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279Discussion
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
e x t r a c r a n i a l I C A s t e n o s i s >80% or an occlusion was found
in 83% versus 40% (Baumgartner et al 2001). Compared
with DSA and/or MRA, the combined use of extracranial
and intracranial hemodynamically ultrasound parameters in patients with presumed ICA dissection yielded
a diagnostic sensitivity of 96%, specifi city of 94%, and
positive and negative predictive values of 92%, and 97%,
respectively (Benninger et al 2006).
Therefore, duplex ultrasound of the brain-supplying
arteries plays an important part in the initial investigation as well as follow-up of patients with ICA dissection
revealing abnormalities in more than 90% of cases, mostly
of hemodynamic character. A combination of unilateral
high-grade stenosis, absence of atherosclerosis (seen in
80–90% of dissections), and the young age of the aff ect-
ed patient make the diagnosis of an ICA dissection very
likely. However, if atherosclerosis is present, the use of
ultrasound carries the risk of overlooking a dissection
and presuming an atherosclerotic stenosis instead.
In young patients with clinically suspected dissection
who did not have ischemic stroke, the incidence of stenoses or occlusions is lower. This implies that further radiologic diagnostics must be performed even if ultrasound
fi ndings are normal.
Our case of unilateral distal extracranial ICA stenosis
illustrates the diagnostic diffi culties in evaluating the
craniocervical region. Ultrasonography is a well-established method for the evaluation of the extracranial ICA
at the level of the carotid bifurcation as these vessel segments can be visualized directly. The combination with
hemodynamic parameters permits excellent determination of grades of stenosis. However, analysis of the distal
extracranial ICA, which lies deep to the mandibular angle,
may cause considerable problems. In addition, the ICA
often follows an elongated vessel course. Evaluation has
therefore often to rely on the assessment of indirect hemodynamic parameters. In our case, these were the high
pulsatility of the CCA and proximal ICA with well-preserved diastolic fl ow, which indicated a distal fl ow ob-
struction. Direct imaging of the more distal ICA can be
attempted using the linear transducer in an axial plane
pointing toward the base of the skull, or a 2-MHz TCCS
probe using the same approach, neither of which was
done in our case. Another technique that can be used to
visualize the distal ICA is the transoral approach, with a
5–9-MHz convex array transducer (Kishikawa et al 2002,
Yasaka et al 1998).
Conventional angiography has long been the gold standard in the diagnosis of arterial dissections, since it can show
the arterial lumen and allows extensive characterization of
carotid and vertebral arteries. The most common fi nding in
ICA dissection is the smooth or irregularly tapered midcervical stenosis (string sign) or occlusion. A dissection may be
assumed if a rat-tail or fl ame-shaped occlusion is present.
Dissecting aneurysm is a further typical sign. Pathognomonic features such as an intima fl ap or a double lumen are
rarely detected. Pelkonen and coworkers found that most
of their patients had irregular stenoses (47%), followed by
occlusions (29%), dissecting aneurysms (17%), or irregular
(Pelkonen et al 2003). The main problem of catheter angiography is its invasiveness. In high-risk populations it carries
a 4% risk of causing a permanent neurologic defi cit (for fur-
ther discussion, see Case 24). Today, DSA has to be regarded
as a second-line method, not only because of the above limitations but also because alternative and less invasive methods yielding similar or even greater diagnostic accuracy are
available.
MRI in combination with vascular ultrasound is now
mostly replacing conventional angiography in the diagnosis and follow-up of dissections of the carotid and
vertebral arteries. In particular, the option of directly
visualizing the intramural hematoma on cross-sectional
images renders MRI a very useful technique in presumptive vessel dissection. The size and shape of the intramural hematoma depends on the surrounding structures,
e.g., bone, fat, or venous plexus. The signal intensity of
the wall hematoma depends on its maturity and MRI
sequences. This crescent hematoma sign succeeds in
a large number of patients using blood-sensitive MRI,
especially T1-weighted (with or without fat suppression) and T2-weighted sequences. Rarely, the hematoma may appear oval or circumferential. Over time, it
shows a typical evolution of signal intensity related to
the paramagnetic impact of the components of hemoglobin breakdown (Kitanaka et al 1994b). Importantly,
the wall hematoma may be missed in the hyperacute
stage in the fi rst days because the isointense hematoma
may be obscured when surrounded by isointense tissues
and the imaging might need to be repeated. In the subacute phase, the hematoma appears characteristically
as a crescent-shaped hyperintense area around an eccentric fl ow void corresponding to the vessel lumen in
dissecting stenosis. In occlusive dissection, the whole
cross-sectional area may present a more full moon appearance. In our patient, the wall hematoma was already
present on the imaging on initial presentation.
This distinct signal increase subsequently fades
within 2 months after acute dissection and a dissecting lesion may then be diffi cult to prove (Paciaroni et al
2005). A fresh intraluminal thrombus may mimic wall
hematoma but often reveals varying signal intensities,
caused by its specifi c components (Schwaighofer et al
1990). Therefore, a crescent-shaped formation with homogeneously increased signal intensity is highly suggestive for dissection but not specifi c, while an additional
widening of the external vessel lumen is confi rmatory
of dissection and should be searched for if a dissection is
suspected. If additionally performed, the fl ow-sensitive
TOF-MRA is able to show fl ow reduction, due for exam-
ple to a sub-basal extracranial stenosis, in the form of a
reduced intracranial ICA signal intensity. In-plane fl ow
due to the tortuous course of the ICA, as well as turbulent fl ow, may impair the image quality and may lead to
an overestimation of the stenosis or to a false diagnosis
of occlusion. In the subacute stage a high-intensity signal of the intraluminal clot may mimic intact blood fl ow.
The diagnostic yield to detect dissecting stenoses and
aneurysms is improved if contrast-enhanced (ce)-MRA
is used (Touzé et al 2001). Because of its better spatial
resolution and better visualization of lumen narrowing, vessel occlusion, and dissecting aneurysm, ce-MRA
should be performed whenever MRI is used for diagnosis of presumed dissection.

280 Case 11 Secondary Occlusion in Left-sided Extracranial Internal Carotid Artery Dissection
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
CT angiography (CTA) sensitively depicts the characteristic imaging appearance of a tapering vessel following dissection. However, it cannot directly visualize
the intramural hematoma, due to limited soft tissue
contrast. It needs careful interpretation within the
base of the skull because of the interference with the
bone structures surrounding the ICA. A critical evaluation of the source images and careful postprocessing of
the images aiming to remove or reduce the bone signal
might help to evaluate the vessel continuity and integrity. Results similar to those of MR techniques have
been reported for the detection and follow-up of ICA
dissections (Leclerc et al 1996). Because of the short
investigation time, multislice CTA is currently the fi rst-
line modality in presumed cervicocerebral vascular
pathology, especially in stroke patients. This technique
provides comprehensive and high-resolution vessel assessment, superior to current MRA modes. Like MRI,
the CTA technique allows visualization of the frequently
seen enlarged external vessel diameter of the dissected
vessels. An initial small study comparing CTA and MRI/
TOF-MRA demonstrated CTA superiority as it depicted
all seven dissections, of which two were missed by the
MRI technique. In the same series, CTA identifi ed a dis-
secting aneurysm missed by MRI (Elijovich et al 2006).
Diff erent bone subtraction techniques have been used,
e.g., threshold-based bone removal in single-energy
CTA as well as dual-energy postprocessing, all aiming to
achieve an image quality similar to conventional DSA.
Currently the most promising approach is dual-source
CTA, which makes it possible to separate bone, calcifi ed
plaques, hemorrhage, and contrast medium by their different absorption spectra (Postma et al 2015). Yet, even
dual-source CTA suff ers from pitfalls such as overesti-
mation of vessel stenosis (Watanabe et al 2009), thus
failing to attain conventional DSA image quality.

Case 12
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
Extracranial Bilateral Internal Carotid Artery and Right Vertebral
Artery Occlusion, and Left Vertebral Artery Stenosis
281
Clinical Presentation
A 79-year-old man, who had never sought medical attention before, was admitted with unusual dizziness
that had occurred intermittently over the past 3 days.
The patient had no vascular risk factors apart from
a history of heavy smoking and alcohol abuse. The
neurologic examination on admission revealed mild gait
ataxia (National Institutes of Health Stroke Scale [NIHSS]
score: 1).
Initial Neuroradiologic Findings
Unenhanced cranial CT was unremarkable. Diff u-
sion-weighted (DW) MRI showed a small subacute right
posterior inferior cerebellar arter y (PICA) infarction at
the cortical–subcortical border (Fig. B12.1). Intracranial time-of-fl ight MR angiography (TOF-MRA) showed
bilateral internal carotid artery (ICA) and right-sided
vertebral arter y (VA) occlusion. No further intracranial pathology was observed. Contrast-enhanced (ce)
MRA of the brain-supplying arteries revealed bilateral
proximal ICA occlusions, a moderate distal stenosis of
the left common carotid artery (CCA), and a high-grade
stenosis of the left VA origin. The right VA signal was
missing over the entire length of the artery (Fig. B12.2
and Fig. B12.3).
Suspected Diagnosis
Right-sided cerebellar ischemic infarction likely caused
by a periocclusional artery-to-artery embolism in right
distal VA occlusion. Clinically asymptomatic bilateral ICA
occlusion and high-grade left proximal VA stenosis, likely
caused by severe generalized extracranial atherosclerosis.
Questions to Answer by
Ultrasound Techniques
• Was the burden of extracranial steno-occlusive lesions
confi rmed by ultrasound?
• Could ultrasound distinguish between a proximal and
distal left VA occlusion?
• If a distal VA occlusion was present, could ultrasound
be used to diff erentiate occlusions located proximally
versus distally to the PICA origin?
• What was the pattern of collateral blood fl ow?
Initial Neurosonologic Findings
Extracranial Duplex Sonography
B-mode sonography revealed generalized atherosclerosis
in both carotid arteries. Both CCAs had a high-resistance
fl ow signal with increased pulsatility. The left distal CCA
showed a 40% local lumen reduction in the cross-sectional
plane caused by a largely hypoechoic plaque. Despite this
lumen reduction, no fl ow disturbances or increased fl ow
velocities were observed. Flow was absent in both ICAs.
The left ICA had a roughly 5 mm residual small-vessel
lumen (“blind sack”). No residual lumen was observed
in the right ICA. Both external carotid arteries (ECAs)
had mildly internalized fl ow signals. The dominant left
V2-VA (diameter 5.4 mm) had a mild poststenotic fl ow
pattern. Nevertheless, fl ow velocities were high and
the blood volume fl ow measured 580 mL/min. At the
V1-VA segment, the fl ow was turbulent and had a fl ow
velocity similar to the V2-VA. The origin was not detected. Only minimal systolic spikes were registered in the
right V1- and V2-VA segments, which otherwise had a
normal diameter (3.9 mm). No cervical spinal collaterals were observed throughout the entire V2-VA length
(Figs. B12.4–B12.9).
Transcranial Duplex Sonography
All intracranial vessels had mild poststenotic fl ow pat-
terns. Blood fl ow in both anterior cerebral arteries (ACAs)
was antegrade. Increased and turbulent fl ow velocities
were seen in both P1-PCAs and posterior communicating
arteries (PCoAs). This, in combination with the normal
fl ow velocities in both P2-PCAs, was considered indicative
of collateral fl ow function from the posterior toward
the anterior circulation. Transforaminal insonation revealed a marked fl ow signal in the left V4-VA and basi-
lar artery. On the right side, no proximal or distal V4-VA
signals were observed. Transorbital insonation revealed
a retrograde fl ow in both ophthalmic arteries (OAs)
(Figs. B12.10–B12.18).
Conclusion
Bilateral proximal extracranial ICA occlusion and distal right VA occlusion proximal to the PICA origin. Left
proximal VA stenosis sonographically assumed to be of
beginning hemodynamic relevance or a collateral fl ow
due to the mild poststenotic downstream fl ow pattern.

282 Case 12 Extracranial Bilateral Internal Carotid Artery and Right Vertebral Artery Occlusion, and Left Vertebral Artery Stenosis
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
Considering the ce-MRA fi ndings, the fi nal diagnosis
was determined to be a high-grade VA stenosis of incipient hemodynamic relevance. Intracranial collateral
blood fl ow to both the middle cerebral artery (MCA) and
ACA territories occurred primarily via both PCoAs, and
to a lesser extent via both OAs.
Fig. B12.19 shows a schematic of the patient’s extra-
and intracranial brain-supplying arteries.
Clinical Course
The patient was send to rehabilitation with aspirin and
statin medication. There, his unsteadiness remitted but
the dizziness remained. The etiology of the three and a
half-vessel disease was severe atherosclerosis caused by
severe, long-standing, combined heavy smoking and alcohol misuse. Stenting of the left VA stenosis was discussed
but refused by the patient. Regular 6-month follow-ups
revealed no further clinical events, and the neurosonologic fi ndings remained unchanged over an observational
period of 2 years.
AB
Fig. B12.1 Diff usion-weighted MRI, axial plane, revealing a small
fresh infarct in the right PICA territory (arrow).
AB C
Fig. B12.2 3D MRA, coronal maximal intensity projection (MIP), TOFMRA (A) and contrast-enhanced (ce) MRA (B). Missing bilateral ICA
signals. Prominent PCoA on both sides (arrowheads). Small right distal V4-VA fi lling, indicating a possible retrograde fl ow toward the PICA
or to relevant V4-VA perforator arteries (arrow). Note that ce-MRA is
superior to TOF-MRA in visualizing the right distal V4-VA lumen.
Fig. B12.3 Extracranial 3D
ce-MRA, coronal MIP, diff er-
ent views (A–C). Bilateral ICA
occlusion with large residual
lumen on the left ICA (A, arrowhead). Residual lumen of
the distal right V4-VA (A, small
arrow). Moderate CCA stenosis, best identifi ed in B (large
arrow). (C) Proximal left VA
stenosis (arrow) and right ICA
occlusion (arrowhead).

283Clinical Course
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
CCA-L
Fig. B12.4 Extracranial duplex, longitudinal plane (left), and crosssectional plane (right). Left CCA with 40% lumen reduction (arrows).
ICA-R
ICA-L
Fig. B12.5 Extracranial duplex, longitudinal plane. Left ICA with
a large “blind sack” several centimeters long and a weak, almost
r e t r o g r a d e fl ow signal.
V2-VA-L
Fig. B12.6 Extracranial duplex, longitudinal plane. Right ICA fi lled
with homogeneous hypoechoic material (arrow).
V1-VA-L
Fig. B12.8 Extracranial duplex, longitudinal plane. V1-VA fl ow sig-
nal similar to the V2-VA segment. The direct VA origin could not be
visualized (fl ow velocity 95/33 cm/s).
Fig. B12.7 Extracranial duplex, longitudinal plane. Mild poststenotic fl ow pattern in the dominant left V2-VA with marked increased
blood volume fl ow (580 mL/min, diameter 5.4 mm).
V2-VA-R
Fig. B12.9 Extracranial duplex, longitudinal plane. Missing fl ow sig-
nal in the right V2-VA (Diameter 3.9 mm).

284 Case 12 Extracranial Bilateral Internal Carotid Artery and Right Vertebral Artery Occlusion, and Left Vertebral Artery Stenosis
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
M1-MCA-L
Fig. B12.10 TCCS (trans temp oral ap proa ch), lef t-sid ed i nson ation ,
midbrain plane. Mild poststenotic fl ow pattern in the left M1-MCA
(fl ow velocity 74/30 cm/s).
P1-PCA- / PCoA-L
M1-MCA-R
Fig. B12.11 TCCS (transtemporal approach), right-sided insonation, midbrain plane. Mild poststenotic fl ow pattern in the right
M1-MCA (fl ow velocity 57/24 cm/s).
P2-PCA-L
Fig. B12.12 TCCS (trans temp oral ap proa ch), lef t-sid ed i nson ation ,
midbrain plane. Raised fl ow velocity in the junction of left PCoA and
P1-PCA indicating collateral fl ow (fl ow velocity 140/40 cm/s).
PCoA-R
Fig. B12.14 TCCS (transtemporal approach), right-sided insonation, midbrain plane. Turbulent fl ow in the right PCoA.
Fig. B12.13 TCCS (trans temp oral ap proa ch), lef t-sid ed i nson ation ,
midbrain plane. Normal fl ow velocity and mild poststenotic fl ow
pattern in the left distal P2-PCA (fl ow velocity 55/24 cm/s).
P2-PCA-R
Fig. B12.15 TCCS (transtemporal approach), right-sided insonation, midbrain plane. Normal fl ow velocity and mild poststenotic
fl ow pattern in the right distal P2-PCA (fl ow velocity 61/27 cm/s).

285Discussion
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
BA
Fig. B12.16 TCCS (t rans foramin al app roac h). Str ong fl ow signal
with increased fl ow velocity in the basilar artery (BA) (fl ow velocity
137/53 cm/s).
OA-R
OA-L
Fig. B12.17 TCCS ( tran sorbital a pproach ), lef t-si ded in sona tion.
Reversed left OA fl ow (fl ow velocity 30/11 cm/s). Note the elon-
gated course of the left OA revealing a bidirectional color signal.
When in doubt, the fl ow pattern has to be considered to decide if
an antegrade or retrograde fl ow is present.
Fig. B12.18 TCCS ( tran sorbital appr oach ), righ t-si ded insonat ion.
Reversed right OA fl ow (fl ow velocity 37/11 cm/s).
Final Diagnosis
Periocclusional embolic right-sided PICA infarct in right
distal VA occlusion and asymptomatic bilateral ICA occlusion. High-grade left proximal VA stenosis and moderate
left CCA stenosis based on a severe generalized extracranial atherosclerosis Acceptable collateral fl ow via both
PCoAs and OAs.
Discussion
Clinical Aspects
Here we discuss a 79-year-old patient with a rare severe
atherosclerotic vascular disease—namely, occlusion of
three out of four extracranial brain-supplying arteries
RL
Fig. B12.19 Schematic of the patient’s extra- and intracranial
brain-supplying arteries. Bilateral ICA and right distal VA occlusion.
Proximal left VA stenosis as well as left CCA stenosis (circles). Collateral blood fl ow toward the anterior circulation mainly via both
PCoAs assisted by both OAs.
and stenosis of the remaining VA. Bilateral extracranial ICA occlusion of atherosclerotic origin alone is rare,
and there are no precise epidemiologic data regarding
incidence or prevalence (for further reading on unilateral ICA occlusion, see Case 28). A duplex sonographic
study reported a bilateral ICA occlusion in 15 of 3,200
unselected patients (0.47%) (Lazarides et al 1991). These
data are in line with another study, which found that 8
of 2,228 patients had transient ischemic attacks (TIA)/
stroke and bilateral ICA occlusion (0.37%) (Mead et al
2006). Except rare cases of bilateral dissecting ICA occlusions, most bilateral occlusions are, as with our patient, of atherosclerotic origin. The combined results of
two studies (total of 95 patients with bilateral occlusion) revealed that 93–100% of patients with bilateral
occlusion reported heavy smoking and usually also had
at least one additional risk factor (e.g., hypertension,

286 Case 12 Extracranial Bilateral Internal Carotid Artery and Right Vertebral Artery Occlusion, and Left Vertebral Artery Stenosis
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
ischemic heart disease, hyperlipidemia, or diabetes)
(AbuRahma and Copeland 1998). Moreover, bilateral occlusion seems to predominantly aff ect males, who make
up 81–91% of the reported cases (Persoon et al 2009).
Vertebrobasilar TIAs, which manifested clinically as
syncope, vertigo, and drop attacks, were also observed
in 14% of cases (Wade et al 1987). As these symptoms
sometimes occurred in association with hyperextension
of the neck or with orthostatic maneuvers (rising from a
sitting or lying position, exercise, postprandial hypotension, or transition from cold to warm environment), a
steal phenomenon in the posterior circulation as a result
of the collateral function was suspected. The “shaking
limb sign”—a rare but prototypic sign of hemodynamic
TIA, caused by ICA occlusion and other orthostatically
induced TIA—has been seen in up to 18% of patients with
bilateral ICA occlusion (Persoon et al 2009). There is no
clear prognostic data. One large study in medically treated patients reported an annual combined TIA and stroke
risk of 15%, a stroke risk of 13%, and a mortality rate of 8%
over a mean observational period of 42 months (Wade
et al 1987). Higher overall mortality was reported in a
smaller case series in which six of eight (75%) medically
treated patients died during a mean follow-up of 6 years
(AbuRahma and Copeland 1998). A better outcome with
a calculated annual stroke rate of 1.2% was reported in
a diff erent study covering a mean observational period
of 5.9 years. The relatively low stroke rate might be related to the high percentage of patients included on the
basis of retinal ischemia (Persoon et al 2009). Unilateral
ICA occlusion combined with isolated retinal ischemia
is known to be associated with lower stroke recurrence
rates than occlusion combined with cerebral ischemia
symptoms (Grubb et al 1998, Klijn et al 2000). Other
possible explanations include both improved medical
secondary prevention therapy and more eff ective man-
agement of cardiovascular risk factors (compared, e.g.,
to the 1980s).
Extracranial–intracranial (EC–IC) bypass surgery has
been suggested because of the high stroke recurrence
rates estimated for patients with bilateral ICA occlusion
(el-Fiki et al 1985, Friedman et al 1987). Interestingly,
however, the prognosis in bilateral ICA occlusion seems in
fact to be better than unilateral ICA occlusion (Klijn et al
2001). Compensation of a bilateral ICA occlusion requires
the presence or development of stable collaterals either
before occurrence of the fi rst symptoms or after fi rst is-
chemia. Interestingly, a subgroup analysis of the ACAS
study demonstrated that patients with an asymptomatic
ICA stenosis ≥60% and a contralateral ICA occlusion had a
lower stroke recurrence risk compared to those without
contralateral occlusion (Baker et al 2000).
Within this context, the question arises as to how
these patients should be managed in terms of blood
pressure. Carotid endarterectomy trials have shown
that conservatively treated patients with bilateral occlusive processes (bilateral high-grade stenosis or
high-grade stenosis and contralateral occlusion) have a
higher stroke risks if blood pressure is reduced below
140 mm Hg (Rothwell et al 2003a).
In our patient, the diagnosis was made after cerebellar
stroke occurrence, which manifested clinically as gait ataxia.
The only symptom suggestive of persistent brainstem malperfusion was chronic dizziness. Transient visual complaints
were not reported. Because of his age, bypass surgery was
not recommended. He did not have increased blood pressure and therefore no antihypertensive medications could
be discontinued to improve his cerebral perfusion.
Our patient additionally had an asymptomatic left VA
stenosis at its origin (V0-VA segment). A contralateral
steno-occlusive lesion or a PICA-ending VA can lead to dizziness, vertigo, blurred vision, and ataxia—i.e., symptoms
resulting from hypoperfusion in the posterior circulation.
Here, the clinical prognosis also depends on the effi ciency
of the collaterals. For instance, anastomoses to deep cervical arteries usually starting at the V2-VA segment may,
in part, compensate for reduced fl ow. These collaterals
can be found in up to 31% of patients with proximal VA
occlusions and in 9% of patients with proximal VA stenosis (Wityk et al 1998). In our case, the above anastomoses
were not detected. (For further reading on V0-VA stenosis
and collateral pathways, see Chapter 5, “VA Occlusion”
under “Extracranial Pathology,” and Case 45.)
Angiologic and Anatomic Aspects
ICA occlusions are easily diagnosed using duplex ultrasound. The characteristic fi ndings are both absent color
and absent Doppler fl ow signals along the extracranial
ICA course. If the occlusion is located further distally, a
proximal stump signal with alternating fl ow and a miss-
ing diastolic fl ow component might be found. A com-
parative study between duplex ultrasound and digital
subtraction angiography (DSA) in 91 patients with ICA occlusion, reported that duplex ultrasound had a sensitivity
of 91%, specifi city of 99%, and positive and negative pre-
dictive values of 96%, and 98%, respectively (AbuRahma et
al 1997). The relatively high rate of false-positive results
is best explained by the pitfalls in detecting a nearly occluded ICA. The use of echo contrast agents may improve
(1) the detection of minimal fl ow within a severe steno-
sis, (2) the evaluation of fl ow in the presence of severely
calcifi ed plaque, and therefore (3) the diff erentiation be-
tween true and near occlusion (Fürst et al 1999, Ohm et
al 2005) (for further discussion on ICA near occlusion, see
also Case 15). Of note, ICA occlusions may reopen over
time. In dissecting ICA disorders, reopening has been observed in up to 90% of cases (Steinke et al 1994). In cardiac
or aortic-to artery embolism, vessel reopening should be
expected in all cases. If no recanalization occurs, reevaluation of its etiology may be necessary. Even in atherosclerosis-related proximal ICA occlusions, a reopening may
rarely occur and has been observed in 16 of 696 patients
(2.3%). As duplex ultrasound and DSA have shown, this
occurred after a mean time interval of 38 months from
occlusion diagnosis (Camporese et al 2011).
The OA is usually a second-line collateral pathway in
ICA steno-occlusive lesions. It may, however, become a
relevant collateral vessel with retrograde fl ow and a fl ow
pattern similar to that of an intracranial artery if hypoplasia of the communicating arteries, or as in our case,
severe contralateral hemodynamic restrictions are present. In our case, the only remaining brain-supplying artery, the left VA, revealed a blood volume fl ow (BVF) of

287Discussion
Valdueza et al., Neurosonology and Neuroimaging of Stroke: A Comprehensive Reference, Second Edition (ISBN 978-3-13-141872-2), copyright © 2017 Thieme Medical Publishers
All rights reserved. Usage subject to terms and conditions of license.
580 mL/min. This is remarkably high, but remains lower
than the global BVF of healthy subjects—reported to be
733 ± 54 mL/min (Schreiber et al 2005b). The physiologic centrifugal BVF of the OA is 10 - 11 mL/min (Ambarki
et al 2013). Collateral activation results in centripetal,
increased OA fl ow. Its fl ow should at least reach values
know from bypass surgery when the superfi cial tempo-
ral artery is used to supply the brain. Here, BVF of 84 ±
32 mL/min (range 14–177 mL/min) have been reported
(Neff et al 2004). To date, no data regarding BVF in ICA
occlusion has been reported, but it seems plausible to
assume that both OAs may compensate the missing fl ow
volume in our presented case.
Compared with the proximal ICA, VA origin assessment
is usually more diffi cult (for further reading on proximal
VA stenosis, see Case 45). The detectable V1- and V2-VA
segments revealed a mild poststenotic fl ow pattern, indi-
rectly suggestive of a hemodynamically relevant proximal
stenosis. When in doubt, extracranial continuous-wave
Doppler sonography may depict the VA off shoot signal
as the small Doppler probe facilitates signal detection behind the clavicle. However, our patient’s concomitant bilateral ICA and contralateral VA occlusion made it unclear
whether part of the poststenotic appearance was related
to hyperperfusion in the single remaining brain-supplying
artery. Finally, MRA clearly disclosed a high-grade stenosis,
leading to readjusted evaluation of ultrasound fi ndings.
The exact defi nition of VA occlusion is equally prob-
lematic (not only using the ultrasound technique) and
requires specifi c anatomic knowledge to avoid diagnostic
errors. As with ICA occlusion, an occluded vessel might
be depicted by color-mode sonography with an absent
color signal (see Fig. A5.75). Within the V2-VA segment,
the blood fl ow in the concomitant vertebral vein is usu-
ally preserved and might be of diagnostic aid (see Fig.
A5.76). In our case, only systolic spikes were seen in the
examined V1- and V2-VA segments. VA hypoplasia was
ruled out as the diameter was 3.9 mm. Distinguishing
short systolic spikes from vessel wall movements may
be diffi cult and may also be interpreted as a proximal VA
occlusion. However, proximal occlusion usually leads to
distal collateral activation. The VA has numerous extracranial anastomoses at all levels of its extracranial course
which can potentially serve as collaterals and prevent
occlusion over its entire length. These are anastomoses
from the thyrocervical trunk and muscular rami of ECA
branches, especially from the occipital artery. Spinal
rami of the contralateral VA can also participate in collateral blood supply. During a proximal VA occlusion, these
collaterals, depending on their quantity and quality, may
cause a secondary VA fi lling with “postocclusional” VA
fl ow of varying magnitude detectable in the distal VA
segments (see Fig. A5.77, Fig. A5.78, A5.79). Complete
occlusion of the entire extracranial VA is rare. In the presence of two equivalent VAs, the above-mentioned collateral pathways are rarely of importance as the contralateral VA will provide the blood supply to the posterior
circulation and also retrograde via a vertebro-vertebral
overfl ow toward the PICA of the aff ected VA. However, if
contralateral VA hypoplasia or PICA termination is present, the extracranial anastomoses become relevant. In
these instances, the distal VA fl ow is usually antegrade
with a typical poststenotic fl ow pattern. Although the
term “poststenotic” seems slightly inaccurate in a vessel
segment distal to an occlusion, we would suggest its use
nevertheless, as it clearly illustrates the common problem of hemodynamic impairment in stenoses and occlusions. A distal extracranial VA occlusion, in contrast,
may cause a stump signal (as seen in our patient), a high
pulsatile fl ow signal with absent end-diastolic fl ow, or
a biphasic or triphasic waveform comparable to an ECA
branch, as its communication to these vessels usually remains open (see Fig. A5.80). For example, one study that
included 10 distal VA occlusions (proximal to the origin
of the PICA), reported that all subjects had zero diastolic
fl ow (Saito et al 2004). From both radiologic and clinical viewpoints, it has to be emphasized that the term
“VA occlusion” alone is not suffi cient. Ideally, it should
be complemented by the exact location of the occlusion
and information regarding possible secondary VA fi lling
by collaterals distal to the occlusion. For further reading,
see also Case 19 and Case 45.

288
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All rights reserved. Usage subject to terms and conditions of license.
Case 13
Right Internal Carotid Artery Stenosis in Fibromuscular
Dysplasia and Granulomatosis with Polyangiitis
(formerly Wegener’s Granulomatosis)
Clinical Presentation
A 51-year-old woman presented with stepwise deterioration of a left hemiparesis that had started 3 days before
admission. The medical history revealed chronic rhinitis,
sinusitis, and bronchitis but no vascular risk factors. On neurologic examination, the patient had a severe left-sided brachiofacial hemiparesis (National Institutes of Health Stroke
Scale [NIHSS] score: 8). In addition, she had nasal congestion.
Initial Neuroradiologic Findings
Cerebral CT (CCT) scan on the day of admission revealed
ischemic infarction in the anterior and posterior territories of the right middle cerebral artery (MCA). MRI was
not performed (Fig. B13.1).
Suspected Diagnosis
Ischemic brain infarction in the right MCA territory of
unknown origin.
Question to Answer by
Ultrasound Techniques
• Was there evidence of a stenotic process, particularly in
the right internal carotid artery (ICA) or MCA?
Initial Neurosonologic Findings (Day 1)
Extracranial Duplex Sonography
B-mode sonography revealed only mild atherosclerosis of the carotid arteries. Color imaging showed
elongation and caliber variations of both distal ICAs,
but predominantly aff ecting the right side. Blood fl ow
velocity in the proximal right ICA was mildly reduced
(fl ow velocity: 47/24 cm/s) and the pulsatility slightly
increased. Within the distal segment of the right ICA,
a non-angle-corrected peak systolic fl ow velocity of
250 cm/s was observed. Normal fl ow velocities were
seen in the remaining extracranial vessels (Fig. B13.2
and Fig. B13.3).
Transcranial Duplex Sonography
The temporal window was inadequate for a complete
insonation of the anterior circulation. Punctual signals
of the proximal M1-MCA could be obtained, revealing
an obvious fl ow velocity diff erence between the two
sides (fl ow velocity: right M1-MCA, 57/25 cm/s; left
M1-MCA, 111/55 cm/s) (not shown). The calculated
Zanette asymmetry index for the systolic velocities was
64 and the right-to-left ratio (= velocity of the aff ected
M1/normal M1 velocity) was 0.51. Assessment of the
other intracranial vessels showed normal and symmetric fl ow signals.
Conclusion
Distal extracranial right ICA stenosis, ~70%. Suspected
right distal M1-MCA occlusion, probably embolic in
nature.
Clinical Course (1)
Thrombolysis was not indicated because of the time
delay and the signs of infarction on CT. The above vascular changes in a relatively young patient without
classic vascular risk factors did not favor an atherosclerotic etiology. In particular, no arterial hypertension
was present. Normal 24-hour ECG and echocardiography made a cardioembolic source unlikely. There
was no coagulopathy involving proteins C and S, anticardiolipin antibodies, activated protein C resistance,
and lupus inhibitor. The cerebrospinal fl uid (CSF) was
normal. In view of the history of chronic rhinitis, sinusitis, and bronchitis, specifi c laboratory tests were
performed which revealed an increased erythrocyte
sedimentation rate (ESR) (70 mm/hour, Westergren),
mild anemia, and thrombocytosis of 1,200/nL (normal
range 130–340/nL), but normal white blood cell counts.
In addition, the level of cytoplasmic antineutrophilic
cytoplasmic antibody (cANCA) was increased to 98 E/
mL (normal value <15 E/mL), and urine proteins and
erythrocytes were elevated. Finally, a nasal mucosa biopsy confi rmed granulomatosis with polyangiitis (GPA;
formerly known as Wegener’s granulomatosis).
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