CME

A 16-Year-Old Male with Dizziness,
Parasthesias, and Ataxia
Margaret Z. Tsien, MD; James R. Brorson, MD; Rimas V. Lukas, MD; and Emily C. Dawson, MD

Abstract
Margaret Z. Tsien, MD, is a Resident, Department of Pediatrics at Comer Children’s
Hospital and Department of Internal Medicine, University of Chicago Medical Center.
James R. Brorson, MD, is Associate Professor, Department of Neurology, University
of Chicago Medical Center. Rimas V. Lukas,
MD, is Assistant Professor, Department of
Neurology, University of Chicago Medical
Center. Emily C. Dawson, MD, is Assistant
Professor, Department of Pediatrics at Comer Children’s Hospital.
Address correspondence to: Margaret Z.
Tsien, MD, 5841 S. Maryland Avenue, Chicago, IL 60637; email: Margaret.Tsien@gmail.
com.
Disclosure: James R. Brorson, MD, has
performed consulting work for the National
Peer Review Corporation and for CVS/Caremark Inc. Rimas V. Lukas, MD, has received
consulting fees from Novocare, and has
been a medical reviewer of published materials for EBSCO and ECRI. The other authors
have no relevant financial relationships to
disclose.
doi: 10.3928/00904481-20140417-10
Acknowledgments: The authors thank
Valerie G. Press, MD, MPH; Fernando Goldenberg, MD; Gregory Christoforidis, MD;
and Joseph R. Hageman, MD.

© Shutterstock

A 16-year-old African-American male with no past medical history presented with gait
instability and somnolence. He had intermittent neurological complaints during the prior 4
months, including dizziness, left arm paresthesias, decreased hearing, and inability to control
his hands. After an initial diagnosis of vertigo, his symptoms progressed, leading to reevaluation and a second emergency department head computed tomography (CT) scan, which
revealed a large area of hypodensity in the cerebellum. Repeat head CT on arrival to the intensive care unit showed a large, left cerebellar hemispheric stroke. This case study discusses
the findings of the patient’s cerebral angiogram, the diagnosis of fibromuscular dysplasia, and
the aggressive treatment that likely prevented further devastating strokes in the brainstem,
thalamus, and occipital lobe. This case serves as a reminder that strokes are not just an adult
disease and that classic presentations can occur even in unconventional patients.

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A

16-year-old African-American
male with no past medical history presented with gait instability and somnolence. He had intermittent neurological complaints during the
prior 4 months, including dizziness, left
arm paresthesias, decreased hearing,
and inability to control his hands. During these 4 months, his primary physician ordered a brain magnetic resonance
imaging (MRI) in the second month
and a head computed tomography (CT)
scan in the third month; both were unremarkable. There was no history of
recent trauma or of sickle cell disease.
The night prior to presentation, his family noted gait instability and decreased
level of arousal. He had decreased oral
intake and vomiting with no fevers. He
was taken to an emergency department
(ED), where he was diagnosed with
vertigo and sent home with prescriptions for meclizine and ondansetron. His
symptoms progressed, leading to reevaluation in a second ED. Head CT scan
revealed a large area of hypodensity in
the cerebellum. The boy was transferred
for intensive care unit (ICU)-level care.
On admission to the pediatric ICU,
the patient was sleepy but able to be
roused, responding slowly to questions
with the need for frequent redirection.
Horizontal nystagmus, decreased hearing on the left side, left facial droop,
left-sided weakness, and dysarthria were
present. Repeat head CT on arrival to
the ICU showed a large, left cerebellar
hemispheric stroke (Figure 1). An acute
stroke protocol was initiated and the
neurocritical care service was consulted.
His symptoms progressed with poor gag
reflex, inability to swallow oral secretions, and agitation requiring intubation
for airway protection. He was taken for
“stat” CT angiography of the head and
neck, which showed bilateral vertebral
artery occlusions. To further define his
pathology and for potential intervention, a conventional angiogram was performed.

PEDIATRIC ANNALS 43:5 | MAY 2014

The angiogram showed evidence
of bilateral vertebral artery dissections
with complete thrombotic occlusion of
the right vertebral artery, large thrombus
with sluggish flow and a pseudoaneurysm in the left vertebral artery, as well
as complete occlusion of the basilar artery, both posterior cerebral arteries, and
cerebellar arteries (Figure 2). A stent
retriever device was used. Embolectomy with complete recanalization of the
basilar artery was achieved, and clot retrieval from the left vertebral artery and
posterior cerebral arteries was also performed. These interventions were successful in restoring adequate flow to the
posterior cerebral circulation; however,
a large infarction in the cerebellum was
already irreversible.

Images courtesy of Margaret Z. Tsien, MD.

CME

Figure 1. Computed tomography of head without contrast demonstrating a cerebellar stroke.

Fibromuscular Dysplasia

including dysarthria, ataxic eye movements, decreased fine motor performance, and a severely ataxic gait. He
requires assistance with all activities of
daily living but is no longer dependent
on tracheostomy and gastrostomy tubes.

The findings on the cerebral angiogram, including stenosis and dilations in
the internal carotid arteries forming the
characteristic “string-of-beads” appearance (Figure 3), suggested fibromuscular dysplasia (FMD) as the etiology of
his spontaneous dissections, leading to
his stroke. Because of the extracranial
bilateral vertebral artery dissections and
the presence of artery-to-artery embolism, he was placed on systemic anticoagulation. His course was complicated
by significant cerebellar swelling due to
strokes and possible reperfusion injury,
requiring decompressive suboccipital
craniectomy and duroplasty. During his
ICU course, the boy had hemorrhagic
conversion of his strokes, fever, hypertension, and tachycardia thought to be
secondary to dysautonomic syndrome.
He required tracheostomy and gastrostomy placement. In the most recent clinic
visit, approximately 5 months after his
hospitalization, the patient continued
to have significant neurologic sequelae,

DISCUSSION
FMD is a non-inflammatory, nonatherosclerotic
vasculopathy
that
causes deformation of arteries, resulting in stenosis, occlusion, aneurysm
formation, or dissection. The pathogenesis of the disease remains unknown
despite suggestions of a genetic, hormonal, or mechanical origin. The epidemiology of FMD is still being evaluated; however, a national registry for
FMD established in 2003 has shown the
following characteristics of those diagnosed: 91% female, mean age of 55.7
years, and 95.4% white.1 Our patient
does not meet this classic phenotype.
FMD in the pediatric population has
focused mainly on renal artery stenosis
presenting with hypertension. There are
a few existing case reports of strokes in
pediatric patients caused by FMD.2-6
FMD presents with stroke, aneurysm,
or dissection in 17% to 20% of cases.1
Most often, FMD presents with nonspecific complaints, such as headaches

Diagnosis:

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CME

Figure 3. Classic “string-of-beads” appearance of
right internal carotid artery.

Figure 2. Left vertebral artery with pseudoaneurysm (1) and large thrombus (2) partially obstructing
flow with possible intimal flap indicating likely dissection. Left posterior cerebral artery with recurrent
flow obstruction (3) but with multiple collaterals. Right vertebral artery with retrograde flow from dye
injected into the left vertebral artery (4).

or hypertension, making it very difficult to diagnosis.
Classically, the diagnosis is made
by conventional angiography, but it
can also be made by pathology. The increase in the general use of noninvasive
imaging like CT angiogram and MR
angiogram has likely contributed to the
increasing rate of incidental diagnosis.
Of note, CT angiograms do not always
detect dissections, as demonstrated in
our patient. The definitive diagnosis of
dissections and FMD in our patient was
made via conventional angiography.
Eighty to 90% of FMD cases have the
classic “string-of-beads” radiographic
finding. FMD can also present in imaging as tubular stenosis, dissection, or
aneurysm.9 The “string-of-beads” angiographic finding represents multifocal
stenosis and mural dilation. This usue118 | Healio.com/Pediatrics

ally correlates with pathologic findings
of medial fibroplasia: disorganization
and replacement of smooth muscle by
a chaotic composition of fibroblasts and
collagen.10,11 Other pathologic findings,
such as intimal fibroplasia and medial
hyperplasia, are rarely found and can
sometimes be seen in other pathologies
such as arterial injury from trauma or
post-arteritis. Given the relative lack of
pathologic samples taken from patients,
the nearest thing to a gold standard for
diagnosing FMD is angiography.
FMD can involve almost any artery in
the body, and the treatment depends on the
presentation. Data from the national registry show 66% renal artery involvement,
56% carotid artery involvement, and
18% with vertebral artery involvement.1
Our patient had angiographic FMD findings involving both carotid arteries, both

vertebral arteries, and multiple areas in
the posterior cerebral circulation. More
than 90% of infarcts from dissection are
thromboembolic rather than from lowflow states; therefore, anticoagulation
was initiated for prevention of both clot
propagation and new ischemic strokes.
The use of anticoagulation for extra-cranial carotid or vertebral dissections has
been in practice since the 1970s, although
the treatment remains somewhat controversial.12 Our patient had a hemorrhagic
conversion of his strokes, and his anticoagulation was discontinued.
CONCLUSION
FMD is a rare disorder with poorly
described pathophysiology. Our patient
had unique demographics and pattern of
involvement. His intermittent symptoms
and negative imaging suggest he was
likely experiencing transient ischemic
attacks (TIAs) during the 4 months preceding his diagnosis. When our patient
presented to the ED, he was initially sent
home with a diagnosis of vertigo. Had
this patient been an adult, TIA/stroke
would have been higher on the differential diagnosis, as his presentation was
classic for both. His age, atypical demo-

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graphics for FMD, and overall decreased
awareness of FMD likely contributed to
the delayed diagnosis. The aggressive
treatment provided to our patient probably prevented the development of devastating brainstem, thalamic, and occipital strokes as commonly seen in cases of
complete acute basilar artery occlusions.
Unfortunately, despite multiple interventions, by the time of his diagnosis
he had already infarcted his cerebellum,
for which treatment is largely supportive. This case reminds us that strokes are
not just an adult disease and that classic
presentations can occur even in unconventional patients. In pediatric patients,
stroke leads to mortality approximately
10% to 25% of the time and commonly
leads to persistent neurologic deficits.13
Early recognition of stroke syndromes in
the pediatric population becomes of par-

PEDIATRIC ANNALS 43:5 | MAY 2014

amount importance given the evolving
availability of acute reperfusion strategies for acute ischemic stroke.
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