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An unusual cause of intracranial hemorrhage

Published:October 04, 2018DOI:https://doi.org/10.1016/j.ejim.2018.09.024

      Keywords

      1. Case description

      A 39-year-old woman with no significant medical history was admitted in the emergency room with loss of consciousness. Computed tomography revealed right-sided putamen hemorrhage (Fig. 1, Panel A). The patient had no risk factors for intracranial hemorrhage, including hypertension, older age, alcohol intake, and dyslipidemia. Cerebral angiography showed steno-occlusive changes at the terminal internal carotid artery (ICA) portion bilaterally, with the development of collateral circulation and no aneurysm (Fig. 1, Panels B and C). What is the diagnosis?
      Fig. 1
      Fig. 1Panel A: Computed tomography revealing right-sided putamen hemorrhage.
      Panels B and C: Cerebral angiography showing steno-occlusive changes at the terminal internal carotid artery portion bilaterally, with the development of collateral circulation.

      2. Discussion section

      Moyamoya disease (MMD) was diagnosed by the abovementioned angiographic findings. MMD is a poorly understood occlusive disease involving large intracranial arteries, especially the distal ICA and the stem of the middle cerebral artery and anterior cerebral artery. The lenticulostriate arteries develop a rich collateral circulation around the occlusive lesion, which gives the impression of a “puff of smoke” (moyamoya in Japanese) on conventional x-ray angiography.
      MMD has several unique clinical features, which include two peaks of age distribution at 5 years and at about 40 years [
      • Kuroda S.
      • Houkin K.
      Moyamoya disease: current concepts and future perspectives.
      ]. The main clinical manifestations of MMD are stroke and hemorrhage; unlike pediatric patients, who usually present with transient ischemic attacks or cerebral infarction, almost one-half of adult patients have intracranial hemorrhage that seriously affects their prognosis [
      • Miyamoto S.
      • Yoshimoto T.
      • Hashimoto N.
      • Okada Y.
      • Tsuji I.
      • Tominaga T.
      • et al.
      Effects of extracranial-intracranial bypass for patients with hemorrhagic moyamoya disease: results of the Japan Adult Moyamoya Trial.
      ]. Extracranial-intracranial bypass surgery, which is often used for ischemic MMD, has preventive effects for adult patients with hemorrhagic MMD [
      • Miyamoto S.
      • Yoshimoto T.
      • Hashimoto N.
      • Okada Y.
      • Tsuji I.
      • Tominaga T.
      • et al.
      Effects of extracranial-intracranial bypass for patients with hemorrhagic moyamoya disease: results of the Japan Adult Moyamoya Trial.
      ].
      The incidence of MMD is high in East Asia, such as Japan [
      • Kuroda S.
      • Houkin K.
      Moyamoya disease: current concepts and future perspectives.
      ]. In Europe, the incidence of MMD has been estimated approximately one-tenth of that in Japan [
      • Yonekawa Y.
      • Ogata N.
      • Kaku Y.
      • Taub E.
      • Imhof H.G.
      Moyamoya disease in Europe, past and present status.
      ]. MMD is a chronic progressive cerebrovascular disease and can cause permanent neurologic impairment. Therefore, early diagnosis and prompt treatment of MMD are important.

      Conflict of interest

      None declared.

      References

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        Effects of extracranial-intracranial bypass for patients with hemorrhagic moyamoya disease: results of the Japan Adult Moyamoya Trial.
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