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Retropharyngeal cellulitis/abscesses are deep neck infections that may become life-threatening if airway compromise occurs. This condition is more common in children than in adults, and associated intracranial vessel narrowing has been reported. We report an adult patient with extensive retropharyngeal cellulitis and intracranial vasospasm. The patient was a 62-year-old woman who presented with fever, sore throat, and neck pain. She also had uncontrolled type 2 diabetes mellitus. Leukocytosis, prolonged erythrocyte sedimentation rate, elevated C-reactive protein, and hyperglycemia were present on admission. Computed tomography and contrast-enhanced magnetic resonance imaging revealed severe swelling in the nasopharyngeal, retropharyngeal, prevertebral, and bilateral carotid spaces. Gadolinium enhancement extended to the middle cranial fossa and visceral space. Multiple stenoses in several intracranial vessels was also identified. Intravenous antibiotic therapy was initiated, the patient's symptoms resolved, and repeat imaging confirmed improvement. Intracranial vasospasm should be considered in patients with retropharyngeal cellulitis.

Intravenous lipoma of the inferior vena cava is an incidental finding on contrast-enhanced computed tomography in 0.5% of individuals. We report a case of multiple intravenous lipomas discovered during diagnosis of cholangitis in a 39-year-old woman. Imaging revealed three fatty masses that appeared connected by cordlike structures: one in the left renal vein with wide mural attachment and two in the inferior vena cava, the higher of which was mobile. We hypothesize that these originated as a single lipoma that subsequently divided into three distinct masses. Because mobile masses may cause pulmonary thromboembolism, surgery is recommended in these cases.

The genetic causes of combined pituitary hormone deficiency remain elusive in most patients. Recently, incompletely penetrant heterozygous mutations in ROBO1 have been described in patients with pituitary stalk interruption syndrome. Herein, we identified a novel homozygous slice site mutation in ROBO1(c.1342+1G>A) using a trio whole-exome sequencing strategy in a 5-year-old Japanese boy who had combined pituitary hormone deficiency, psychomotor developmental delay, severe intellectual disability,sensorineural hearing loss, strabismus, and characteristic facial features, including a broad forehead, micrognathia, and arched eyebrows. Magnetic resonance imaging delineated anterior pituitary hypoplasia, ectopic posterior pituitary, invisible pituitary stalk, thinning of the corpus callosum, and hypoplasia of the pons and midbrain. The phenotypically normal parents (first cousins) were heterozygous for the mutation. The results provide further evidence of ROBO1 being involved in the development of the pituitary gland.A recessive mutation of ROBO1 is a potential novel cause of a syndromic disorder associated with combined pituitary hormone deficiency., Journal of Human Genetics, 64, pp.341?346; 2019

Purpose: It is sometimes challenging to diagnose hemangioblastoma by MRI when the tumor is a solid mass in the posterior fossa. We therefore evaluated perfusion images and diffusion-weighted images to diagnose hemangioblastoma in order to obtain the most useful quantitative features. Methods: Thirty-one posterior fossa tumors whose pathological diagnosis was confirmed by surgery were included in groups A (12 hemangioblastomas) and B (non-hemangioblastoma 19 cases: metastatic tumor 6 cases, pilocytic astrocytoma 3 cases, malignant lymphoma 3 cases, glioblastoma 2 cases, medulloblastoma 2 cases, and other 3 cases). All cases were imaged by 3.0-Tesla MRI, with the apparent diffusion coefficient (ADC) on diffusion-weighted imaging as the parameter, arterial spin labeling (ASL) as the relative value from the CBF map to the region of interest (ROI) in the contralateral hemisphere as perfusion image, dynamic susceptibility contrast (DSC) as rCBF, rCBV, corrected CBV, and K2. The ROI was set to match the contrast-enhanced part, and the two groups were compared and examined. Results: The relative ASL value of group A and the corrected CBV in DSC were significantly higher than group B (p

BACKGROUND Pituitary apoplexy associated with aneurysmal rupture is extremely rare and may be misdiagnosed as primary pituitary adenoma apoplexy. The authors present a case of a patient with pituitary apoplexy caused by rupture of an anterior cerebral artery aneurysm embedded within a giant pituitary adenoma, and they review the relevant literature. OBSERVATIONS A 78-year-old man experienced sudden headache with progressive vision loss. Magnetic resonance imaging (MRI) revealed a giant pituitary tumor with abnormal signal intensity. Magnetic resonance angiography immediately before surgery showed a right A1 segment aneurysm, suggesting coexisting pituitary apoplexy and ruptured aneurysm. The patient underwent urgent transsphenoidal surgery for pituitary apoplexy. The tumor was partially removed, but the perianeurysmal component was left behind. Subsequent cerebral angiography showed a 5-mm right A1 aneurysm with a bleb that was successfully embolized with coils. Retrospective review of preoperative dynamic MRI showed extravasation of contrast medium from the ruptured aneurysm into the pituitary adenoma. Histopathologic examination showed gonadotroph adenoma with hemorrhagic necrosis. Postoperatively, the patient’s visual function improved. LESSONS MRI identification of pituitary apoplexy caused by aneurysmal rupture has not been reported previously. Aneurysmal rupture should be considered in the differential diagnosis of pituitary apoplexy. When a ruptured aneurysm is encountered, the authors recommend treating it before addressing pituitary apoplexy.

BACKGROUND: Vacuolar encephalomyelopathy, a disregarded diagnosis lately, was a major neurological disease in the terminal stages of human immunodeficiency virus (HIV)-1 infection in the pre-antiretroviral therapy (ART) era. Granulomatous-lymphocytic interstitial lung disease (GLILD) was classically identified as a non-infectious complication of common variable immunodeficiency; however, it is now being recognized in other immunodeficiency disorders. Here, we report the first case of GLILD accompanied by vacuolar encephalomyelopathy in a newly diagnosed HIV-infected man. CASE PRESENTATION: A 40-year-old Japanese man presented with chronic dry cough and progressing paraplegia. Radiological examination revealed diffuse pulmonary abnormalities in bilateral lungs, focal demyelinating lesions of the spinal cord, and white matter lesions in the brain. He was diagnosed with GLILD based on marked lymphocytosis detecting in bronchoalveolar lavage, and transbronchial-biopsy proven T-cellular interstitial lung disease with granulomas. Microbiological examinations did not reveal an etiologic agent. The patient was also diagnosed with HIV-associated vacuolar encephalomyelopathy on the basis of an elevated HIV viral load in cerebrospinal fluid. After initiating ART, the brain lesions and paraplegia improved significantly, and interstitial abnormalities of the lungs and cough disappeared. CONCLUSION: This report highlights that even in the post-ART era in developed countries with advanced healthcare services, HIV-associated vacuolar encephalomyelopathy should be considered in the differential diagnosis of a progressive neurological disorder during the first visit. Furthermore, GLILD may represent an HIV-associated pulmonary manifestation that can be treated by ART., AIDS research and therapy, 17(1), art.no.38; 2020

Background: Although transvenous embolization (TVE) is an effective method for treating dural arteriovenous fistula (AVF), directing the catheter to the lesion site is difficult. Objective: We report on the utility of a quadruple coaxial catheter system for TVE. Materials and methods: The quadruple catheter system comprised a 6 Fr guiding sheath, 6 Fr guiding catheter, 4 Fr intermediate catheter, and a regular microcatheter. The system was utilized in 27 consecutive dural AVF cases treated with TVE. In this study, we reviewed our experience with this system, including the theory, method of use, and complications. Results: Stenosis or obstruction of the vascular access was identified in 12 cases. The catheter could not reach to the lesion in three cases of cavernous sinus (7.4%); therefore, transarterial embolization was employed. Angiographic results revealed that the cases consist of total occlusion (n = 16, 59.5%), subtotal (n = 10, 37.0%), and partial occlusion (n = 1, 3.7%). Complete resolution or improvement of symptoms was observed in 23 patients (85.2%), no improvement of symptoms was observed in three patients (7.4%), and deterioration of symptoms was observed in one patient (3.7%). Venous perforation occurred in one patient without any neurological deficit. The catheter system provided access to the lesion and provided stability during the mechanically demanding process navigating the catheter and placing the coils. Conclusion: We determined that the quadruple coaxial system was safe and efficient for TVE for dural AVF., Neurological Research, 37(4), pp.328-331; 2015

Objective: Carotid artery stenting (CAS) is a well-accepted treatment for atherosclerotic stenosis of carotid arteries. Since the occurrence of distal embolization with CAS is still a major concern embolus protection devices (EPD) are usually employed during the procedure. We examined two types of embolus protection filters (Angioguard XP (AG); Filterwire EZ (FW)) and evaluated the function. Thus, the filter was examined postoperatively and the cause of intraoperative flow impairment was evaluated. Materials and methods: CAS was performed for 54 patients with carotid artery stenosis (55 lesions: 25 AG; 27 FW; 3 others). After completing CAS the filter membrane was stained with hematoxylin-eosin (HE) solution and removed from the filter strut. Once mounted on a glass slide the filter was evaluated under a microscope. The area occupied with debris was measured and the relationship to intraoperative flow impairment was evaluated. Furthermore, the relationship between perioperative ischemic complications and intraoperative flow impairment was statistically analyzed. Results: Microscopic observation of the slide revealed the pore density of the FW was 1.5 times higher than that of the AG and the filter area of the FW was 2.5 times wider than than the AG. HE staining facilitated characterization of the debris composition. The area occupied with debris was significantly more in the AG (0.241 ±0.13 cm2) than in the FW (0.129 ±0.093 cm2). Thus, fibrin was significantly more precipitated in the AG. Flow impairment occurred in 6 AG cases (24.0 %) and 4 FW cases (14.8 %). It was induced by filter obstruction in the AG and by vasospasms in the FW. Three cases treated with AG (12.0 %) were complicated with cerebral infarction and all of them were related to flow impairment. One FW case (3.7 %) was complicated with cerebral infarction in presence of preserved flow throughout the intervention. Conclusion: Filter function is different according to each design. The cause of flow impairment was attributable to filter obstruction in the AG group and to vasospasms in the FW group. Filter obstruction tends to result in cerebral infarction., Acta Neurochirurgica, 156(9), pp.1721-1728; 2014

C-stopper coil (CSC) which are available for 0.018-inch inner diameter microcenter have been used for neurointervention such as transarterial embolization (TAE) of feeding artery. Although various shapes of pushable microcoils have been developed, microcoils are usually short to embolize the lesion and require lots of coils. The most specific feature of CSC is the extended length of 18 cm. To evaluate the usefulness of CSC, we reviewed our experience of CSC. Neurointervention using CSC was performed for 28 patients (31 treatments). Intervention procedures were TAE for dural arteriovenous fistula (AVF) (n = 15), transvenous embolization for dural AVF (n = 4), parent artery occlusion for cerebral aneurysm, dissection and carotidcavernous fistula (n = 8), TAE for epistaxis (n = 2), and preoperative embolization for tumor (n = 2). CSCs were deployed with push technique through microcatheter. CSCs were successfully placed into the lesion namely feeding artery, venous sinus, parent artery of aneurysm, or dissection. There were no major technical complications resulting in morbidity. Postoperative course was uneventful. No recanalization of the occluded vessel occurred during follow-up. Use of CSCs was safe and feasible for embolization of cerebrovascular lesion., Neurologia medico-chirurgica, 54(6), pp.450-456; 2014

Endovascular coil embolization for intracranial aneurysms, arteriovenous malformations (AVMs), dural arteriovenous fistulas (AVFs), and hypervascular tumors are recognized as an effective adjunctive or curative treatment. In this setting, it is sometimes difficult to navigate a coil delivery microcatheter to the target point of a tiny, tortuous vessel. We herein present a case series of a novel method that enabled super-selective coil embolization using an extremely soft bare, electrodetachable coil (ED extrasoft(®) coil) through a liquid embolic delivery microcatheter (Marathon(®)). The Marathon(®) catheter was successfully placed at the target point of the tiny, tortuous vessel, and coil embolization was achieved in all 16 patients with 9 AVMs, 2 distal aneurysms, 2 AVFs, and 3 meningiomas. The primary ED extrasoft(®) coil and delivery wire have a very small radius, and the coil is rapidly detachable with an alarm notice from the generator even under Marathon(®) with one marker. We believe that this technique can provide safe and efficient embolization for selected patients.

Background: We aimed to evaluate the validity of duplex ultrasonography (DUS) using a microconvex array transducer (MAT) with enhanced flow imaging (EFI) for visualization of the distal, internal carotid artery (ICA) and the accurate assessment of ICA stenosis. Methods: Patients who underwent both DUS and digital subtraction angiography (DSA) were registered for this study. DUS was performed by using a linear array transducer (LAT) and an MAT with EFI. The visibility of the ICA was compared between the 2 transducers. ICA stenosis was evaluated by the North American Symptomatic Carotid Endarterectomy Trial (NASCET) method on DUS, and the peak systolic flow velocity (PSV) was evaluated by using an MAT. These results were compared with DSA. Results: In 238 internal carotid arteries, the average length of visualized ICA was longer for DUS using an MAT than an LAT (38.7 ± 11.7 mm versus 25.8 ± 9.8 mm, P, Journal of Stroke and Cerebrovascular Diseases, 22(8), pp.e360-e365; 2013

A 63-year-old woman presented with right hearing disturbance and vertigo. Magnetic resonance (MR) imaging revealed the presence of right vestibular schwannoma (VS). Stereotactic radiosurgery (SRS) was performed with a tumor marginal dose of 14 Gy using two isocenters. She was followed up clinically and neuroradiologically using three-dimensional spoiled gradient-echo MR imaging. She experienced temporal neurological deterioration due to peritumoral edema in her right cerebellar peduncle and pons for a few months beginning 1.5 years after SRS, when she experienced transient right facial dysesthesia and hearing deterioration. Ten years after SRS, the patient presented with sudden onset of vertigo, gait disturbance, diplopia, dysarthria, and nausea. MR imaging demonstrated a new lesion in the right cerebellar peduncle, which was diagnosed as radiation-induced stroke. The patient was followed up conservatively and her symptoms disappeared within a few months.Multiple delayed onset radiation injuries are possible sequelae of SRS for VS., Neurologia medico-chirurgica, 52(12), pp.933-936; 2012

Background and purpose: T2*-weighted magnetic resonance angiography (SWAN) detects hemodynamic insufficiency as hypointense areas in medullary or cortical veins. We therefore investigated whether SWAN can help predict ischemic penumbra-like lesions in patients with acute ischemic stroke (AIS). Materials and methods: Magnetic resonance imaging (MRI) records?including SWAN, diffusion-weighted imaging (DWI), and magnetic resonance angiography (MRA)?of consecutive patients with major vessel occlusion within 6 h from AIS onset were analyzed. Acute recanalization was defined as an arterial occlusive lesion score of 2?3. A modified Alberta Stroke Program Early CT Score (mASPECTS) was used to evaluate ischemic areas revealed by SWAN and DWI. SWAN- and DWI-based mASPECTSs were calculated, and correlations between DWI-SWAN mismatches with final infarct lesions or clinical outcomes were evaluated. Results: Among the 35 patients included in this study, we confirmed cardioembolic stroke in 26, atherothrombotic stroke in 4, and unknown stroke etiology in 5. Overall, recanalization was achieved in 23 patients, who showed a higher follow-up DWI-based mASPECTS and lower modified Rankin Scale (mRS) score at 90 days than patients without recanalization. Initial SWAN- and follow-up DWI-based mASPECTSs were significantly higher for atherothrombotic stroke than for cardioembolic stroke. Of 12 patients without recanalization, DWI-SWAN mismatch was significantly correlated with infarct growth. Patients with recanalization showed no such correlation. In the assessment of clinical outcome, follow-up DWI-based mASPECTS and patient's age were significantly correlated with mRS at 90 days after stroke. A multivariate logistic regression analysis revealed that the follow-up DWI-based mASPECTS was independently associated with a favorable outcome 90 days after stroke. Conclusions: For patients with AIS, DWI-SWAN mismatch might show penumbra-like lesions that would predict infarct growth without acute recanalization. Assessment of ischemic lesions from the venous side appears to be useful for considering the etiology and revascularization therapy., PLoS ONE, 13(4), e0195554; 2018

BACKGROUND: Although aneurysm formation accompanying parenchymal hemorrhage is one of devastating complications in the central nerves system (CNS), imaging studies of the brain are not routinely warranted in patients with infective endocarditis (IE). To assess the clinical importance for detecting silent lesions in the central nervous system, we investigated hypointense signal spots detected on the brain T2*-weighted MR imaging in patients with IE. METHODS AND RESULTS: Eleven patients with IE were retrospectively reviewed. Seven patients (63.6%) showed hypointense signal spots on T2*-weighted MR images. The number of hypointense signal spots increased within only a few weeks in five patients. CONCLUSION: The brain T2*-weighted MR imaging in patients with IE may have a potential role to detect CNS lesions with clinical significance of potentially high risk of intracranial hemorrhage. T2*-weighted hypointense signal spots may be specific to brain involvement, and be quite useful in monitoring CNS lesions associated with IE, even if they are asymptomatic., Clinical neurology and neurosurgery, 112(5), pp.436-440; 2010

BACKGROUND: Diffusion-weighted imaging (DWI) has been reported to be a useful technique for diagnosing Creutzfeldt-Jakob disease (CJD). The present study reported DWI results in cases of familial CJD with a V180I mutation (CJD180) in the prion protein gene as well as neurological findings. METHODS: A retrospective analysis of 3 patients with V180I was performed. Cerebrospinal fluid (CSF) analysis, brain MRI, single-photon emission computed tomography (SPECT), and magnetic resonance spectroscopy (MRS) were included. CSF was analyzed for biochemical markers, and each patient underwent brain MRI, SPECT, and MRS analysis. A brain biopsy from the frontal cortex, which corresponded to the area of increased DWI signals, was utilized for neuropathological analysis. RESULTS: CSF analysis results revealed elevated total tau protein and the absence of 14-3-3 protein, as well as decreased concentrations of neuron-specific enolase, S100 protein, and prostaglandin E(2). All patients presented with unique MRI features. Brain biopsy showed severe spongiform morphology, but comparatively preserved neurons and mild astrocytic gliosis. Accumulations of PrP(Sc) were not detected using the 3F4 antibody, and microglial activation was subtle. SPECT revealed hypoperfusion throughout both hemispheres. MRS revealed a reduced N-acetyl aspartate/creatine ratio. CONCLUSION: Results from this study suggested that increased DWI signals could reflect severe spongiform changes in CJD180 patients., Dementia and geriatric cognitive disorders, 28(6), pp.550-557; 2009

BACKGROUND AND PURPOSE: Identifying the precise hemodynamic features, including the fistulous point, is essential for treatments of dural arteriovenous fistulas (DAVFs). This study illustrates the efficacy of DynaCT digital angiograms obtained from a 3D C-arm CT to directly visualize the location of the fistulous points in DAVFs. MATERIALS AND METHODS: This retrospective study observed 14 consecutive patients with DAVFs, which included 7 cavernous sinuses, 4 transverse-sigmoid sinuses, 2 convexity-superior sagittal sinuses, and 1 tentorial sinus. In the assessment of the practical applicability for the diagnosis of DAVFs, images obtained from 2D digital subtraction angiography (DSA) and DynaCT were comparatively evaluated. RESULTS: In all patients, DynaCT digital angiography could clearly demonstrate the feeding arteries, the fistulous points, and the draining veins. Significant anatomic landmarks for the fistulous points with relationships to osseous structures were also provided. Compared with 2D DSA, DynaCT digital angiograms demonstrated 12 additional findings in 8 patients (57%), including the detection of the fistulous points (n = 7), the feeders (n = 1), the retrograde leptomeningeal drainage (n = 1), the draining veins (n = 1), and the venous anomaly (n = 2). CONCLUSIONS: In comparison with 2D DSA, DynaCT may provide more detailed information to evaluate DAVFs. DynaCT digital angiograms have a high contrast and isotropic spatial resolution, allowing a reliable visualization of small vessels and fine osseous structures. Such detailed information, especially for the location of the fistulous points, could be very useful for either the endovascular or the surgical treatments of DAVFs., AJNR. American journal of neuroradiology, 30(3), pp.487-491; 2009

Percutaneous transluminal angioplasty with stenting (PTA/stenting) for intracranial atherosclerotic stenoses is usually performed without any protection devices. We report a unique case of atherothrombotic stenosis with the vulnerable plaque in the cavernous portion of the internal carotid artery (ICA), which was successfully treated by PTA/stenting under cerebral protection with the flow reversal system. A 68-year-old woman presented repetitive transient ischemic attacks in the right ICA territory. Cerebral angiography revealed 80% stenosis in the cavernous portion of the right ICA. High-resolution magnetic resonance imaging (HR-MRI) demonstrated lipid-rich plaques at this lesion. PTA/stenting was performed with a proximal protection device under flow reversal. A filter device captured much amount of atherothrombotic debris with lipid-rich macrophages and leukocytes, which was consistent with HR-MRI findings. Some selected cases of intracranial atherothrombotic ICA stenoses may need endovascular treatment with cerebral protection system. HR-MRI is useful to evaluate plaque characteristics even in the cavernous portion of the ICA., Clinical neurology and neurosurgery, 110(10), pp.1031-1034; 2008

We present a unique case of a cavernous sinus (CS) dural arteriovenous fistula (DAVF), which recurred at adjacent sinuses following repeated transvenous embolizations (TVEs). A 68-year-old woman presented with progressive left conjunctival chemosis and diplopia. Cerebral angiography revealed a left CS DAVF, which was completely obliterated by TVE via the left inferior petrosal sinus (IPS). Two years later, the DAVF recurred in the left IPS, and again in the left sigmoid sinus (SS) 3 years after the initial treatment in spite of a second TVE. Moreover, the left SS and the left internal jugular vein, which had been previously stenotic, had been occluded. The third TVE resulted in the complete obliteration of the SS DAVF. CS DAVFs may recur at adjacent sinuses even after complete obliteration by TVE. Careful follow-up is necessary to check for the recurrence of DAVFs, especially in cases with venous flow changes, such as sinus occlusion, following endovascular treatment., The original publication is available at www.springerlink.com, Radiation medicine, 26(7), pp.431-437; 2008