p.Arg332Cys mutation of NOTCH3 gene in two unrelated Japanese families with CADASIL.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy is a cerebrovasuclar disease caused by NOTCH3 mutations, usually localized to exons 3 and 4. This report describes the clinical and neuroradiological findings of 2 subjects of two unrelated Japanese families who shared a common p.Arg332Cys mutation.

Recovered neuronal viability revealed by Iodine-123-iomazenil SPECT following traumatic brain injury.

We evaluated cortical damages following traumatic brain injury (TBI) in the acute phase with [(123)I] iomazenil (IMZ) single photon emission computed tomography (SPECT). In all, 12 patients with cerebral contusion following TBI were recruited. All patients underwent IMZ SPECT within 1 week after TBI.

Operative open intra-aneurysmal thrombectomy for ruptured cerebral aneurysm and peripheral ischemia associated with vasospasm.

The endovascular approach has become the standard treatment for ruptured aneurysms during the vasospasm risk period following subarachnoid hemorrhage; however, it may be disadvantageous under certain conditions. We report a patient with a ruptured middle cerebral artery aneurysm with severe vasospasm and thrombosis within the aneurysm immediately after angiography. Emergent operative open thrombectomy of the intra-aneurysmal thrombus restored blood flow to the ischemic penumbra territory demonstrated by single photon emission CT scan and diffusion-weighted MRI.

Cerebrovascular moyamoya disease in a 5-year-old girl who underwent interruption of the right common carotid artery in the neonatal period.

The authors report on the case of a girl with cerebrovascular moyamoya disease born with severe respiratory failure caused by a congenital diaphragmatic hernia. Cardiopulmonary management included extracorporeal membrane oxygenation until the diaphragm defect was repaired. The right common carotid artery (CA) was interrupted and cannulated for extracorporeal membrane oxygenation.

Inhibitory effects of eicosapentaenoic acid on chronic cerebral vasospasm after subarachnoid hemorrhage: possible involvement of a sphingosylphosphorylcholine-rho-kinase pathway.

Background And Purpose: Rho-kinase (ROK)-mediated Ca2+ sensitization of vascular smooth muscle (VSM) contraction plays a pivotal role in cerebral vasospasm (CV). We previously demonstrated that sphingosylphosphorylcholine (SPC) induces Ca2+ sensitization through sequential activation of the Src family protein tyrosine kinases (Src-PTKs) and ROK in vitro, and that Ca2+ sensitization is inhibited by eicosapentaenoic acid (EPA) through the selective inactivation of Src-PTK. In this study, we examined whether SPC induced CV in vivo, and, if it did, whether EPA would inhibit CV, as induced by SPC or in an in vivo model of subarachnoid hemorrhage (SAH).

De novo aneurysm formation on middle cerebral artery branches adjacent to the anastomotic site of superficial temporal artery-middle cerebral artery bypass surgery in two patients: technical case report.

Objective: Aneurysm formation is a complication of superficial temporal artery-middle cerebral artery bypass surgery occurring as pseudoaneurysms caused by technical failure, but also as true aneurysms discovered after long-term follow-up.

Clinical Presentation: A 53-year-old woman presented with a left internal carotid artery cavernous aneurysm manifesting as double vision. Superficial temporal artery-middle cerebral artery bypass, internal trapping of the internal carotid artery, and embolization were performed.

Sphingosylphosphorylcholine is a novel messenger for Rho-kinase-mediated Ca2+ sensitization in the bovine cerebral artery: unimportant role for protein kinase C.

Although recent investigations have suggested that a Rho-kinase-mediated Ca2+ sensitization of vascular smooth muscle contraction plays a critical role in the pathogenesis of cerebral and coronary vasospasm, the upstream of this signal transduction has not been elucidated. In addition, the involvement of protein kinase C (PKC) may also be related to cerebral vasospasm. We recently reported that sphingosylphosphorylcholine (SPC), a sphingolipid, induces Rho-kinase-mediated Ca2+ sensitization in pig coronary arteries.