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Research ArticlePediatrics

Imaging Characteristics of Cerebrovascular Arteriopathy and Stroke in Hutchinson-Gilford Progeria Syndrome

V.M. Silvera, L.B. Gordon, D.B. Orbach, S.E. Campbell, J.T. Machan and N.J. Ullrich
American Journal of Neuroradiology May 2013, 34 (5) 1091-1097; DOI: https://doi.org/10.3174/ajnr.A3341
V.M. Silvera
aFrom the Departments of Radiology (V.M.S., D.B.O.)
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L.B. Gordon
bAnesthesia (L.B.G.)
dDepartment of Pediatrics (L.B.G.), Hasbro Children's Hospital, Providence, Rhode Island
eWarren Alpert Medical School (L.B.G., J.T.M.)
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D.B. Orbach
aFrom the Departments of Radiology (V.M.S., D.B.O.)
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S.E. Campbell
fCenter for Gerontology and Healthcare Research (S.E.C.), Brown University, Providence, Rhode Island
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J.T. Machan
eWarren Alpert Medical School (L.B.G., J.T.M.)
gDepartments of Biostatistics, (J.T.M.)
hOrthopaedics, Surgery, and Psychology (J.T.M.), Rhode Island Hospital, Providence, Rhode Island.
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N.J. Ullrich
cNeurology (N.J.U.), Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts
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Abstract

BACKGROUND AND PURPOSE: HGPS is a rare disorder of segmental aging, with early morbidity from cardiovascular and cerebrovascular disease. The goal of this study was to identify the neurovascular features, infarct type, topography, and natural history of stroke in the only neurovascular imaging cohort study of HGPS.

MATERIALS AND METHODS: We studied 25 children with confirmed diagnoses of HGPS and neuroimaging studies available for review. Relevant clinical information was abstracted from medical records.

RESULTS: We identified features suggestive of a vasculopathy unique to HGPS, including distinctive intracranial steno-occlusive arterial lesions, basal cistern collateral vessels, and slow compensatory collateral flow over the cerebral convexities. The arterial pathology in the neck consisted of distal vertebral artery stenosis with prominent collateral vessel formation as well as stenosis and calcification of both the cervical internal and common carotid arteries. Radiographic evidence of infarction was found in 60% of patients, of which half were likely clinically silent. Both large- and small-vessel disease was observed, characterized by arterial territorial, white matter, lacunar, and watershed infarcts.

CONCLUSIONS: We report a unique intracranial and superior cervical arteriopathy in HGPS distinct from other vasculopathies of childhood, such as Moyamoya, and cerebrovascular disease of aging, including atherosclerosis. Arterial features of the mid and lower neck are less distinctive. For the first time, we identified early and clinically silent strokes as a prevalent disease characteristic in HGPS. Longitudinal analysis of stroke incidence and vasculopathy may provide an outcome measure for future treatment interventions for children with HGPS.

ABBREVIATIONS:

ACA
anterior cerebral artery
CCA
common carotid artery
HGPS
Hutchinson-Gilford Progeria Syndrome
PCA
posterior cerebral artery
VA
vertebral artery
  • © 2013 by American Journal of Neuroradiology
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American Journal of Neuroradiology: 34 (5)
American Journal of Neuroradiology
Vol. 34, Issue 5
1 May 2013
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Imaging Characteristics of Cerebrovascular Arteriopathy and Stroke in Hutchinson-Gilford Progeria Syndrome
V.M. Silvera, L.B. Gordon, D.B. Orbach, S.E. Campbell, J.T. Machan, N.J. Ullrich
American Journal of Neuroradiology May 2013, 34 (5) 1091-1097; DOI: 10.3174/ajnr.A3341

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Imaging Characteristics of Cerebrovascular Arteriopathy and Stroke in Hutchinson-Gilford Progeria Syndrome
V.M. Silvera, L.B. Gordon, D.B. Orbach, S.E. Campbell, J.T. Machan, N.J. Ullrich
American Journal of Neuroradiology May 2013, 34 (5) 1091-1097; DOI: 10.3174/ajnr.A3341
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