, hypoxia leads to neuronal damage. There is a fall in ATP
with release of glutamate, which opens calcium channels with release of free radicals.
These alterations lead to inflammatory damage, necrosis and apoptotic cell death.
Clinical features
The stroke most typically seen is caused by infarction in the internal capsule
following thromboembolism in a middle cerebral artery branch. A similar picture is
caused by internal carotid occlusion .Limb weakness on the opposite side to the
infarct develops over seconds, minutes or hours. There is a contralateral hemiplegia or
hemiparesis with facial weakness. Aphasia is usual when the dominant hemisphere is
affected. Weak limbs are at first flaccid and areflexic. Headache is unusual.
Consciousness is usually preserved. Exceptionally, an epileptic seizure occurs at the
onset. After a variable interval, usually several days, reflexes return, becoming
exaggerated. An extensor plantar response appears. Weakness is maximal at first;
recovery occurs gradually over days, weeks or many months.
Brainstem infarction
 The lateral medullary syndrome (thrombosis of posterior inferior cerebellar
artery (PICA) or its branches, and Wallenberg's syndrome) is a common
example of brainstem infarction presenting as acute vertigo with cerebellar
and other signs. It can follow vertebral artery thromboembolism or dissection.
 Coma follows damage to the brainstem reticular activating system.
 The locked-in syndrome is caused by upper brainstem infarction.
 Pseudobulbar palsy can follow lower brainstem infarction.

Table 21-27. Features of brainstem infarction

Clinical feature Structure involved
Hemiparesis or tetraparesis Corticospinal tracts
Sensory loss Medial lemniscus and spinothalamic
tracts
Diplopia Oculomotor system
 Facial numbness Vth nerve nuclei
Facial weakness VIIth nerve nucleus
Nystagmus, vertigo Vestibular connections
Dysphagia, dysarthria IXth and Xth nerve nuclei
Dysarthria, ataxia, hiccups, vomiting Brainstem and cerebellar connections
Horner's syndrome Sympathetic fibres
Coma, altered consciousness Reticular formation
Table 21-28. Clinical signs in the lateral medullary syndrome (PICA thrombosis)
Ipsilateral Contralateral
Facial numbness (Vth) Spinothalamic sensory loss
Diplopia (VIth) Hemiparesis (mild, unusual)
Nystagmus  
Ataxia (cerebellar)  
Horner's syndrome  
IXth and Xth nerve lesions  

Other patterns of infarction

1. Lacunar infarction
Lacunes are small (<1.5 cm3) infarcts seen on MRI or at autopsy. Hypertension is
commonly present. Lacunar infarction is often symptomless.
2. Hypertensive encephalopathy
This is due to cerebral oedema, causing severe headaches, nausea and vomiting.
Agitation, confusion, fits and coma occur if the hypertension is not treated.
Papilloedema develops, either due to ischaemic optic neuropathy or following the
brain swelling due to multiple acute infarcts. MRI shows oedematous white matter in
the parieto-occipital regions.
3. Multi-infarct dementia (vascular dementia)
Multiple lacunes or larger infarcts cause generalized intellectual loss seen with
advanced cerebrovascular disease. In the late stages, there is dementia, pseudobulbar
palsy and a shuffling gait (small steps), sometimes called atherosclerotic
parkinsonism. Binswanger's disease is a term for widespread low attenuation in
cerebral white matter, usually with dementia, TIAs and stroke episodes in
hypertensive patients (the changes being seen on imaging/autopsy).
4. Visual cortex infarction(Anton's syndrome)
PCA infarction or infarction of the MCA macular branch causes combinations of
hemianopic visual loss and cortical blindness.
5. Weber's syndrome
is due to a unilateral infarct in the midbrain, there are
Ipsilateral IIIrd nerve palsy with contralateral hemiplegia. Paralysis of upward gaze is
usually present.
6. Watershed (borderzone) infarction
Cortical infarcts, often multiple, follow prolonged periods of low perfusion (e.g.
hypotension after massive myocardial infarction or cardiac bypass surgery) between
areas supplied by the anterior, middle and posterior cerebral arteries. Cortical visual
loss, memory loss and intellectual impairment are typical. In some cases a vegetative
state or minimal conscious state follows.
Acute stroke: immediate care, and thrombolysis
Paramedics and members of the public are encouraged to make the diagnosis of stroke
on a simple history and examination - FAST:

 Face - sudden weakness of the face

 Arm - sudden weakness of one or both arms
 Speech - difficulty speaking, slurred speech
 Time - the sooner treatment can be started, the better

The benefit of thrombolysis is shown on CT perfusion scans and decreases with time,
even within the time window of 4.5 hours. Every minute counts. If thrombolysis is not
given, aspirin 300 mg daily should be given as soon as a diagnosis of ischaemic stroke
or thromboembolic TIA is confirmed, reducing to 75 mg after several days. Following
thrombolysis aspirin should not be started until 24-48 hours later.
Investigations
The purpose of investigations in stroke is:

 to confirm the clinical diagnosis and distinguish between haemorrhage and

thromboembolic infarction
 to look for underlying causes and to direct therapy
 To exclude other causes, e.g. tumour.

Sources of embolus should be sought (e.g. carotid bruit, atrial fibrillation, valve
lesion, evidence of endocarditis, previous emboli or TIA) and hypertension/postural
hypotension assessed. Brachial BP should be measured on each side; >20 mmHg
differences are suggestive of subclavian artery stenosis.
Table 21-29. Thrombolysis in acute ischaemic stroke
Eligibility
Age ≥ 18 years
Clinical diagnosis of acute ischaemic stroke
Assessed by experienced team
Measurable neurological deficit
Blood tests: results available
CT or MRI consistent with acute ischaemic stroke
Timing of onset well established
Thrombolysis should commence as soon as possible and up to 4.5 hours after acute
stroke
Exclusion criteria
Historical
Stroke or head trauma within the prior 3 months
Any prior history of intracranial haemorrhage
Major surgery within 14 days
Gastrointestinal or genitourinary bleeding within the previous 21 days
Myocardial infarction in the prior 3 months
Arterial puncture at a non-compressible site within 7 days
Lumbar puncture within 7 days
Clinical
Rapidly improving stroke syndrome
Minor and isolated neurological signs
Seizure at the onset of stroke if the residual impairments are due to Postictal phenomena
Symptoms suggestive of subarachnoid haemorrhage, even if the CT is normal
Acute MI or post-MI pericarditis
Persistent systolic BP > 185, diastolic BP > 110 mmHg, or requiring aggressive therapy
to control BP
Pregnancy or lactation
Active bleeding or acute trauma (fracture)
Laboratory
Platelets < 100 000/mm3
Serum glucose < 2.8 mmol/L or > 22.2 mmol/L
INR > 1.7 if on warfarin
Elevated partial thromboplastin time if on heparin
Dose of i.v. alteplase (tissue plasminogen activator)
Total dose 0.9 mg/kg (maximum 90 mg)
10% of total dose by initial i.v. bolus over 1 minute
Remainder infused i.v. over 60 minutes
Imaging in acute stroke
CT and MRI:
 Non-contrast CT will demonstrate haemorrhage immediately but cerebral
infarction is often not detected or only subtle changes are seen initially.MRI
shows changes early in infarction and a later MRI shows the full extent of the
damaged area or penumbra.
 Diffusion-weighted MRI (DWI) can detect cerebral infarction immediately but
is as accurate as CT for the detection of haemorrhage.

Box 21.1 Stroke: further investigation and management

Further investigations

 Routine bloods (for ESR, polycythaemia, infection,

vasculitis, thrombophilia, syphilitic serology, clotting
studies, autoantibodies, lipids)
 Chest X-ray
 ECG
 Carotid Doppler studies
 MR angiography, if appropriate

Further management

 Drugs for hypertension, heart disease, diabetes, other medical

conditions
 Other antiplatelet agents, e.g. dipyridamole
 Question of endarterectomy
 Question of anticoagulation.
 Speech therapy, dysphagia care, physiotherapy, occupational
therapy
 Specific issues, e.g. epilepsy, pain, incontinence
  Preparations for future care

CT is still more widely available than MRI and should be performed if MRI is
unavailable so that there is no delay in giving thrombolysis for cerebral infarction.
More detailed studies involving perfusion-weighted images and diffusion-weighted
MRI will differentiate the infarct core and the penumbral area which is potentially
recoverable.
Treatment of acute stroke
. Thrombolysis has been shown to improve outcome and should be used immediately
if there are no contraindications. In a massive middle cerebral artery infarct,
hemispheric swelling occurs with oedema. Decompressive hemicraniectomy reduces
the intracranial pressure and the mortality but extensive neurological deficits remain.
Later investigations
MR angiography (MRA) or CT angiography
is valuable in anterior circulation TIAs to confirm surgically accessible arterial
stenoses, mainly internal carotid stenosis. If ultrasound suggests carotid stenosis,
normotensive patients with TIA or stroke in the anterior circulation should have
vascular imaging.
Box 21.2 Stroke: immediate management
1. Admit to multidisciplinary stroke unit
2. General medical measures
o Airway: confirm patency and monitor
o Continue care of the unconscious or stuporose patient
o Oxygen by mask
o Monitor BP
o Look for source of emboli
o Assess swallowing
3. Is thrombolysis appropriate?
If so immediate brain imaging is essential.
4. Brain imaging
CT should always be available. This will indicate
haemorrhage, other pathology and sometimes infarction. MR
is better overall, if immediately available
5. Cerebral infarction
If CT excludes haemorrhage, give immediate thrombolyte
therapy. Aspirin, 300 mg/day is given if thrombolytic therapy
is contraindicated
6. Cerebral haemorrhage
If CT shows haemorrhage, give no drugs that could interfere
with clotting. Neurosurgery may occasionally be needed

Carotid Doppler and duplex scanning.

This screen for carotid (and vertebral) stenosis and occlusion: in skilled hands they
demonstrate accurately the degree of internal carotid stenosis.
Long-term management
Medical therapy
Risk factors should be identified and addressed.
1. Antihypertensive therapy
Control of high blood pressure is the major factor in primary and secondary stroke
prevention. Transient hypertension, often seen following stroke, usually does not
require treatment provided diastolic pressure does not rise >100 mmHg. Sustained
severe hypertension needs treatment; BP should be lowered slowly to avoid any
sudden fall in perfusion.
2. Antiplatelet therapy
Long-term soluble aspirin (75 mg daily) reduces the incidence of further infarction
following thromboembolic TIA or stroke. Aspirin inhibits cyclo-oxygenase, which
converts arachidonic acid to prostaglandins and thromboxanes; predominant
therapeutic effects are reduction of platelet aggregation. Clopidogrel and
dipyridamole are also used. Combined aspirin 75 mg daily and dipyridamole 200 mg
twice daily possibly provide optimal prophylaxis against further thromboembolic
stroke or TIA.
3. Anticoagulants
Heparin and warfarin should be given when there is atrial fibrillation, other
paroxysmal dysrhythmias, cardiac valve lesions (uninfected) or cardiomyopathies.
Brain haemorrhage must be excluded by CT/MRI. Patients must be aware of the small
risk of cerebral (and other) haemorrhage. Anticoagulants are potentially dangerous in
the two weeks following infarction because of the risk of provoking cerebral
haemorrhage.
4. Other measures
Polycythaemia and any clotting abnormalities should be treated. Statin therapy should
be given for all.
5. Surgical approaches
 Internal carotid endarterectomy
Surgery is usually recommended in TIA or stroke patients with internal carotid artery
stenosis >70%. Successful surgery reduces the risk of further TIA/stroke by around
75%. Endarterectomy has a mortality around 3%, and a similar risk of stroke. PCTA
(stenting) is an alternative. The value of surgery for asymptomatic carotid stenosis is
debatable.
Stroke in the elderly
Table 21-30. Anticoagulants and stroke prevention
Indication Comment
Valvular heart disease Heparin/warfarin of benefit in chronic rheumatic heart disease,
particularly mitral stenosis
Recent MI
  Intracardiac Heparin/warfarin if there is evidence of Intracardiac thrombus
thrombus
  Atrial fibrillation Anticoagulants long term reduce stroke incidence in atrial
fibrillation
Acute internal carotid Anticoagulants reserved for imaging-confirmed cases of
artery thrombus arterial thrombosis or dissection. They have not been shown to
Acute basilar artery be beneficial in stroke prevention after thromboembolism from
thrombus carotid or vertebrobasilar sources
Internal carotid artery
dissection
Extracranial vertebral
artery dissection
Prothrombic states, Consider anticoagulation, in consultation with haematologist
e.g. protein C
deficiency
Recurrent TIAs or If no remediable cause, a trial of anticoagulants may be
stroke on full justified
antiplatelet therapy
Cerebral venous Benefits of anticoagulation outweigh risks of haemorrhage
thrombosis including
sinus thrombosis
Whilst in the elderly the yield of investigation in stroke diminishes, age is no barrier
to recovery; elderly patients benefit the most from good rehabilitation. Consider social
isolation, pre-existing cognitive impairment, nutrition, skin and sphincter care, and
reassess swallowing. Carotid endarterectomy over 75 years carries little more risk
than in younger cases.
 Rehabilitation: multidisciplinary approach
Of particular value in the first few weeks after stroke to relieve spasticity, prevent
contractures and teach patients to use walking aids. Baclofen and/or botulinum toxin
are sometimes helpful in the management of severe spasticity.
.
Prognosis
About 25% of patients die within 2 years of a stroke, nearly 10% within the first
month. This early mortality is higher following intracranial haemorrhage than
thromboembolic infarction. Poor outcome is likely when there is coma, a defect in
conjugate gaze and hemiplegia.
Recurrent strokes are common (10% in the first year) and many patients die
subsequently from MI. Of initial stroke survivors, some 30-40% remains alive at 3
years.
Intracranial haemorrhage
This comprises:

 intracerebral and cerebellar haemorrhage

 subarachnoid haemorrhage
 Subdural and extradural haemorrhage/haematoma.

 Intracerebral haemorrhage
Etiology
Causes approximately 10% of strokes. Rupture of microaneurysms (Charcot-
Bouchard aneurysms, 0.8-1.0 mm diameter) and degeneration of small deep
penetrating arteries are the principal pathology. Such haemorrhage is usually
massive, often fatal, and occurs in chronic hypertension and at well-defined sites -
basal ganglia, pons, cerebellum and subcortical white matter.
In normotensive patients, particularly over 60 years, lobar intracerebral haemorrhage
occurs in the cerebral cortex. Cerebral amyloid angiopathy (rare) is the cause in
some of these haemorrhages, and the tendency to rebleed is associated with particular
apolipoprotein E genotypes.
Recognition
Intracerebral haemorrhage tends to be dramatic with severe headache. It is more likely
to lead to coma than thromboembolism.
Brain haemorrhage is seen on CT imaging immediately as intraparenchymal,
intraventricular or subarachnoid blood. Routine MRI may not identify an acute small
haemorrhage correctly in the first few hours but MRI diffusion weighted (MRI DW)
is as good as CT.
Management: haemorrhagic stroke
The principles are those for cerebral infarction. The immediate prognosis is less good.
Antiplatelet drugs and, of course, anticoagulants are contraindicated. Control of
hypertension is vital. Urgent neurosurgical clot evacuation is occasionally necessary
when there is deepening coma and coning (particularly in cerebellar haemorrhage).
Cerebellar haemorrhage
There is headache, often followed by stupor/coma and signs of cerebellar/brainstem
origin (e.g. nystagmus, ocular palsies). Gaze deviates towards the haemorrhage. Skew
deviation may develop. Cerebellar haemorrhage sometimes causes acute
hydrocephalus, a potential surgical emergency.
Subarachnoid haemorrhage (SAH)
Of dramatic onset. SAH accounts for some 5% of strokes and has an annual incidence
of 6 per 100 000.
Causes
The causes of SAH are shown in Table 21.31; it is unusual to find any contributing
disease.
Saccular aneurysms develop within the circle of Willis and adjacent arteries. Common
sites are at arterial junctions:

 between posterior communicating and internal carotid artery - posterior

communicating artery aneurysm( is the commonest cause of a painful IIIrd
nerve palsy)
 between anterior communicating and anterior cerebral artery - anterior
communicating and anterior cerebral artery aneurysm
 At the trifurcation or a bifurcation of the middle cerebral artery - middle
cerebral artery aneurysm.

Other aneurysm sites are on the basilar, PICA, intracavernous internal carotid and
ophthalmic arteries. Saccular aneurysms are an incidental finding in 1% of autopsies
and can be multiple.
Aneurysms cause symptoms either by spontaneous rupture, when there is usually no
preceding history, or by direct pressure on surrounding structures.
Arteriovenous malformation (AVM)
AVMs are collections of arteries and veins of developmental origin. An AVM may
also cause epilepsy, often focal. Once an AVM has ruptured, the tendency is to
rebleed - 10% will then do so annually.
Cavernous haemangiomas (cavernomas) are common (<0.5% of population) and
consist of collections of capillary vessels; they are frequently symptomless and seen
incidentally on imaging. Rarely cavernomas cause seizures or bleed; exceptionally
they cause sudden death from massive haemorrhage. Surgery is rarely appropriate.
Table 21-31. Underlying causes of subarachnoid haemorrhage
Saccular (berry) aneurysms 70%
Arteriovenous malformation (AVM) 10%
No arterial lesion found 15%
Rare associations (< 5%)  
Bleeding disorders  
Mycotic aneurysms - endocarditis  
 Acute bacterial meningitis  
Tumours, e.g. metastatic melanoma, oligodendroglioma  
Arteritis (e.g. SLE)  
Spinal AVM → spinal SAH  
Coarctation of the aorta  
Marfan's, Ehlers-Danlos syndrome  
Polycystic kidneys  
Clinical features of subarachnoid haemorrhage
There is a sudden devastating headache, often occipital. usually followed by
vomiting , coma and death. SAH is a possible diagnosis in any sudden headache.
Following major SAH there is neck stiffness and a positive Kernig's sign.
Papilloedema is sometimes present, with retinal and/or subhyaloid haemorrhage .
Minor bleeds cause few signs, but almost invariably headache.
Investigations
CT imaging is the immediate investigation needed. LP is not necessary if SAH is
confirmed by CT. CSF becomes yellow (xanthochromic) several hours after SAH.
Visual inspection of supernatant CSF is usually sufficiently reliable for diagnosis.
Spectrophotometry to estimate bilirubin in the CSF released from lysed cells is used
to define SAH with certainty. MR angiography is usually performed in all
potentially fit for surgery, i.e. generally below 65 years and awake. In some, no
aneurysm or source of bleeding is found, despite a definite SAH.
Differential diagnosis
From migraine. Thunderclap headache is used (confusingly) to describe either SAH
or a sudden (benign) headache for which no cause is ever found. Acute bacterial
meningitis occasionally when a meningeal microabscess ruptures or at the onset.
Cervical arterial dissection can present with a sudden headache.
Complications
Obstructive hydrocephalus, seen on CT. Shunting may be necessary. Arterial
spasm (visible on angiography and a cause of coma or hemiparesis) is a serious
complication of SAH and a poor prognostic feature.
Management
Immediate treatment is bed rest and supportive measures. Hypertension control.
Dexamethasone to reduce cerebral oedema; it also is believed to stabilize the blood-
brain barrier. Nimodipine, a calcium-channel blocker, reduces mortality.
Nearly half of cases are die before reaching hospital. Of the remainder, a further 10-
20% rebleeds and dies within several weeks.
Subdural and extradural bleeding
These conditions can cause death following head injuries unless treated promptly.
Subdural haematoma (SDH)
This follows rupture of a vein. Usually following a head injury, sometimes trivial.
The interval between injury and symptoms can be days, or extend to weeks or months.
Chronic, apparently spontaneous SDH is common in the elderly, and also occurs
with anticoagulants.
Headache, drowsiness and confusion are common; symptoms are indolent and can
fluctuate. Focal deficits develop. Epilepsy occasionally occurs. Stupor, coma and
coning may follow.
Extradural haemorrhage (EDH)
Typically follows a linear skull vault fracture tearing a branch of the middle
meningeal artery. Extradural blood accumulates rapidly over minutes or hours. A
characteristic picture is of a head injury with a brief duration of unconsciousness,
followed by improvement (the lucid interval). The patient then becomes stuporose,
with an ipsilateral dilated pupil and contralateral hemiparesis, with rapid
transtentorial coning. Bilateral fixed dilated pupils, tetraplegia and respiratory
arrest follow. An acute progressive SDH presents similarly.
Management
Needs immediate imaging. CT is the mostly used. MRI is more sensitive for the
detection of small hematomas. T1 weighted MRI shows bright images due to the
presence of methaemoglobin.
EDHs require urgent neurosurgery: if N/A drainage through skull burr-holes has
been lifesaving.
Subdural bleeding usually needs less immediate attention. Even large collections can
resolve spontaneously without drainage. Serial imaging is needed to assess progress.
CORTICAL VENOUS THROMBOSIS AND DURAL VENOUS SINUS
THROMBOSIS (intracranial venous thrombosis)
Are usually (>50%) associated with a pro-thrombotic risk factor, e.g. OCPs,
pregnancy, genetic or acquired pro-thrombotic states, dehydration and head injury.
Infection, e.g. from a paranasal sinus, may be present. Venous thromboses can also
arise spontaneously.
Cortical venous thrombosis
Can leads to headache, focal signs and/or epilepsy, often with fever.
Dural venous sinus thromboses
 Cavernous sinus thrombosis causes ocular pain, fever, proptosis and chemosis.
External and internal ophthalmoplegia with papilloedema develops.
 Sagittal and lateral sinus thrombosis cause raised ICP with headache, fever,
papilloedema and often epilepsy.
Management
MRI, MRA and MRV show occluded sinuses and/or veins. Treatment is with
heparin followed by warfarin for 6 months. Anticonvulsants are given if necessary.