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The authors report the case of a 71-year-old woman presented to the Emergency Department with acute ischemic stroke. She was treated with rt-PA and interventional endovascular revascularization and developed rapidly progressing angioedema that led to emergency intubation. The standard treatment was not very effective and the swelling improved after infusion of fresh frozen plasma. Angioedema after rt-PA infusion could be a life-threatening emergency that requires quick airway management by skilled professionals. As this condition is triggered by several factors, such as unregulated histamine and bradykinin production, the traditional treatment recommended by the guidelines may not be sufficient and the use of FFP can be considered as a safe and valuable aid.

Major cerebral vessels have been proposed as a target of defective mitochondrial metabolism in patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes syndrome (MELAS). Cerebral angiographic techniques are not routinely performed in MELAS patients. A systematic literature review was performed to identify studies describing major vessel caliber alterations in MELAS. Twenty-three studies reporting on 46 MELAS patients were included. Alterations in major caliber vessels were present in 59% (27/46) of patients. Dilation occurred in 37% (17/46) of patients, and in 88% (15/17) of them during a stroke-like episode (SLE). Stenosis was reported in 24% (11/46) of patients: 36% (4/11) related to an SLE and 64% (7/11) to dissections or degenerative changes. During an SLE, identification of intracranial vessels dilation or stenosis could be a selection tool for new treatment protocols. Outside SLE, identification of major cerebral vessels dissections and degenerative changes may help to prevent subsequent complications. (c) 2021 Elsevier Masson SAS. All rights reserved.

BackgroundThere is no consensus on the most effective endovascular technique to use in patients with acute ischemic stroke due to terminal internal carotid artery (ICA) occlusion. The aim of this study was to compare safety and efficacy of the aspiration technique (AT) and combined technique (CT) as first-line approach in terminal ICA occlusions.MethodsWe performed a retrospective analysis of prospectively collected databases from seven Italian stroke centers. Patients were divided into two subgroups according to the first-line approach: AT group or CT group. We followed the STROBE guidelines for cohort studies. We used Chi-square test, one-way and multivariate ANOVA analysis, together with contrast analysis and post hoc tests, logistic regression and Pearson’s bivariate correlation for the statistical analyses.ResultsBetween January 2018 and August 2020, 353 patients were treated for a terminal ICA occlusion, with either AT or CT. CT was associated with a higher Thrombolysis in Cerebral Infarction (TICI) 2B-3 after the first pass (51.0% vs 26.9%) and at the end of the procedure (84% vs 73.3%) and with an improved clinical outcome at discharge (modified Rankin Scale (mRs) 0–2 of 47.8% vs 34.0%) and at 3 months’ follow-up (mRs 0–2 of 56.5% vs 38.9%) compared with AT.ConclusionThrombectomy of terminal ICA occlusions obtained using CT as first-line approach demonstrated better technical and functional outcomes in comparison with AT.

OBJECTIVESurgical treatment of orbital lesions is challenging because complex approaches with a high risk of postoperative sequelae are required. Recently, minimally invasive endoscopic approaches through endonasal (EEA) and transpalpebral (ETP) routes have been proposed. The objective of this study was to assess outcomes of EEA and ETP in the authors’ series of patients with orbital lesions.METHODSData from all patients who underwent operations for an orbital tumor through an endoscopic approach at the authors’ institution from 2002 to 2018 were retrospectively collected. All patients underwent preoperative MRI and ophthalmological evaluation, which was repeated 3 months after surgery and then at regular follow-up intervals. A systematic review of the literature was also performed using Medline, Embase, and Web of Science databases.RESULTSThe series includes 23 patients (14 males); the mean patient age was 48 ± 23.9 years. Most of the lesions were intraconal (n = 19, 83%). The more frequent histotype was cavernous hemangioma (n = 5, 22%). Exophthalmos was the most common symptom (21 of 23 patients). EEA was performed in 16 cases (70%) and ETP in 7 (30%). The aim of the surgery was achieved in 94% of the cases after an EEA (successful biopsy in 5 of 6 cases and radical resection in all 10 remaining patients), and in 86% after an ETP (successful biopsy in 2 cases and radical tumor resection in 4 of 5 cases). Complications consisted of 3 cases (13%) of transitory diplopia. One recurrence (4%) was observed at follow-up (mean 59 ± 55 months).CONCLUSIONSThe EEA and ETP have demonstrated to be safe and effective for tumors located respectively in medial and lateral quadrants, permitting one to approach orbital lesions endoscopically at 360°. Innovative surgical tools, including intraoperative ultrasonography, may be useful to potentially reduce surgical morbidity. Larger series are needed to validate these preliminary suggestions.

Herniated intervertebral disc is one of the common causes of neck/low back pain and/or neuralgia. The vast majority of such symptoms improve with conservative treatments that include rest, medications, and physical therapy. In case of failure, many minimally invasive disc techniques have been emergedas alternative optionsto surgical treatments, to avoid their potential complications and possiblytheir suboptimal results. Chemonucleolysis is one of the minimally invasive disc decompression techniques thatare based on the intradiscal injection of chemonucleolytic agents causing dehydration of the disc matrix, resulting into a reduction of the intradiscal pressure and disc voulme, and hence, alleviation of the nerve root compression. These chemonucleolytic materials include chymopapain, collagenase, oxygen-ozone mixture,radio-opaque jellified ethanol “Discogel, chondroitinasesulphateABC, and matrix metalloproteinase (MMP). Nowadays, chemonucleolysis using the oxygen-ozone mixture and radiopaque gelified ethanol have gathered the most support for use in clinical practice.This article aims to clarify the mechanisms of action, selection criteria, procedural technique, outcome assessment, efficacy, and safety of a variety of intradiscalchemonucleolytictherapiesused for the treatment of herniated discs.

Introduction A reduction of the hospitalization and reperfusion treatments was reported during COVID-19 pandemic. However, high variability in results emerged, potentially due to logistic paradigms adopted. Here, we analyze stroke code admissions, hospitalizations, and stroke belt performance for ischemic stroke patients in the metropolitan Bologna region, comparing temporal trends between 2019 and 2020 to define the impact of COVID-19 on the stroke network. Methods This retrospective observational study included all people admitted at the Bologna Metropolitan Stroke Center in timeframes 1 March 2019–30 April 2019 (cohort-2019) and 1 March 2020–30 April 2020 (cohort-2020). Diagnosis, treatment strategy, and timing were compared between the two cohorts to define temporal trends. Results Overall, 283 patients were admitted to the Stroke Center, with no differences in demographic factors between cohort-2019 and cohort-2020. In cohort-2020, transient ischemic attack (TIA) was significantly less prevalent than 2019 (6.9% vs 14.4%, p = .04). Among 216 ischemic stroke patients, moderate-to-severe stroke was more represented in cohort-2020 (17.8% vs 6.2%, p = .027). Similar proportions of patients underwent reperfusion (45.9% in 2019 vs 53.4% in 2020), although a slight increase in combined treatment was detected (14.4% vs 25.4%, p = .05). Door-to-scan timing was significantly prolonged in 2020 compared with 2019 (28.4 ± 12.6 vs 36.7 ± 14.6, p = .03), although overall timing from stroke to treatment was preserved. Conclusion During COVID-19 pandemic, TIA and minor stroke consistently reduced compared to the same timeframe in 2019. Longer stroke-to-call and door-to-scan times, attributable to change in citizen behavior and screening at hospital arrival, did not impact on stroke-to-treatment time. Mothership model might have minimized the effects of the pandemic on the stroke care organization.

We present the neuroimaging findings of three cases of non-ketotic hyperglycaemia (NKH) associated with focal seizures and a review of the previous cases and series reported in literature. NKH is a cause of seizures in both long-standing and newly diagnosed diabetic patients. They are usually focal motor seizures, rarely with a secondary generalisation. This condition does not fully respond to anticonvulsant therapy if glycaemic levels are not normalised. Of interest, magnetic resonance imaging (MRI) of NKH could be different from those observed during other kinds of seizures. Indeed, seizure-related MRI abnormalities mainly involve the cortical grey matter, while NKH-related seizures usually appear as reversible subcortical T2/fluid attenuation inversion recovery (FLAIR) hypointensity. This latter abnormality shows a good spatial correlation with the area of the ictal focus on electroencephalogram and could be associated with other more common post-ictal MRI changes (cortical grey matter T2/FLAIR hyperintensity, cortical or leptomeningeal enhancement). Although these abnormalities tend to be transient, a focal volume loss or gliosis can result on follow-up imaging. Our cases confirm T2/FLAIR subcortical hypointensity as a main neuroradiological hallmark of NKH-induced seizures.

Background Occlusion of the internal carotid artery (ICA), whether isolated or in the setting of a tandem lesion (TL) have a poor response to treatment with intravenous thrombolysis. Previous studies ​​have demonstrated the superiority of mechanical thrombectomy in the treatment of acute ischemic stroke (AIS) following large vessel occlusion, compared to standard intravenous fibrinolysis. The aim of our study was to describe endovascular treatment (EVT) in AIS due to isolated ICA occlusion or TL. Methods We assessed the association between 90-day outcome and clinical, demographic, imaging, and procedure data in 51 consecutive patients with acute isolated ICA occlusion or TL who underwent EVT. We evaluated baseline NIHSS and mRS, ASPECTS, type of occlusion, stent placement, use of stent retrievers and/or thromboaspiration, duration of the procedure, mTICI, postprocedural therapy and complications. Results A favorable 90-day outcome (mRS 0–2) was achieved in 34 patients (67 %) and was significantly associated with the use of dual antiplatelet therapy after the procedure (p = 0.008), shorter procedure duration (p = 0.031), TICI 2b-3 (p p = 0.001). Four patients did not survive, resulting in a mortality rate of 8 %. Conclusions Our study has shown that EVT in the treatment of AIS due to ICA occlusion is safe, and effective in determining a good functional outcome. ICA stenting led to good angiographic results and therapy with a glycoprotein IIb / IIIa inhibitor immediately after stent release did not result in a greater risk of hemorrhage. The use of post-procedural dual antiplatelet therapy was associated with favorable outcome, without a significant increase in hemorrhagic transformation.

Background and Purpose: Acute ischemic stroke and large vessel occlusion can be concurrent with the coronavirus disease 2019 (COVID-19) infection. Outcomes after mechanical thrombectomy (MT) for large vessel occlusion in patients with COVID-19 are substantially unknown. Our aim was to study early outcomes after MT in patients with COVID-19. Methods: Multicenter, European, cohort study involving 34 stroke centers in France, Italy, Spain, and Belgium. Data were collected between March 1, 2020 and May 5, 2020. Consecutive laboratory-confirmed COVID-19 cases with large vessel occlusion, who were treated with MT, were included. Primary investigated outcome: 30-day mortality. Secondary outcomes: early neurological improvement (National Institutes of Health Stroke Scale improvement ≥8 points or 24 hours National Institutes of Health Stroke Scale 0–1), successful reperfusion (modified Thrombolysis in Cerebral Infarction grade ≥2b), and symptomatic intracranial hemorrhage. Results: We evaluated 93 patients with COVID-19 with large vessel occlusion who underwent MT (median age, 71 years [interquartile range, 59–79]; 63 men [67.7%]). Median pretreatment National Institutes of Health Stroke Scale and Alberta Stroke Program Early CT Score were 17 (interquartile range, 11–21) and 8 (interquartile range, 7–9), respectively. Anterior circulation acute ischemic stroke represented 93.5% of cases. The rate modified Thrombolysis in Cerebral Infarction 2b to 3 was 79.6% (74 patients [95% CI, 71.3–87.8]). Thirty-day mortality was 29% (27 patients [95% CI, 20–39.4]). Early neurological improvement was 19.5% (17 patients [95% CI, 11.8–29.5]), and symptomatic intracranial hemorrhage was 5.4% (5 patients [95% CI, 1.7–12.1]). Patients who died at 30 days exhibited significantly lower lymphocyte count, higher levels of aspartate, and LDH (lactate dehydrogenase). After adjustment for age, initial National Institutes of Health Stroke Scale, Alberta Stroke Program Early CT Score, and successful reperfusion, these biological markers remained associated with increased odds of 30-day mortality (adjusted odds ratio of 2.70 [95% CI, 1.21–5.98] per SD-log decrease in lymphocyte count, 2.66 [95% CI, 1.22–5.77] per SD-log increase in aspartate, and 4.30 [95% CI, 1.43–12.91] per SD-log increase in LDH). Conclusions: The 29% rate of 30-day mortality after MT among patients with COVID-19 is not negligible. Abnormalities of lymphocyte count, LDH and aspartate may depict a patient’s profiles with poorer outcomes after MT. Registration: URL: https://www.clinicaltrials.gov . Unique identifier: NCT04406090.

Spontaneous intracranial hypotension (SIH) results from low cerebrospinal fluid (CSF) volume, typically secondary to a spinal CSF leak. CSF hypovolemia leads to brain sagging, particularly in orthostatic position, and expansion of the intracranial venous compartment. These pathophysiological mechanisms are responsible for the clinical presentation that usually consists of postural headache variably associated with other neurological symptoms. The wide range of clinical manifestations, including the absence of typical headache, may delay diagnosis. Magnetic resonance imaging (MRI) supports diagnosis showing typical findings including subdural fluid collections, pachymeningeal enhancement and brain sagging.

Background and Aims C.septicum is an anaerobic bacterium that resides in the gastrointestinal tract of herbivores. Its presence in human fecal flora is still debated, being found in only 2.8% of healthy adults. Transmitted by oral-fecal route, it reaches the systemic circulation, causing bacteremia, myonecrosis, bowel necrosis, intracranial abscesses/encephalitis. Shiga-toxin producing E. Coli related hemolytic uremic syndrome (STEC-HUS) is a thrombotic microangiopathy characterized by hemolytic anemia, thrombocytopenia and kidney damage, related to STEC infection. Severe presentation includes gastrointestinal and neurologic involvement. Microangiopathic lesions in the colonic mucosa can facilitate the colonization of systemic circulation by C.septicum, spreading the superinfection. Since the first report in 1988, very few cases of STEC-HUS complicated by C.septicum superinfection have been described in literature and thus the factors predicting clinical outcome are not clear. Method We describe the case of a 5-years-old girl admitted for anemia, AKI, thrombocytopenia and diarrhea. Previous history was unremarkable. STEC-HUS diagnosis was made. Despite prompt conservative treatment, 12h later her clinical condition worsened, showing ESKD needing dialysis, abdominal pain, progressive hyporesponsiveness. The brain CT scan showed pneumocephalus and encephalitis; blood cultures were drawn and broad-spectrum antibiotic therapy was set (Tab.1). Cardiovascular instability prevented surgical treatment of pneumocephalus to be done. The patient died shortly after because of septic shock. Post-mortem stool and blood cultures confirmed STEC infection complicated by C.Septicum. Previous literature on this specific clinical picture has been reviewed and summarized. Results Eleven additional cases (7 males) have been previously described. Mean age of presentation was 2.9 years. A; abdominal symptoms (commonly distention and pain) were reported in all cases, while ESRD was present in 10 cases. Six patients presented fever. Central nervous system presentation as pneumocephalus was associated with poor prognosis, while surgical treatment availability of brain lesions to survival. In one case the infection caused myonecrosis (Tab. 1). The mortality rate was about 60%. In 7 cases, clinical conditions worsened in less than 24h and this was in all cases related to poor outcome. Conclusion C.septicum superinfection complicating STEC-HUS is a rare but fearsome complication to be aware of. Rapid diagnosis and surgical treatment are essential for the successful management of this particular condition.

Background and Aims Bartter (BS) and Gitelman syndrome (GS) are autosomal recessive rare inherited disorders characterized by hypokalemic metabolic alkalosis and secondary hyperaldosteronism. The primary defect is a genetically determined impairment of sodium chloride reabsorption in the renal tubule, thus resulting in salt loss, dehydration and acid-base homeostasis perturbations. Although the diagnosis can be suspected based on presenting features, the clinical diagnosis of BS and GS can be challenging, as they are rare and phenotypically overlapping. As a consequence, the current clinical classification lacks of specificity and genetic testing represents the gold standard for the diagnosis. Driven by the rapidly decreasing costs and turn-around time, next-generation sequencing technologies are increasingly utilized in diagnostics and research of inherited tubulopathies, including BS and GS. Recently, sequencing of selected gene panels provided the advantage of achieving high coverage of genes of interest at lower costs, providing high diagnostic yield and new insights into the phenotypic spectrum of these rare disorders. However, whole-exome (WES) is not routinely performed for the molecular diagnosis of BS and GS. The aim of our study was to assess the diagnostic performance of WES in BS and GS and to establish genotype-phenotype correlations. Method We performed WES in all consecutive patients referred for genetic testing with a clinical suspect of BS or GS. Variant prioritization was carried out according to the American College of Medical Genetics and Genomics guidelines (ACMG). Parents and first-degree relatives were included, whenever available. Demographic, clinical and laboratory data were collected retrospectively, in order to establish genotype-phenotype correlations. Results We enrolled 50 patients (22 males, 46 Caucasians) with a clinical diagnosis of BS (19), GS (24) or BS/GS (7). All the patients showed hypokalemic metabolic alkalosis at onset (serum bicarbonate=29.5 mEq/l ± 4.4, potassium= 2.7 mEq/l ± 0.6). The median age at clinical diagnosis was 7 years (range 0-67 years). Three patients had familial history of tubulopathies. WES showed pathogenic variants in 42/50 patients (84%), thus establishing a conclusive diagnosis. Interestingly, a dedicated analytic pipeline allowed us to identify copy number variations (CNVs) in 7/42 patients with a confirmed genetic diagnosis. In detail, WES allowed us to confirm the clinical diagnosis in 33/50 patients, with an improvement in classification in at least 14 cases (i.e. subtype I-V of BS). In 9 additional patients, genetic testing changed the clinical diagnosis: 6 patients with a clinical of BS turned out to have pathogenic variants in SLC12A3, resulting in GS; in 3 patients, genetic testing revised the clinical diagnosis indicating inherited disorders outside the BS/GS spectrum (HELIX syndrome, Primary familial hypoparatiroidism, Type 2 renal hypomagnesemia). Only 38% of patients with a genetic diagnosis of BS showed nephrocalcinosis. Strikingly, this was present in 8% of patients with GS. On the other hand, hypomagnesemia, a distinctive feature of GS, was similarly distributed among BS and GS patients (45% vs. 68%, respectively). Finally, although patients with GS showed a median age at onset higher than patients with BS, some overlap did exist, making differential diagnosis challenging at single-patient level. Conclusion The results of our study demonstrate that WES ensures a high diagnostic yield (84%) in patients with a clinical diagnosis of BS or GS, especially if coupled with analysis of CNVs. This approach showed to be useful in dealing with the phenotypic heterogeneity typical of these rare disorders, improving differential diagnosis by detecting phenocopies also outside the BS/GS spectrum, enabling additional specific work-up, genetic counseling, and screening of at-risk relatives.

BACKGROUND: In many clinical trials endovascular procedures are suggested as the treatment of choice for aneurysmal Subarachnoid Hemorrhage (aSAH) whenever possible. However, in clinical practice this management is often controversial. The aim of this study is to analyze factors involved in this decision. METHODS: Our study included 317 consecutive cases of aSAH between 2010 and 2016, assessing clinical and neuroradiological features to evaluate their role in this choice. RESULTS: In our series coiling was preferred in 119 (37.6%) patients, while 198 (62.4%) were treated surgically. On univariate analysis location of aneurysms (p < 0.001), GCS score on admission (p: 0.105), degree of midline shift (p:0.015), Fisher' score (p: 0.002) and presence of vessels in the aneurysmal neck (p: 0.071) proved the most relevant factors in the choice. Also multivariate analysis confirmed the location and Fisher' grade as influential factors. Conversely, other radiological parameters, such as morphology, aspect and dome-neck ratio, presence of pre-operative vasospasm or hydrocephalus were not associated with this decision. CONCLUSIONS: The decision process in aSAH requires a multidisciplinary team, to singularly evaluate each patient. We found that the location of aneurysms in Vertebro-Basilar circulation, PCoM and ICA, greater GCS score, absence of vessels in the aneurysmal neck, lower midline shift and Fisher' score are factors influencing in choosing coiling. Conversely, morphology, Aspect and Dome-Neck ratio proved not relevant to this decision, due to technological improvement and increasing skills in the endovascular treatment.

Spontaneous intracranial hypotension results from a spinal CSF leak and usually presents with orthostatic headache, although acephalgic presentations have anecdotally been reported. The underlying low CSF volume, rarely, leads to serious complications such as cerebral venous thrombosis and coma. We report a patient presenting with cerebral venous thrombosis secondary to acephalgic spontaneous intracranial hypotension. An epidural blood patch was performed; nonetheless, the patient intracracal condition deteriorated to coma and neuroimages showed a deep brain swelling with midbrain distortion, subsequently complicated by intracranial pontine hemorrhage. © 2020, Fondazione Società Italiana di Neurologia.

Background Finding an intracranial aneurysm (IA) during a thunderclap headache (TCH) attack, represents a problem because it is necessary to distinguish whether the aneurysm is responsible for the headache as a warning leak or as an incidental finding. High-Resolution Vessel-Wall (HRVW) MRI sequences have been proposed to assess the stability of the wall, as it permits to detect the presence of aneurysmal wall enhancement (AWE). In fact, AWE has been confirmed due to inflammation, recognizable preceding rupture. Case 1: A 37-year-old woman with a migraine more intense than her usual. A CTA revealed a 10 mm AComA aneurysm without subarachnoid hemorrhage (SAH) and HRVW-MRI excluded AWE. The patient’s headache improved, and therefore, the aneurysm was considered an incidental finding, and the headache diagnosed as TCH attack. Subsequently, the aneurysm was surgically clipped, and typical migraine relapsed was reported at follow-up (FU). Case 2: A 67-year-old woman with no history of headaches underwent CTA for an abrupt onset of headache. A 7 mm right carotid-ophthalmic aneurysm with no sign of SAH was discovered. HRVW-MRI demonstrated AWE and thus, a TCH attack for a warning leak of an unstable wall was suspected. Endovascular coiling was immediately performed and at FU any further headache attack was reported. Conclusions HRVW-MRI is useful in case of finding aneurysm as the cause of headaches, particularly the TCH attack. In fact, HRVW-MRI could assess the stability of the aneurysms wall, allowing different patient management and eventually the aneurysmal treatment.

Introduction: Neurological manifestations are emerging as relatively frequent complications of corona virus disease 2019 (COVID-19), including stroke and encephalopathy. Clinical characteristics of the latter are heterogeneous and not yet fully elucidated, while the pathogenesis appears related to neuroinflammation in a subset of patients. Case: A middle-aged man presented with acute language disturbance at the emergency department. Examination revealed expressive aphasia, mild ideomotor slowing, and severe hypocapnic hypoxemia. Multimodal CT assessment and electroencephalogram (EEG) did not reveal any abnormalities. COVID-19 was diagnosed based on chest CT findings and positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) reverse transcription PCR (RT-PCR) on nasopharyngeal swab. The following day, neurological symptoms progressed to agitated delirium and respiratory status worsened, requiring admission to the ICU and mechanical ventilation. Brain MRI and cerebrospinal fluid (CSF) studies were unremarkable. RT-PCR for SARS-CoV-2 on CSF was negative. He received supportive treatment and intravenous low-dose steroids. His neurological and respiratory status resolved completely within 2 weeks. Conclusions: We report a patient with reversible COVID-19-related encephalopathy presenting as acute aphasia, mimicking stroke or status epilepticus, eventually evolving into delirium. Although large-vessel stroke is frequently encountered in COVID-19, our case suggests that focal neurological deficits may occur as the earliest feature of encephalopathy. Neurological status reversibility and the absence of abnormalities on brain MRI are consistent with a functional rather than a structural neuronal network impairment.

Noninvasive tools for diagnosis or prediction of acute kidney allograft rejection have been extensively investigated in recent years. Biochemical and molecular analyses of blood and urine provide a liquid biopsy that could offer new possibilities for rejection prevention, monitoring, and therefore, treatment. Nevertheless, these tools are not yet available for routine use in clinical practice. In this systematic review, MEDLINE was searched for articles assessing urinary biomarkers for diagnosis or prediction of kidney allograft acute rejection published in the last five years (from 1 January 2015 to 31 May 2020). This review follows the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. Articles providing targeted or unbiased urine sample analysis for the diagnosis or prediction of both acute cellular and antibody-mediated kidney allograft rejection were included, analyzed, and graded for methodological quality with a particular focus on study design and diagnostic test accuracy measures. Urinary C-X-C motif chemokine ligands were the most promising and frequently studied biomarkers. The combination of precise diagnostic reference in training sets with accurate validation in real-life cohorts provided the most relevant results and exciting groundwork for future studies.

Background and Aims Congenital anomalies of the kidney and urinary tract (CAKUT) are the major cause of chronic kidney disease and end-stage kidney disease in childhood. Solitary functioning kidney (SFK) is part of the spectrum of CAKUT. Congenital SFK is mainly due to unilateral renal agenesis (URA) and multi-cystic dysplastic kidney (MCDK). With the implementation of routine fetal ultrasound screening, SFK is increasingly recognized before birth, significantly raising the number of patients referred to paediatric nephrology units for clinical monitoring during childhood. Understanding the pathophysiology of SFK is pivotal for guiding the clinical management and informing long-term outcome. However, observational studies performed in children with SFK are controversial, especially about the need, methodology and timing of functional assessment. This may be at least in part due to the fact that different models of SFK, including congenital and acquired after unilateral nephrectomy, are often grouped together. The aim of this study was to assess the clinical, laboratory and functional features of congenital SFK caused by URA and MCDK in children, with a particular focus on the role of renal scintigraphy in estimating kidney function during childhood and adolescence. Method We retrospectively collected clinical, laboratory and instrumental records of all consecutive pediatric patients (aged 0-18 years) affected by congenital SFK caused by URA or MCDK referred to the Nephrology and Dialysis Unit of Meyer Children’s Hospital of Florence (Italy) from 1992 to 2019. Patients with unilateral kidney hypodysplasia were excluded. In particular, we reviewed data from ultrasound scanning and sequential renal scintigraphy over time. URA and MCDK were compared for clinical features, long-term course and outcome. Results A total of 155 patients with congenital SFK were included in the study and divided in two groups according to the cause of SFK (URA, n=100; MCDK, n=55). The median length of follow-up was 47 and 45 months, respectively. Male sex and ethnicity were equally distributed in the two groups. Prenatal diagnosis was more frequent in MCDK group. We did not observe either preterm birth or low birth weight in patients enrolled. Overall, the clinical features were not statistically different between the two groups. In particular, SFK associated CAKUT, including vesicoureteral reflux, occurred at a comparable frequency. Also, measurement of kidney length by ultrasound scanning, which is often considered suggestive of compensatory hypertrophy, did not differ between groups. Although renal clearance from sequential renal scintigraphy appeared not statistically different between URA and MCDK, the latter seems to reach complete functional adaptation more rapidly and earlier in the first two years of life. Conclusion The clinical course and long-term outcome of SFK has been a topic of extensive debate. Due to poor-quality of data (unclear inclusion/exclusion criteria, lack of uniformity in data collection and outcome definition), generalization of findings from observational studies to all patients with SFK could be inappropriate. Congenital SFK could represent the most unbiased group to analyze and this study provides a thorough clinical characterization of a large and strictly selected cohort. Insights from sequential renal scintigraphy suggest a different trend in reaching single kidney complete functional adaptation in URA and MCDK. These results could potentially reveal significant differences in the pathophysiologic mechanisms of reaching compensatory hypertrophy and functional adaptation by the solitary kidney in the two models. Whether confirmed in larger cohorts, these findings could provide important implications for follow-up planning, informing the need, methodology and timing for function assessment, tailoring the clinical management and understanding long-term prognosis.

Background and Aims Secondary hemolytic-uremic syndrome (HUS) is currently defined in the absence of genetic defects in complement genes (primary or atypical HUS, aHUS) or Shiga-toxin producing E. Coli infection (STEC-HUS). However, the role of complement genes abnormalities in secondary HUS is still a matter of debate as results coming from studies performed in adults are controversial. Moreover, genetic defects of complement regulation have been recently described in children with STEC-HUS, thus challenging the current HUS classification. The identification of a role of complement genes abnormalities in these patients could have important clinical implications for diagnosis and treatment. In this study, we assessed the presence of complement genes abnormalities in children with secondary HUS. Method We describe a case series of pediatric patients diagnosed with secondary HUS. All patients were screened for the presence of ADAMTS13 auto-antibody and ADAMTS13 deficiency. We performed next-generation sequencing for a panel of complement genes reported in association with aHUS (CFH, CFI, MCP, C3, FB, and THBD). Copy number variations and hybrid genes assessment were included in the analysis. Results Four patients aged 1-4 years with a diagnosis of sporadic secondary HUS were included in the study. HUS was secondary to glomerulopathy, cobalamin deficiency, bone marrow transplantation, and pneumococcal pneumoniae. Two out of four patients showed hypocomplementaemia (both C3 and C4). Diarrhea was a common clinical feature in all patients at onset, and none of these patients showed neurologic involvement. Renal replacement therapy was required in two patients at onset. Only one patient did not recover kidney function, and subsequently underwent kidney transplantation. Next-generation sequencing showed genetic abnormalities in all the patients (Table 1). All but one genetic variants have already been described in association with atypical HUS. Interestingly, haplotypes in CHF and MCP were present in three patients, all of Caucasian ethnicity. Conclusion According to the current HUS classification, complement abnormalities are diagnostic of aHUS. However, results coming from pediatric cohorts of STEC-HUS patients already claimed a role for genetic background also outside the aHUS spectrum, suggesting that the current terminology lacks both specificity and suggestion of cause. In this case series, complement genes abnormalities were present in all patients. Whether confirmed in larger cohorts, these findings would support the role of genetic background even in secondary HUS, suggesting the need for a revision of the current HUS classification, in agreement with the pathogenic mechanisms, in order to tailor the optimal treatment.

Introduction: CKD is associated with a reduction of patients’ health-related quality of life. Considering the time spent in dialysis, satisfaction with care is essential for patients QOL. Objective: Since the possible association between satisfaction with the dialysis care and QOL has never been studied, in this study, we explore this plausible link. Methods: One hundred three patients on hemodialysis (HD) and peritoneal dialysis (PD) filled-in patient-reported outcome measures (PROMs) and patient-reported experience measures (PREMs). QOL was assessed by Kidney Disease Quality of Life-36 and satisfaction by Choices for Healthy Outcomes in Caring for End-Stage Renal Disease (CHOICE) questionnaire. The analysis was conducted on patient-level, considering for single patient sociodemographic characteristics and presence of depression/anxiety. One-way ANOVA was used to compare QOL mean scores for patients who answered “excellent” and for those who answered “other ratings” in CHOICE questionnaire and the Pearson χ2 test to compare the patients’ characteristics between these 2 groups of patients. Results: The analysis showed a significant positive association between PREM and PROM scores for 8 out of 23 CHOICE items. Six of them were related to the figure of nephrologist, 1 to dialysis access site, and 1 to the social worker support. Significant association (p < 0.05) were between frequency of seeing nephrologist and physical component plus mental component, accuracy of information from nephrologist and burden of disease, accuracy of instructions from nephrologist and burden of disease, coordination between nephrologist and other physicians plus mental component, attention to cleanliness of access site and mental component, amount of dialysis information from staff and burden of disease, information from staff when choosing between HD or PD and physical component plus burden of disease, and ease of seeing social worker and burden of disease. Conclusions: The study provides support for the relationship between the care satisfaction and QOL, highlighting the central role of the nephrologist-patient communication in the QOL of dialysis patients.

INTRODUCTION: A direct aspiration first pass technique (ADAPT) is an alternative technique as first-line thrombectomy for large vessel occlusion in acute ischemic stroke, still debated when compared to first-line stent retriever. To retrospectively evaluate technical and clinical outcomes of the ADAPT as first-line treatment for anterior circulation acute ischemic stroke with large-bore reperfusion catheters. METHODS: A multicentric data collection from 14 medical centers was retrospectively analyzed. Large-bore catheters had a distal diameter between 0.64 and 0.71 in; stent retriever was added when aspiration alone failed. Baseline characteristics, technical and clinical variables were collected, including NIHSS, thrombolysis in cerebral infarction (TICI), peri-procedural complications, 90-day mRS and 90-day mortality. RESULTS: Overall, 501 patients were treated. No statistically significant difference in terms of baseline features or tPA administration was recorded between patients treated with ADAPT and with additional stent retriever. ADAPT alone was successful in achieving TICI ≥ 2b in 71.8% with a median of 1.55 aspiration attempts. In terms of TICI ≥ 2b, ADAPT alone was better than additional stent retriever (p

Purpose: Intracranial carotid artery occlusion represents an underinvestigated cause of acute ischemic stroke as well as an indication for mechanical thrombectomy. We investigated baseline and procedural characteristics, outcomes and predictors of outcome in patients with acute ischemic stroke secondary to intracranial carotid artery occlusion. Methods: A retrospective analysis of the Italian Registry of Endovascular Treatment in Acute Stroke was performed. Patients with intracranial carotid artery occlusion (infraclinoid and supraclinoid) with or without cervical artery occlusion but with patent intracranial arteries were included. The 3‑month functional independence, mortality, successful reperfusion and symptomatic intracranial hemorrhage were evaluated. Results: Intracranial carotid artery occlusion with patent intracranial arteries was diagnosed in 387 out of 4940 (7.8%) patients. The median age was 74 years and median baseline National Institute of Health Stroke Scale (NIHSS) was 18. Functional independence was achieved in 130 (34%) patients, successful reperfusion in 289 (75%) and symptomatic intracranial hemorrhage in 33 (9%), whereas mortality occurred in 111 (29%) patients. In univariate analysis functional independence was associated with lower age, lower NIHSS at presentation, higher rate of successful reperfusion and lower rate of symptomatic intracranial hemorrhage. Multivariable regression analysis found age (odds ratio, OR:1.03; P = 0.006), NIHSS at presentation (OR: 1.07; P < 0.001), diabetes (OR: 2.60; P = 0.002), successful reperfusion (OR:0.20; P < 0.001) and symptomatic intracranial hemorrhage (OR: 4.17; P < 0.001) as the best independent predictors of outcome. Conclusion: Our study showed a not negligible rate of intracranial carotid artery occlusion with patent intracranial arteries, presenting mostly as severe stroke, with an acceptable rate of 3‑month functional independence. Age, NIHSS at presentation and successful reperfusion were the best independent predictors of outcome. © 2020, Springer-Verlag GmbH Germany, part of Springer Nature.

Patient with surgically treated right temporo-occipito-parietal glioblastoma multiforme. Postoperative follow-up performed early (24 hours and 2 weeks) with MR morphologic sequences.

Background: Transverse sinus stenosis is a common brain MRI finding in chronic migraine (CM) and chronic tension-type headache (CTTH) patients in clinical practice; however, its clinical and diagnostic role is unclear. The aim of the study is to determine the frequency of transverse sinus stenosis in these headache patients resistant to preventive treatments and to verify whether this is a useful finding for identifying patients with intracranial hypertension. Methods: This is an observational study. Patients with refractory CM and CTTH underwent a 3T-magnetic resonance venography (MRV) before cerebrospinal fluid (CSF) opening pressure measurement. Transverse sinus stenosis was determined using the combined conduit score. Patients with opening pressure >200 repeated MRV study 1 month after CSF withdrawal to evaluate changes in neuroimaging findings. Results: We analyzed MRV studies of 40 patients (32 F, 8 M; mean age, 49.4 ± 10.8; mean body mass index, 26.7 ± 6.4; 39 CM and 1 CTTH with concomitant episodic migraine). Nineteen cases (47.5%) had evidence of transverse sinus stenosis: bilateral in seven patients (17.5%) and unilateral in 12 cases (30%). No statistically significant differences in transverse sinus stenosis distribution were found between patients with opening pressure 200 mmH2O. On Spearman bivariate test, there was no correlation between opening pressure and combined conduit score. No changes in neuroimaging findings were found 1 month after CSF withdrawal. Conclusion: Transverse sinus stenosis is a frequent radiological finding (47.5%) in CM and CTTH patients refractory to preventive treatments. However, this finding is not suggestive of intracranial hypertension. Whether transverse sinus stenosis may be a possible risk factor for chronic headache or a comorbidity needs to be evaluated in larger epidemiological studies.

Giant intracranial aneurysms still represent a serious threat because of high mortality and morbidity and an absence of a widely accepted treatment. Herein, we illustrate the case of a 54-yr-old woman with progressive neurological decline who was found to have an unruptured, giant, partially thrombosed aneurysm of the left supraclinoid portion of internal carotid artery (ICA) with brainstem compression. In this video, we demonstrate the successful stepwise treatment of this lesion with endovascular embolization of the patent portion of the aneurysm, followed by microsurgical debulking and clipping. At 3-yr follow-up, the patient experienced a neurological improvement and performed all normal activity. For patients in which single-modality therapy does not assure a safe treatment option, multimodal management with endovascular and open techniques should be considered as a therapeutic option for complex giant intracranial aneurysms. Consent was given by the patient prior to the surgical procedure. Consent and approval for this operative video, in light of the retrospective nature and use of anonymized material, were waived.

// Alba A. Brandes 1 , Gaetano Finocchiaro 2 , Vittorina Zagonel 3 , Michele Reni 4 , Alessandra Fabi 5 , Claudia Caserta 6 , Alicia Tosoni 1 , Marica Eoli 2 , Giuseppe Lombardi 3 , Matteo Clavarezza 7 , Alexandro Paccapelo 1 , Stefania Bartolini 1 , Luigi Cirillo 8 , Raffaele Agati 8 and Enrico Franceschi 1 1 Department of Medical Oncology, Bellaria-Maggiore Hospitals, Azienda USL, IRCCS Institute of Neurological Sciences, Bologna, Italy 2 Molecular Neuro-Oncology Unit, IRCCS Foundation Carlo Besta, Milan, Italy 3 Department of Clinical and Experimental Oncology, Medical Oncology 1, Veneto Institute of Oncology, IRCCS, Padua, Italy 4 Department of Medical Oncology, IRCCS San Raffaele, Milan, Italy 5 Medical Oncology 1, Regina Elena National Cancer Institute, Rome, Italy 6 Oncology Department, Santa Maria Hospital, Terni, Italy 7 Medical Oncology Unit, Ente Ospedaliero Ospedali Galliera, Genova, Italy 8 Department of Neuroradiology, Bellaria Hospital, IRCCS Institute of Neurological Sciences, Bologna, Italy Correspondence to: Alba A. Brandes, email: // Keywords : glioblastoma, RANO, bevacizumab, fotemustine, ETS Received : January 21, 2017 Accepted : February 08, 2017 Published : February 25, 2017 Abstract BACKGROUND: Disease assessment for recurrent glioblastoma (GBM) represents a challenge, especially with the use of antiangiogenic agents. Moreover, validated neuroradiological predictors of outcome are lacking. Recently, the concept of early tumor shrinkage (ETS) has been developed to better assess the ability of treatments in determining a rapid and remarkable tumor response. The aim of the study was to evaluate the role of ETS in predicting survival of GBM patients treated with BEV METHODS: We examined the radiological data of patients with recurrent GBM treated with bevacizumab (BEV) or fotemustine (FTM) in the randomized phase II AVAREG trial (EudraCT: 2011-001363-46). Radiologic assessments at first disease assessment (day 46) were used to calculate the relative change in the sum of the products of perpendicular diameters of all measurable lesions determined by either T1 contrast and T2/FLAIR. RESULTS: In patients treated with BEV, the best ETS cut-off was reduction of 15% with T1 contrast and of 40% with T2/FLAIR. Adopting this cut-off for T1 contrast radiological changes, ETS was a significant predictor of OS for patients treated with BEV (HR = 0.511, 95%CI:0.269-0.971, p = 0.040). The cut-off obtained for T2/FLAIR was not significantly correlated with OS ( p = 0.102), but we found a trend for correlation with survival when considering the variable as continuous ( p = 0.058). CONCLUSIONS: ETS evaluating T1 contrast reduction is a helpful predictor of survival in patients with recurrent GBM treated with BEV, and if validated in a larger prospective trial could be a helpful surrogate endpoint.

Increasing evidence has demonstrated the bidirectional link between acute kidney injury (AKI) and chronic kidney disease (CKD) such that, in the clinical setting, the new concept of a unified syndrome has been proposed. The pathophysiological reasons, along with the cellular and molecular mechanisms, behind the ability of a single, acute, apparently self-limiting event to drive chronic kidney disease progression are yet to be explained. This acute injury could promote progression to chronic disease through different pathways involving the endothelium, the inflammatory response and the development of fibrosis. The interplay among endothelial cells, macrophages and other immune cells, pericytes and fibroblasts often converge in the tubular epithelial cells that play a central role. Recent evidence has strengthened this concept by demonstrating that injured tubules respond to acute tubular necrosis through two main mechanisms: The polyploidization of tubular cells and the proliferation of a small population of self-renewing renal progenitors. This alternative pathophysiological interpretation could better characterize functional recovery after AKI.

Background and objectives Nephrotic syndrome is a typical presentation of genetic podocytopathies but occasionally other genetic nephropathies can present as clinically indistinguishable phenocopies. We hypothesized that extended genetic testing followed by reverse phenotyping would increase the diagnostic rate for these patients. Design, setting, participants, & measurements All patients diagnosed with nephrotic syndrome and referred to our center between 2000 and 2018 were assessed in this retrospective study. When indicated, whole-exome sequencing and in silico filtering of 298 genes related to CKD were combined with subsequent reverse phenotyping in patients and families. Pathogenic variants were defined according to current guidelines of the American College of Medical Genetics. Results A total of 111 patients (64 steroid-resistant and 47 steroid-sensitive) were included in the study. Not a single pathogenic variant was detected in the steroid-sensitive group. Overall, 30% (19 out of 64) of steroid-resistant patients had pathogenic variants in podocytopathy genes, whereas a substantial number of variants were identified in other genes, not commonly associated with isolated nephrotic syndrome. Reverse phenotyping, on the basis of a personalized diagnostic workflow, permitted to identify previously unrecognized clinical signs of an unexpected underlying genetic nephropathy in a further 28% (18 out of 64) of patients. These patients showed similar multidrug resistance, but different long-term outcome, when compared with genetic podocytopathies. Conclusions Reverse phenotyping increased the diagnostic accuracy in patients referred with the diagnosis of steroid-resistant nephrotic syndrome.

Steroid-resistant nephrotic syndrome (SRNS) is a clinical picture defined by the lack of response to standard steroid treatment, frequently progressing toward end-stage kidney disease. The genetic basis of SRNS has been thoroughly explored since the end of the 1990s and especially with the advent of next-generation sequencing. Genetic forms represent about 30% of cases of SRNS. However, recent evidence supports the hypothesis that “phenocopies” could account for a non-negligible fraction of SRNS patients who are currently classified as non-genetic, paving the way for a more comprehensive understanding of the genetic background of the disease. The identification of phenocopies is mandatory in order to provide patients with appropriate clinical management and to inform therapy. Extended genetic testing including phenocopy genes, coupled with reverse phenotyping, is recommended for all young patients with SRNS to avoid unnecessary and potentially harmful diagnostic procedures and treatment, and for the reclassification of the disease. The aim of this work is to review the main steps of the evolution of genetic testing in SRNS, demonstrating how a paradigm shifting from “forward” to “reverse” genetics could significantly improve the identification of the molecular mechanisms of the disease, as well as the overall clinical management of affected patients.

Brainstem arteriovenous malformations (AVMs) are complex and life-threatening lesions. In our video (Video 1), we illustrate resection of a posterior midbrain hemorrhaged AVM (Spetzler-Martin grade III, Lawton-Young grade III, Supplementary Spetzler-Martin grade 6) in a 55-year-old woman presenting with ventricular hemorrhage and coma. Multimodal therapy with endovascular embolization of an associated prenidal aneurysm of the right posteromedial choroidal artery, followed by microsurgical resection via supracerebellar-infratentorial approach, is demonstrated. A total resection of the AVM was achieved. At the 4-year follow-up, the patient had a modified Rankin Scale score of 3. AVMs in eloquent brain regions require a carefully tailored, multimodal diagnostic and therapeutic approach to limit procedure-related complications and obtain acceptable outcomes. Herein, we illustrate such a situation and discuss some key points for success.

Background: To determine the prevalence of Idiopathic intracranial hypertension without papilledema (IIHWOP) testing revised diagnostic criteria by Friedman in refractory chronic headache (CH) patients. Methods: This is a prospective observational study. Each patient underwent ophthalmologic evaluation and Optical Coherence Tomography; brain magnetic resonance venography (MRV) and a lumbar puncture (LP) with opening pressure (OP) measurement. CSF withdrawal was performed in patients with CSF OP > 200 mmH20. IIHWOP was defined according Friedman's diagnostic criteria. Effect of CSF withdrawal was evaluated clinically in a 6-month follow-up and with a MRV study at 1 month. Results: Forty-five consecutive patients were enrolled. Five were excluded due to protocol violations. Analyses were conducted in 40 patients (32 F, 8 M; mean age 49.4 ± 10.8). None had papilledema. Nine patients (22.5%) had OP greater than 200 mmH2O, two of them above 250 mmH2O. Two (5%) had neuroimaging findings suggestive of elevated intracranial pressure. One of them (2.5%) met the newly proposed diagnostic criteria by Friedman for IIHWOP. After CSF withdrawal seven (77.8%) of the nine patients improved. No changes in neuroimaging findings were found. Conclusions: We found a low prevalence (2.5%) of IIHWOP in refractory CH patients according to current diagnostic criteria. In agreement with Friedman's criteria, our results confirm that a diagnosis of IIHWOP should be based on CSF OP and the combination of neuroradiological findings. However, where to set the CSF OP upper limit in IIHWOP needs further field testing. Although IIHWOP is a rare clinical condition, it should be considered and treated in refractory CH patients.

Background: Depression is the most common psychiatric disorder in long-term dialysis patients and a risk factor for morbidity and mortality. Although there is a relevance of the issue in the dialysis setting, we still know little about possible relationships between depression and uraemia-related biochemical abnormalities. Our aims were to evaluate (1) the prevalence of depression in our haemodialysis (HD) and peritoneal dialysis (PD) population using a validated and easy-to-implement screening tool and (2) the association between depression and the main uraemia-related clinical and biochemical parameter changes. Methods: In this monocentric cross-sectional study, all patients of our centre with at least 3 months of dialysis were screened by Patient Health Questionnaire-9 (PHQ-9), a self-administered depression-screening questionnaire validated in dialysis setting. The impact of depressive symptoms on daily life was also assessed. We then analysed relationships between the PHQ-9-derived depressive score, functional impairment score, demographic, clinical and laboratory variables. Results: In our cohort of 145 patients, depressive symptoms were found in 69 patients (46%). Stratifying for severity, mild, moderate and severe grade accounted for 31, 13 and 2% respectively. Depressive symptoms affected 36% of patients on PD versus 52% of patients on HD. Moreover, the PD patients had significantly less functional impairment derived from depressive symptoms than the HD patients. Simple and multiple regression analysis identified serum phosphorus as the only uraemia-related laboratory parameter that was high statistically associated with depressive score. Conclusions: Using a reliable, simple and fast tool, we found that depressive symptoms affect almost half of dialysis patients, particularly so the HD cohort. Severity of depressive symptoms seems related to serum levels of phosphorus possibly because depression affects compliance to therapy.

Purpose All percutaneous minimally invasive disc treatments are typically indicated to contained disc herniations. Our study’s aim is to evaluate prospectively the efficacy of ozone nucleolysis in the treatment of either contained or uncontained lumbar disc herniations. Methods Fifty-two patients, aged 27–87 years, with symptomatic herniated lumbar discs, without migration, sequestration, or severe degenerative disc changes, who failed conservative treatment, were included in our study. The patients underwent fluoroscopic-guided intradiscal oxygen-ozone mixture injection (5 ml) at a concentration of 27–30 μg/ml and periradicular injection of the same O2-O3 mixture (10 ml), steroid (1 ml), and local anesthetic (1 ml). Clinical outcomes were evaluated, based on the Oswestry Disability Index (ODI) and pain intensity (0–5) scale results, obtained initially and at 2- and 6-month controls. Our results were analyzed by ANOVA and chi-squared (χ 2) tests. Results Our initial results obtained at 2-month control were promising, indicating a significant decrease in pain disability and intensity in 74% (37) and 76% (38) of the patients respectively, and minimally increased to 76% (38) and 78% (39) at 6-month control (P ). The mean preprocedure ODI and pain intensity scores were 35 ± 14.36 and 2.38 ± 0.90, respectively, which were reduced to 19.36 ± 13.12 and 1.04 ± 0.92 at 6-month control. Our failure had been mostly related to long symptoms duration of more than 1 year. No complications were recorded. Conclusion Ozone nucleolysis is a safe cost-effective minimally invasive technique for treatment of contained and uncontained lumbar disc herniations.

The advent of flow diverter (FD) stents has deeply changed the endovascular treatment of cerebral aneurysms. Endosac- cular devices have been replaced by endoluminal stents and the management of FD-treated patients differs markedly from that of patients treated with metal coils. Whichever technique or devices are used, a thrombus forms within the aneurysmal sac after treatment. The ensuing events are well known: clot formation and inflammation develop in a physiological healing process that takes from months to years These new devices have also given rise to several unpre- dictable complications, including still unexplained fatal case of spontaneous rupture of treated aneurysms The mechanism of post-flow-diversion haemorrhage remains difficult to clearly understand: aneurysm thrombosis, fol lowed by mural inflammation, leading to autolysis and rupture has been described.Nevertheless thrombosis followed by organization and neo-intimal closure of the neck is required for successful occlusion of treated aneurysms, thus there must be further elements to consider for thrombosis leading to rupture rather than occlusion. This report describes the favourable outcome of a focal wall rupture affecting a large carotid-ophthalmic artery aneurysm treated with FD and coils deployment. It displays the potential interaction between thrombosis, inflammation and residual flow in the aneurysm.

Intradiscal oxygen-ozone (O2-O3) chemonucleolysis is a well-known effective treatment for pain caused by protruding disc disease and nerve root compression due to bulging or herniated disc. The most widely used therapeutic combination is intradiscal injection of an O2-O3 mixture (chemonucleolysis), followed by periradicular injection of O2-O3, steroid and local anaesthetic to enhance the anti-inflammatory and analgesic effect. The treatment is designed to resolve pain and is administered to patients without motor weakness, whereas patients with acute paralysis caused by nerve root compression undergo surgery 24–48h after the onset of neurological deficit. This paper reports on the efficacy of O2-O3 chemonucleolysis associated with anti-inflammatory foraminal injection in 13 patients with low back pain and cruralgia, low back pain and sciatica and subacute partial motor weakness caused by nerve root compression unresponsive to medical treatment. All patients were managed in conjunction with our colleagues in the Neurosurgery Unit of Bellaria Hospital and the IRCCS Institute of Neurological Sciences, Bologna. The outcomes obtained are promising: 100% patients had a resolution of motor weakness, while 84.6% had complete pain relief. Our results demonstrate that O2-O3 therapy can be considered a valid treatment option for this category of patients.

The published version of this article unfortunately contained a mistake. Affiliation 2 was presented incorrectly in the original article. The updated affiliation is Neuroradiology Unit, Bellaria Hospital, IRCCS Institute of Neurological Sciences, Bologna, Italy.

Endovascular treatment of giant, fusiform and dissecting aneurysms with flow diverter stents is becoming more and more popular. However, very few studies on the follow-up have been published. We describe a patient with a dissecting aneurysm of the right vertebral artery treated with flow diverter stent placement. The patient was followed up with CT angiography (CTA), time-resolved contrast-enhanced MR angiography (CE-MRA) and digital subtraction angiography (DSA). CTA had false negative results in two instances, whereas time-resolved CE-MRA and DSA were the most accurate in depicting the residual flow in the aneurysmal sac. However, in the case of DSA the demonstration of residual flow proved quite difficult and required a very thorough examination with oblique projections. Our 2.5-year experience with this patient led us to believe that time-resolved CE-MRA is a valuable tool in the follow-up of flow diverter-treated stents.

A multitude of therapies is available to treat disc herniation, ranging from conservative methods (medication and physical therapy) to minimally invasive (percutaneous) treatments and surgery. O2-O3 chemonucleolysis (O2-O3 therapy) is one of the minimally invasive treatments with the best cost/benefit ratio and lowest complication rate. Another substance recently made available exploiting the chemical properties of pure ethanol is DiscoGel®, a radiopaque gelified ethanol more viscous than absolute alcohol 8,9. The present study aimed to assess the therapeutic outcome of DiscoGel® chemonucleolysis in patients with lumbar disc herniation unresponsive to O2-O3 therapy. Thirty-two patients aged between 20 and 79 years were treated by DiscoGel® chemonucleolysis between December 2008 and January 2010. The treatment was successful (improvement in pain) in 24 out of 32 patients. DiscoGel® is safe and easy to handle and there were no complications related to product diffusivity outside the treatment site. The therapeutic success rate of DiscoGel® chemonucleolysis in patients unresponsive to O2-O3 therapy was satisfactory. Among other methods used to treat lumbar disc herniation, DiscoGel® chemonucleolysis can be deemed an intermediate procedure bridging conservative medical treatments and surgery.

The Silk stent (Balt, Montmorency, France) is a retractable device designed to achieve curative reconstruction of the parent artery associated with an intracranial aneurysm. We present our initial experience with the Silk flow-diverting stent in the management and follow-up of 25 patients presenting with intracranial aneurysms. Twenty-five patients (age range, 34–81 years; 24 female) were treated with the Silk flow-diverting device. Aneurysms ranged in size from small (5), large (10) and giant (10) and included wide-necked aneurysms, multiple, nonsaccular, and recurrent intracranial aneurysms. Nine aneurysms were treated for headache, 14 for mass effect. None presented with haemorrhage. All patients were pretreated with dual antiplatelet medications for at least 72 hours before surgery and continued taking both agents for at least three months after treatment. A total of 25 Silk stents were used. Control MR angiography and/or CT angiography was typically performed prior to discharge and at one, three, six and 12 months post treatment. A follow-up digital subtraction angiogram was performed between six and 19 months post treatment. Complete angiographic occlusion or subtotal occlusion was achieved in 15 patients in a time frame from three days to 12 months. Three deaths and one major complication were encountered during the study period. Two patients, all with cavernous giant aneurysms, experienced transient exacerbations of preexisting cranial neuropathies and headache after the Silk treatment. Both were treated with corticosteroids, and symptoms resolved completely within a month. In our experience the Silk stent has proven to be a valuable tool in the endovascular treatment of intracranial giant partially thrombosed aneurysms and aneurysms of the internal carotid artery cavernous segment presenting with mass effect. The time of complete occlusion of the aneurysms and the risk of the bleeding is currently not predictable.

Spinal dural arteriovenous fistulae (SDAVF) are the most common vascular malformations of the spine. Although digital subtraction angiography (DSA) remains the standard of reference to diagnose and classify vascular spinal lesions, we investigated the clinical value of contrast-enhanced MR angiography (CE-MRA), equipped with TRICKS sequences, in localizing SDAVF before selective catheter angiography and possible subsequent treatment. We studied 16 consecutive patients suspected of harbouring vascular spinal cord malformations and we tried to determine the level and the side of the arterial feeder to the arteriovenous abnormality. In 12 cases the results were compared with DSA and/or possible post-operative findings. In nine cases CE-MRA correctly depicted the origin of the fistula: in particular one patient was treated surgically only on the basis of MRA results. Thanks to its elevated spatial and temporal resolution, spinal contrast-enhanced MRA using TRICKS sequences proved reliable in detecting and localizing the SDAVF arterial feeders and can be used as a guide to subsequent selective DSA examination.

Background A diagnosis of idiopathic intracranial hypertension without papilledema (IIHWOP) should be considered in unresponsive chronic daily headache (CDH) patients [1]. A CSF opening pressure (OP) above 200 mm H2O has been detected in chronic migraine patients with conflicting result, ranging from 10% to 86% of patients [1,2]. Moreover, controversies exist regarding the OP cut-off value greater than 200 or 250 mm H2O and the role of transverse sinus stenosis (TSS) [3,4].

A 34-year-old man developed since age 18 years epilepsia partialis continua followed over the years by progressive ataxic hemiparesis involving the left side (figure 1). Structural cerebral abnormalities, mitochondrial dis- eases, and known autoimmune disorders were excluded. 1 Cerebral MRI showed progressive right cerebral and crossed left cerebellar hemiatrophy (figure 2, A and B). Tractography of cortico-ponto-cerebellar (CPC) path- ways 2 (figure 2, C and D) demonstrated marked involvement of the right tract, suggesting that a dying-back mechanism of efferent CPC fibers may cause crossed cerebellar atrophy. We infer that crossed cerebellar atro- phy is a distinctive feature of long-term Rasmussen encephalitis evolution.

Purpose: To compare 3 T elliptical-centric CE MRA with 3 T TOF MRA for the detection and characterizationof unruptured intracranial aneurysms (UIAs), by using digital subtracted angiography (DSA) as reference.Materials and methods: Twenty-nine patients (12 male, 17 female; mean age: 62 years) with 41 aneurysms(34 saccular, 7 fusiform; mean diameter: 8.85 mm [range 2.0-26.4 mm]) were evaluated with MRA at3 T each underwent 3D TOF-MRA examination without contrast and then a 3D contrast-enhanced (CE-MRA) examination with 0.1 mmol/kg bodyweight gadobenate dimeglumine and k-space elliptic mapping(Contrast ENhanced Timing Robust Angiography [CENTRA]). Both TOF and CE-MRA images were used toevaluate morphologic features that impact the risk of rupture and the selection of a treatment. Almosthalf (20/41) of UIAs were located in the internal carotid artery, 7 in the anterior communicating artery,9 in the middle cerebral artery and 4 in the vertebro-basilar arterial system.All patients also underwent DSA before or after the MR examination.Results: The CE-MRA results were in all cases consistent with the DSA dataset. No differences werenoted between 3D TOF-MRA and CE-MRA concerning the detection and location of the 41 aneurysmsor visualization of the parental artery. Differences were apparent concerning the visualization of mor-phologic features, especially for large aneurysms (>13 mm). An irregular sac shape was demonstratedfor 21 aneurysms on CE-MRA but only 13/21 aneurysms on 3D TOF-MRA. Likewise, CE-MRA permittedvisualization of an aneurismal neck and calculation of the sac/neck ratio for all 34 aneurysms with aneck demonstrated at DSA. Conversely, a neck was visible for only 24/34 aneurysms at 3D TOF-MRA. 3DCE-MRA detected 15 aneurysms with branches originating from the sac and/or neck, whereas brancheswere recognized in only 12/15 aneurysms at 3D TOF-MRA.Conclusion: For evaluation of intracranial aneurysms at 3 T, 3D CE-MRA is superior to 3D TOF-MRA forassessment of sac shape, detection of aneurysmal neck, and visualization of branches originating fromthe sac or neck itself, if the size of the aneurysm is greater than 13 mm. 3 T 3D CE-MRA is as accurate andeffective as DSA for the evaluation of UIAs. © 2013 Published by Elsevier Ireland Ltd.

We describe a rare case of giant intracranial chordoma, emphasizing the patient's long survival and his excellent response to radiotherapy that led to a progressive regression of neurological symptomatology up to disappearance, in the absence of cerebral white matter damages.

The occurrence of a pituitary adenoma located entirely outside the sella turcica, so-called ectopic adenoma, is extremely rare. We report a case of a non secreting-pituitary adenoma located above the diaphragma sellae, with no invasion into the sella turcica, confirmed at surgery. The tumor was initially treated unsuccessfully by operations via the transphenoidal route. After initial negative exploration by the transphenoidal route, the patient was successfully treated by an endoscopic endonasal transphenoidal approach extended to the tuberculum sellae and the posterior planum sphenoidale to access the suprasellar supraglandular region. A brief review of ectopic adenomas and a discussion of the preoperative diagnosis are presented.

Aim The optimal acquisition time for staging colo-rectal carcinoma with a contrast enhanced multidetector CT colonography (CE CTC) has not yet been established. A dual phase with both arterial and portal venous acquisition has been proposed. The purpose of our study is to assess the value of single portal venous phase CE CTC in the preoperative staging of colo-rectal carcinoma. Materials and methods Fifty two (30 M, 22 F; aged 35–82 years) consecutive patients with a histologically proven diagnosis of colo-rectal adenocarcinoma or a highly suspected colo-rectal cancer on conventional colonoscopy underwent a four-slice CE CTC. The procedure was performed 70 s (portal phase) after the intravenous bolus (3 ml/s) administration of 120 ml iodinated non-ionic contrast agent (370 mg iodine/ml). Scans were performed using the following parameters: 2.5 mm beam collimation, pitch 1.25, 120 kV, 200 mAs, rotation time 0.75 s. Images were reconstructed with an effective thickness of 3.2 mm at intervals of 1.6 mm. Two radiologists independently evaluated the depth of tumour invasion into the colo-rectal wall (T), regional lymph node involvement (N), and extracolonic metastases (M). Disagreement was resolved by means of a consensus decision. The pathological results served as the standard of reference. Assessment was made of sensitivity, specificity and accuracy, as well as positive and negative predictive values were assessed. Results CE CTC correctly staged the pT of 52/56 (93%) and the N of 40/56 (71%) lesions, as well as properly identifying 13/14 (93%) extracolonic findings. Conclusion The single portal venous phase CE CTC scanning protocol enables satisfactory preoperative assessment of T, N and M staging in patients with colo-rectal cancer.