CASE STUDY

Moyamoya disease: A case of vanishing cerebral vessels

Joanne L. Thanavaro, DNP, ANP-BC, ACNP-BC, DCC (Associate Professor)1 , Nimish Nemani, MD (Director)2 ,
& Hilton I. Price, MD (Chief)3
1
School of Nursing, St. Louis University, St. Louis, Missouri
2
Intensive Care Unit, Midwest Acute Care Consultants, St. Anthony’s Medical Center, St. Louis, Missouri
3
Department of Radiology, Christian Northeast Hospital, St. Louis, Missouri

Keywords Abstract
Neurology; neuroradiology; neurosurgery;
vascular disease. Purpose: To provide an overview of moyamoya disease (MMD) including
pathophysiology, epidemiology, clinical presentation, diagnosis, treatment,
Correspondence and prognosis.
Joanne L. Thanavaro, DNP, ANP-BC, ACNP-BC, Data sources: Selected clinical and epidemiological studies, review articles,
DCC, School of Nursing, St. Louis University, and diagnostic guidelines for MMD.
3525 Caroline Avenue, Room 321, St. Louis,
Conclusions: MMD is a rare cerebrovascular disease characterized by progres-
Missouri 63104. Tel: 314-977-8993;
Fax: 314-977-8840;
sive stenosis of the distal internal carotid arteries and their major branches. The
E-mail: jthanava@slu.edu dilated and fragile basal collateral circulations display a “puff of smoke” appear-
ance and thus are called moyamoya vessels. Other unique features of MMD
Received: October 2010; include 2:1 female preponderance and its peak incidence in two age groups:
accepted: March 2011
early childhood and adults in their mid-40s. The pathophysiology of MMD is
doi: 10.1111/j.1745-7599.2012.00782.x unclear and possible causes include genetic linkage, angiogenesis, autoimmune
disease, cranial radiation, and infection of the head and neck. Most patients are
symptomatic and may present with ischemic or hemorrhagic strokes, seizure,
or headache. The diagnosis depends on clinical presentation and radiographic
imaging, and disease progression may be halted with direct or indirect cerebral
revascularization.
Implications for practice: It is important to make a correct diagnosis and
provide appropriate treatment to reduce the morbidity and mortality associ-
ated with MMD. A prompt referral for possible surgical revascularization of-
fers the best chance to reduce additional cerebral injuries and improve clinical
outcomes.

Case presentation
ES is a 26-year-old female with a history of diabetes
mellitus, Von Willebrand disease, and a seizure disor-
der. She presented to the emergency department (ED)
with nonspecific symptoms including headache, dizzi-
ness, lightheadedness, nausea, and vertigo-type symp-
toms. She was discharged home with symptomatic
treatment since there was no indication of a serious
illness. She returned to the ED 2 days later because
of a progressively worsening headache and stiff neck.
The patient was afebrile and normotensive (120/70
mmHg). She was alert and oriented to person, place,
and time; and she had no meningismus or focal neuro-
logical deficits. Complete blood count (including white
blood cells and platelets), prothrombin time, partial
thromboplastin time, and complete metabolic profiles
were within normal limits. She had an episode of
seizure in the ED, treated with intravenous lorazepam
administration.
A lumbar puncture was performed as an initial workup
because of the presenting symptoms of progressive
headache, nausea, and stiff neck. The cerebrospinal fluid
(CSF) was grossly bloody, indicative of intracerebral hem-
orrhage (ICH). A cerebral computed tomography (CT)
scan was obtained based on prior studies indicating that
this imaging technique readily identifies cerebral in-
travascular etiologies of ICH, the most likely causes of
ICH in a young female patient without hypertension or
coagulation abnormalities (Chalela et al., 2007; Delgado
Almandoz, 2009). The patient’s CT scan demonstrated

Journal of the American Association of Nurse Practitioners 25 (2013) 173–179 

C 2012 The Author(s) 173
Journal compilation 
C 2012 American Association of Nurse Practitioners
Moyamoya disease J. L. Thanavaro et al.

Figure 1 Anterior–posterior views of carotid an-

giograms demonstrate occluded right internal
carotid artery (A) at the supraclinoid portion and
occluded left internal carotid artery (B) at the
parasellar portion.

evidence of intraventricular hemorrhage, an old right

frontal and parietal lobe infarct, and right periventricular
white matter hypodensity. CT angiogram showed dimin-
ished blood flow within the intracranial distribution of
the internal carotid arteries (ICAs), suggestive of vascu-
lar occlusive disease such as moyamoya disease (MMD).
The cerebral angiography revealed bilateral occlusion of
terminal ICAs and absent flow into both anterior cere-
bral artery (ACA) and middle cerebral artery (MCA)
(Figure 1). The networks of moyamoya vessels charac-
teristic of the diagnosis of MMD were not present and
both ACA and MCA were filled by the collateral cir-
culations from the vertebral artery, compatible with an
advanced stage of MMD (Figure 2). Based on the pres-
ence of bilateral occlusion of terminal ICAs, absent ma-
jor cerebral arteries (ACA and MCA), absent moyamoya
vessels, and evidence of extracranial collateral circula-
tions, the patient was diagnosed with Stage V–VI MMD
on the Suzuki Grading System. ES was admitted to
the intensive care unit and was eventually discharged
from the hospital with full recovery and without resid-
ual neurological deficits. She was subsequently referred Figure 2 Lateral view of left vertebral artery (dark arrow) providing col-
lateral circulations to the anterior (thick white arrow) and middle cerebral
to a University hospital for additional evaluation and
arteries (thin white arrow).
had a successful right superficial temporal artery (STA)
to right MCA revascularization procedure 2 months
later. tients to transient ischemic attacks (TIAs) and ischemic or
hemorrhagic strokes (Burke et al., 2009; Lee et al., 2009;
Smith & Scott, 2005). At the early stage of the disease, the
Background
intracranial collateral circulations that develop at the base
MMD is a rare chronic cerebrovascular disease which of the brain display a “puff of smoke” appearance and are
is characterized by progressive bilateral stenosis or oc- called moyamoya vessels (Figure 3; Burke et al., 2009;
clusion of the terminal part of the ICAs or their main Kuroda & Houkin, 2008; Smith & Scott, 2005; Suzuki &
branches including the ACA and MCA (Burke et al., Takaku, 1969). Moyamoya is Japanese and translates to
2009; Kuroda & Houkin, 2008; Scott & Smith, 2009; “puff of smoke.” The condition was initially identified in
Suzuki & Takaku, 1969). Many collateral networks pro- Japan, thus the reason for a Japanese name (Caldarelli
vide an alternative route for cerebral perfusion; however, et al., 2001; Kraemer et al., 2008; Suzuki & Takaku,
they are imperfect and fragile and may predispose pa- 1969; Yilmaz et al., 2001). As the disease progresses, the

174
J. L. Thanavaro et al.
Figure 3 Left carotid angiogram from a 53-year-old oriental woman who
became unresponsive with left hemiparalysis from a massive intracerebral
hemorrhage. The angiogram shows an occluded left internal carotid artery
(thick dark arrow) with collateral circulation to an anterior cerebral artery
(white thick arrow) and a middle cerebral artery (white thin arrow) via
moyamoya vessels (surrounded by dark thin arrows).
antegrade blood flow decreases with gradually dimin-
ishing moyamoya vessels and the cerebral blood flow is
more dependent on the development of extracranial col-
lateral circulations.
MMD is most prevalent in Japan and even though this
disease is now recognized throughout the world, it re-
mains underdiagnosed as a cause of ischemic or hem-
orrhagic stroke in Western countries (Kraemer et al.,
2008; Kuriyama et al., 2008; Yilmaz et al., 2001). The
clinical diagnosis of MMD requires a strong suspicion
for the disease when treating ischemic or hemorrhagic
cerebrovascular accident (CVA) in children or young fe-
males without other risk factors for strokes such as age
>55 years, history of atrial fibrillation or heart dis-
ease, hypertension, dyslipidemia, smoking history, di-
abetes mellitus, contraceptive pills, or hormone ther-
apy (Reeves et al., 2009; Smith & Scott, 2005). While
some advanced practice nurses (APNs) may encounter
MMD patients with suspected strokes in the acute
care setting, other APNs may care for these patients
with milder ischemic and nonischemic MMD symptoms
in a primary care setting before or after the diagno-
sis is established. Additionally, more patients are sur-
viving the acute event and doing well after cerebral
revascularization. These patients may appear at vari-
ous pediatric or adult primary care practices for other
healthcare needs. It is important that APNs are well-
armed with knowledge of MMD so they will be able
to make a correct diagnosis and provide appropriate
treatment.
Moyamoya disease
Pathophysiology
The pathophysiology of MMD is unclear. The patho-
logical findings of thickened intima, smooth muscle cell
hyperplasia, and luminal thrombosis without atheroscle-
rotic or inflammatory changes in the affected vessels in-
dicate a significant role of angiogenesis in the develop-
ment of MMD (Scott & Smith, 2009; Ullrich et al., 2007).
Luminal microthrombi may lead to endothelial injury,
thickening of the intima, and smooth muscle cell pro-
liferation. Protein S deficiency, lupus anticoagulant, and
anticardiolipin antibodies have been found in other cases,
suggesting a possible autoimmune mechanism in MMD
(Kuroda & Houkin, 2008). A possible genetic association
of MMD to chromosome 17 has been demonstrated in fa-
milial MMD (Burke et al., 2009; Kuroda & Houkin, 2008;
Yamauchi et al., 2000).
Two forms of MMD have been recognized including
primary and secondary forms of the disease. The primary
form is more common and is also referred to as true
MMD. While most cases are idiopathic, familial MMD
has been reported in 12–15% of cases in the Japanese
population (Burke et al., 2009; Kuriyama et al., 2008;
Kuroda & Houkin, 2008; Yamauchi et al., 2000). The
secondary form of this disease is known as moyamoya
syndrome or quasi-moyamoya. This secondary form has
been described in association with sickle cell disease, neu-
rofibromatosis type I, Down’s syndrome, infection of the
head and neck, and cranial therapeutic irradiation (Baba,
Houkin, & Kuroda, 2008; Scott & Smith, 2009; Uchino
et al., 2005 Ullrich et al., 2007; Yamada et al., 1997).
MMD is often associated with bilateral angiographic find-
ings and unilateral findings are more commonly found in
moyamoya syndrome (Fukui, 1997; Ueki et al., 1994).
Epidemiology
Prior reported incidence and prevalence of this dis-
ease varies depending on the population selected for the
study (Baba et al., 2008; Kuriyama et al., 2008; Kuroda
& Houkin, 2008). An all-inclusive survey of a large
Japanese island reported an annual incidence of 0.94 per
100,000 people and a prevalence of 10.5 per 100,000 peo-
ple (Baba et al., 2008). However, a recent nationwide sur-
vey of the Japanese population reported an annual de-
tection rate of the disease of 0.54 per 100,000 patients
and a prevalence of 6 per 100,000 patients (Kuriyama
et al., 2008). A gender difference has been established
with a female to male ratio of approximately 2:1 (Baba
et al., 2008; Kuroda & Houkin, 2008). There are two
peaks of age distribution; prior studies have reported the
highest peak in children between the ages of 5 and 9 years
but recent data reveal a significant increase in the adult
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Moyamoya disease
population with the largest peak between 45 and 49 years
old (Baba et al., 2008; Kuroda & Houkin, 2008; Wakai
et al., 1997).
One study from the western United States (Washing-
ton and California) that included patients from various
ethnic backgrounds reported a lower incidence (0.086
per 100,000), a similar female to male ratio and a sim-
ilar early peak but a later second peak between 55 and
59 years old (Uchino et al., 2005). The incidence was
higher in Asian Americans (4.6 times) and African Amer-
icans (2.2 times) than the Caucasian population. Forty-
eight percent of African Americans in the study also had a
diagnosis of sickle cell disease. The data for MMD in Cau-
casians are limited with the incidence of this disease es-
timated to be one-tenth of that observed in the Japanese
population (Kraemer et al., 2008; Uchino et al., 2005). A
retrospective study in 21 Caucasian patients treated for
MMD in a German institution reported a female predom-
inance of 4.25:1 and all the females presented with an
ischemic event (Kraemer et al., 2008). The available data
indicate that Caucasian patients do not demonstrate the
two peaks of age distributions at the onset of the disease,
have more benign symptoms at presentation, have more
ischemic symptoms at all ages, and respond better to sur-
gical treatment (Hallemeier et al., 2006; Kraemer et al.,
2008; Mesiwala et al., 2008; Yilmaz et al., 2001).
Clinical presentation
Over 80% of patients with MMD are symptomatic and
their clinical presentation may include ischemic, hemor-
rhagic or “other” symptoms such as headache, seizure,
choreiform movements, cognitive, or psychiatric changes
(Baba et al., 2008; Burke et al., 2009). Most childhood
MMD cases present with ischemic events that are of-
ten precipitated by hyperventilation or crying and oc-
cur repetitively over several years (Burke et al., 2009;
Kuroda & Houkin, 2008; Han et al., 2000; Smith & Scott,
2005). Adult patients may present with ischemic symp-
toms, intracranial bleeding, or both (Han et al., 2000;
Kuroda & Houkin, 2008). Two-thirds of these adult pa-
tients suffer from intracranial bleeding which may be at-
tributed to the rupture of moyamoya vessels, aneurysms
in the circle of Willis, dilated perforators, or dilated col-
lateral arteries on the brain surface (Burke et al., 2009;
Kuroda & Houkin, 2008; Osanai et al., 2008). Intracere-
bral hematoma is the major cause of death in patients
with MMD (Burke et al., 2009). Various types of col-
lateral circulation may contribute to other symptoms of
MMD. The dilated meningeal and leptomeningeal col-
lateral vessels may predispose patients to migraine-like
headaches that are frequently unresponsive to medical
therapy (Seol et al., 2005). Choreiform movements in
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J. L. Thanavaro et al.
some patients may be attributed to dilated moyamoya
vessels in the basal ganglion (Parmar et al., 2000; Scott
et al., 2004).
Diagnosis
Strict guidelines for the diagnosis of MMD have been
established (Burke et al., 2009; Fukui, 1997). The di-
agnosis is normally made on the basis of clinical pre-
sentation of ischemic or hemorrhagic stroke in chil-
dren or young female patients together with primary
and secondary radiographic findings (Burke et al., 2009;
Kuriyama et al., 2008; Scott & Smith, 2009; Smith &
Scott 2005). The presenting symptoms and physical find-
ings depend on the age of patients as previously described
(Burke et al., 2009; Kuroda & Houkin, 2008; Han et al.,
2000). Fundoscopic examination may reveal a “morn-
ing glory disk,” which is an enlarged optic disk occasion-
ally seen in those who develop vascular anomalies in the
retina (Lee et al., 2009, Scott & Smith, 2009). The elec-
troencephalogram (EEG) in 50% of the children with this
disease may demonstrate a distinctive pattern of ”build-
up” and ”rebuild-up” phenomenon that occurs when the
child is asked to hyperventilate. Specifically at the be-
ginning of hyperventilation, the EEG displays high volt-
age slow waves which disappear during hyperventilation
but return within 20–60 s after stopping hyperventilation
(Kodama et al., 1979; Smith & Scott, 2005).
The workup of a suspected case of MMD usually begins
with cerebral CT scan, which can readily identify the sec-
ondary findings (Burke et al., 2009; Smith & Scott, 2005).
It is possible that patients with TIAs may not demonstrate
any abnormalities (Scott & Smith, 2009). CT angiogra-
phy may be used to visualize the intracranial stenoses but
cerebral angiography remains the gold-standard imaging
technique that will best demonstrate the primary find-
ings of MMD (Burke et al., 2009; Kuriyama et al., 2008;
Scott & Smith, 2009). These primary findings consist of
bilateral stenosis or occlusion of the terminal portion of
the ICAs or their major branches and abundant collat-
eral formation with basal moyamoya vessels (Burke et al.,
2009; Fukui, 1997; Kuriyama et al., 2008). Magnetic res-
onance imaging (MRI) and angiography (MRA) are re-
liable methods for the visualization of the primary and
secondary findings as well as postoperative results of
revascularization procedure for MMD (Burke et al.,
2009). Because of its excellent diagnostic yield and non-
invasive nature of the procedure, MRA is now recom-
mended as the primary diagnostic imaging procedure
for MMD (Smith & Scott 2005). Conventional cerebral
angiography may be necessary in a few patients when
smaller vessel occlusions and moyamoya collateral vessels
are not well visualized with MRI and MRA (Kuriyama
J. L. Thanavaro et al.
Table 1 Suzuki Staging System of moyamoya disease
Stage I Bilateral internal carotid artery (ICA) stenosis distally at the
carotid forks
Stage II Development of basal moyamoya vessels
Stage III Increasing ICA stenosis involving anterior and middle
cerebral arteries
Intensification of moyamoya vessels
Stage IV Occlusion of ICAs and the entire circle of
Willis—disappearance of all major cerebral arteries
Diminished basal moyamoya vessels
Development of extracranial collaterals
Stage V Further reduction of basal moyamoya vessels
Intensification of extracranial collaterals
Stage VI Disappearance of basal moyamoya vessels
Collateral circulation solely from the external carotid arteries
Note. Burke et al., 2009; Scott & Smith, 2009; Suzuki & Kodama, 1983;
Zipfel et al., 2005.
et al., 2008; Smith & Scott 2005). A definitive diagnosis of
MMD requires the presence of bilateral distal ICA stenosis
or occlusion and intra- or extracranial collateral circula-
tions demonstrated by cerebral angiography or MRI and
MRA (Kuriyama et al., 2008).
While bilateral ICA obstruction is characteristic of
MMD, some patients may initially present with unilateral
vascular abnormalities and approximately one-third of
them will eventually develop contralateral disease (Kelly
et al., 2006; Kuroda et al., 2005). In a study of 120
adults, female sex was the only risk for progression of
the disease, whereas a study with predominantly children
in the sample reported the association of progression of
the disease to contralateral abnormalities on initial imag-
ing, congenital cardiac anomaly, previous cranial radia-
tion, Asian ancestry, and familial moyamoya syndrome
(Kuroda et al., 2005; Smith & Scott, 2008). The secondary
radiographic findings include cerebral infarction, white
matter lesions, atrophy, and hemorrhage (Burke et al.,
2009; Scott et al., 2004; Smith & Scott 2005; Suzuki &
Kodama, 1983).
The Suzuki Grading System describes six angiographic
stages, which indicate the severity of the disease (Burke
et al., 2009; Scott & Smith, 2009; Suzuki & Kodama,
1983). The grading system begins with bilateral ICA
stenosis distally at the carotid forks in Stage I, progres-
sive development and disappearance of basal moyamoya
vessels over Stage II–V, and final deterioration to com-
plete absence of major cerebral arteries and basal moy-
amoya vessels with collateral circulation produced solely
from the external carotid arteries in Stage VI (Table 1).
Most symptomatic cases of MMD are diagnosed at Stage
III of this grading system (Burke et al., 2009). Extra
to intracranial collaterals in some patients may form net-
like vessels; two forms have been described including eth-
Moyamoya disease
moidal and vault moyamoya (Suzuki & Kodama, 1983).
Ethmoidal moyamoya communicates with basal moy-
amoya via ophthalmic, anterior, and posterior ethmoidal
arteries. Vault moyamoya develops transdural anastomo-
sis from the middle meningeal artery and STA. These
extra- to intracranial moyamoya vessels poorly develop
in adults as compared to children and this may be at-
tributed to the loss of the capacity to form these collat-
erals with increasing age (Suzuki & Kodama, 1983).
The case presentation in this report demonstrates that
the diagnosis of MMD can be difficult and requires
a strong clinical suspicion. The initial presentation of
headache, dizziness, and nausea without any other neu-
rological deficits were subtle and nonspecific. Even with
a progressive worsening headache and stiff neck, the pa-
tient continued to have a normal neurological exami-
nation without meningismus. The diagnosis of ICH was
made primarily because of grossly bloody CSF, which was
attributed to MMD on the basis of the primary findings of
complete ICA occlusion involving ACA and MCA with
extra- to intracranial collateral circulations, as well as the
secondary findings of prior infarct and intraventricular
hemorrhage on CT scan.
Treatment
Patients with milder symptoms are usually treated con-
servatively with medical therapy but there is little evi-
dence of short-term or long-term efficacy (Burke et al.,
2009). Antiplatelet agents have been used to prevent em-
boli but chronic anticoagulation with warfarin is rarely
used (Bowen et al., 2005; Scott et al., 2004; Smith &
Scott, 2005). Calcium-channel blockers may relieve in-
tractable headaches or reduce the incidence and severity
of TIAs (Scott & Smith, 2009; Shirane et al., 1997).
Patients with more severe symptoms are treated with
one of the three surgical revascularization procedures:
direct, indirect, and combined techniques (Burke et al.,
2009; Hallemeier et al., 2006; Kuroda & Houkin, 2008;
Smith & Scott, 2005; Yilmaz et al., 2001). Direct revas-
cularization involves extra- to intracranial arterial by-
pass with anastomosis of the STA to MCA or other graft-
type procedures (Burke et al., 2009; Kuroda & Houkin,
2008; Smith & Scott, 2005). This type of revasculariza-
tion is limited to adults or older children with accept-
able calibers of donor and recipient vessels (Burke et al.,
2009). Indirect revascularization involves placing vascu-
larized tissues such as dura, temporalis muscle, or STA
itself, in direct contact with the brain to promote neo-
vascularization to the underlying cerebral cortex (Burke
et al., 2009; Kuroda & Houkin, 2008; Smith & Scott,
2005). This procedure is more suitable for patients with
poor cortical branches for anastomosis or for younger
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Moyamoya disease
patients with smaller donor or recipient vessels (Burke
et al., 2009). Encephaloduroarteriosynangiosis (EDAS) is
the most commonly performed indirect revascularization
procedure, in which a segment of a scalp artery is trans-
posed on to the surface of the brain to improve collateral
blood flow (Yilmaz et al., 2001). Combined direct and in-
direct procedures have been employed to promote better
revascularization and prevent ischemia in some patients
(Burke et al., 2009).
The revascularization procedure is usually recom-
mended for ischemic strokes or TIAs in patients with good
neurological ability to perform daily activities, small area
of cerebral infarctions (<2 cm in diameter) or disease
severity at Stage II–IV (Zipfel et al., 2005). However, the
efficacy of cerebral revascularization for the prevention
of recurrent cerebral hemorrhage is unclear and requires
further evaluation (Isono et al., 2002; Kawaguchi et al.,
2000; Zipfel et al., 2005). The patient in this case pre-
sentation had rather far advanced occlusive cerebrovas-
cular disease (Stage V–VI); cerebral revascularization for
an ischemic event is generally not recommended at this
stage (Zipfel et al., 2005). It is not unclear if the clini-
cal findings of simultaneous ischemic (old right frontal
and parietal lobe infarcts) and hemorrhagic disease pro-
cesses could have influenced the neurosurgeon’s deci-
sion to proceed with cerebral revascularization in this
patient. An intracranial carotid stent insertion is an un-
proven nonsurgical revascularization treatment for MMD
that may be beneficial to selected patients. Successful ICA
stent insertion in a patient who was suffering from recur-
rent TIAs associated with severe distal left ICA obstruc-
tion prevented any additional neurological events over a
period of 46 months follow-up (Kornblihtt et al., 2005).
This procedure may provide a temporary relief of the
stenosis but its use needs further study.
Prognosis
The natural history of MMD is variable and disease
progression can be slow or fulminant with rapid neu-
rological deterioration (Burke et al., 2009; Scott et al.,
2004; Smith & Scott, 2005). MMD eventually progresses
in most patients and medical therapy alone does not
stop disease progression (Kuroda et al., 2005; Smith &
Scott, 2005). Two-thirds of patients have symptomatic
progression over 5 years and surgery reduces the rate of
progression to 2.6–5.6% (Choi et al., 1997; Fung et al.,
2005; Guzman et al., 2009; Scott & Smith, 2009). MMD
in children progresses more rapidly than in adults and
most adult cases are more advanced when the diagno-
sis is made (Burke et al., 2009). Neurological status at
the time of the diagnosis rather than the patient’s age
predicts the long-term prognosis and early diagnosis and
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J. L. Thanavaro et al.
treatment substantially improve the patient’s long-term
outcome (Scott et al., 2004; Smith & Scott, 2005).
Summary
MMD is a rare progressive cerebrovascular disease
which requires a strong clinical suspicion to make a cor-
rect diagnosis, particularly if a child or a young female
patient without typical risks for CVA presents with CVA
or seizure disorder. The diagnosis may be confirmed with
radiographic imaging. Surgical treatment with direct or
indirect cerebral revascularization halts the progression
of the disease in the majority of patients and a poten-
tially good outcome is expected. An understanding of this
clinical entity is essential for making the correct diagno-
sis, explaining the nature of the disease to patients and
their families, providing proper care in the primary and
acute care setting, and making an appropriate referral for
definitive surgical correction.
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