Ultrasonography monitoring with Superb Microvascular Imaging during cerebrovascular surgery.

INTRODUCTION: Ultrasonography (US) is used as a real-time dynamic imaging modality during neurosurgery. A novel Doppler US technique, Superb Microvascular Imaging (SMI), can be used to visualize low-velocity flow of small vessels at high resolution with high frame rates. We visualized vessel flow using this US SMI technique and contrast agent during cerebrovascular surgery. METHODS: Forty-three patients with an unruptured cerebral aneurysm (control), ischemic and hemorrhagic moyamoya disease, carotid artery stenosis, hemangioblastoma, severe stenosis of the middle cerebral artery, venous angioma, and intracerebral hemorrhage (ICH) underwent neurosurgery with US SMI monitoring using a contrast agent. The diameter, length, and number of penetrating vessels were analyzed in patients with an unruptured cerebral aneurysm (control), moyamoya disease, and ICH. RESULTS: Diameter and length of cerebral penetrating vessels were significantly increased in patients with moyamoya disease and ICH compared to control patients. The number of penetrating vessels was increased in moyamoya disease patients compared to control and ICH patients. In hemorrhagic moyamoya disease, flow in the penetrating vessels originated from a deep periventricular point and extended to the cerebral surface. Pulsatile cerebral aneurysms during clipping surgery and carotid artery stenosis during carotid endarterectomy were easily identified by SMI. Drastically increased vessel flow in patients with a hemangioblastoma or a venous angioma was observed. CONCLUSION: Using the US SMI technique and contrast agent, we obtained useful flow information of the vascular disease structure and intracerebral deep small vessels during cerebrovascular surgery. Further quantitative analysis will be informative and helpful for cerebrovascular surgery.

A case of a rosette-forming glioneuronal tumor with clinicopathological features of a dysembryoplastic neuroepithelial tumor and fibroblast growth factor receptor 1 internal tandem duplication.

Rosette-forming glioneuronal tumors (RGNTs) are benign WHO grade 1 tumors that occur in the ventricular system, particularly the fourth ventricle. RGNTs and dysembryoplastic neuroepithelial tumors (DNTs) are both categorized as neuronal and mixed neuronal-glial tumors and may be difficult to distinguish. Coexistence of the two tumor types has been reported. Here, we report a pediatric case of RGNT with DNT-like features showing intraventricular dissemination. The tumor occurred in the medial temporal lobe and presented with specific pathological glioneuronal elements including floating neurons, which are typical in DNTs, but was diagnosed as RGNT because of the presence of neurocytic rosettes. Genetic analysis detected fibroblast growth factor receptor 1 internal tandem duplication (FGFR1-ITD) of the tyrosine kinase domain, which was previously reported to be specific for DNT. RGNTs with FGFR1-ITD may show atypical clinical presentation and pathological features.

Nodular Pseudogout of the Skull Base Arising From the Temporomandibular Joint.

ABSTRACT: The authors report a case of a rare, large, nodular pseudogout which developed from the temporomandibular joint (TMJ).An 83-year-old female was referred to the hospital with swelling on her left cheek. Imaging studies revealed a large calcified mass surrounding the left mandibular condyle, partially destroying the adjoining bone and extending to the skull base. Magnetic resonance imaging (MRI) revealed soft tissue masses with low signal intensity on T1- and T2-weighted images and were enhanced after intravenous gadolinium injection.The mass was clinically and radiologically suspected to be a neoplastic lesion, such as chondrosarcoma or fibrous dysplasia. However, histological analysis showed that the mass contained granulomatous lesions with numerous multiple nodular rod-shaped and diamond-shaped crystal deposits, which validated the diagnosis of pseudogout. X-ray diffraction (XRD) was performed to identify the tumor's deposited materials and revealed that these were calcium pyrophosphate dihydrate (CPPD) crystals. This result confirmed the histopathological diagnosis of "tophaceous pseudogout."

An extracellular [NiFe] hydrogenase mediating iron corrosion is encoded in a genetically unstable genomic island in Methanococcus maripaludis.

Certain methanogens deteriorate steel surfaces through a process called microbiologically influenced corrosion (MIC). However, the mechanisms of MIC, whereby methanogens oxidize zerovalent iron (Fe0), are largely unknown. In this study, Fe0-corroding Methanococcus maripaludis strain OS7 and its derivative (strain OS7mut1) defective in Fe0-corroding activity were isolated. Genomic analysis of these strains demonstrated that the strain OS7mut1 contained a 12-kb chromosomal deletion. The deleted region, termed "MIC island", encoded the genes for the large and small subunits of a [NiFe] hydrogenase, the TatA/TatC genes necessary for the secretion of the [NiFe] hydrogenase, and a gene for the hydrogenase maturation protease. Thus, the [NiFe] hydrogenase may be secreted outside the cytoplasmic membrane, where the [NiFe] hydrogenase can make direct contact with Fe0, and oxidize it, generating hydrogen gas: Fe0 + 2 H+ → Fe2+ + H2. Comparative analysis of extracellular and intracellular proteomes of strain OS7 supported this hypothesis. The identification of the MIC genes enables the development of molecular tools to monitor epidemiology, and to perform surveillance and risk assessment of MIC-inducing M. maripaludis.

[A Case of Aplastic or Twig-Like Middle Cerebral Artery Presenting with an Intracranial Hemorrhage Two Years after a Transient Ischemic Attack].

Aplastic or twig-like middle cerebral artery (Ap/T-MCA) is a rare anatomical anomaly, which can be associated with intracranial hemorrhage and cerebral ischemia. A 52-year-old woman who presented with sudden headache was admitted to our hospital. Computed tomography (CT) and magnetic resonance imaging showed no abnormality; however, magnetic resonance angiogram revealed an occlusion or severe stenosis in the left middle cerebral artery. Three-dimensional CT angiography demonstrated severe stenosis in the left middle cerebral artery. The patient was discharged without any neurological deficit; however, she subsequently complained of temporary weakness in the right hand. It was possibly due to a transient ischemic attack; therefore, cilostazol 200 mg/day was administered for prevention of cerebral ischemia. Single photon emission computed tomography(with or without administration of acetazolamide)showed neither significant decrease in the cerebral blood flow nor cerebrovascular reactivity; hence, surgical revascularization was not performed. However, two years after the initial admission, she was urgently admitted to our hospital with sudden headache and nausea followed by aphasia and weakness of the right extremities. CT images showed diffuse subarachnoid hemorrhage and intracerebral hemorrhage in the left temporo-parietal lobe. Cerebral angiography revealed that the left middle cerebral artery was Ap/T-MCA without cerebral aneurysms. The patient was treated conservatively, and she eventually recovered without any neurological deficit except mild aphasia. Since Ap/T-MCA is associated with both hemorrhagic and ischemic stroke, antiplatelet therapy should be administered carefully. Moreover, it is necessary to consider extracranial-intracranial bypass to reduce hemodynamic stress on the abnormal vessels.

[A case of cerebellar variant of posterior reversible encephalopathy syndrome in puerperium].

A 29-year-old woman complained of headache and nausea several hours after delivery, followed by mild disturbance of consciousness. Physical examination revealed hypertension, systemic edema, nystagmus, dysarthria, and cerebellar ataxia. Computed tomography showed low attenuation areas in the cerebellum, and MR imaging revealed vasogenic edema in the cerebellum. MR angiography and MR venography demonstrated no significant abnormalities. We diagnosed a cerebellar variant of posterior reversible encephalopathy syndrome(PRES), and treated the patient immediately with antihypertensive drug and diuretic. The symptoms dramatically improved and MR imaging ten days after admission revealed disappearance of the vasogenic edema in the cerebellum. She was discharged without any sequelae. Though a cerebellar variant of PRES is very rare, rapid diagnosis and treatment is important for good prognosis when the disease is encountered.

[Blood blister-like aneurysm on the posterior wall of the internal carotid artery causing subarachnoid hemorrhage: a case report].

We report a case of blood blister-like aneurysm(BBA)on the posterior wall of the internal carotid artery(ICA)causing subarachnoid hemorrhage(SAH). A 45-year-old man suffering from SAH(WFNS grade 5)was referred to our hospital. 3D-CT angiography showed a BBA on the posterior wall of the ICA. The aneurysm had been enlarged for a few days. Therefore we performed coil embolization. Four weeks after the operation, the aneurysm was found to have regrown. In the second operation the aneurysm was successfully treated by the ICA proximal occlusion with extracranial-intracranial bypass. Postoperative follow-up cerebral angiography detected no aneurysm. To our knowledge, BBA on the posterior wall of the ICA is rare. Radical surgery with bypass should be performed as soon as possible when the BBA is found to regrow. We discuss the clinical characteristics with a review of the literature.

[A case of cervicothoracic subpial lipoma].

We report the case of a 24-year-old woman with cervicothoracic subpial lipoma not associated with spinal dysraphism. She complained of back pain, gait disturbance, and sensory disturbance of the both lower extremities. MRI revealed a hyperintense mass lesion that was dorsolateral to the spinal cord in the intradural region between C7 and Th4 on both T1 and T2 weighted images. Axial T2 weighted images showed dorsal nerve roots passing through the mass. A thoracic laminectomy between Th1 and Th4 was performed. A yellowish subpial mass was found after the dura was opened, and the borderline between the tumor and the normal spinal cord was unclear. The mass was partially resected and intraoperative SEP monitoring data remained unchanged. Dural plasty using artificial dura was performed. Histological examination revealed mature adipose and connective tissues, a fact which was compatible with a diagnosis of lipoma. Postoperatively, no complications occurred. The patient's gait disturbance and hypesthesia of the right lower extremity slightly improved. Subpial spinal lipoma not associated with dysraphism is rare and accounts for only 1% of all spinal tumors. A slow ascending spastic monoparesis or paraparesis is a common initial symptom. However, once symptoms progress, further deterioration is rapid. Therefore, early surgical decompression and debulking of the subpial lipoma in patients with neurological abnormalities is recommended because most symptomatic patients do not improve after surgery.

Iron-corroding methanogen isolated from a crude-oil storage tank.

Microbiologically influenced corrosion of steel in anaerobic environments has been attributed to hydrogenotrophic microorganisms. A sludge sample collected from the bottom plate of a crude-oil storage tank was used to inoculate a medium containing iron (Fe(0)) granules, which was then incubated anaerobically at 37 degrees C under an N(2)-CO(2) atmosphere to enrich for microorganisms capable of using iron as the sole source of electrons. A methanogen, designated strain KA1, was isolated from the enrichment culture. An analysis of its 16S rRNA gene sequence revealed that strain KA1 is a Methanococcus maripaludis strain. Strain KA1 produced methane and oxidized iron much faster than did the type strain of M. maripaludis, strain JJ(T), which produced methane at a rate expected from the abiotic H(2) production rate from iron. Scanning electron micrographs of iron coupons that had been immersed in either a KA1 culture, a JJ(T) culture, or an aseptic medium showed that only coupons from the KA1 culture had corroded substantially, and these were covered with crystalline deposits that consisted mainly of FeCO(3).

[A case of adolescent adrenoleukodystrophy with clinical improvement after allogeneic bone marrow transplantation (allo-BMT)].

We reported a case of adolescent type adrenoleukodystrophy. A 20-year old man noticed slight hemiparesis on his right side. After admission, high level of serum VCLFA and ACTH and a point mutation in ALDP codon 606 were recognized along with white matter lesions in the left medulla, basal ganglia, splenium of the corpus callosum on brain MRI. Then we diagnosed the patient as having adolescent type adrenoleukodystrophy. We started Lorenzo's oil and seaching for BMT donor. Six months later, he received allo-BMT at our hospital. His clinical symptoms gradually deteriorated during waiting allo-BMT, and just before allo-BMT, right hemiparesis, gait disturbance, exaggerated deep tendon reflexes on his right side, right lower quadrantanopia and mild cognitive deterioration. Two months after allo-BMT, gait disturbance and right hemiparesis were alleviated. We also observed improvement of abnormal findings on brain MRI and magnetic stimulation. We believed that allo-BMT is effective for the treatment of adolescent type adrenoleukodystrophy.