[Monitoring and Neurocritical Care of Severe Traumatic Brain Injury].

The main objectives of critical care of severe traumatic brain injury(TBI)are the prevention and treatment of intracranial hypertension and secondary brain insults, preservation of cerebral perfusion pressure, and optimization of cerebral oxygenation. The critical care management of severe TBI will be discussed with a focus on the monitoring and avoidance or minimization of secondary brain insults, with emphasis on understanding the underlying physiology and pathophysiology. The evolution of critical care of severe traumatic brain injury will also be discussed along with the accumulating knowledge and experience.

Application of neuro-endoscopic target aspiration of the necrotic core for cerebral contusion with delayed progression: technical note.

BACKGROUND: The optimal management strategy for cerebral contusion remains controversial, especially when standard craniotomy could not be used. We performed neuro-endoscopic target lesionectomy for the delayed progression of cerebral contusion in order to aspirate the necrotic core, which is the primal source of contusional edema. METHODS: The present study included 10 consecutive patients (2 women and 8 men, with a mean age of 67 years old) with traumatic brain injury presenting with delayed deterioration of cerebral contusion where standard craniotomy could not be used. Neuro-endoscopic aspiration of the necrotic core was prospectively performed for all patients. We assessed the computed tomography findings after surgery to evaluate the efficacy of this procedure. RESULTS: Endoscopic surgery was performed promptly after neurological deterioration, with a mean interval between trauma and surgery of 7 days, ranging from 2 to 16 days. During the operation, the centers of contusions comprised serous liquid components in all 10 patients and were easily aspirated by endoscopy. Contusional edemas were markedly decreased in all within 3 days after surgery. CONCLUSIONS: Progression of contusional edema can be caused by the accumulation of water into the necrotic core due to the rapid increase in osmolality. In light of the highly liquefied demarcated characteristics of the necrotic core, neuro-endoscopic aspiration could be an optional treatment strategy for cerebral contusion with delayed progression in a minimally invasive manner.

Case Report: Vertebro-vertebral arteriovenous fistula showing symptoms mimicking ALS: Diagnostic imaging supports accurate differentiation between ALS and mimicking conditions.

We report a rare case of a vertebro-vertebral arteriovenous fistula (VVAVF) manifesting as amyotrophic lateral sclerosis (ALS). A 76-year-old female patient presented with progressive weakness, muscle atrophy, fasciculation, and preserved deep tendon reflexes in the right upper limb. Electrophysiological testing showed lower motor neuron dysfunction. The patient was suspected to have ALS, but cervical magnetic resonance imaging (MRI) revealed enlarged blood vessels in the spinal canal, which compressed the cervical spinal cord and nerve roots. Angiography showed a shunt from the right vertebral artery to the right intervertebral vein and the vertebral venous plexus; therefore, the patient was diagnosed with VVAVF. Transarterial embolization was performed to obliterate the shunt, and weakness in the patient's right upper limb subsequently improved. It is worth considering VVAVF as a differential diagnosis of ALS-like diseases.

A Case of Telangiectatic Osteosarcoma in the Frontal Bone.

Telangiectatic osteosarcoma (TOS) is a subtype of osteosarcoma. TOS in the elderly and TOS in the skull are very rare. Here, we report a case of TOS in the frontal bone of an elderly patient. The patient was a 79-year-old woman who was identified as having a right frontal bone lesion. The patient was initially diagnosed with an intradiploic epidermoid cyst (IEC). A 60mm cystic lesion with bone destruction appeared 5 years later, which enlarged over 2 months. The fluid in the cyst seemed to be blood that had lost its clotting ability. Tumor and the surrounding tissue were resected. The postoperative course was favorable. Postoperative magnetic resonance imaging (MRI) showed no evidence of residual lesions. There was no metastases. Histopathologically, the cyst wall was composed of fibrous connective tissue and did not contain epithelial components. There were no skin appendages and keratinized tissues in the lesion. Based on these findings, the diagnosis changed to aneurysmal bone cyst (ABC). Subsequent immunohistochemical examinations confirmed that the MIB-1 index was 50% in some sections and there were atypical cells showing osteogenic properties in other sections. Based on these results, the patient was finally diagnosed with TOS. The differential diagnoses for cystic lesion presenting bone destruction include TOS, ABC, and IEC. In case of cystic lesion with bone destruction, early diagnosis based on histopathological study is important and complete resection with surrounding tissues is required, given the possibility of TOS.

Trans-Cell Technique through Mesh of Pipeline Embolization Device: A Case Report.

Objective: We report a case of coil embolization using trans-cell technique through mesh of a pipeline embolization device (PED). Case Presentation: A 55-year-old female developed a left cavernous carotid aneurysm (CCA) with left abducens nerve palsy. The abducens nerve palsy improved gradually after PED deployment for the aneurysm. Sixty-nine days after the procedure, the patient suddenly presented with a severe headache, left abducens nerve palsy, left eyelid edema, and left pulsatile tinnitus. Digital subtraction angiography (DSA) revealed left direct carotid cavernous fistula (dCCF) due to rupture of the aneurysm, and the patient underwent endovascular treatment. A Marathon was guided into the left internal carotid artery, and a guidewire via the Marathon passed through the mesh of the PED. Then the Marathon advanced over the guidewire into the aneurysm through the mesh of the PED, with assistance of a distal access catheter and a balloon catheter. Transarterial intra-aneurysmal coil embolization using trans-cell technique was performed, and the shunt blood flow was diminished. After subsequent transvenous embolization (TVE), the shunt blood flow disappeared, and all neurological symptoms improved. When PED is deployed linearly at a diameter 0.5 mm smaller than the nominal diameter, the average strand spacing is calculated to be approximately 0.2 mm. Since PED is a braided stent, the spacing can be large. It is theoretically reasonable for Marathon with an outer diameter of 0.59 mm to pass through the mesh of the PED. Conclusion: In some cases, trans-cell technique through mesh of PED can be performed using a small diameter microcatheter.

Digital intravascular pressure wave recording during endovascular treatment reveals abnormal shunting flow in vertebral venous fistula of the vertebral artery: illustrative case.

BACKGROUND: An arteriovenous fistula is an abnormal arteriovenous shunt between an artery and a vein, which often leads to venous congestion in the central nervous system. The blood flow near the fistula is different from normal artery flow. A novel method to detect the abnormal shunting flow or pressure near the fistula is needed. OBSERVATIONS: A 76-year-old woman presented to the authors' institute with progressive right upper limb weakness. Right vertebral angiography showed a fistula between the right extracranial vertebral artery (VA) and the right vertebral venous plexus at the C7 level. The patient underwent endovascular treatment for shunt flow reduction. Before the procedure, blood pressures were measured at the proximal VA, distal VA near the fistula, and just at the fistula and drainer using a microcatheter. The blood pressure waveforms were characteristically different in terms of resistance index, half-decay time, and appearance of dicrotic notch. The fistula was embolized with coils and N-butyl cyanoacrylate solution. LESSONS: During endovascular treatment, the authors were able to digitally record the vascular pressure waveform from the tip of the microcatheter and succeeded in calculating several parameters that characterize the shunting flow. Furthermore, these parameters could help recognize the abnormal blood flow, allowing a safer endovascular surgery.

Ruptured Basilar Artery Dissection Diagnosed Using Magnetic Resonance Vessel Wall Imaging and Treated with Coil Embolization with Overlapping LVIS Stents: A Case Report.

The diagnosis and treatment of patients with ruptured basilar artery dissection (rBAD) are often difficult. We present a case of rBAD diagnosed with magnetic resonance vessel wall imaging (MR-VWI) and treated with coil embolization with overlapping low-profile visualized intraluminal support (LVIS) stents. The case is of a 49-year-old woman with subarachnoid hemorrhage. digital subtraction angiography (DSA) showed irregularity in an anterior wall of the middle portion of the basilar artery, indicating the presence of a false lumen. MR-VWI showed local enhancement in an arterial wall, which was consistent with the wall irregularity observed in DSA. Overlapping stents (two LVIS stents) was performed in the basilar artery and coils were placed in the false lumen. The false lumen was completely thrombosed, and anterograde blood flow of the basilar artery was preserved. Dual antiplatelet therapy was administered, and the patient underwent an uneventful postoperative course. DSA performed 6 months later showed a white-collar sign. MR-VWI has attracted attention as a useful modality for detecting a ruptured lesion in patients with subarachnoid hemorrhage. This is the first report, to the best of our knowledge, describing the practical use of MR-VWI for rBAD. MR-VWI is suggested to improve diagnostic accuracy for rBAD. There are no established treatments for rBAD; reconstructive endovascular treatments comprising stent placement and coil embolization of a false lumen are promising. The LVIS stent has a braided design and high metal coverage ratio and is considered to be reasonable for use in rBAD. Coil embolization of a false lumen with overlapping LVIS stents may be effective for rBAD.

[C2 neurinoma with intracranial extension: a case report].

We report a rare case presenting with a C2 neurinoma, which extended beyond the foramen magnum, producing a huge mass at the clivus. A 10-year-old boy with medical history of glycogenosis type VIII and attention-deficit hyperactivity disorder had been suffering from progressing foramen magnum syndrome for 36 months. MRI demonstrated a huge tumor, 70 mm in a diameter, which distributed in both the intra- and extra-spinal canal at the C2 level and extended into the intracranial space beyond the foramen magnum. The tumor was totally removed via the trascondylar fossa approach with C1 hemilaminectomy. Operative findings indicated that the tumor arising from extradural C2 nerve intruded into the intradural space, and extended beyond the foramen magnum. Foramen magnum tumors sometimes remain unrecognized until severe neurological deficits develop because of bizarre symptomatology. Surgical strategies with less invasiveness based on early diagnosis lead to successful recovery of neurological function.

Mechanical Thrombectomy for Acute Ischemic Stroke Arising from Thrombus of the Left Superior Pulmonary Vein Stump after Left Pneumonectomy: A Case Report.

Pulmonary vein thrombosis is a rare disease but can cause severe complications. However, pathophysiology of this entity is not fully understood. We report a case of mechanical thrombectomy for acute ischemic stroke arising from thrombus of the left superior pulmonary vein stump after left pneumonectomy. A 67-year-old male without atrial fibrillation presented with middle cerebral artery occlusion 11 days after left pneumonectomy. Contrast-enhanced computed tomography showed thrombus in the left superior pulmonary vein stump. He received mechanical thrombectomy and had a good postoperative course. The retrieved embolus was macroscopically red thrombus and histological examination revealed that the embolus consisted of fibrin and erythrocyte-rich thrombus. Thrombus formation in a pulmonary vein stump after lung resection may be an embolic source of AIS. Mechanical thrombectomy and anticoagulation is effective for the treatment of this pathology.

Spontaneous Development of Encapsulated Subdural Hematoma in the Posterior Cranial Fossa after Cardiac Surgery: A Case Report.

We report a case of an encapsulated subdural hematoma in the posterior cranial fossa, showing spontaneous development and rapid increase in size after cardiac surgery. An 86-year-old woman underwent aortic valve replacement for aortic valve stenosis, followed by anticoagulant therapy with heparin. Three days after the cardiac surgery, she complained of headache. Computed tomography revealed development of a subdural hematoma in the posterior cranial fossa. The hematoma rapidly increased in size within 7 days. Eleven days after cardiac surgery, she underwent removal of the subdural hematoma by craniotomy. Intraoperatively, the subdural hematoma was covered by a thick granulomatous capsule, with histopathological findings similar to those of a chronic subdural hematoma. She was discharged 2 weeks after the craniotomy without any neurological deficits. Encapsulated subdural hematoma in the posterior cranial fossa is rare and its etiology is unknown. In this case, postoperative anticoagulant therapy can promote the rapid growth of thick hematoma capsule. It is possible that previously reported cases of "posterior fossa chronic subdural hematoma" contain similar lesions to that in our patient.

Increased vascular MMP-9 in mice lacking RNF213: moyamoya disease susceptibility gene.

Moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease with unknown etiology. Recent genetic studies have identified RNF213 as an important susceptibility gene for MMD. To evaluate the role of RNF213 in vascular remodeling, RNF213 knockout mice (RNF213-/-) and their wild-type littermates (WT) were subjected to common carotid artery ligation to induce vascular hyperplasia. We examined the vascular expression of matrix metalloproteinase (MMP)-9, known to be increased in MMD. MMP-9 expression was significantly higher in RNF213-/- mice than in wild-type mice 1 and 7 days after common carotid artery ligation. The vascular wall was significantly thinner in RNF213-/- mice at 14 days. The increased vascular expression of MMP-9 and subsequent vascular wall thinning in RNF213-/- mice could reflect the early characteristic of MMD, consistent with the recently proposed constrictive remodeling theory.

Genetics and Biomarkers of Moyamoya Disease: Significance of RNF213 as a Susceptibility Gene.

Moyamoya disease is characterized by a progressive stenosis at the terminal portion of the internal carotid artery and an abnormal vascular network at the base of the brain. Although its etiology is still unknown, recent genome-wide and locus-specific association studies identified RNF213 as an important susceptibility gene of moyamoya disease among East Asian population. A polymorphism in c.14576G>A in RNF213 was identified in 95% of familial patients with moyamoya disease and 79% of sporadic cases, and patients having this polymorphism were found to have significantly earlier disease onset and a more severe form of moyamoya disease, such as the presentation of cerebral infarction and posterior cerebral artery stenosis. The exact mechanism by which the RNF213 abnormality relates to moyamoya disease remains unknown, while recent reports using genetically engineered mice lacking RNF213 by homologous recombination provide new insight for the pathogenesis of this rare entity. Regarding biomarkers of moyamoya disease, moyamoya disease is characterized by an increased expression of angiogenic factors and pro-inflammatory molecules such as vascular endothelial growth factors and matrix metalloproteinase-9, which may partly explain its clinical manifestations of the pathologic angiogenesis, spontaneous hemorrhage, and higher incidence of cerebral hyperperfusion after revascularization surgery. More recently, blockade of these pro-inflammatory molecules during perioperative period is attempted to reduce the potential risk of surgical complication including cerebral hyperperfusion syndrome. In this review article, we focus on the genetics and biomarkers of moyamoya disease, and sought to discuss their clinical implication.

Temporal profile of the vascular anatomy evaluated by 9.4-T magnetic resonance angiography and histopathological analysis in mice lacking RNF213: a susceptibility gene for moyamoya disease.

Moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease with unknown etiology. Recent genome-wide and locus-specific association studies identified RNF213 as an important MMD susceptibility gene. However, the exact mechanism by which an abnormality in RNF213 leads to MMD is unknown. To evaluate the role of RNF213 in the etiology of MMD, we generated RNF213-deficient mice (RNF213-/-) by deleting exon 32 of RNF213 by the Cre-lox system, and investigated whether they developed MMD. The temporal profile of cervical/intracranial arteries was evaluated by 9.4-T magnetic resonance angiography (MRA). The anatomy of the circle of Willis was analyzed by a trans-cardiac injection of carbon black dye. The common carotid arteries (CCA) were sectioned and the arterial wall thickness/thinness was evaluated by Elastica-Masson staining before and after CCA ligation, which selectively induced vascular hyperplasia. As a result, RNF213-/- grew normally, and no significant difference was observed in MRA findings, the anatomy of the circle of Willis, or vascular wall thickness/thinness between RNF-/- and wild-type littermates (Wt.) under normal conditions until 64 weeks of age. However, Elastica-Masson staining demonstrated that both the intima and medial layer were significantly thinner after CCA ligation in RNF213-/- than in Wt. after 14 days (P<0.01). In conclusion, mice lacking the RNF213 gene did not spontaneously develop MMD, indicating that a functional loss of RNF213 did not sufficiently induce MMD. Suppression of vascular remodeling in RNF213-/- requires further examination to clarify the role of RNF213.

Subarachnoid hemorrhage due to ruptured posterior cerebral artery aneurysm simultaneously associated with multiple remote intracerebral hemorrhages--case report.

A 61-year-old woman presented with aneurysmal subarachnoid hemorrhage (SAH) associated with multiple remote intracerebral hemorrhages (ICHs). She had undergone microsurgical neck clipping for ruptured right middle cerebral artery aneurysm and ventriculo-peritoneal shunting 16 years previously. Computed tomography revealed SAH predominantly in the basal cistern and ambient cistern, in association with multiple ICHs in the bilateral tegmentum of the brain stem and right caudate nucleus. Digital subtraction angiography revealed an aneurysm at the P(1) segment of the left posterior cerebral artery. The ruptured aneurysm was microsurgically clipped via a left subtemporal approach without complications. Simultaneous occurrence of aneurysmal SAH and multiple remote ICHs is rare. The spatial pattern of the ICHs in the present case is apparently unique.

Evidence for antagonistic regulation of cell growth by the calcineurin and high osmolarity glycerol pathways in Saccharomyces cerevisiae.

Because Ca(2+) signaling of budding yeast, through the activation of calcineurin and the Mpk1/Slt2 mitogen-activated protein kinase cascade, performs redundant function(s) in the events essential for growth, the simultaneous deletion of both these pathways (Delta cnb1 Delta mpk1) leads to lethality. A PTC4 cDNA that encodes a protein phosphatase belonging to the PP2C family was obtained as a high dosage suppressor of the lethality of Delta cnb1 Delta mpk1 strain. Overexpression of PTC4 led to a decrease in the high osmolarity-induced Hog1 phosphorylation, and HOG1 deletion remarkably suppressed the synthetic lethality, indicating an antagonistic role of the high osmolarity glycerol (HOG) pathway and the Ca(2+) signaling pathway in growth regulation. The calcineurin-Crz1 pathway was required for the down-regulation of the HOG pathway. Analysis of the time course of actin polarization, bud formation, and the onset of mitosis in synchronous cell cultures demonstrated that calcineurin negatively regulates actin polarization at the bud site, whereas the HOG pathway positively regulates bud formation at a later step after actin has polarized.