Child ; Humans ; Male ; Moyamoya Disease ; etiology ; Takayasu Arteritis ; complications ; diagnosis

Moyamoya disease is a cerebrovascular disorder of unknown cause, characterized by slowly progressive bilateral stenosis or occlusion of the internal carotid arteries and produces collateral vessels. Moyamoya syndrome has rarely been reported in association with Graves' disease, especially in children. Several reports suggest that a cerebral infarction might have occurred in patients with clinical and laboratory evidence of hyperthyroid function. We report a case of Moyamoya disease in a girl with Down syndrome and thyrotoxicosis, and we review the relevant literature. To our best knowledge, this is the first report of Moyamoya disease associated with thyrotoxicosis in a young person in Korea.
Down Syndrome/complications ; Female ; Humans ; Korea ; Moyamoya Disease/*complications/*diagnosis ; Thyrotoxicosis/*complications ; Young Adult

Adult ; Hemoglobinuria, Paroxysmal ; complications ; pathology ; Humans ; Male ; Moyamoya Disease ; diagnosis ; etiology ; pathology ; Young Adult

No abstract available.
Adult ; Brain Ischemia/diagnosis/*etiology ; Cerebral Angiography ; Fatal Outcome ; Female ; Humans ; Hyperventilation/complications ; Moyamoya Disease/*complications/diagnosis/therapy ; Risk Factors ; Stroke/diagnosis/*etiology ; Thyroid Crisis/*complications/diagnosis/therapy

We report on a 13-year-old female with systemic lupus erythematosus (SLE) who exhibited symptoms of severe migraine and familial moyamoya disease. Cerebral magnetic resonance angiography (MRA) showed stenosis and occlusion of the bilateral internal carotid arteries associated with the development of collateral circulation (moyamoya vessels). In a child, as in this case, headaches with cerebral infarction associated with moyamoya disease are unusual. Few cases of SLE associated with familial moyamoya disease have been reported, with no previous reports of such cases from Korea. There were no evidences of antiphospholipid syndrome, and activity of SLE or other risk factors for cerebral occlusion were also absent.
Adolescent ; Carotid Artery, Internal/pathology ; Female ; Human ; Lupus Erythematosus, Systemic/*complications ; Magnetic Resonance Angiography ; Migraine/etiology ; Moyamoya Disease/*diagnosis/*genetics

A 36-year-old man was diagnosed with a right temporal lobe grade II cerebral arteriovenous malformation (cAVM) and was treated with radiosurgery. At nine months after the cAVM radiosurgery, the patient began to develop bilateral focal narrowing at the M1 segments of the bilateral middle cerebral arteries. The narrowing progressively deteriorated as was demonstrated on longitudinal serial follow-up MR imaging. X-ray angiography performed at 51 months after radiosurgery confirmed that the cAVM was cured and a diagnosis of moyamoya disease. To the best of our knowledge, this is the first case of cAVM-associated moyamoya disease that developed after radiosurgery. Given the chronological sequence of disease development and radiation dose distribution of radiosurgery, it is proposed that humoral or unknown predisposing factors, rather than direct radiation effects, are the cause of moyamoya disease associated with cAVM.
Adult ; Humans ; Intracranial Arteriovenous Malformations/diagnosis/*surgery ; Magnetic Resonance Imaging ; Male ; Moyamoya Disease/*etiology ; Postoperative Complications ; Radiosurgery

The brain MR images of 23 patients with angiographically proved moyamoya disease were reviewed to evaluate the capability of MR to demonstrate vascular and parenchymal abnormalities. All the MR images were obtained on a 2.0 T superconducting system and included T1-weighted sagittal and T2-weighted axial images without implementation of flow compensation (FC). The vascular abnormalities demonstrated on MR images were narrowing of the cavernous internal carotid artery (ICA) (73%), narrowing or occlusion of the supraclinoid ICA (87%) and proximal middle cerebral artery (MCA) (91%), and multiple collateral vessels in the basal ganglia and/or thalamus (96%). The parenchymal abnormalities included ischemic infarctions (74%), predominantly located in watershed areas, hemorrhagic infarctions (26%), intracerebral hematomas (13%), and intraventricular hemorrhage (13%). In conclusion, MR imaging was a useful diagnostic modality for detecting both vascular and parenchymal abnormalities associated with moyamoya disease. This may obviate the need for invasive angiography as far as the diagnosis is wanted at the non-quantitative level.
Adolescent ; Adult ; Basal Ganglia/pathology/radiography ; Carotid Artery, Internal/pathology/radiography ; Cerebral Angiography ; Child ; Female ; Hematoma/complications/diagnosis/pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Moyamoya Disease/complications/*diagnosis/pathology ; Myocardial Infarction/complications/diagnosis/pathology

Progressive narrowing of distal carotid arteries and the development of compensatory fine networks are the characteristic findings of moyamoya disease. Cerebral infarction in moyamoya disease is due to a decreased blood flow and shows an uneven distribution in the distal bed of the anterior and middle cerebral arteries. The progression of disease in the posterior circulation follows that in the anterior circulation. Posterior circulation symptoms due to cerebral infarction usually occur in the advanced stage of the disease and follow the anterior circulation symptoms. We encountered an unusual case of moyamoya disease which initially presented with a transient visual field defect. One month later our patient developed blindness and her cerebral angiography showed advanced moyamoya disease.
Adult ; Blindness/etiology ; Case Report ; Cerebral Angiography ; Female ; Human ; Magnetic Resonance Imaging ; Moyamoya Disease/*complications/*diagnosis ; Tomography, Emission-Computed, Single-Photon ; Vision Disorders/*etiology ; *Visual Fields

OBJECTIVETo study the clinical manifestations and neuroimaging characteristics of pediatric moyamoya disease.

METHODSThe clinical data of 17 children with moyamoya disease were retrospectively studied.

RESULTSThe onset age was between 3 and 14 years. The main clinical manifestations included motor weakness of extremities or hemiplegia, sensory disturbance and headache. Cranial CT or/and MRI examinations predominately showed cerebral infarct. Magnetic resonance angiography (MRA) and digital subtraction angiography (DSA) showed stenosis or occlusion at the terminus of the siphon portions of internal carotid arteries and proximal portions of anterior or middle cerebral arteries, and abnormal vascular networks at the base of brain.

CONCLUSIONSCerebral ischemia is main clinical manifestations in children with moyamoya disease, presenting motor weakness of extremities or hemiplegia, sensory disturbance and headache. DSA is essential to the diagnosis of the disease.

Adolescent ; Angiography, Digital Subtraction ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Moyamoya Disease ; complications ; diagnosis ; therapy ; Tomography, X-Ray Computed

PURPOSE: Moyamoya disease (MMD) occurs predominantly in Korean and Japanese women. The aim of this study was to investigate clinical features and pregnancy outcomes in women with MMD. MATERIALS AND METHODS: We conducted a retrospective chart review of women with MMD who visited our Department of Obstetrics and Gynecology between January 2005 and October 2013. For all study subjects, clinical features, demographic characteristics, and perinatal outcomes were recorded. RESULTS: We identified 28 pregnancies in 22 patients who had been diagnosed with MMD. The mean maternal age at delivery was 31.9+/-3.5 years old. The mean gestational age at delivery was 38.0+/-0.9 weeks. Among the 28 pregnancies, 25 (92.5%) underwent cesarean section; 19 (76.0%) of them were performed under regional anesthesia and six (24.0%) under general anesthesia. The mean newborn weight was 3233.7+/-348.2 g. The 5-minute Apgar score in 85% of the newborns was higher than 8, with no other apparent complications. During the puerperal period, transient ischemic attack symptom or seizure occurred in 4 cases, although patients recovered within a few days. CONCLUSION: For pregnant women with MMD, it is important to control blood pressure and prevent hyperventilation during the intrapartum period, and the best methods of delivery and anesthesia should be considered to avoid unfavorable sequelae. Additionally, a multidisciplinary approach (i.e., neurosurgery) is necessary to constantly manage underlying diseases.
Adult ; Blood Pressure/physiology ; Cesarean Section ; Female ; Gestational Age ; Humans ; Infant, Newborn ; Maternal Age ; Moyamoya Disease/*diagnosis/epidemiology ; Pregnancy ; *Pregnancy Complications ; Pregnancy Outcome/*epidemiology ; Republic of Korea/epidemiology ; Retrospective Studies