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Several genetic investigations were conducted to identify germline and somatic mutations in somatotropinomas, a subtype of pituitary tumors. To our knowledge, we report the first acromegaly patient carrying a RET pathogenic variant: c.2410G>A (rs79658334), p.Val804Met. Alongside the fact that the patient’s father and daughter carried the same variant, we investigated the clinical significance of this variant in the context of somatotropinomas and other endocrine tumors, reviewing the RET mutations’ oncogenic mechanisms. The aim was to search for new targets to precisely manage and treat acromegaly. Our case describes a new phenotype associated with the RET pathogenic variant, represented by aggressive acromegaly, and suggests consideration for RET mutation screening if NGS for well-established PitNET-associated gene mutations renders negative.

Background: We aim to assess the role of a multidisciplinary approach in pituitary adenomas (PitNETs) classification, evaluate criteria concordance, and compare intraoperative assessments with post-operative MRIs for tumor remnants. Methods: Clinical, radiological, histological, and intra- and post-operative data of the treated PitNETs were extracted from prospectively created records. PitNETs were graded according to Trouillas, and the evaluation of the tumor remnants was recorded. Results: Of 362 PitNETs, 306 underwent surgery, with Trouillas grading assigned to 296. Eight-nine radiologically non-invasive PitNETs progressed to grades 1b (27), 2a (42), or 2b (20) due to proliferative or surgical invasiveness criteria. Twenty-six radiologically invasive tumors were graded 2b due to proliferative criteria. Surgical resection details and post-surgical MRI findings revealed that residual tumors were more common in grades 2a and 2b. During surgery, small tumor remnants were documented in 14 patients which were not visible on post-surgical MRI. Post-surgical MRIs identified remnants in 19 PitNETs not seen during surgery, located in lateral recesses of the sella (4), retrosellar (2), or suprasellar regions (7), along the medial wall of the cavernous sinus (6). Conclusions: The Pituitary Board allows for the correct grading of PitNETs to be obtained and an accurate identification of high-risk patients who should undergo closer surveillance due to tumor remnants.

Large language models (LLMs), especially those based on the Generative Pre-trained Transformer (GPT) architecture, have becomewidely popularand have beenappliedinvarious fields due to their ability to provide written responses to a diverse range of queries swiftly and accurately. LLMs have demonstrated a transformative and potentially revolutionary capacity in multiple medical subfields, including radiology.1 A promising utilisation of these models is to streamline free-text radiology reports into concise or structured formats, 2,3 thereby enhancing accessibility and organisation of extensive information, potentially facilitating communication among medical professionals. Furthermore, incorporating automated radiological structured reporting systems could enhance clinical procedures, standardising language across institutions, promoting effective communication among healthcare experts, and improving the efficiency of data extraction for research purposes. The present authors share their preliminary results with three LLMs evaluating their accuracy in extracting emergency data recognition within a human-generated emergency radiology report.

BackgroundThe glioblastoma's bad prognosis is primarily due to intra-tumor heterogeneity, demonstrated from several studies that collected molecular biology, cytogenetic data and more recently radiomic features for a better prognostic stratification. The GLIFA project (GLIoblastoma Feature Analysis) is a multicentric project planned to investigate the role of radiomic analysis in GB management, to verify if radiomic features in the tissue around the resection cavity may guide the radiation target volume delineation. Materials and methodsWe retrospectively analyze from three centers radiomic features extracted from 90 patients with total or near total resection, who completed the standard adjuvant treatment and for whom we had post-operative images available for features extraction. The Manual segmentation was performed on post gadolinium T1w MRI sequence by 2 radiation oncologists and reviewed by a neuroradiologist, both with at least 10 years of experience. The Regions of interest (ROI) considered for the analysis were: the surgical cavity +/- post-surgical residual mass (CTV_cavity); the CTV a margin of 1.5 cm added to CTV_cavity and the volume resulting from subtracting the CTV_cavity from the CTV was defined as CTV_Ring. Radiomic analysis and modeling were conducted in RStudio. Z-score normalization was applied to each radiomic feature. A radiomic model was generated using features extracted from the Ring to perform a binary classification and predict the PFS at 6 months. A 3-fold cross-validation repeated five times was implemented for internal validation of the model. ResultsTwo-hundred and seventy ROIs were contoured. The proposed radiomic model was given by the best fitting logistic regression model, and included the following 3 features: F_cm_merged.contrast, F_cm_merged.info.corr.2, F_rlm_merged.rlnu. A good agreement between model predicted probabilities and observed outcome probabilities was obtained (p-value of 0.49 by Hosmer and Lemeshow statistical te

BACKGROUND: The oculomotor cistern (OMC) is a cerebrospinal fluid space bound by meningeal layers that surrounds the oculomotor nerve as it crosses the oculomotor triangle to reach the lateral wall of the cavernous sinus at the level of the anterior clinoid process. Although several anatomical and radiological studies are available, its anatomy and relationship with pituitary adenomas (PAs) are still matter of discussion. - OBJECTIVE: The aim of the study is to provide an updated and focused overview of the OMC, highlighting the different perspectives and descriptions from anatomical, radiological, and clinical points of view. - METHODS: A scoping review was conducted up to 29th October 2022, according to PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews) criteria. PubMed, Web of Science, Scopus databases, and correlated citations were investigated. - RESULTS: Of the 562 records identified, 22 were included in the present analysis. There were 13, 5, and 4 anatomo-surgical, radiological, and clinical studies, respectively. Though there is general consensus on its definition, data are variable on different features of OMC. Defects or absence of dural layers adjacent to the oculomotor nerve were described in only 4 papers. The transition from meningeal to neural layers is still unclear. PAs with OMC involvement are poorly studied and have unique clinical characteristics. To date, 21 patients have been described; the reported prevalence of OMC involvement by PAs ranges from 4.1% to 14.6%.

Letter to the Editor

Recently, advances in molecular biology and bioinformatics have allowed a more thorough understanding of tumorigenesis in aggressive PitNETs (pituitary neuroendocrine tumors) through the identification of specific essential genes, crucial molecular pathways, regulators, and effects of the tumoral microenvironment. Target therapies have been developed to cure oncology patients refractory to traditional treatments, introducing the concept of precision medicine. Preliminary data on PitNETs are derived from preclinical studies conducted on cell cultures, animal models, and a few case reports or small case series. This study comprehensively reviews the principal pathways involved in aggressive PitNETs, describing the potential target therapies. A search was conducted on Pubmed, Scopus, and Web of Science for English papers published between 1 January 2004, and 15 June 2023. 254 were selected, and the topics related to aggressive PitNETs were recorded and discussed in detail: epigenetic aspects, membrane proteins and receptors, metalloprotease, molecular pathways, PPRK, and the immune microenvironment. A comprehensive comprehension of the molecular mechanisms linked to PitNETs' aggressiveness and invasiveness is crucial. Despite promising preliminary findings, additional research and clinical trials are necessary to confirm the indications and effectiveness of target therapies for PitNETs.

Predictive factors for response to regorafenib in recurrent glioblastoma, IDH-wildtype, are scarcely recognized. The objective of this study was to identify molecular predictive factors for response to regorafenib using a clinically available platform. We analyzed a prospective cohort of 30 patients harboring recurrent glioblastoma, IDH-wildtype, and treated with regorafenib. Next-generation sequencing (NGS) analysis was performed on DNA extracted from paraffin-embedded tissues using a clinically available platform. Moreover, MGMT methylation and EGFRvIII expression analyses were performed. Six-month progression-free survival (PFS) was 30% and median overall survival (OS) was 7.5 months, in line with literature data. NGS analysis revealed a mutation in the EGFR pathway in 18% of cases and a mutation in the mitogen-activated protein-kinase (MAPK) pathway in 18% of cases. In the remaining cases, no mutations were detected. Patients carrying MAPK pathway mutation had a poor response to regorafenib treatment, with a significantly shorter PFS and a nonsignificantly shorter OS compared to EGFR-mutated patients (for PFS, 2.5 vs 4.5 months, p = 0.0061; for OS, 7 vs 9 months, p = 0.1076). Multivariate analysis confirmed that MAPK pathway mutations independently predicted a shorter PFS after regorafenib treatment (p = 0.0188). The negative prognostic role of MAPK pathway alteration was reinforced when we combined EGFR-mutated with EGFRvIII-positive cases. Recurrent glioblastoma tumors with an alteration in MAPK pathway could belong to the mesenchymal subtype and respond poorly to regorafenib treatment, while EGFR-altered cases have a better response to regorafenib. We thus provide a molecular selection criterion easy to implement in the clinical practice.

Magnetic resonance imaging (MRI) is the gold standard for glioblastoma (GBM) patient evaluation. Additional non‐invasive diagnostic modalities are needed. GBM is heavily infiltrated with tumor‐associated macrophages (TAMs) that can be found in peripheral blood. FKBP51s supports alternative‐macrophage polarization. Herein, we assessed FKBP51s expression in circulating monocytes from 14 GBM patients. The M2 monocyte phenotype was investigated by qPCR and flow cytometry using antibodies against PD‐L1, CD163, FKBP51s, and CD14. MRI assessed morphologic features of the tumors that were aligned to flow cytometry data. PD‐L1 expression on circulating monocytes correlated with MRI tumor necrosis score. A wider expansion in circulating CD163/monocytes was measured. These monocytes resulted in a dramatic decrease in patients with an MRI diagnosis of complete but not partial surgical removal of the tumor. Importantly, in patients with residual tumor, most of the peripheral monocytes that in the preoperative stage were CD163/FKBP51s− had turned into CD163/FKBP51s+. After Stupp therapy, CD163/FKBP51s+ monocytes were almost absent in a case of pseudoprogression, while two patients with stable or true disease progression showed sustained levels in such circulating monocytes. Our work provides preliminary but meaningful and novel results that deserve to be confirmed in a larger patient cohort, in support of potential usefulness in GBM monitoring of CD163/FKBP51s/CD14 immunophenotype in adjunct to MRI.

Objective: Ability to thrive after invasive and intensive treatment is an important parameter to assess in patients with glioblastoma multiforme (GBM). Karnofsky Performance Status (KPS) is used to identify those patients suitable for postoperative radiochemotherapy. The aim of the present study is to investigate whether machine learning (ML)-based models can reliably predict patients' KPS 6 months after surgery. Methods: A cohort of 416 patients undergoing surgery for a histopathologically confirmed GBM were collected from a multicentric database and split into a training and hold-out test set in an 80:20 ratio. Worsening of KPS at 6 months after surgery (compared with preoperative KPS) occurred in 138 patients (33.2%). Relevant preoperative, intraoperative, and immediately postoperative variables were selected by a recursive features selection algorithm (Boruta) and used to build 2 ML-based predictive models. Results: A random forest classifier and a random forest regressor were trained to predict 6 months postoperative KPS as a categorical (worsening vs. stable/improving) and continuous variables; they achieved, respectively, an area under the curve of 0.81 (95% confidence interval, 0.76–0.84) and a mean absolute error of 4.4 (95% confidence interval, 4.0–4.7). Leveraging the predictive value resulting from the combination of independent variables, the random forest classifier outperformed conventional statistics (area under the curve improvement of +21%). Conclusions: Two robust ML-based prediction models were successfully trained and internally validated. Considerable effort remains to improve the interpretation of the results when these predictions are used in a patient-centered care context.

OBJECTIVE: Mild Traumatic Brain Injury (MTBI) in anticoagulated patients is a common challenge for Emergency Department (ED) Physicians. Anticoagulation is considered a risk factor for developing delayed intracranial hemorrhage (ICH) after MTBI. The occurrence of this event in patients on Vitamin K Antagonists (VKA) or Direct Oral Anticoagulants (DOACs) remains unclear. Primary endpoint: to analyze the role of anticoagulants as risk factors for developing delayed ICH after MTBI and evaluate the indications to repeat a cranial computed tomography (CT) after a period of observation. Secondary endpoint: to assess the difference in the prevalence rate of delayed ICH in patients on VKA versus those on DOACs. PATIENTS AND METHODS: We evaluated all consecutive patients admitted to our ED for MTBI, which had a control CT for late ICH after a negative CT at admission. We used a propensity score match (PSM) on factors affecting the need for oral anticoagulation to adjust the comparison between anticoagulated vs. non-anticoagulated patients for the baseline clinical characteristics. RESULTS: Among 685 patients enrolled, 15 (2.2%) developed ICH at control CT. After PSM, the incidence of ICH, although slightly higher, was not statistically different in anticoagulated patients vs. non-anticoagulated (2.3% vs. 0.6%, p=0.371). Among the 111 patients on VKA, 5 (4.5%) had a late ICH, compared to 4 out of 99 (4.0%) on DOACs; the difference was not statistically significant (p=0.868). CONCLUSIONS: The risk of developing delayed ICH after MTBI in patients on anticoagulation therapy is low. After correction for baseline covariates, the risk does not appear higher compared to non-anticoagulated patients. Thus, a routine control CT scan seems advisable only for patients presenting a clinical deterioration. Larger, prospective trials are required to clarify the safety profile of DOACs vs. VKA in MTBI.

Brain biopsy is the gold standard in order to establish the diagnosis of unresectable brain tumors. Few studies have investigated the long-term outcomes of biopsy patients. The aim of this single-institution-based study was to assess the concordance between radiological and histopathological diagnoses, and the long-term patient outcome. Ninety-three patients who underwent brain biopsy in the last 5 years were analyzed. We included patients treated with stereotactically guided needle, open, and neuroendoscopic biopsies. Most patients (86%) received needle biopsy. Gliomas and primary brain lymphomas comprised 88.2% of cases. The diagnostic yield was 95.7%. Serious complication and death rates were 3.2% and 2.1%, respectively. The concordance rate between radiological and histological diagnoses was 93%. Notably, the positive predictive value of radiological diagnosis of lymphoma was 100%. Biopsy allowed specific treatment in 72% of cases. Disease-related neurological worsening was the main reason that precluded adjuvant treatment. Adjuvant treatment, in turn, was the strongest prognostic factor, since the median overall survival was 11 months with vs. 2 months without treatment (p = 0.0002). Finally, advanced molecular evaluations can be obtained on glioma biopsy specimens to provide integrated diagnoses and individually tailored treatments. We conclude that, despite the huge advances in imaging techniques, biopsy is required when an adjuvant treatment is recommended, particularly in gliomas.

Background: JAM3 gene, located on human chromosome 11q25, encodes a member of the junctional adhesion molecule (JAM) family. Mutations of this gene are associated with hemorrhagic destruction of the brain, subependymal calcification, and congenital cataracts (HDBSCC). Case report: Herein, we present a newborn male with a prenatal suspicion of bilateral cataracts but without fetal ultrasound findings of cortical malformations. He was postnatally diagnosed with a clinical picture of HDBSCC and Early-onset Developmental and Epileptic Encephalopathy (DEE), associated to a homozygous variant of JAM3 gene. Conclusion: Identification of this variant in affected individuals has implications for perinatal and postnatal management and genetic counseling. To the best of our knowledge, this is the first case reported of a child with a JAM3 variant in Italy, from a different ethnic background than the other reported children until now (Saudi Arabian, Turkish, Afghani, and Moroccan origin). JAM3 screening could be requested in prenatal diagnosis of fetal congenital cataracts and included in Next-Generation DNA Sequencing panels.

This article is comment on: Vakrakou et al. Clinico-radiologic features and therapeutic strategies in tumefactive demyelination: a retrospective analysis of 50 consecutive cases. Ther Adv Neurol Disord 2021; 14: https://journals.sagepub.com/doi/10.1177/17562864211006503.

We describe the case of a male newborn presenting with a prenatal diagnosis of persistent hyperextension of the fetal neck and severe hypotonia and respiratory insufficiency at birth. Facial weakness, increased serum creatine kinase levels, and abnormal feeding, together with other signs, such as severe contractures, also classically associated with congenital myopathies prompted to perform a muscle biopsy showing internal rods suggestive of a possible nemaline myopathy. These findings suggest that a careful neurological examination should be performed in infants with persistent hyperextension of the fetal neck to exclude weakness and a possible underlying muscle disorder.

Introduction: We sought to verify the predicting role of a favorable profile on computed tomography perfusion (CTP) in the outcome of patients with acute ischemic stroke (AIS) due to large vessel occlusion (LVO) undergoing effective mechanical thrombectomy (MT). Methods: We retrospectively enrolled 25 patients with AIS due to LVO and with a CTP study showing the presence of ischemic penumbra who underwent effective MT, regardless of the time of onset. The controls were 25 AIS patients with overlapping demographics and clinical and computed tomography angiography features at admission who had undergone successful MT within 6 h from onset and without a previous CTP study. The outcome measure was the modified Rankin Scale (mRS) score at 90 days. Results: Sixty-four percent of the study patients had an mRS score of 0-1 at 90 days versus 12% of the control patients (p < 0.001). Patients of the study group had a more favorable distribution of disability scores (median mRS [IQR] score of 0 [0-2] vs. 2 [2-3]). Multivariate analysis showed that the selection of patients based on a favorable CTP study was strongly associated (p < 0.001) with a better neurological outcome. Conclusions: In our small-sized and retrospective study, the presence of ischemic penumbra was associated with a better clinical outcome in patients with AIS due to LVO after MT. In the future, a larger and controlled study with similar criteria of enrollment is needed to further validate the role of CTP in patient selection for MT, regardless of the time from the onset of symptoms.

Purpose: To assess the accuracy of dynamic susceptibility contrast-enhanced perfusion-weighted magnetic resonance imaging in glioma grading and brain tumor characterization of infratentorial tumors, and to investigate differences from supratentorial tumors. Methods: This retrospective study, approved by the institutional review board, included 246 patients with brain tumors (184 supratentorial, 62 infratentorial), grouped by tumor type: high-grade gliomas (HGG), low-grade gliomas (LGG), metastases (Met), and primary central nervous system lymphoma (PCNSL). Relative cerebral blood volume (rCBV) and mean percentage of signal recovery (PSR) were calculated. For statistical analyses, lesions were grouped by location and histology. Differences were tested with Mann–Whitney U tests. From ROC curves, we calculated accuracy, sensitivity, specificity, PPV, and NPV, for rCBV and PSR. Results: For infratentorial tumors, rCBV was highly accurate in differentiating HGG from LGG (AUC = 0.938). Mean PSR showed high accuracy in differentiating PCNSL and HGG from Met (AUC = 0.978 and AUC = 0.881, respectively). Infratentorial and supratentorial tumors had similarly high rCBV in HGG, high mean PSR in PCNSL, and low mean PSR in Met. The main differences were the optimum threshold rCBV values (3.04 for supratentorial, 1.77 for infratentorial tumors) and the mean PSR, which was significantly higher in LGG than in HGG in supratentorial (p = 0.035), but not infratentorial gliomas. Using infratentorial rCBV threshold values for supratentorial tumors decreased the sensitivity and specificity. Conclusion: rCBV and mean PSR were useful in grading and differentiating infratentorial tumors. Proper cutoff values were important in the accuracy of perfusion-weighted imaging in posterior fossa tumors.

Despite recent discoveries in genetics and molecular fields, glioblastoma (GBM) prognosis still remains unfavorable with less than 10% of patients alive 5 years after diagnosis. Numerous studies have focused on the research of biological biomarkers to stratify GBM patients. We addressed this issue in our study by using clinical/molecular and image data, which is generally available to Neurosurgical Departments in order to create a prognostic score that can be useful to stratify GBM patients undergoing surgical resection. By using the random forest approach [CART analysis (classification and regression tree)] on Survival time data of 465 cases, we developed a new prediction score resulting in 10 groups based on extent of resection (EOR), age, tumor volumetric features, intraoperative protocols and tumor molecular classes. The resulting tree was trimmed according to similarities in the relative hazard ratios amongst groups, giving rise to a 5-group classification tree. These 5 groups were different in terms of overall survival (OS) (p < 0.000). The score performance in predicting death was defined by a Harrell’s c-index of 0.79 (95% confidence interval [0.76–0.81]). The proposed score could be useful in a clinical setting to refine the prognosis of GBM patients after surgery and prior to postoperative treatment.

Objective: We studied the correlation of magnetic resonance imaging (MRI) findings with the prognosis of patients with multiple sclerosis (MS)-related trigeminal neuralgia (TN) who had undergone percutaneous balloon compression (PBC). To the best of our knowledge, no studies have previously reported on this topic. We also performed a review of pertinent studies. Methods: From a surgical series of 82 patients with TN who had undergone PBC from January 2013 to June 2017, we identified 16 patients with MS (mean age, 54.81 ± 8.51 years; mean follow-up, 38.12 ± 19.86 months). Using brain MRI, we evaluated the presence of a lesion in the trigeminal nerve root entry zone and trigeminal nuclei. With other clinical and procedure-related factors, we studied its effect on acute pain relief (pain free at hospital discharge) and pain-free survival (PFS). Results: Of the 16 patients, 15 (93.75%) reported acute pain relief; 8 patients (50.00%) experienced a recurrence of pain. At the latest follow-up visit, an excellent outcome (Barrow Neurological Institute grade 1–2) was obtained for 75% of patients and a good outcome (Barrow Neurological Institute grade 3) for 25%. No patient experienced uncontrolled pain. The presence of a pear-like balloon shape during surgery (P = 0.0053) and a MRI detectable lesion in the trigeminal pathway (P = 0.049) were associated with greater PFS. Conclusions: PBC can be efficient in treating MS-related TN. Some factors, such as a pear-like balloon shape during surgery and evidence of a MRI-detectable lesion in the trigeminal pathway were associated with longer PFS.

BACKGROUND AND PURPOSE: Dynamic susceptibility contrast MR perfusion imaging has limited results in children due to difficulties in reproducing technical standards derived from adults. This prospective, multicenter study aimed to determine DSC feasibility and quality in children using custom administration of a standard dose of gadolinium. MATERIALS AND METHODS: Eighty-three consecutive children with brain tumors underwent DSC perfusion with a standard dose of gadobutrol administered by an automated power injector. The location and size of intravenous catheters and gadobutrol volume and flow rates were reported, and local and/or systemic adverse effects were recorded. DSC was qualitatively evaluated by CBV maps and signal intensity-time curves and quantitatively by the percentage of signal drop and full width at half-maximum, and the data were compared with the standards reported for adults. Quantitative data were grouped by flow rate, and differences among groups were assessed by analysis of covariance and tested for statistical significance with a t test. RESULTS: No local or systemic adverse events were recorded independent of catheter location (63 arm, 14 hand, 6 foot), size (24-18 ga), and flow rates (1-5 mL/s). High-quality CBV maps and signal intensity-time curves were achieved in all patients, and quantitative evaluations were equal or superior to those reported for adults. No significant differences (P ≥ .05) were identified among the higher-flow-rate groups in the quantitative data. CONCLUSIONS: A custom administration of a standard dose of gadobutrol allows safe and high-quality DSC MR perfusion imaging in children.

A 36-year-old female patient was referred to our hospital for a six-months clinical history of episodes of right-sided focal motor seizures and absence. MRI scan showed a multi-nodular lesion in the subcortical white matter of left superior temporal gyrus. After a complete resection, the neuropathological examination revealed a lesion with marked cell vacuolation which was composed of neuroepithelial cells ranging from small to medium sized elements to larger, neuron-like cells, occasionally bi-nucleated. The neuropathological features of the lesion were compatible with the diagnosis of multinodular and vacuolating neuronal tumor (MVNT) of the CNS. MVNT is very rare, recently described entity among the large spectrum of glioneuronal tumors. It is characterized by benign clinical behavior and neurosurgical resection usually provide control of disease.

PURPOSE: Craniosynostostic syndromes are due to multisuture synostoses and affect the entire craniofacial skeleton. This study analyzed the facial complex and airways to quantify the relationship between insufficient facial growth, airways obstruction, and the sutural pattern of the splanchnocranium and cranial fossae. METHODS: Preoperative high-resolution CT images in 19 infants with syndromic craniosynostosis were quantitatively analyzed. Because all children showed involvement of minor sutures/synchondroses coursing in the posterior cranial fossa, they were divided into three groups according to the synostotic involvement of "minor" sutures/synchondroses coursing in anterior (ACF) and middle (MCF) cranial fossae: group 1 (ACF), group 2 (MCF), and group 3 (ACF-MCF). Analysis of the facial complex and airway was performed. Each group was compared with age-matched healthy subjects. RESULTS: Premature closure of skull base synchondroses of ACF and MCF was found only in groups MCF and ACF-MCF. Group MCF showed synostosis in the posterior branch of the coronal ring and reduced anterior hemifossae lengths while group ACF-MCF showed synostosis in the anterior branch of the coronal ring and reduced middle hemifossae lengths. No group showed reduced maxillary or mandibular volumes but group MCF showed synostosis of the zygomaticomaxillary sutures and maxillary retrusion. All groups showed reduced airway volume but group 2 had a higher degree of airway hypoplasia. CONCLUSION: The skull base synostotic process drives the changes in facial complex growth and airway obstruction. Premature closure of synchondroses/sutures in the posterior branch of the coronal ring causes insufficient facial growth, maxillary retrusion, and more severe airway reduction.

We report on a symptomatic case in which the whole intracranial blood supply was provided by a single vertebral artery as both internal carotid arteries were occluded and the contralateral vertebral artery was severely hypoplasic. The patient was treated by a flow-augmentation extracranial-intracranial bypass. Preoperative perfusion studies were essential in tailoring surgical strategy. Keypoints of the paper are contralateral perfusion changes after unilateral bypass surgery. The patient experienced a total recovery from symptoms and a bilateral improvement in brain perfusion, probably as consequence of post-operative hemodynamic rearrangement.

Vertebral hemangiomas (VHs) are a frequent and often incidental finding on computed tomography (CT) and magnetic resonance (MR) imaging of the spine. When their imaging appearance is "typical" (coarsened vertical trabeculae on radiographic and CT images, hyperintensity on T1- and T2-weighted MR images), the radiological diagnosis is straightforward. Nonetheless, VHs might also display an "atypical" appearance on MR imaging because of their histological features (amount of fat, vessels, and interstitial edema). Although the majority of VHs are asymptomatic and quiescent lesions, they can exhibit active behaviors, including growing quickly, extending beyond the vertebral body, and invading the paravertebral and/or epidural space with possible compression of the spinal cord and/or nerve roots ("aggressive" VHs). These "atypical" and "aggressive" VHs are a radiological challenge since they can mimic primary bony malignancies or metastases. CT plays a central role in the workup of atypical VHs, being the most appropriate imaging modality to highlight the polka-dot appearance that is representative of them. When aggressive VHs are suspected, both CT and MR are needed. MR is the best imaging modality to characterize the epidural and/or soft-tissue component, helping in the differential diagnosis. Angiography is a useful imaging adjunct for evaluating and even treating aggressive VHs. The primary objectives of this review article are to summarize the clinical, pathological, and imaging features of VHs, as well as the treatment options, and to provide a practical guide for the differential diagnosis, focusing on the rationale assessment of the findings from radiography, CT, and MR imaging.

This study aims to identify the premature synostosis of "major" and "minor" sutures of the four "sutural arches" of the skull and to perform a morphometric analysis in children with syndromic craniosynostosis in order to evaluate changes in the skull base linked with premature suture synostosis.

Craniosynostosis is a condition characterised by the premature fusion of one or more of the cranial sutures. The aim of the study was to identify, by multidetector computed tomography (CT), the involvement of vault sutures as well as of the skull base sutures (named "minor" sutures). The latter ones are involved in development of craniofacial and skull base deformities.

Introduction MRI abnormalities in the postictal period might represent the effect of the seizure activity, rather than its structural cause. Material and Methods Retrospective review of clinical and neuroimaging charts of 26 patients diagnosed with seizure-related MR-signal changes. All patients underwent brain-MRI (1.5-Tesla, standard pre- and post-contrast brain imaging, including DWI-ADC in 19/26) within 7 days from a seizure and at least one follow-up MRI, showing partial or complete reversibility of the MR-signal changes. Extensive clinical work-up and follow-up, ranging from 3 months to 5 years, ruled out infection or other possible causes of brain damage. Seizure-induced brain-MRI abnormalities remained a diagnosis of exclusion. Site, characteristics and reversibility of MRI changes, and association with characteristics of seizures were determined. Results MRI showed unilateral (13/26) and bilateral abnormalities, with high (24/26) and low (2/26) T2-signal, leptomeningeal contrast-enhancement (2/26), restricted diffusion (9/19). Location of abnormality was cortical/subcortical, basal ganglia, white matter, corpus callosum, cerebellum. Hippocampus was involved in 10/26 patients. Reversibility of MRI changes was complete in 15, and with residual gliosis or focal atrophy in 11 patients. Reversibility was noted between 15 and 150 days (average, 62 days). Partial simple and complex seizures were associated with hippocampal involvement (p=0.015), status epilepticus with incomplete reversibility of MRI abnormalities (p=0.041). Conclusions Seizure or epileptic status can induce transient, variably reversible MRI brain abnormalities. Partial seizures are frequently associated with hippocampal involvement and status epilepticus with incompletely reversible lesions. These seizure-induced MRI abnormalities pose a broad differential diagnosis; increased awareness may reduce the risk of misdiagnosis and unnecessary intervention.

Hydrocephalus affects 7% to 10% of patients with Chiari I malformation (CIM). It can be successfully treated by endoscopic third ventriculostomy (ETV), possibly improving related CIM and syringomyelia.

Background. This study was conceived to explore the correspondence between scintigraphic imaging of the sympathetic innervation of human postischemic left ventricular aneurysms and direct immunohistochemical localization of the nerve fibers in the same area. Methods and Results. In 7 patients undergoing left ventricular aneurysmectomy for postischemic ventricular aneurysm, the findings of thallium 201 and metaiodobenzylguanidine myocardial scintigraphy were compared with direct immunohistochemical localization of the nerve fibers in the same area. This comparison showed good correspondence between scintigraphic and immunohistochemical data, although scintigraphy failed to detect areas of minimal sympathetic innervation. Moreover, microscopic analysis showed sympathetic nerve fibers with peculiar morphology and distribution in the aneurysmal zone. Conclusions. There is a good correspondence between immunohistochemical and scintigraphic imaging in the detection of sympathetic cardiac nerves in human left ventricular aneurysms; a morphologically abnormal sympathetic reinnervation can be found in the aneurysmal area (although denervation can persist in some zones).