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

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

Adolescent ; Cerebellum ; diagnostic imaging ; pathology ; Cerebral Angiography ; Female ; Humans ; Intracranial Arteriovenous Malformations ; complications ; diagnostic imaging ; pathology ; Moyamoya Disease ; complications ; diagnostic imaging ; pathology

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

PURPOSE: Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance of ivy signs and whether they correlate with old brain lesions and the severity of clinical symptoms in patients with MMD. MATERIALS AND METHODS: FLAIR images of 83 patients were reviewed. Each cerebral hemisphere was divided into 4 regions and each region was scored based on the prominence of the ivy sign. Total ivy score (TIS) was defined as the sum of the scores from the eight regions and dominant hemispheric ivy sign (DHI) was determined by comparing the ivy scores from each hemisphere. According to the degree of ischemic symptoms, patients were classified into four subgroups: 1) nonspecific symptoms without motor weakness, 2) single transient ischemic attack (TIA), 3) recurrent TIA, or 4) complete stroke. RESULTS: TIS was significantly different as follows: 4.86+/-2.55 in patients with nonspecific symptoms, 5.89+/-3.10 in patients with single TIA, 9.60+/-3.98 in patients with recurrent TIA and 8.37+/-3.39 in patients with complete stroke (p=0.003). TIS associated with old lesions was significantly higher than those not associated with old lesions (9.35+/-4.22 vs. 7.49+/-3.37, p=0.032). We found a significant correlation between DHI and motor symptoms (p=0.001). CONCLUSION: Because TIS has a strong tendency with severity of ischemic motor symptom and the presence of old lesions, the ivy sign may be useful in predicting severity of disease progression.
Adolescent ; Adult ; Aged ; Brain/metabolism/*pathology ; Cerebral Arteries/*pathology ; Child ; Child, Preschool ; Collateral Circulation ; Disease Progression ; Female ; Humans ; Magnetic Resonance Imaging/*methods ; Male ; Meninges/*pathology ; Middle Aged ; Moyamoya Disease/complications/*pathology ; Severity of Illness Index ; Stroke ; Young Adult