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Case Reports / Journal of Clinical Neuroscience 48 (2018) 104–105

Hyperkinetic transient ischemic attacks preceding deep ganglionic
infarction in a patient with a treated parasellar chondrosarcoma
Michael W. Ruff a, Adip G. Bhargav b, Stephen W. English a, James P. Klaas a,⇑
a
b

Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA
Mayo Clinic School of Medicine, Mayo Clinic, Rochester, MN 55905, USA

a r t i c l e

i n f o

Article history:
Received 13 June 2017
Accepted 23 October 2017

Keywords:
Chondrosarcoma
Radiation-induced atherosclerosis
Hyperkinetic transient ischemic attack (TIA)

a b s t r a c t
A 44-year-old right-handed female with a past medical history of parasellar chondrosarcoma status postsurgical debulking and proton beam therapy (70 Gy) three years prior to presentation experienced several hours of brief, repetitive episodes of transient hemiballism and dystonia; this was followed by abrupt
onset of fixed hemiparesis and dysarthria weeks later, ipsilateral to her prior hyperkinetic movements.
She was found to have total occlusion of the right middle cerebral artery with focal stenosis of the proximal right A-1 segment of the anterior cerebral artery adjacent to the remnants of the chondrosarcoma.
These focal areas of narrowing were attributed to accelerated atherosclerotic disease, an adverse effect of
the radiotherapy used to treat her chondrosarcoma. As treatments improve and mean survival increases
for intracranial malignancy, radiation-induced atherosclerotic disease with protean manifestations such
as those presented in this case may be encountered more frequently.
Ó 2017 Elsevier Ltd. All rights reserved.

1. Case description
A 44 year-old right-handed female with a history of parasellar
chondrosarcoma, treated three years prior with surgical debulking,
and a combination of proton and photon radiotherapy at an outside institution (70 Gy total), developed abrupt onset of recurrent
‘‘jumping, twisting” and ‘‘flinging” movements of the left arm
and leg lasting 20–30 s. Motor function and coordination were
intact between recurrent episodes. She was treated with 1 mg of
lorazepam and the movements resolved. An emergent CT head
demonstrated stability of the chondrosarcoma remnants and no
evidence of infarction. Limited sequence MRI (without diffusionweighted sequences) performed the following day showed no
change from previous post-treatment imaging. Seventeen days
later, the patient presented with left-sided hemiparesis, left facial
droop, and dysarthria. She was noted to have mild left hemineglect
and a flattened affect.
MRI brain demonstrated a well-demarcated acute infarction in
the right basal ganglia with evidence of slow flow in the right
hemisphere and absence of the right middle cerebral artery
(MCA) flow void on T2-weighted images (Fig. 1A–C). The patient
was transferred to our institution where conventional angiography
was performed which demonstrated occlusion of the right MCA at
its origin with robust leptomeningeal collateral supply from the
right anterior cerebral artery (ACA) and posterior cerebral artery.
Additionally there was focal stenosis of the proximal A-1 segment
of the right ACA (Fig. 1D and E). The areas of stenosis and occlusion
were adjacent to the remnants of the chondrosarcoma and were
suggestive of chronic focal atherosclerosis. The patient was started
on aspirin and atorvastatin and was able to return to work one year
later.

2. Discussion
Chondrosarcomas are rare intracranial neoplasms that result in
morbidity through local compression or invasion. They typically

respond well to surgical debulking and high dose (typically proton)
radiotherapy and have low five year mortality [1].
Radiotherapy may contribute to vasculopathy of medium and
large vessels resulting in symptomatic cerebrovascular disease
years after exposure. Animal models suggest that acute
radiation-induced endothelial injury followed by chronic inflammation, or injury to the vasa vasorum may result in accelerated
atherosclerosis leading to focal narrowing of the vessel lumen
[2,3]. The patient’s MRI demonstrated the ‘‘Ivy sign”, a radiographic sign initially described in patients with Moyamoya, which
refers to diffuse leptomeningeal enhancement on post-contrast
MRI and/or hyperintensity on FLAIR imaging, suggesting robust
leptomeningeal collateralization, in our case due to chronic
hypoperfusion of the MCA territories. These findings are in line
with a chronic stenosis leading to occlusion [4]. Alternatively,
cerebral infarction secondary to direct vascular compression from
an intracranial tumor is exceedingly rare and often associated
with rapid tumor expansion or hemorrhage, neither of which were
noted in this case [2,5].
In our patient, the hyperkinetic movements and subsequent
stroke are presumed to be secondary to occlusion of the right
MCA with hypoperfusion to deep ganglionic structures [6,7]. Her
initial presentation to medical attention, in retrospect, was a
hyperkinetic TIA. It is unclear whether initiation of an antiplatelet
agent at the time of initial presentation would have influenced her
outcome. Currently, no trials adequately assess medical treatment
options in primary or secondary stroke prevention in patients
receiving head/neck radiation [2,3]. We recommend a high index
of suspicion for radiation-induced vasculopathy in patients with
transient paroxysmal neurological phenomenon and consideration
for traditional vascular risk factor modification and intervention.
As treatments improve and mean survival increases for intracranial
malignancy, radiation-induced atherosclerotic disease with protean manifestations such as hyperkinetic transient ischemic
attacks may be encountered more frequently.
Sources of support

⇑ Corresponding author at: Department of Neurology, Mayo Clinic, 200 First
Street Southwest, Rochester, MN 55905, USA.
E-mail address: Klaas.James@mayo.edu (J.P. Klaas).

This research did not receive any specific grant from funding
agencies in the public, commercial, or not-for-profit sectors.

Case Reports / Journal of Clinical Neuroscience 48 (2018) 104–105

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Fig. 1. (A) Axial T1-weighted FLAIR with contrast demonstrating residual chondrosarcoma involving the right skull base; (B) Axial DWI image demonstrating acute infarction
of the right basal ganglia and both anterior and posterior limbs of the internal capsule; (C) Axial T2-weighted FLAIR image demonstrating asymmetric increased vascular
signal (yellow arrows) over the right MCA distribution secondary to increased leptomeningeal collateralization from chronic hypoperfusion of the right MCA distribution—
‘‘Ivy sign”; (D and E) Conventional cerebral angiography of the right internal carotid artery demonstrating occlusion of the proximal right MCA M1 segment approximately 5
mm distal to its origin with robust leptomeningeal collateral arteries over the cerebral convexity.

Conflict of interest
The authors declare no conflicts of interest.
References
[1] Bloch OG, Jian BJ, Yang I, et al. A systematic review of intracranial
chondrosarcoma and survival. J Clin Neurosci 2009;16(12):1547–51.
[2] Dearborn J, Urretia V, Zeiler S. Stroke and cancer—a complicated relationship. J
Neurol Transl Neurosci 2014;2(1):1039–51.
[3] Plummer C et al. Ischemic stroke and transient ischemic attack after head and
neck radiotherapy: a review. Stroke 2011;42(9):2410–8.
https://doi.org/10.1016/j.jocn.2017.10.076

[4] Ohta T, Tanaka H, Kuroiwa T. Diffuse leptomeningeal enhancement, ‘‘ivy sign,”
in magnetic resonance images of moyamoya disease in childhood: case report.
Neurosurgery 1995;37(5):1009–12.
[5] Obeid M, Ulane C, Rosenfeld S. Pearls & Oy-sters: large vessel ischemic stroke
secondary to glioblastoma multiforme. Neurology 2010;74:e50–1.
[6] Alonso J et al. Limb-shaking TIA presenting as Hemichorea-Hemiballismus: TIA
chameleons diagnostic challenge in emergency department. J Stroke
Cerebrovasc Dis 2015;24:327–31.
[7] Mehanna R, Jankovic J. Movement disorders in cerebrovascular disease. Lancet
Neurol 2013;12(6):597–608.