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Clinical Presentation and Diagnosis of Cerebrovascular Disease

Chapter 30 Clinical Presentation and Diagnosis of Cerebrovascular Disease

Mark J. Alberts

Stroke is a common and serious disorder. Each year stroke aects almost 800,000 people in the United States and about 16 million people throughout
the world.1 Associated high morbidity and mortality provide impetus for improving diagnosis, acute management, and prevention of strokes. A full
understanding of how patients with stroke and cerebrovascular disease come to medical attention, along with a logical approach for dening the
mechanism of stroke, are needed for safe and eective implementation of acute therapies and prevention strategies. This chapter will focus on clinical
manifestations of all types of cerebrovascular disease and how clinicians can approach diagnostic evaluation.

Overview of Clinical Stroke

Stroke and cerebrovascular disease are caused by some disturbance of the cerebral vessels in almost all cases. In simple terms, we can divide stroke into
two major types: ischemic and hemorrhagic. Ischemic stroke is the most common variety and is responsible for 80% to 85% of all strokes; hemorrhagic
stroke accounts for the remainder.2 On occasion, an ischemic stroke can undergo secondary hemorrhagic transformation; likewise, a cerebral
hemorrhage (particularly a subarachnoid hemorrhage [SAH]) can cause a secondary ischemic stroke via vasospasm.

Ischemic stroke occurs when a blood vessel in or around the brain becomes occluded or has a high-grade stenosis that reduces the perfusion of distal
cerebral tissue. A variety of mechanisms and processes can lead to such occlusions and will be discussed later in more detail. On rare occasions, venous
thrombosis can occlude a cerebral vein and lead to ischemic as well as hemorrhagic strokes (venous infarction).

Hemorrhagic stroke (intracerebral hemorrhage [ICH] and SAH) occurs when a blood vessel in or around the brain ruptures or leaks blood into the brain
parenchyma (ICH) or into the subarachnoid space (SAH). It is not uncommon for there to be some overlap, such as an ICH also causing some degree of
SAH and/or an intraventricular hemorrhage. Likewise, an SAH can produce some elements of an ICH if the aneurysmal rupture directs blood into the
brain parenchyma. As with ischemic stroke, a variety of processes and lesions can produce ICH and SAH, but most aect integrity of the vessel wall in
some way.

Clinical Manifestations of Stroke and Cerebrovascular Disease

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Stroke is similar to real estate in that much of its presentation and prognosis depend on size and location. The area of brain involved dictates presenting
symptoms. Blood vessels that supply dierent parts of the brain are aected by dierent types of cerebrovascular disease and have dierent mechanisms
(pathophysiology) for the stroke. This concept greatly inuences and denes the approach a vascular neurologist or neurosurgeon uses when assessing
patients with a stroke or cerebrovascular disease.3,4

For example, a patient with evidence of involvement of the left hemispheric cortex (e.g., aphasia, visual eld defect, weakness of contralateral face and
arm) is likely to have a process involving the left middle cerebral artery (MCA). If head computed tomography (CT) does not show evidence of a
hemorrhage, likely etiologies would include an embolic event from the heart (e.g., atrial brillation) or an artery-to-artery embolism (as might be seen
with a high-grade lesion at the carotid bifurcation in the neck). Another patient with a pure motor hemiparesis but no other decits is likely to have a
lesion aecting the motor pathways in the internal capsule, often due to occlusion of a small penetrating artery (lenticulostriate vessel) deep in the brain.
Most ischemic strokes will respect the vascular territory of one or more arteries.5 Indeed, lesions that do not respect typical arterial territories lead to
concern for a nonvascular process (e.g., tumor, infection). Common ischemic stroke syndromes can be found in Tables 30-1 and 30-2.

Table 30-1 Common Large-Vessel Ischemic Stroke Syndromes

Table 30-2 Common Lacunar Stroke Syndromes

Evaluation of hemorrhagic stroke follows a similar logical assessment, but is further complicated by spread of the initial bleed, the eects of increased
intracranial pressure, and other secondary eects that lead to neurological manifestations beyond the original injury. In this case, detailed cerebral
imaging is vital for understanding the mechanism of the stroke and reasons for secondary worsening. The discussions that follow oer more detailed
descriptions of common hemorrhagic stroke syndromes correlated with their likely anatomy and most likely pathophysiology.

Besides location of the stroke, the tempo of onset and progression of symptoms often provide valuable information about stroke etiology and mechanism.
Stroke symptoms that progress in a casual manner with gradual onset and worsening over many minutes or longer often suggest a thrombotic process or
hypoperfusion due to occlusion or stenosis of a larger proximal vessel. Such a leisurely progression can also be seen with stroke mimics such as
complicated migraines or partial seizures. The converse is a stroke syndrome with sudden onset of maximal symptoms that remain stable; this suggests
an embolic process such as a cardioembolic stroke due to atrial brillation.

Similar reasoning holds true for most cases of hemorrhagic stroke. Intracerebral hemorrhage often presents with abrupt onset of symptoms, but close
questioning may reveal that symptoms actually progressed over 15 to 30 minutes as the hematoma grew and expanded.6 Subarachnoid hemorrhage is
often characterized by sudden onset of the worst headache of ones life, with signicant nausea, vomiting, and sti neck in many cases. The phrase
worst headache of my life is so characteristic of SAH that a patient who presents to the physician or emergency department with that symptom
complex is assumed to have SAH until proven otherwise.7

Transient Ischemic Attack

A transient ischemic attack (TIA) is often a prodrome to an ischemic stroke. Symptoms of a TIA are identical to those of a stroke, but with resolution
within 24 hours (according to the old denition of a TIA). In reality, most TIA syndromes last just a few minutes, not many hours. In fact, modern brain
imaging using magnetic resonance imaging (MRI) with diusion-weighted sequences has now shown that 25% to 30% of patients with a TIA lasting
30min to 2 hours will have a new diusion-weighted imaging (DWI) lesion on MRI indicating a stroke based on a tissue denition.8 Transient ischemic
attack symptoms lasting 6 hours or longer have a 50% likelihood of having a new stroke on MRI with DWI techniques. Therefore the perceived distinction
between a TIA and a stroke should be viewed more as a continuum from minor transient neuronal dysfunction to permanent brain infarction.

Although it was once thought that the risk of stroke after a TIA was low, new imaging studies as well as epidemiological studies have proven this is not
the case. Based on purely clinical criteria (not MRI results), several recent studies have shown that after a TIA, 10% of patients will have a stroke within
3months, and half those strokes (5%) will occur within 48 hours of the initial TIA. About 25% of patients with a TIA will have a stroke, myocardial
infarction (MI), death, recurrent TIA, or be hospitalized within the next 3 months.9 Based on these poor outcomes, recently published guidelines
recommend hospital admission for patients with a recent TIA.8

Further studies have attempted to better dene those patients with a TIA who are at higher risk of having a stroke within the next 2 to 7days. Several
scoring systems have been developed (Table 30-3) that may be useful for assessing such risks. Of course, any such assessment tool must be tempered
by good clinical judgment and consideration of all clinical factors.

Table 30-3 Transient Ischemic Attack Scoring Systems

ABCD Age, blood pressure, clinical symptoms, duration

ABCD2 Age, blood pressure, clinical symptoms, duration, diabetes

ABCD2I Age, blood pressure, clinical symptoms, duration, infarction

Age: 60years or greater = 1 pointBlood pressure: systolic 140mmHg or greater = 1 point or diastolic 90mmHg or greater = 1 pointClinical symptoms:
unilateral weakness = 2 points; speech disturbance without weakness = 1 pointDuration: 60 minutes or more = 2 points; 10-59 minutes = 1
pointDiabetes: 1 point (on antidiabetic medications)Infarction: evidence for acute ischemic stroke on CT or MRI
CT, computed tomography; MRI, magnetic resonance imaging.

From Johnston SC, Rothwell PM, Nguyen-Huynh MN, et al: Validation and renement of scores to predict very early stroke risk after transient ischaemic
attack. Lancet 369:283292, 2007.50

Several types of TIAs deserve special mention because of their unique presentations. One is sudden blindness in one eye, which typically occurs as a
shade coming down over the eye. Some patients report a graying out of vision in the eye, like looking through a gray haze or cloud. This type of TIA is
often referred to as amaurosis fugax. This symptom complex typically resolves in a few minutes, although it can last for several hours. There is sometimes
pain in or around the eye, but patients usually do not have any other focal neurological complaints. Some cases of amaurosis are due to emboli to the
retinal circulation from an ulcerated plaque in or near the carotid bifurcation in the neck. Other cases can be due to local disease in the ophthalmic artery
or in the posterior ciliary artery that supplies the optic nerve.10

The other unique type of TIA is the limb-shaking TIA. This typically involves the arm or leg on one side of the body. Patients report uncontrollable shaking
of a limb that can be precipitated by movement. These spells can last seconds to minutes. They are not epileptic in origin; electroencephalogram (EEG) is
unremarkable. These TIAs are associated with severe stenosis of the contralateral internal or common carotid artery.11 Once the carotid artery is opened
(usually with an endarterectomy), the spells cease.

Lastly is the topic of crescendo TIAs. This refers to a pattern where TIAs are recurrent, last longer, or are more severe in nature. This is a very worrisome
type of TIA and is associated with a risk of stroke as high as 25% to 50% over the next few weeks.12

Some hemorrhagic strokes may also have a TIA equivalent, namely the sentinel headache before a SAH. The sentinel headache present as an acute
headache that is unusual in terms of its nature, severity, and onset. It typically lasts more than an hour but does not have other impressive focal
neurological ndings and resolves prior to the denitive SAH presentation. Sentinel headaches occur in 25% to 50% of patients with a subsequent
aneurysmal SAH and typically antedate the SAH by days to weeks (average 2weeks).13,14 It is thought that most of these headaches are due to either
minor leakage from a fragile aneurysm or enlargement of the aneurysm, resulting in pressure on a nearby structure that produces pain.

Ischemic Stroke Syndromes

There are numerous manifestations of ischemic stroke, and they can be classied based on brain location involved, artery aected, or symptoms
produced. Although advanced diagnostic techniques have altered some of the clinical rules of stroke symptoms and etiology, there are still some useful
concepts that can guide us in terms of stroke location and mechanism. Tables 30-1 and 30-2 list some classic ischemic stroke syndromes with their
major clinical manifestations, vascular territory, and underlying pathophysiology.5
Broadly speaking, ischemic strokes typically involve one or more vessels or vascular territories and produce a clinical picture of focal neurological decit.
Typically, clinicians look for unilateral weakness or sensory decits, unilateral visual eld abnormalities, speech disturbance (aphasia or dysarthria),
neglect syndromes, unilateral ataxia, ophthalmoplegias, or gaze abnormalities as clues of a stroke. Symptoms such as vague diuse weakness alone,
headaches alone, memory loss, abnormal behavior, or isolated dizziness are rarely caused by an ischemic stroke. The appearance of a lesion in a typical
vascular territory (based on brain imaging) is a key feature of almost all stroke syndromes.4

Presence of cortical decits (aphasia, visual eld cuts, neglect syndromes) often indicates involvement of a major cerebral vessel in the cerebral
hemispheres. Presence of ataxia, bilateral motor or sensory decits, Horners syndrome, ophthalmoplegias, and crossed sensory ndings (one side of the
face and the other side of the body) often indicates a stroke in the posterior (vertebral-basilar) territory. There are specic syndromes that indicate
small-vessel involvement deep in the brain. These so-called lacunar strokes are due to occlusion of small penetrating arteries that arise directly from
larger parent vessels. Favored locations include the deep basal ganglia structures, thalamus, and brainstem (pons). A listing of large-vessel and lacunar
syndromes appears in Tables 30-1 and 30-2.

Atherothrombosis accounts for the majority of ischemic strokes. These lesions can occur anywhere in the cerebral vasculature, but they tend to have a
preference for specic locations such as the bifurcation of the carotid artery in the neck, intracranial carotid siphons, proximal portion of the middle
cerebral artery, mid-portion of the basilar artery, and aortic arch. An atherosclerotic plaque forms over many years, then ruptures causing formation of a
superimposed thrombus.15 This atherothrombotic lesion can totally occlude the vessel, produce severe narrowing (leading to watershed ischemia), or be
a source of embolic material that embolizes to more distal parts of the cerebral vasculature (artery-to-artery emboli).

Cardiac embolism accounts for 15% to 20% of all ischemic strokes. A variety of conditions such as atrial brillation, endocarditis, prior myocardial
infarction, valvular disease, and cardiomyopathy often lead to formation of intracardiac thrombi that subsequently embolize to the brain (and other
organs).4,16 Most lacunar strokes are due to either lipohyalinosis or microatheromata occluding a small penetrating artery.

Special Cases
Ischemic Stroke in Young Adults
All clinicians see young adults (often dened as 45years of age) with ischemic strokes. Such cases often entail a special evaluation because of the
unique processes and conditions that can produce strokes in this age group. Many case series have examined the diseases leading to ischemic strokes in
the young, and in general they fall into a few major categories: (1) premature atherosclerosis, (2) unusual vascular pathologies, (3) cardiac etiology, (4)
coagulopathy, and (5) other diseases.17

Premature atherosclerosis typically occurs in patients with risk factors for atherosclerosis; in some cases these have not been diagnosed or not properly
treated. Examples include hypertension, hyperlipidemia, diabetes, smoking, and obesity. The types of uncommon vascular pathologies often seen in
young adults with a stroke include dissection of a vessel (often not related to any obvious trauma), bromuscular dysplasia, moyamoya disease, or a
vasculitis related to an inammatory condition or drug abuse.17 Numerous cardiac processes can lead to strokes in the young, such as congenital heart
disease, a patent foramen ovale (particularly with evidence of venous thrombi), valvular disease (infectious or inammatory), cardiomyopathy, myxoma,
papillary broelastoma, and many others. Myriad clotting disorders have been associated with strokes in young adults, the most common being lupus
anticoagulants, anticardiolipin antibodies, and protein C and protein S deciency.18 In general, these coagulopathies are more likely to cause venous
thrombosis than arterial thrombosis. Clotting disorders related to hematological malignancies can cause both ischemic and hemorrhagic strokes.19
Various systemic diseases are also associated with hypercoagulable states such as inammatory bowel disease, hemoglobinopathies, elevated
homocysteine, and cancer.

The other category covers a host of conditions, some rare and some common, that cause strokes in young adults. Migraine headaches and pregnancy
are the most common of these. Patients with complex or complicated migraines, prolonged auras, or taking contraceptives have a higher risk of stroke.20
Pregnancy, particularly in the third trimester and up to 3months postpartum is associated with increased stroke risk, particularly venous thrombosis and
cerebral hemorrhage.21,22 Drug abuse is another common cause of ischemic and hemorrhagic strokes in young adults.23 Other rare conditions include
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), MELAS (mitochondrial encephalomyopathy,
lactic acidosis, and stroke), isolated central nervous system (CNS) vasculitis, Sneddon syndrome (combination of a livedo reticular rash, antiphospholipid
antibodies, and ischemic stroke), Marfans syndrome, and a host of others (especially connective tissue disorders) have been known to cause strokes in
this population.

Strokes Related to Systemic Disease

Numerous systemic disorders are important and potent risk factors for stroke: hypertension, diabetes, hyperlipidemia, smoking, heart disease (atrial
brillation, myocardial infarction, valvular disease, etc.), drug abuse, and others. These have been covered in other chapters of this book. Our focus here
is on specic systemic disorders that lead to specic or unusual types of strokes.

There are a number of unique systemic disorders that cause strokes in patients of any age. Autoimmune diseases, such as lupus, can produce strokes
through a variety of mechanisms that include advanced or premature atherosclerosis, vasculitis, hypercoagulable states, and cardioembolic events.24
Sickle cell disease (SCD) also leads to ischemic strokes and hemorrhagic strokes due to myriad processes including a large-vessel arteriopathy, small-
vessel occlusion, rupture of moyamoya vessels (producing ICH and/or SAH), and accelerated atherosclerosis due to hypertension and renal failure.25,26

Drug abuse, particularly cocaine, can produce ischemic strokes via a number of processes including vasospasm, cardiac emboli (due to cardiomyopathy),
hypertension, and endocarditis.27 Drug abuse can produce an ICH or SAH due to extreme hypertension and necrotizing vasculitis. It is a fallacy to
assume that drug abuse only occurs in young patients or those from certain demographic groups. All patients admitted with a stroke should be tested for
drug abuse with urine toxicology screens, not excluding those older than 50years and white-collar professionals.

Human immunodeciency virus/acquired immunodeciency syndrome (HIV/AIDS) is now recognized as increasing the risk of stroke. This is partially
because patients with HIV/AIDS are living longer, and some are having strokes as a result of accelerated development of typical stroke risk factors. It is
also clear that modern drug therapy for AIDS can increase the risk of stroke (particularly ischemic stroke).28,29

Systemic cancer is a commonly overlooked cause of strokes. Sometimes the stroke diagnosis precedes diagnosis of the underlying cancer. Mechanisms
for strokes related to cancer include a hypercoagulable state and nonbacterial thrombotic endocarditis. Oftentimes these strokes are multiple, variable in
size, and in dierent vascular territories.30,31 Such patients may also have deep venous thrombosis (DVT). Liver failure appears to increase risk of
ischemic and hemorrhagic stroke.

Intracerebral Hemorrhage
In broad terms, ICH can be divided into traumatic and nontraumatic etiologies. This chapter will focus on nontraumatic ICH, since ICH related to trauma
is not routinely considered a stroke. Intracerebral hemorrhage is typically caused by rupture of a blood vessel within the brain parenchyma. Patients
typically develop a focal neurological decit suddenly, but symptoms often evolve over 10 to 30 minutes as the hematoma gradually expands. Headache
is commonly present, and the vast majority of patients have markedly elevated blood pressure (often in excess of 200mmHg systolic) even without a
prior history of hypertension. Nausea and vomiting can also occur, particularly with ICH that involves the brainstem and/or cerebellum.

Chronic or acute hypertension is the most common etiology for nontraumatic ICH, and this type of bleed typically occurs in specic brain locations (Table
30-4). As with ischemic stroke, location of the ICH is highly correlated with the type of symptoms produced. Recent studies using serial brain scans have
shown that 30% to 40% of ICHs will expand over the rst 24 hours after admission; such expansion is almost always associated with clinical
worsening.6,32 High blood pressure may be a risk factor for ICH expansion, although this association has not been mechanistically proven.

Table 30-4 Location and Symptoms for Common Types of Intracerebral Hemorrhage

ICH Location Likely Etiology Common Symptoms

Contralateral hemiparesis, speech changes, gaze deviation, altered mentation if

Basal ganglia Hypertension
large

Lobar Hypertension, CAA Cortical syndromes, weakness, visual eld lesions, altered mentation if large

Thalamus Hypertension Altered mentation, sensory changes, gaze abnormalities

Pons Hypertension Coma, gaze and pupil abnormalities, quadriparesis

Cerebellum Hypertension, AVM Ipsilateral ataxia, dizziness, vertigo, nausea/vomiting

Hemispheric AVM, extreme hypertension, mycotic

Headaches, seizures, cortical syndromes
cortex aneurysm

AVM, arteriovenous malformation; CAA, cerebral amyloid angiopathy; ICH, intracerebral hemorrhage.

Another increasingly common etiology for ICH is cerebral amyloid angiopathy (CAA), which typically aects patients older than 70years of age. Cerebral
amyloid angiopathy is caused by deposition of one or more amyloid proteins within the wall of cerebral small arterioles. A typical CAA bleed occurs in a
lobar region (junction of gray matter and white matter), most commonly in the parietal, temporal, and occipital lobes. Intracerebral hemorrhages due to
CAA can be multiple and recurrent.3335 There is a clear association between CAA, ICH, and Alzhemiers disease. Sometimes an ischemic stroke can
undergo hemorrhagic transformation and become an ICH. This occurs in up to 15% of cases of ischemic stroke and is associated with large strokes,
cardioembolic strokes, and the use of anticoagulants and thrombolytic agents.

A variety of vascular malformations can cause an ICH, particularly arteriovenous malformations (AVMs) and cavernous malformations (less commonly,
capillary telangiectasias and developmental venous anomalies). Arteriovenous malformations are the most common and serious type of vascular
malformation that cause an ICH, and recurrent ICHs, as well as producing seizures and local neurological decits.36 The characteristics and hemorrhagic
risk of each of these lesions is shown in Table 30-5.

Table 30-5 Common Types of Central Nervous System Vascular Lesions That Lead to Cerebral Hemorrhage

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