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Development of multiple cerebral arteriovenous malformations documented in an adult by serial angiography

Case report

Yoshio Miyasaka, Kuniaki Nakahara, Hiroshi Takagi, and Hiroyuki Hagiwara

✓ A 50-year-old woman with a parietal intracerebral hematoma was initially treated by hematoma evacuation. Initial preoperative and follow-up angiograms obtained 6 months later demonstrated no pial arteriovenous malformations (AVMs). She suffered a subarachnoid hemorrhage 8 years later. Results of follow-up cerebral angiography revealed the development of previously undetected multiple cerebral AVMs. This appears to be the first reported case of the development of multiple cerebral AVMs in an adult, demonstrated on serial angiography.

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Cranial burr hole for revascularization in moyamoya disease

Masataka Endo, Nobuyuki Kawano, Yoshio Miyasaka, and Kenzoh Yada

✓ Currently, superficial temporal artery-middle cerebral artery (MCA) anastomosis, encephalomyosynangiosis (EMS), and encephalo-duro-arterio-synangiosis are used to treat moyamoya disease and are reported to effectively improve ischemic symptoms. All are methods of reversing the flow of blood from the external carotid artery system into the cortical branches of the MCA. As moyamoya disease advances, these operations alone will predictably not correct the deterioration in blood flow in the territory of the anterior cerebral artery. It was noted in a case of moyamoya disease with intraventricular hemorrhage that a burr hole, made in the frontal region for drainage purposes, induced marked neovascularization. Since then, similar frontal burr holes have been made in five juvenile cases of moyamoya disease; this procedure involved making a burr hole in both frontal bones and incising both the dura and the arachnoid membrane. In two cases a frontal burr hole was placed simultaneously with EMS, and in the others the frontal burr hole was made following EMS. The clinical symptoms improved after the frontal burr hole was made, and dynamic computerized tomography revealed improved circulation in the frontal regions. Together with conventional surgical therapy for juvenile cases of moyamoya disease, this operation is considered beneficial both to the circulation in the frontal region and for the protection of frontal brain function.

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Diffuse cerebrospinal gliomatosis

Case report

Nobuyuki Kawano, Yoshio Miyasaka, Kenzoh Yada, Hideo Atari, and Kenichi Sasaki

✓ A case of diffuse cerebrospinal gliomatosis is presented, with widespread involvement of the brain, cranial nerves, and spinal cord. This case showed a far more extensive distribution of tumor cells than previously reported cases of gliomatosis cerebri. The clinical picture and oncogenesis of gliomatosis cerebri is briefly discussed.

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Intracranial vertebral artery dissection with subarachnoid hemorrhage: clinical characteristics and outcomes in conservatively treated patients

Masaru Yamada, Takao Kitahara, Akira Kurata, Kiyotaka Fujii, and Yoshio Miyasaka

Object. Intracranial vertebral artery (VA) dissection with subarachnoid hemorrhage is notorious for frequent rebleeding and a poor prognosis. Nevertheless, some patients survive with a good final outcome. The factors associated with the prognosis of this disease are not fully understood and appropriate treatment strategies continue to be debated. The authors retrospectively evaluated the clinical features of conservatively treated patients to elucidate the relationship between the clinical and angiographic characteristics of the disease and final outcomes.

Methods. This study includes 24 patients who were treated by conservative methods between 1990 and 2000. Conservative treatment was chosen because of delayed diagnosis, poor clinical condition, or anatomical features such as bilateral lesions and contralateral VA hypoplasia.

Of nine patients with an admission Hunt and Kosnik Grade I or II, eight had good outcomes (mean follow-up period 8 years and 4 months). All 15 patients with Grade III, IV, or V died and in 10 of these the cause of death was rebleeding. Among the 24 patients, 14 suffered a total of 35 rebleeding episodes; in 10 (71.4%) of these 14 patients rebleeding occurred within 6 hours and in 13 (93%) within 24 hours. Compared with the survivors, there was a female preponderance (0.022) among patients who died. These patients also had significantly shorter intervals between onset and hospital admission (p = 0.0067), a higher admission Hunt and Kosnik grade (p = 0.0001), a higher incidence of prehospitalization (p = 0.0296) and postadmission (p = 0.0029) rebleeding episodes, and a higher incidence of angiographically confirmed pearl-and-string structure of the lesion (p = 0.0049).

Conclusions. In our series of preselected patients, poor admission neurological grade, rebleeding episode(s), and lesions with a pearl-and-string structure were predictive of poor outcomes. Our findings indicate that patients with these characteristics may be candidates for aggressive attempts to prevent rebleeding during the acute stage. Patients without these characteristics may be good candidates for conservative treatment, especially those who survive the acute phase without rebleeding.

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Successful stent placement for cervical artery dissection associated with the Ehlers—Danlos syndrome

Case report and review of the literature

Akira Kurata, Hidehiro Oka, Taketomo Ohmomo, Hitoshi Ozawa, Sachio Suzuki, Kiyotaka Fujii, Shinichi Kan, Yoshio Miyasaka, and Harue Arai

✓ This 44-year-old man with Ehlers—Danlos syndrome (EDS) Type IV presented with hemiparesis and the Gerstmann syndrome. Left carotid artery (CA) angiography revealed a dissecting aneurysm with severe stenosis located in the common CA; the lesion was successfully treated with a stent graft. The patient's clinical course after endovascular surgery was uneventful, without occurrence of megacolon. The literature for spontaneous CA dissection in EDS Type IV cases is reviewed and points for investigation and treatment are discussed.

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Venous ischemia caused by dural arteriovenous malformation

Case report

Akira Kurata, Yoshio Miyasaka, Takatomo Yoshida, Masatake Kunh, Kenzoh Yada, and Shinichi Kan

✓ A case is presented of tentorial dural arteriovenous malformation (AVM) associated with visual hallucinations and quadrant hemianopsia. Computerized tomography (CT) and magnetic resonance imaging showed an ischemic region, mainly affecting the white matter of the right occipital lobe, that was defined as an area of increased blood volume on dynamic CT scans and as a decrease in cerebral blood flow on N-isopropylp-123I-iodoamphetamine single-photon emission CT scans. Angiography demonstrated venous congestion, probably because the retrograde arterial inflow from the dural AVM into the corticomedullary vein was direct and not via the sinuses. The symptoms and radiological findings improved immediately after endovascular treatment. The origin of these symptoms was fully evaluated and confirmed to be a reversible ischemic change caused by disturbance of the volume of venous return over an extensive area.

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An analysis of the venous drainage system as a factor in hemorrhage from arteriovenous malformations

Yoshio Miyasaka, Kenzoh Yada, Takashi Ohwada, Takao Kitahara, Akira Kurata, and Katsumi Irikura

✓ The authors studied the venous drainage system and its impairment in relation to risk of hemorrhage in 108 cases of supratentorial arteriovenous malformation (AVM). The proportion of AVM's undergoing hemorrhage (hemorrhagic rate) was calculated in relation to: 1) the number of draining veins (one, two, or three or more); 2) the presence or absence of impairment in venous drainage (severe stenosis or occlusion in draining veins); and 3) the location of draining veins (deep venous drainage alone, superficial venous drainage alone, or a combination of the two). Statistical analysis demonstrated that AVM's with the following characteristics had a high risk of hemorrhage: 1) one draining vein (hemorrhagic rate 89% in 54 patients); 2) severely impaired venous drainage (hemorrhagic rate 94% in 18 patients); and 3) deep venous drainage alone (hemorrhagic rate 94% in 32 patients).

The present study suggests that the venous drainage system of AVM's is significantly associated with the risk of hemorrhage of these lesions. Therefore, careful preoperative angiographic evaluation of the venous drainage system is mandatory for decision making in the management of patients with AVM's.

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Retrograde thrombosis of feeding arteries after removal of arteriovenous malformations

Yoshio Miyasaka, Kenzoh Yada, Takashi Ohwada, Takao Kitahara, Masataka Endoh, Motoyoshi Saito, Akira Kurata, and Hirotoshi Ohtaka

✓ Five cases of retrograde thrombosis of former feeding arteries after removal of an arteriovenous malformation (AVM) are reported. The clinical features of these patients were studied and compared to those of 71 patients without this complication. The following characteristics were found to correlate with retrograde thrombosis: 1) advancing age of the patient; 2) large AVM size; and 3) markedly dilated and elongated feeders. It is suggested that the slow flow in the former feeding arteries that was observed immediately after AVM removal and pathological changes in these vessels due to long-standing hemodynamic stresses contributed to the development of retrograde thrombosis. Neurological manifestations related to retrograde thrombosis were noted in three of the five cases. Although infrequent, this complication should be considered as a serious possibility following removal of an AVM.

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Venous Drainage System and Risk of Hemorrhage from AVM's

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Venous Drainage in Hemorrhage from AVM's