If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
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?
Panels B and C: Cerebral angiography showing steno-occlusive changes at the terminal internal carotid artery portion bilaterally, with the development of collateral circulation.
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 [
]. 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 [
]. 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
Kuroda S.
Houkin K.
Moyamoya disease: current concepts and future perspectives.
30389-3&id=gr1.jpg){kind=link}