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Unilateral Hemispheric Proliferation of Ivy Sign on Fluid-Attenuated Inversion Recovery Images in Moyamoya Disease Correlates Highly with Ipsilateral Hemispheric Decrease of Cerebrovascular Reserve

M. Kawashima, T. Noguchi, Y. Takase, T. Ootsuka, N. Kido and T. Matsushima
American Journal of Neuroradiology October 2009, 30 (9) 1709-1716; DOI: https://doi.org/10.3174/ajnr.A1679

Article Figures & Data

Figures

  • Fig 1.
    Fig 1.

    The proliferative differences of ivy signs on FLAIR images between both hemispheres in each patient was rated as minimal, moderate, or marked. A, Symmetric ivy distribution. We defined the hemispheres as ivy symmetric when a symmetric ivy distribution was observed in both hemispheres. B, Minimal ivy proliferation along the cortical sulci dominantly in the right frontal lobe (dotted circle). C, moderate ivy dominance in the right hemisphere (dotted circle). D, Marked ivy distribution in the left hemisphere (dotted circle).

  • Fig 2.
    Fig 2.

    Case of a 28-year-old man with unsymmetric ivy sign and decreased vascular reserve defined by quantitative SPECT analysis. He had sustained a left TIA. A, FLAIR image showed marked ivy sign in the right hemisphere. B, MRA stages in right and left hemispheres were III and II, respectively. C, Basal brain perfusion SPECT showed mildly decreased CBF in the right hemisphere. D, ACZ stress brain perfusion SPECT showed remarkably decreased CBF in the right hemisphere. E, Quantitative analysis of basal/ACZ stress brain perfusion SPECT. Lower 4 columns show 3D-SSP format view sets of rest CBF, Diamox CBF, vascular reserve, and staging by JFT study from top. Vascular reserve was impaired in most of the right ACA and MCA and in part of the left ACA and MCA territories. The proportions of the stage II area in the right and left hemispheres were 64.9% and 57.9%, respectively. Vascular reserve means in the right and left hemispheres were −9.63 and 2.80, respectively. Vascular reserve less than −30% areas were seen scattered in the right hemisphere. The patient is free from symptoms after cerebral revascularization on the right side.

  • Fig 3.
    Fig 3.

    Quantitative estimation of SPECT in 13 patients with ivy unsymmetric and ivy symmetric distributions. IDH indicates ivy dominant hemisphere; ILDH, ivy less dominant hemisphere. A,B, Proportion of stage II area and mean vascular reserve in IDH and ILDH in patients with an unsymmetric ivy sign (n = 8). A, Proportion of stage II area in IDH and ILDH. Mean proportions of stage II area are 20.71% ± 22.14% in IDH and 13.43% ± 19.91% in ILDH. B, Mean vascular reserve in IDH and ILDH. Mean vascular reserves are 15.06% ± 15.69% in IDH and 23.00% ± 14.59% in ILDH. C,D, Proportion of stage II area and mean vascular reserve in both hemispheres in patients with symmetric ivy sign (n = 5). C, Proportion of stage II area in both hemispheres. Mean proportions of stage II area are 25.64% ± 24.00% in the right hemisphere and 25.64% ± 18.55% in the left hemisphere. D, Mean vascular reserve in both hemispheres. Mean vascular reserves are 12.38% ± 16.69% in the right hemisphere and 15.49% ± 15.77% in the left hemisphere.

  • Fig 4.
    Fig 4.

    A female patient with unsymmetric ivy sign and decreased RI defined by semiquantitative SPECT analysis. She had sustained a right TIA. A, FLAIR image showed marked ivy sign in the left hemisphere. B, Basal brain perfusion SPECT showed decreased CBF in the left hemisphere with an AI of 0.85. C, ACZ stress brain perfusion SPECT showed decreased CBF in the left hemisphere with an AI of 0.73. Semiquantitative analysis of basal/ACZ stress brain perfusion SPECT revealed that rRI decreased in the ivy-dominant hemisphere (RI mean, 0.86). D, Right carotid angiography showed normal filling of vessels in the ACA and MCA regions. E, Left carotid angiography showed delayed filling of vessels in the ACA and MCA regions. A retrograde slow flow of pial vasculature via leptomeningeal collaterals was observed. Regional ACCT in the right and left hemispheres were 3.2 and 5.4 s, respectively (rACCT ratio, 1.69).

  • Fig 5.
    Fig 5.

    A female patient with unsymmetric ivy sign and decreased ivy sign after operation (10 months follow-up period). She had sustained a left TIA. A, Preoperative FLAIR images showed moderate ivy dominance in the right hemisphere (dotted circles). Preoperative MRA grade in the right and left hemispheres were III and II, respectively. She underwent direct bypass surgery in the right side. B, Postoperative FLAIR image obtained 10 months after revascularization surgery revealed decreased ivy sign in the right hemisphere. Postoperative MRA showed well-developed collateral vessels via bypass in the right MCA region (arrow). The patient had no symptoms after the operation.

Tables

  • Table 1:

    MRA score in Moyamoya patient*

    ICANormal0
    Stenosis at supraclinoid portion1
    Loss of continuous signal intensity at supraclinoid portion2
    Invisible3
    MCANormal0
    Stenosis at M11
    Loss of continuous signal intensity at M12
    Invisible3
    ACADistal to A2 is normal0
    Stenosis of ACA distal to A21
    Invisible2
    PCADistal to P2 is normal0
    Stenosis of PCA distal to P21
    Invisible2

    • Note:—MRA indicates MR angiography; ICA, internal carotid artery; MCA, middle cerebral artery; ACA, anterior cerebral artery; PCA, posterior cerebral artery.

    • * MRA score 0–1, MRA grade I; MRA score 2–4, MRA grade II; MRA score 5–7, MRA grade III; MRA score 8–10, MRA grade IV.

  • Table 2:

    Ivy sign and clinical characteristics of 34 patients

    Ivy DominancePatients (n)*TIA (Right/Left)Bypass Surgery (Right/Left)Postoperative Ivy Decrease
    Left side127 (58.3%)/00/8 (66.6%)5/8 (62.5%)
        Minimal42/00/1
        Moderate53/00/5
        Marked32/00/2
    Right side100/9 (90%)7 (70%)/04/7 (66.7%)
        Minimal20/22/0
        Moderate70/65/0
        Marked10/10
    Symmetric122/3/1 (50%) (right/left/bilateral)1/1/1 (25%) (right/left/bilateral)1/3 (33.3%)

    • Note:—TIA indicates transient ischemic attack.

    • * TIA refers to symptomatic body side.

  • Table 3:

    Semiquantitative estimation of vascular reactivity by SPECT in 21 patients with ivy unsymmetric and ivy symmetric proliferation

    RI Mean
    Ivy unsymmetric (n = 14)0.80 ± 0.10* (Ivy dominant/ivy less dominant)
    Ivy symmetric (n = 7)1.05 ± 0.16 (right/left)

    • Note:—RI indicates (vascular) reactivity index; SPECT, single-photon emission CT.

    • * P < .05.

  • Table 4:

    Correlation between ivy proliferation and MRA stage and angiographic findings

    MRA Stage (IDH)MRA Stage (ILDH)rACCT Ratio
    Ivy unsymmetric (n = 22)2.95 ± 0.39*2.60 ± 0.502.10 ± 0.24* (n = 9)
    Ivy symmetric (n = 12)2.78 ± 0.39 (left side)2.88 ± 0.33 (right side)1.10 ± 0.23 (n = 7)

    • Note:—IDH indicates ivy-dominant hemisphere; ILDH, ivy less-dominant hemisphere; rACCT, regional arteriocapillary circulation time.

    • * P < .05 (comparison between IDH and ILDH in ivy unsymmetric or right and left in patients with symmetric ivy sign on MRA and comparison between patients with ivy unsymmetric sign and those with ivy symmetric sign of rACTT ratio on angiography).

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Unilateral Hemispheric Proliferation of Ivy Sign on Fluid-Attenuated Inversion Recovery Images in Moyamoya Disease Correlates Highly with Ipsilateral Hemispheric Decrease of Cerebrovascular Reserve
M. Kawashima, T. Noguchi, Y. Takase, T. Ootsuka, N. Kido, T. Matsushima
American Journal of Neuroradiology Oct 2009, 30 (9) 1709-1716; DOI: 10.3174/ajnr.A1679
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