1/19. moyamoya disease of adult onset brain stem haemorrhage associated with bilateral occlusion of the vertebral arteries--case report.An unusual and first case of moyamoya disease of adult onset brain stem haemorrhage associated with occlusion of both vertebral arteries is reported. A 30-year-old man suddenly suffered from dyspnea, dysphagia, and left-sided hemisensory disturbance. Computed tomography and magnetic resonance imaging revealed a fresh haematoma in the left medulla oblongata and various-sized old infarcts in both parietal lobes. Cerebral angiograms disclosed occlusion of the bilateral internal carotid arteries on both sides at their intracranial portion, accompanied with the developed basal moyamoya vessels. The right vertebral artery occluded at its V2-V3 segment, in which the posterior inferior cerebellar artery was opacified via the posterior spinal artery, and the basilar artery was filled from the anterior spinal artery. The left vertebral artery was also occluded at the craniovertebral junction (V4) with collateral flow. Only one case of moyamoya disease associated with bilateral occlusion of the vertebral artery has been reported previously, and a haemorrhage into the medulla oblongata in moyamoya disease has never been described.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/19. association of cerebral arteriovenous malformations and spontaneous occlusion of major feeding arteries: clinical and therapeutic implications.OBJECTIVE: The spontaneous occlusion of a cerebral arteriovenous malformation (AVM) occurs rarely. Occlusion of a parent artery feeding the AVM is even more rare, and its incidence is unknown. We undertook this study to determine the incidence of occlusion of a major artery feeding an AVM and to recommend a management strategy for such an AVM. methods: We identified AVMs associated with an occluded artery by performing a retrospective angiographic analysis of 500 patients with AVMs who presented to Henry Ford Hospital from 1976 to 1998. RESULTS: A review of the angiograms revealed that 7 (1.4%) of 500 patients with an AVM had occlusion of one or more major arteries feeding the nidus. In four patients, an internal carotid artery and its bifurcation were occluded; in two patients, the M1 segment of the middle cerebral artery was occluded, and in one patient, a vertebral artery was occluded. Pial collaterals and/or a moyamoya pattern of anastomoses developed in all patients, with the exception of one who had vertebral artery occlusion. Five patients underwent definitive treatment: one received radiosurgery, and four underwent surgical excision. One of the surgically treated patients died of complications from excessive blood loss and coagulopathy, but the other three had no postoperative complications. CONCLUSION: The occlusion of a major artery feeding an AVM occurs rarely (1.4%). These AVMs are moderate to large in size (>3 cm). To prevent collateral flow-related complications of cortical "steal" and hemorrhage, as well as the usual risk of hemorrhage from the AVM itself, surgical management should be considered for these AVMs.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
3/19. Intraventricular aneurysms--three case reports.Three rare cases of purely intraventricular aneurysms are described, including a unique aneurysm in the fourth ventricle. A 30-year-old female, a 47-year-old male, and an 11-year-old girl presented with disturbance of consciousness due to massive intraventricular hemorrhage. Digital subtraction angiography revealed an idiopathic peripheral aneurysm in the fourth ventricle in the first patient, and aneurysms in the lateral ventricle associated with moyamoya disease in the latter two patients. The former two aneurysms were treated surgically and histologically confirmed to be pseudoaneurysms. The latter aneurysm disappeared spontaneously within 2 months after onset. The aneurysm in the lateral ventricle was resected via a parietal corticotomy with stereotactic insertion of an 8-Fr silicone tube to guide the approach route. This method was very useful because computerized neuronavigation was not available. The aneurysm in the fourth ventricle was resected via a midline suboccipital approach with C-1 laminectomy. Conservative treatment is usually recommended initially for patients with intraventricular aneurysm because spontaneous cure often occurs. We recommend direct surgery if the size of the aneurysm remains unchanged, because the risk of surgery has decreased recently owing to new techniques for neuronavigation.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
4/19. moyamoya disease associated with a brain stem glioma.An 8-year-old boy was found to have primary moyamoya disease associated with a brain stem glioma. For over 3 years the child had experienced transient ischemic attacks induced by hyperventilation. One month before referral to our hospital he had presented with progressive left facial nerve palsy. magnetic resonance imaging showed a cystic mass in the lower pons. Angiography revealed severe bilateral stenosis of the internal carotid arteries and prominent moyamoya vessels in the basal ganglia. Partial resection of the tumor yielded a histological diagnosis of pilocytic astrocytoma. Local radiation therapy reduced the size of the tumor. Anastomosis of the superficial temporal arteries and middle cerebral arteries on both sides was then performed. After direct bypass surgery, the patient remained in a good condition for a 5-year follow-up period. Clinical investigation of the coincidence of primary moyamoya disease and brain stem glioma led the authors to conclude that these two diseases coexisted independently.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
5/19. diffusion-weighted imaging in moyamoya disease.moyamoya disease is characterized by progressive intracranial vascular stenoses of the circle of willis, resulting in successive ischemic events. We report serial diffusion-weighted imaging studies in a case of moyamoya disease. A 4-year-old right-handed patient presented with multiple infarcts in the right and left hemispheres. Each new infarct was unambiguously recognized as bright on diffusion-weighted imaging. Previous infarcts, readily detected on other magnetic resonance imaging sequences, were not bright on diffusion-weighted imaging. The patient subsequently underwent bilateral synangiosis. In this case, the diffusion-weighted images were helpful in assessing the extent of infarcts, determining the age of the lesion, and correlation with new clinical findings. We emphasize the usefulness of diffusion-weighted imaging for following the clinical course of children with moyamoya disease, in whom new focal deficits are highly suspicious of new infarcts.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
6/19. Radiosurgical treatment of a cerebral arteriovenous malformation in a patient with moyamoya disease: case report.OBJECTIVE AND IMPORTANCE: A case of a cerebral arteriovenous malformation (AVM), which was treated with gamma knife surgery, in a patient with moyamoya disease is described. This is the first report of radiosurgical treatment of a cerebral AVM in a patient with moyamoya disease; the outcome was satisfactory. CLINICAL PRESENTATION: The patient presented with frequent episodes of transient ischemic attacks and seizures. INTERVENTION: Staged encephaloduroarteriosynangiosis was performed on both sides, with an interval of 10 months. Postoperative cerebral angiography demonstrated augmented cerebral perfusion, which increased the size of the AVM nidus. The patient subsequently underwent gamma knife surgery, and the AVM completely disappeared 2 years later. CONCLUSION: radiosurgery may be a good treatment option for AVMs accompanying moyamoya disease, allowing the preservation of collateral vessels and the prevention of possible misery perfusion.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
7/19. Obstruction of cerebral arteries in childhood stroke.middle cerebral artery obstruction in children is reviewed by our two cases. Ischemic childhood stroke was caused by moyamoya disease in the first, and by fibromuscular dysplasia in the second patient. In both cases transcranial Doppler sonography and cranial CT were performed, but the final diagnosis was made by angiography. The importance of angiography in childhood stroke is emphasized.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
8/19. Moyamoya-induced paroxysmal dyskinesia.moyamoya disease (MMD) is an uncommon intracranial vasculopathy that typically presents with ischemic or hemorrhagic stroke. Persistent choreoathetosis has been identified as a rare early manifestation of MMD. We present 2 patients with paroxysmal dyskinesia as the initial symptom of MMD, one resembling paroxysmal kinesigenic dyskinesia (PKD) and the other paroxysmal non-kinesigenic dyskinesia (PNKD). We also review the cases of moyamoya-induced chorea reported previously, none of which resembled PKD or PNKD. We hypothesize that both hormonal and ischemic factors may be implicated in the pathogenesis of these abnormal involuntary movements. These cases suggest that MMD should be included in the differential diagnosis of PKD and PNKD.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/19. Pathological and immunohistochemical findings of an autopsy case of adult moyamoya disease.moyamoya disease is vaso-occlusive disease involving the arteries of the circle of willis that is accompanied by a compensatory recruitment of a vascular network. The pathological and immunohistochemical findings of an autopsy case of hemorrhagic moyamoya disease in a 69-year-old woman are described in the present report. The autopsy findings of the brain revealed cerebral and intraventricular hemorrhage with edema. The left anterior cerebral artery, bilateral middle cerebral arteries and left posterior cerebral artery were marked narrowing, and the other arteries revealed mild narrowing. Microscopically, the arteries of the circle of willis showed narrowed lumen, fibrocellular intimal thickening, marked tortuousness of internal elastic lamina and attenuation of media. The thickened intima was composed of smooth muscle cells. The vessels with dilated or irregular-shaped lumen suggested abnormal vascular networks demonstrated by angiography. In this case, no correlation between the abnormal vascular network and expression of VEGF or VEGF receptor was disclosed. It was hypothesized that abnormal vascular networks might be composed of collateral vessels in relation to various pathological changes of the arteries, such as occlusion and stenosis, and intracranial hemorrhage in patients with moyamoya disease might occur as a result of rupture of arteries including abnormal vascular networks.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
10/19. Elastosis perforans serpiginosa-like pseudoxanthoma elasticum in a child with severe Moya Moya disease.A 2-year-old girl with Moya Moya disease who had relapsing cerebrovascular strokes presented with loose skin folds, 'chicken' skin appearance and perforating elastosis serpiginosa-like lesions in the genitocrural region. Histologically, calcified material perforating the epidermis and adjacent short curled and mineralized elastic fibres suggested a variant of pseudoxanthoma elasticum (PXE). As PXE is known to be caused by various mutations in the transmembrane transporter ABCC6 gene, we hypothesized that a novel ABCC6 mutation may underlie this unique combination of PXE and elastopathic vascular damage. Therefore, the complete ABCC6 coding region of the patient and her parents was screened for genetic alterations. No bona fide disease-causing mutation of ABCC6 could be found in the child and in her parents. However, two novel allelic amino acid substitutions (Arg1273Lys and Glu1293Lys; exon 27) were found in the girl and her father, localized in close proximity to the region that codes for the functionally critical second nucleotide-binding fold of ABCC6. Although a causal involvement of these amino acid substitutions could not be proven based on this study, both heterozygote substitutions may possibly have interacted with other undetected recessive maternal ABCC6 changes in the child. To the best of our knowledge, this is the first report of an association between early-onset PXE and severe Moya Moya syndrome possibly related to ABCC6 changes.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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