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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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  • Fig 1.
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    Fig 1.

    Kaplan-Meier model of stroke-free probability depicts the youngest ages of patients at which imaging demonstrated ≥1 infarct, acute or chronic. The solid line shows the estimated stroke-free probability; dashed lines, the 95% CIs; and the dotted lines, the estimated age at which 50% of children have radiographically detectable stroke.

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    Fig 2.

    Graphic representation of infarct distribution.

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    Fig 3.

    Patterns of infarction. Axial FLAIR images of 2 different patients. A, Chronic watershed (white arrows) and white matter infarcts (black arrow). B, Acute gyral infarcts (black arrows). Bright signal in the sulci indicates slow cortical collateral flow (white arrows).

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    Fig 4.

    Arterial calcification. CTA reformatted images of the same patient demonstrate right VA calcification (A) and ICA and external carotid artery (B) calcifications.

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    Fig 5.

    VA stenoses and collaterals. A, Lateral projection of a conventional angiogram demonstrates a short-segment high-grade stenosis of the distal V2 segment of the VA. B, Lateral projection of a 3D model of the cervical MRA in a different patient demonstrates a high-grade stenosis of the V2 segment of the VA (single white arrow), an enlarged deep cervical artery (double white arrows), and enlargement of the occipital artery (curved white arrow).

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    Fig 6.

    Collateral vessels. A, Coronal T2WI of an enlarged ASA (white arrow). B, Collapsed view of an MRA in a different patient demonstrates M1 (single black arrow) and A1 stenoses (black double arrows), internal maxillary artery collaterals (black wavy arrows), subfrontal collaterals (single white arrow), and an enlarged ASA and posterior spinal artery (white double arrows).

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    Fig 7.

    ICA stenosis. 3D model of the MRA of the circle of Willis demonstrates bilateral short-segment high-grade stenoses of the cavernous ICAs (white arrows).

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    Fig 8.

    Collateral vessels. A, Axial T2WI shows collateral vessels in the basal cistern (arrow). Source images from an MRA in a different patient demonstrate subfrontal (B) and perisplenial (C) collateral vessels (arrows.)

Tables

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  • Indications for neuroimaging

    IndicationsaNo. of Scans
    Acute neurologic symptoms suspicious for TIA/stroke50
    Screening/diagnostic31
    Follow-up on prior imaging21
    Seizure13
    Unknown8
    Trauma7
    Headaches5
    Otherb5
    Extra-axial hemorrhage/subdural hematoma3
    Papilledema2
    Growth hormone initiation1
    • ↵a Some patients had >1 indication for imaging and/or multiple studies done at 1 study date.

    • ↵b Eye pain, stent placement, cyst evaluation.

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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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Cite this article
V.M. Silvera, L.B. Gordon, D.B. Orbach, S.E. Campbell, J.T. Machan, N.J. Ullrich
Imaging Characteristics of Cerebrovascular Arteriopathy and Stroke in Hutchinson-Gilford Progeria Syndrome
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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  • Neurologic features of Hutchinson-Gilford progeria syndrome after lonafarnib treatment
    Nicole J. Ullrich, Mark W. Kieran, David T. Miller, Leslie B. Gordon, Yoon-Jae Cho, V. Michelle Silvera, Anita Giobbie-Hurder, Donna Neuberg, Monica E. Kleinman
    Neurology 2013 81 5
  • Accelerated Vascular Aging as a Paradigm for Hypertensive Vascular Disease: Prevention and Therapy
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    Canadian Journal of Cardiology 2016 32 5
  • Hutchinson–Gilford Progeria Syndrome: A Premature Aging Disease
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  • An Upregulation in the Expression of Vanilloid Transient Potential Channels 2 Enhances Hypotonicity-Induced Cytosolic Ca2+ Rise in Human Induced Pluripotent Stem Cell Model of Hutchinson Gillford Progeria
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