Long-Term Survival and Factors Associated with Increased Mortality in Patients with Ocular Adnexal Lymphomas.

Orbital and ocular adnexal lymphoma (OAL) affects the orbit and the surrounding structures and can arise as several subtypes with variable prognoses. We performed an observational study on the relationship between OAL subtype, diagnostic features, and prognosis to offer valuable insights into imaging techniques, such as Positron Emission Tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with Computed Tomography (18F-FDG PET-CT), in predicting outcomes. With this aim, we retrospectively reviewed 99 patients with OALs, recording demographics, cancer subtype, location and treatment, 18FDG avidity, and bone marrow positivity. We divided patients into Group 1 (those presenting with extranodal marginal zone lymphoma-EMZL) and Group 2, including all other subtypes. The primary outcome was long-term cancer-specific survival (CSS) based on key predictors, performed through Kaplan-Meier curves and the log-rank test, with a p < 0.05 significance threshold. The mean patient age was 67 years (57-75.5). The most frequent histopathologic subtypes were EMZL lymphoma in 69 patients (69.7%), small lymphocytic lymphoma (11.1%) and diffuse-large B-cell lymphoma (10.1%). Patients of Group 1 showed a better prognosis (CSS = 80%) compared to those of Group 2 (CSS = 60%) (p = 0.01). In patients with high-grade lymphoma, the occurrence of 18FDG avidity (p = 0.003) and bone marrow positivity (p = 0.005) were related to a worse prognosis. In our group, EMZL was the most prominent subtype of OALs and exhibited the best prognosis, low 18FDG avidity, and bone marrow negativity. By observing specific patterns in radiological findings, it is possible to increase our understanding of disease progression, treatment response, and the overall prognosis in OAL patients.

A pharmacoeconomic analysis from Italian guidelines for the management of prolactinomas.

Background: Prolactinoma, the most common pituitary adenoma, is usually treated with dopamine agonist (DA) therapy like cabergoline. Surgery is second-line therapy, and radiotherapy is used if surgical treatment fails or in relapsing macroprolactinoma. Objective: This study aimed to provide economic evidence for the management of prolactinoma in Italy, using a cost-of-illness and cost-utility analysis that considered various treatment options, including cabergoline, bromocriptine, temozolomide, radiation therapy, and surgical strategies. Methods: The researchers conducted a systematic literature review for each research question on scientific databases and surveyed a panel of experts for each therapeutic procedure's specific drivers that contributed to its total cost. Results: The average cost of the first year of treatment was €2,558.91 and €3,287.40 for subjects with microprolactinoma and macroprolactinoma, respectively. Follow-up costs from the second to the fifth year after initial treatment were €798.13 and €1,084.59 per year in both groups. Cabergoline had an adequate cost-utility profile, with an incremental cost-effectiveness ratio (ICER) of €3,201.15 compared to bromocriptine, based on a willingness-to-pay of €40,000 per quality-adjusted life year (QALY) in the reference economy. Endoscopic surgery was more cost-effective than cabergoline, with an ICER of €44,846.64. Considering a willingness-to-pay of €40,000/QALY, the baseline findings show cabergoline to have high cost utility and endoscopic surgery just a tad above that. Conclusions: Due to the favorable cost-utility profile and safety of surgical treatment, pituitary surgery should be considered more frequently as the initial therapeutic approach. This management choice could lead to better outcomes and an appropriate allocation of healthcare resources.

Italian Guidelines for the Management of Prolactinomas.

INTRODUCTION: This guideline (GL) is aimed at providing a reference for the management of prolactin (PRL)-secreting pituitary adenoma in adults. However, pregnancy is not considered. METHODS: This GL has been developed following the methods described in the Manual of the Italian National Guideline System. For each question, the panel appointed by Associazione Medici Endocrinologi (AME) has identified potentially relevant outcomes, which have then been rated for their impact on therapeutic choices. Only outcomes classified as "critical" and "important" have been considered in the systematic review of evidence and only those classified as "critical" have been considered in the formulation of recommendations. RESULTS: The present GL provides recommendations regarding the role of pharmacological and neurosurgical treatment in the management of prolactinomas. We recommend cabergoline (Cab) vs. bromocriptine (Br) as the firstchoice pharmacological treatment to be employed at the minimal effective dose capable of achieving the regression of the clinical picture. We suggest that medication and surgery are offered as suitable alternative first-line treatments to patients with non-invasive PRL-secreting adenoma, regardless of size. We suggest Br as an alternative drug in patients who are intolerant to Cab and are not candidates for surgery. We recommend pituitary tumor resection in patients 1) without any significant neuro-ophthalmologic improvement within two weeks from the start of Cab, 2) who are resistant or do not tolerate Cab or other dopamine-agonist drugs (DA), 3) who escape from previous efficacy of DA, and 4) who are unwilling to undergo a chronic DA treatment. We recommend that patients with progressive disease notwithstanding previous tumor resection and ongoing DA should be managed by a multidisciplinary team with specific expertise in pituitary diseases using a multimodal approach that includes repeated surgery, radiotherapy, DA, and possibly, the use of temozolomide. CONCLUSION: The present GL is directed to endocrinologists, neurosurgeons, and gynecologists working in hospitals, in territorial services or private practice, and to general practitioners and patients.

Vasari Scoring System in Discerning between Different Degrees of Glioma and IDH Status Prediction: A Possible Machine Learning Application?

(1) The aim of our study is to evaluate the capacity of the Visually AcceSAble Rembrandt Images (VASARI) scoring system in discerning between the different degrees of glioma and Isocitrate Dehydrogenase (IDH) status predictions, with a possible application in machine learning. (2) A retrospective study was conducted on 126 patients with gliomas (M/F = 75/51; mean age: 55.30), from which we obtained their histological grade and molecular status. Each patient was analyzed with all 25 features of VASARI, blinded by two residents and three neuroradiologists. The interobserver agreement was assessed. A statistical analysis was conducted to evaluate the distribution of the observations using a box plot and a bar plot. We then performed univariate and multivariate logistic regressions and a Wald test. We also calculated the odds ratios and confidence intervals for each variable and the evaluation matrices with receiver operating characteristic (ROC) curves in order to identify cut-off values that are predictive of a diagnosis. Finally, we did the Pearson correlation test to see if the variables grade and IDH were correlated. (3) An excellent ICC estimate was obtained. For the grade and IDH status prediction, there were statistically significant results by evaluation of the degree of post-contrast impregnation (F4) and the percentage of impregnated area (F5), not impregnated area (F6), and necrotic (F7) tissue. These models showed good performances according to the area under the curve (AUC) values (>70%). (4) Specific MRI features can be used to predict the grade and IDH status of gliomas, with important prognostic implications. The standardization and improvement of these data (aim: AUC > 80%) can be used for programming machine learning software.

Neoplasms and tumor-like lesions of the sellar region: imaging findings with correlation to pathology and 2021 WHO classification.

The sellar region represents a complex anatomical area, composed of multiple structures of different embryological derivation, including the skull base and the pituitary gland, along with vascular, nervous, and meningeal structures. Masses arising in this region include benign and malignant lesions arising from the pituitary gland itself, but also from vestigial embryological residues or surrounding tissues, that may require different therapeutic approaches. While assessing sellar region masses, the combination of clinical presentation and imaging features is fundamental to define hypotheses about their nature. MR represents the imaging modality of choice, providing information about the site of the lesion, its imaging features, and relation with adjacent structures, while CT is useful to confirm the presence of lesion calcifications or to reveal tumor invasion of bony structures. The aim of this pictorial review is to provide an overview of the common neoplasms and tumor-like conditions of the sellar region, according to the 2021 WHO Classification of Tumors of the Central Nervous System (fifth edition), with an emphasis on the radiologic-pathologic correlation. After a brief introduction on the anatomy of this region and the imaging and pathological techniques currently used, the most relevant MRI characteristics, clinical findings, and pathological data, including histologic and molecular features, will be shown and discussed, with the aim of facilitating an appropriate differential diagnosis among these entities.

Clinical correlates of R1 relaxometry and magnetic susceptibility changes in multiple sclerosis: a multi-parameter quantitative MRI study of brain iron and myelin.

OBJECTIVES: The clinical impact of brain microstructural abnormalities in multiple sclerosis (MS) remains elusive. We aimed to characterize the topography of longitudinal relaxation rate (R1) and quantitative susceptibility (χ) changes, as indices of iron and myelin, together with brain atrophy, and to clarify their contribution to cognitive and motor disability in MS. METHODS: In this cross-sectional study, voxel-based morphometry, and voxel-based quantification analyses of R1 and χ maps were conducted in gray matter (GM) and white matter (WM) of 117 MS patients and 53 healthy controls. Voxel-wise between-group differences were assessed with nonparametric permutation tests, while correlations between MRI metrics and clinical variables (global disability, cognitive and motor performance) were assessed both globally and voxel-wise within clusters emerging from the between-group comparisons. RESULTS: MS patients showed widespread R1 decrease associated with more limited modifications of χ, with atrophy mainly involving deep GM, posterior and infratentorial regions (p < 0.02). While R1 and χ showed a parallel reduction in several WM tracts (p < 0.001), reduced GM R1 values (p < 0.001) were associated with decreased thalamic χ (p < 0.001) and small clusters of increased χ in the caudate nucleus and prefrontal cortex (p < 0.02). In addition to the atrophy, χ values in the cingulum and corona radiata correlated with global disability and motor performance, while focal demyelination correlated with cognitive performance (p < 0.04). CONCLUSIONS: We confirmed the presence of widespread R1 changes, involving both GM and WM, and atrophy in MS, with less extensive modifications of tissue χ. While atrophy and χ changes are related to global and motor disability, R1 changes are meaningful correlates of cognition. KEY POINTS: • Compared to healthy controls, multiple sclerosis patients showed R1 and χ changes suggestive of iron increase within the basal ganglia and reduced iron and myelin content within (subnuclei of) the thalamus. • Thalamic volume and χ changes significantly predicted clinical disability, as well as pulvinar R1 and χ changes, independently from atrophy. • Atrophy-independent R1 and χ changes, suggestive of thalamic iron and myelin depletion, may represent a sensitive marker of subclinical inflammation.

Extra-Neural Metastases of Late Recurrent Myxopapillary Ependymoma to Left Lumbar Paravertebral Muscles: Case Report and Review of the Literature.

Ependymomas are commonly classified as low-grade tumors, although they may harbor a malignant behavior characterized by distant neural dissemination and spinal drop metastasis. Extra-CNS ependymoma metastases are extremely rare and only few cases have been reported in the lung, lymph nodes, pleura, mediastinum, liver, bone, and diaphragmatic, abdominal, and pelvic muscles. A review of the literature yielded 14 other case reports metastasizing outside the central nervous system, but to our knowledge, no studies describe metastasis in the paravertebral muscles. Herein, we report the case of a 39-year-old patient with a paraspinal muscles metastasis from a myxopapillary ependymoma. The neoplasm was surgically excised and histologically and molecularly analyzed. Both the analyses were consistent with the diagnosis of muscle metastases of myxopapillary ependymoma. The here-presented case report is first case in the literature of a paraspinal muscles metastasis of myxopapillary ependymoma.

The Fate of Fat Graft in Extended Endoscopic Transtuberculum-Transplanum Approaches.

OBJECTIVE: To analyze the long-term fate of autologous fat graft in skull base reconstruction after an extended endoscopic transtuberculum-transplanum approach. METHODS: Data from 98 consecutive patients undergoing a transtuberculum-transplanum approach and skull base reconstruction using the 3F technique between June 2017 and January 2022 were retrospectively analyzed. Fat graft volume was measured on postoperative day 1 computed tomography scan and early (≤15 days), 3-month, and 1-year magnetic resonance imaging scans. Fat graft volumes and resorption rate were calculated in patients with a complete radiological follow-up and correlated to demographic, pathological, and surgical features. RESULTS: Fat volumes and resorption rate were calculated in 55 patients. Mean volume of fat on postoperative day 1 computed tomography scan was 3.58 ± 1.89 cm3 and on early, 3-month, and 1-year postoperative magnetic resonance imaging scans was 2.45 ± 1.57 cm3, 1.40 ± 0.76 cm3, and 0.92 ± 0.56 cm3, respectively. Resorption rate was 44% after 3 months and 67% after 1 year. Resorption rate did not significantly correlate with demographic, pathological, and surgical features. Cerebrospinal fluid leak requiring redo surgery occurred in 1 patient (1/98, 1.2%). There were 6 cases of visual worsening (6/98, 6.1%); no cases correlated to fat overpacking. No delayed complications at the donor site or at the grafting site were noted. CONCLUSIONS: Autologous fat graft in skull base reconstruction is observed to shrink significantly over time, reaching 67% in 1 year. Its use is associated with excellent outcomes, making it a favored material for skull base reconstruction.

Diagnosis and management of intralabyrinthine schwannoma: case series and review of the literature.

Intralabyrinthine schwannoma (ILS) is a rare benign tumor affecting cochlear and vestibular nerves, whose symptoms are generally unspecific and frequently responsible for a late diagnosis. Radiological examinations, with particular reference to magnetic resonance imaging (MRI), represent the only diagnostic technique to identify ILS. On computed tomography ILS can only be indirectly suspected by the presence of surrounding bone remodeling, whereas MRI provides direct visualization of the neoplasm as a filling defect within the labyrinth with vivid contrast enhancement. At the same time, MRI is also helpful in defining ILS anatomical extension into adjacent structures and in planning therapeutic management. Here we report three representative cases of ILS with new pictorial imaging features to improve ILS early detection and optimize subsequent therapeutic management.

Upper Eyelid Isolated Arterio-Venous Malformation Treated With Embolization in a Patient With Keloid-Prone Skin.

Ocular adnexal aterio-venous malformations (AVMs) are rare congenital disabling anomalies, which may enlarge causing disfiguring deformity and rarely severe hemorrhage. These lesions are generally treated by preliminary endovascular embolization to shrink the arterio-venous malformation, followed by surgical gross total resection. The authors report a case of eyelid arterio-venous malformation in a 12-year-old girl, which progressively increased in size in few months. The patient complained mild itching, blurring of the vision, and mild tenderness. Magnetic resonance imaging showed an expansive mass with multiple arterial vessels at the left superior eyelid and left forehead. The diagnosis of arterio-venous malformation was then confirmed by digital subtraction angiography. Primary surgical excision was excluded because of the high risk of intrasurgical bleeding. Embolization through superselective cannulation of the left external carotid feeder vessels was performed resulting in flow exclusion up to the 80% of the nidus. Subsequent surgical resection was not recommended due to clinical evidence of keloid-prone skin.

Pituitary magnetic resonance imaging vs. bilateral inferior petrosal sinus sampling: comparison between non-invasive and invasive diagnostic techniques for Cushing's disease-a narrative review.

Cushing's syndrome is a pathological clinical condition caused by an exposure of elevated cortisol levels over a long period of time. It is therefore essential to establish what the cause of hypercortisolism is. In most cases (about 80%) the pathological process is due to adrenocorticotropic hormone (ACTH), while in a minor part of the cases (about 20%) the cause is represented by a pathology of the adrenal glands and therefore not related to ACTH. Most patients with ACTH dependent Cushing's syndrome have a pituitary microadenoma; in the remaining cases (30%), the high level of cortisol is linked to an ectopic secretion of ACTH. Surgical removal of the pituitary adenoma represents the treatment of choice in Cushing's disease (CD) patients; it is therefore necessary to identify and precisely locate the pituitary tumour responsible for the secretion of ACTH. Adequate diagnostic information is very often, even with magnetic resonance imaging (MRI), and in these cases we rely on bilateral inferior petrosal sinuses sampling (BIPSS). This procedure is considered the gold standard method for the diagnosis, but like any other diagnostic method it is not free from erroneous results such as false positives or false negatives.

Imaging findings in hypophysitis: a review.

Hypophysitis (HP) is a rare acute or chronic inflammatory condition of the pituitary gland. The greatest challenge in the management of HP is establishing a diagnosis through clinical criteria and non-invasive methods and predicting the patients' clinical outcome. The aim of this review is to describe the neuroradiological findings of this rare disease, providing some information regarding the possible differential diagnosis in order to avoid unnecessary surgery. Gadolinium-enhanced pituitary magnetic resonance imaging (MRI) is considered the neuroradiological investigation of choice. The features suggestive for HP include an enlarged triangular- or dumbbell-shaped gland with a thickened and not obviously deviated stalk, further supported by the absence of posterior pituitary bright spot on T1weighted images, particularly in patients presenting with diabetes insipidus. Contrast enhancement pattern is quite variable; dural enhancement has been reported in some cases after intravenous contrast administration. The characterization of the unusual sellar mass is not straightforward and generally results in a wide differential. HP should be primarily differentiated from pituitary adenomas (including pituitary apoplexy), from pituitary metastases, and from other sellar and parasellar tumors, e.g., craniopharyngiomas, germinomas, gliomas, lymphomas, meningiomas, pituicytomas, chordomas, teratomas, dermoids and epidermoids, Rathke's cleft cysts, and abscesses. In patients suspected for secondary forms related to systemic pathology, additional imaging is helpful in identifying other involved sites. Neuroradiologists need to know MRI appearance of this rare disease, as well as its typical symptoms and serological markers. A strict collaboration with endocrinologists and neurosurgeons is mandatory in order to reach a definitive diagnosis, allowing to promptly initiating an appropriate treatment.

Pituitary magnetic resonance imaging predictive role in the therapeutic response of growth hormone-secreting pituitary adenomas.

BACKGROUND: Growth hormone (GH)-secreting pituitary adenomas, responsible for the development of acromegaly, are the second most frequent type of secreting pituitary adenomas and are characterized by very variable T2-weighted signal intensity on pituitary magnetic resonance imaging (MRI). Previous data have demonstrated a correlation between T2-weighted tumor signal intensity and response to therapy with conventional somatostatin analogs (SSA) in patients with acromegaly. The aim of the current retrospective study was to investigate the correlation between the T2-weighted tumor signal on pituitary MRI and both biochemical and radiological response to first-line SSA therapy. METHODS: Twenty-two naive patients with acromegaly were eligible for the study (14 females and 8 males, mean age ± SD: 58.8±15.74). A biochemical evaluation (GH and IGF-I levels) and an MRI assessment (volume and signal intensity analysis of adenoma) were conducted in each patient at diagnosis and after 12 months of SSA therapy. RESULTS: On diagnostic pituitary MRI, 16 (72.7%) adenomas were T2- hypointense and 6 (27.2%) T2-hyperintense. After 12 months of SSA therapy, IGF-I levels decreased by more than 50% from baseline in 62.5% of patients with T2-hypointense and 33.3% of patients with T2- hyperintense tumor signal, respectively (P=0.03). Moreover, GH levels decreased by more than 80% from baseline in 81.3% and 33.3% of patients with T2-hypointense and T2-hyperintense tumor signal (P=0.02). A significant tumor volume reduction (≥20%) was observed in 75% of the T2-hypointense and 33.3% of the T2-hyperintense adenomas (P=0.001). CONCLUSIONS: In naive patients with acromegaly, first-line SSA therapy is associated with a better biochemical response and greater tumor shrinkage in T2-hypointense compared to T2-hyperintense adenomas. Therefore, T2-weighted sequences of pituitary MRI can help to classify GH-secreting pituitary adenomas into a T2-hypointense and T2-hyperintense type and, therefore, to identify patients who can better respond to first-line SSA therapy.

Characteristics of meningitis following transsphenoidal endoscopic surgery: a case series and a systematic literature review.

BACKGROUND: Meningitis occurs in 0.8-1.5% of patients undergoing neurosurgery. The aim of the study was to evaluate the characteristics of meningitis after endoscopic endonasal transsphenoidal surgery (EETS) comparing the findings retrieved to those highlighted by literature search. MATERIALS AND METHODS: Patients treated by EETS during an 18-year period in the Department of Neurosurgery of 'Federico II' University of Naples were evaluated and included in the study if they fulfilled criteria for meningitis. Epidemiological, demographic, laboratory, and microbiological findings were evaluated. A literature research according to PRISMA methodology completed the study. RESULTS: EETS was performed on 1450 patients, 8 of them (0.6%) had meningitis [median age 46 years (range 33-73)]. Endoscopic surgery was performed 1-15 days (median 4 days) before diagnosis. Meningeal signs were always present. CSF examination revealed elevated cells [median 501 cells/µL (range 30-5728)], high protein [median 445 mg/dL (range 230-1210)], and low glucose [median 10 mg/dL (range 1-39)]. CSF culture revealed Gram-negative bacteria in four cases (Klebsiella pneumoniae, Escherichia coli, Alcaligenes spp., and Haemophilus influenzae), Streptococcus pneumoniae in two cases, Aspergillus fumigatus in one case. An abscess occupying the surgical site was observed in two cases. Six cases reported a favorable outcome; two died. Incidence of meningitis approached to 2%, as assessed by the literature search. CONCLUSIONS: Incidence of meningitis after EETS is low despite endoscope goes through non-sterile structures; microorganisms retrieved are those present within sinus microenvironment. Meningitis must be suspected in patients with persistent fever and impaired conscience status after EETS.

Pituitary magnetic resonance imaging in Cushing's disease.

Adrenocorticotropin-secreting pituitary tumor represents about 10 % of pituitary adenomas and at the time of diagnosis most of them are microadenomas. Transsphenoidal surgery is the first-line treatment of Cushing's disease and accurate localization of the tumor within the gland is essential for selectively removing the lesion and preserving normal pituitary function. Magnetic resonance imaging is the best imaging modality for the detection of pituitary tumors, but adrenocorticotropin-secreting pituitary microadenomas are not correctly identified in 30-50 % of cases, because of their size, location, and enhancing characteristics. Several recent studies were performed with the purpose of better localizing the adrenocorticotropin-secreting microadenomas through the use in magnetic resonance imaging of specific sequences, reduced contrast medium dose and high-field technology. Therefore, an improved imaging technique for pituitary disease is mandatory in the suspect of Cushing's disease. The aims of this paper are to present an overview of pituitary magnetic resonance imaging in the diagnosis of Cushing's disease and to provide a magnetic resonance imaging protocol to be followed in case of suspicion adrenocorticotropin-secreting pituitary adenoma.

Magnetic resonance imaging in the pre-operative evaluation of obstructive epiphora: true-FISP and VIBE vs gadolinium.

PURPOSE: To assess unenhanced magnetic resonance imaging (MRI) in the preoperative evaluation of obstructive epiphora in patients undergoing dacryocystorhinostomy (DCR) and in particular, to evaluate the efficacy of this technique in the detection of the exact level of obstruction occurring in the naso-lachrymal duct (NLD). The correct identification and characterization of the NLD and its obstructions lead to a more effective surgery, preventing recurrent dacryocystitis after the surgical treatment. METHODS: From January 2009 to December 2014, 127 obstructive epiphoras were diagnosed and treated in 127 patients (35 M, 92 F; mean age 60.7 ± 7.48 years, range 42-75 years) with endoscopic DCR, in a IRB-approved protocol. To precisely define the morphology of the NLD and the site of obstruction, some of these patients (67/127) underwent unenhanced 1.5-T MR with TrueFISP and VIBE sequences, while the remaining (60/127) underwent Gadolinium-enhanced 1.5-T MR. Afterwards, surgery checked the real site of obstruction in both groups of patients (enhanced and unenhanced MR), with surgical outcomes matched with previous MR reports. RESULTS: In all cases, unenhanced MRI was able to detect the exact site of obstruction along the NLD, allowing a correct planning of surgical endoscopic procedures. On the contrary, enhanced MRI wrongly diagnosed six patients with proximal stenosis (6/60, 10.0%) as intermediate NLD obstruction. Unenhanced MRI was found to be more accurate than enhanced MRI with a statistical significant difference (p value = 0.0256) and obviously cheaper and easier to perform. All imaging reports were verified with surgery. The correct identification of the level of obstruction allowed successful surgery in around 73% (93/127) of patients, who had no recurrence during 6-month follow-up. CONCLUSION: In patients with epiphora, unenhanced MR showed to be highly reliable and even more effective than enhanced MR in the preoperative characterization of NLD stenosis, with no need of performing complex, time-wasting and expensive procedures for the administration of topical contrast media.

Flow diverter device for the treatment of small middle cerebral artery aneurysms.

PURPOSE: Experience with the endovascular treatment of middle cerebral artery (MCA) aneurysms by flow diverter devices (FDD) is still limited. This study examines the results and complications of FDD for small aneurysms at this location. METHODS: From February 2010 to December 2013, 14 patients (10 women; mean age 59 years) with 15 small MCA aneurysms were treated with FDD. All procedures were performed with the Pipeline embolization device (PED). RESULTS: Complete occlusion was obtained in 12/15 aneurysms (80%) and partial occlusion in 3 (20%). Among 13 aneurysms with a side branch, this was patent at the angiographic control in 4 cases, showed decreased filling in 6, and was occluded in 3 (with neurological deficits in 2). All PEDs were patent at follow-up. Post-procedural ischemic complications occurred in 4 (27%) procedures with permanent neurological deficit (modified Rankin score 2) in 3 (21%). No early or delayed aneurysm rupture, no subarachnoid or intraparenchymal hemorrhage and no deaths occurred. CONCLUSIONS: Endovascular treatment with FDD is a relatively safe treatment for small MCA aneurysms resulting in a high occlusion rate. The findings of this study suggest that complete occlusion after endovascular treatment with FDD can be delayed (>6 months). Ischemic complications may occur as early or delayed, particularly at clopidogrel interruption.

Pituitary function and morphology in Fabry disease.

Endocrine abnormalities are known to affect patients with Fabry disease (FD). Pituitary gland theoretically represents an ideal target for FD because of high vascularization and low proliferation rate. We explored pituitary morphology and function in a cohort of FD patients through a prospectic, monocentric study at an Academic Tertiary Center. The study population included 28 FD patients and 42 sex and age-matched normal subjects. The protocol included a contrast enhancement pituitary MRI, the assessment of pituitary hormones, anti-pituitary, and anti-hypothalamus antibodies. At pituitary MRI, an empty sella was found in 11 (39%) FD patients, and in 2 (5%) controls (p < 0.001). Pituitary volume was significantly smaller in FD than in controls (p < 0.001). Determinants of pituitary volume were age and alpha-galactosidase enzyme activity. Both parameters resulted independently correlated at multivariate analysis. Pituitary function was substantially preserved in FD patients. Empty sella is a common finding in patients with FD. The major prevalence in the elderly supports the hypothesis of a progressive pituitary shrinkage overtime. Pituitary function seems not to be impaired in FD. An endocrine workup with pituitary hormone assessment should be periodically performed in FD patients, who are already at risk of cardiovascular complications.