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Rapidly Progressive Fatal Bihemispheric Infarction Secondary to Moyamoya Syndrome in Association with Graves Thyrotoxicosis

S.W. Hsu, J.C. Chaloupka and D. Fattal
American Journal of Neuroradiology March 2006, 27 (3) 643-647;
S.W. Hsu
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J.C. Chaloupka
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D. Fattal
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    Fig 1.

    Axial cranial CT scans obtained with intravenous contrast enhancement (A, -B) show multiple areas of abnormal low attenuation, which are most dramatic within both frontal lobes as well as within the bilateral basal ganglia.

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    Fig 2.

    The anteroposterior projections of bilateral internal carotid artery (ICA) angiography in early (A, right; -B, left) and late arterial phases (C, right; -D, left) and lateral projections of bilateral ICA angiography (E, right; -F, left) show nearly complete occlusion of the bilateral supraclinoid ICA as well as bilateral proximal segments of the ACAs and MCAs. Only minimal delay antegrade flows of the bilateral MCAs are noted. Collateral flows are seen from the posterior cerebral artery via the posterior choroidal plexus to the pericallosal artery.

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    Fig 3.

    Magnified lateral projections of bilateral ICA angiography (A, right; -B, left) show only minimal collateral flows (arrows) from lenticulostriates over the base of brain, which reconstitute into the postocclusive or highly stenotic portions of the MCAs.

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    Table 1:

    Summary of diseases associated with Graves disease and with Moyamoya syndrome

    Diseases associated with Graves diseaseUlcerative colitis, inflammatory bowel disease, pulmonary hypertension, aplastic anemia, primary sclerosing cholangitis, retroperitoneal fibrosis, membranous gromerulonephritis, immunoglobulin A/minimal change nephropathy, myasthenia gravis, cerebral venous thrombosis, thyroid carcinoma, breast carcinoma, Moyamoya syndrome
    Diseases associated with Moyamoya syndromeSickle cell anemia, aplastic anemia, systemic lupus erythematous, antiphospholipid syndrome, ulcerative colitis, tuberculosis, leptospirosis, Down syndrome, Apert syndrome, neurofibromatosis, tuberous sclerosis, cranial irradiation, Graves disease
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    Table 2:

    Summary of reported cases of Moyamoya syndrome in association with Graves disease

    StudyAge/Sex (y)RaceClinical PresentationsCerebral Angiographic FindingsOutcome
    Kushima et al326/FJapaneseThyrotoxicosis, recurrent hemiparesis, speech disturbanceTypical netlike vessels at the base of brain, occlusion of right ACA and MCARecovery
    Kushima et al322/FJapaneseThyrotoxicosis, hemiparesisNarrowing of bilateral ICA, netlike vessels at the base of brainRecovery
    Liu et al428/FChineseThyrotoxicosis, hemiparesisBilateral ACA and MCA occlusion with Moyamoya vessels, tubular stenosis of bilateral cervical ICANot described
    Tendler et al537/FHispanicThyrotoxicosis, hemiparesisRight distal ICA, proximal MCA, and ACA occlusion, collateral from PCARecovery
    Tendler et al547/FCaucasianMoyamoya disease was diagnosed 10 years before thyrotoxicosisBilateral MCA occlusion, “puff of smoke” vessels on left basal gangliaRecovery
    Leno et al621/MHispanicDown syndrome, hemiparesis, thyrotoxicosisMarked stenoses of both supraclinoid ICA, decreased flow over MCA, occluded ACA, prominent lenticulostratesRecovery
    Kim et al737/FKoreanThyrotoxicosis, cardiomegaly, pulmonary edema, seizureBilateral ICA and left MCA occlusion (by MR angiography)Recovery
    Nakamura et al 823/FJapaneseThyrotoxicosis, cardiomegaly, cerebral infarctionMultiple intracranial arterial stenoses, netlike collaterals around circle of WillisRecovery after revasculization
    Nakamura et al854/FJapaneseUpper limits of normal range of thyroid function, hemiparesisMultiple intracranial arterial stenosis around circle of WillisRecovery after revasculization
    Hsu et al (this study)40/FCaucasianThyrotoxicosis, rapid progressive bilateral cerebral infarctionBilateral distal ICA and proximal MCA and ACA occlusionDied
    • Note:—ACA indicates anterior cerebral artery; MCA, middle cerebral artery; ICA, internal carotid artery; PCA, posterior cerebral artery.

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American Journal of Neuroradiology: 27 (3)
American Journal of Neuroradiology
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S.W. Hsu, J.C. Chaloupka, D. Fattal
Rapidly Progressive Fatal Bihemispheric Infarction Secondary to Moyamoya Syndrome in Association with Graves Thyrotoxicosis
American Journal of Neuroradiology Mar 2006, 27 (3) 643-647;

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Rapidly Progressive Fatal Bihemispheric Infarction Secondary to Moyamoya Syndrome in Association with Graves Thyrotoxicosis
S.W. Hsu, J.C. Chaloupka, D. Fattal
American Journal of Neuroradiology Mar 2006, 27 (3) 643-647;
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