Search

ObjectiveAfter a previous survey on the incidence of diabetic ketoacidosis (DKA) at onset of type 1 diabetes in children in 2013–2014 in Italy, we aimed to verify a possible decline in the incidence of DKA at onset during a national prevention campaign.DesignProspective observational study.SettingMulticentre study throughout Italy.InterventionNational awareness campaign started in November 2015 and held until December 2017.PatientsDuring 2016 and 2017 we collected data on all patients aged 0–18 years with new-onset diabetes.Main outcome measuresDKA (pH ResultsRecords (n=2361) of children with newly diagnosed type 1 diabetes were collected from 58 out of 68 (85.3%) centres of the original survey participants and 100% of the previously surveyed tertiary centres. Overall, DKA was observed in 1124 patients, with an increased rate when compared with the previous survey (47.6% vs 38.5%, p=0.002), and severe DKA in 15.3%. In children below 6 years, DKA was observed in 323 out of 617 (52.5%) and severe DKA in 16.7%; in this age group, occurrence of DKA reduced by 21.3% (p=0.009). DKA treatment according to the ISPED guidelines was adopted in 95% of the centres, with a 27% improvement (p=0.025).ConclusionsDuring a 2-year awareness campaign, DKA at onset of diabetes in children and adolescents 0–18 years is still common and increased when compared with the 2013–2014 survey.

As of January 2020, a new pandemic has spread from Wuhan and caused thousands of deaths worldwide. Several studies have observed a relationship between coronavirus disease (COVID-19) infection and the cardiovascular system with the appearance of myocardial damage, myocarditis, pericarditis, heart failure and various arrhythmic manifestations, as well as an increase in thromboembolic risk. Cardiovascular manifestations have been highlighted especially in older and more fragile patients and in those with multiple cardiovascular risk factors such as cancer, diabetes, obesity and hypertension. In this review, we will examine the cardiac involvement associated with SARS-CoV-2 infection, focusing on the pathophysiological mechanism underlying manifestations and their clinical implication, taking into account the main scientific papers published to date.

Objectives Forty sites were involved in this multicenter and multivendor registry, which sought to evaluate indications, spectrum of protocols, impact on clinical decision making and safety profile of cardiac magnetic resonance (CMR). Materials and methods Data were prospectively collected on a 6-month period and included 3376 patients (47.2 ± 19 years; range 1–92 years). Recruited centers were asked to complete a preliminary general report followed by a single form/patient. Referral physicians were not required to exhibit any specific certificate of competency in CMR imaging. Results Exams were performed with 1.5 T scanners in 96% of cases followed by 3 T (3%) and 1 T (1%) magnets and contrast was administered in 84% of cases. The majority of cases were performed for the workup of inflammatory heart disease/cardiomyopathies representing overall 55.7% of exams followed by the assessment of myocardial viability and acute infarction (respectively 6.9% and 5.9% of patients). In 49% of cases the final diagnosis provided was considered relevant and with impact on patient's clinical/therapeutic management. Safety evaluation revealed 30 (0.88%) clinical events, most of which due to patient's preexisting conditions. Radiological reporting was recorded in 73% of exams. Conclusions CMR is performed in a large number of centers in Italy with relevant impact on clinical decision making and high safety profile.

INTRODUCTION: We retrospectively evaluated the contribution of the positron emission tomography (PET) combined with computed tomography (PET/CT) and contrast enhanced computed tomography (ceCT) in the staging and in the prognostication of untreated advanced Hodgkin’s lymphoma (HL). METHODS: One-hundred-sixty-two HL patients from Italian, Poland and Danish centers entered this study. Treatment consisted on ABVD with or without radiotherapy. All patients were staged at baseline with ceCT and PET/CT (PET-0) and after 2 ABVD with PET/CT (PET-2). All the ceCT and FDG/PET scans were centrally reviewed. PET-2 was reported according to the 5-point Deauville scale: score 1 to 3 considered negative and score 4-5 positive. RESULTS: Patients breakdown into (a) stage I-II; (b) III or (c) IV for CeCT and PET/CT-staged patients was as follows: 49.1%, 31.0%, 19.9% and 46.6%, 21.7%, 31.7%, respectively. ceCT and PET-0 were concordant or discordant in 117 (72.2%) and 45 (27.8%) patients, respectively. Combining the PET-0 and ceCT data we demonstrated an extra-nodal sites (ENS) in 57 (35.2%) of the 162 patients. Most (27/57) extra-nodal sites (ENS) were detected by PET-0 alone while ENS were concordantly detected by ceCT and PET-0 in other 25 cases and by ceCT alone in the remaining 5 patients. These latter three patient groups showed similar 2-y EFS rates of 62%, 83% and 56%, respectively. In patients with PET/CT-documented ENS, a pathological metabolic uptake was documented, respectively, in the BM (18 cases), lung (7 cases) and in two other parenchymal sites (2 cases). Overall, the 2 and 5-year OS and EFS for the entire patient cohort were 97.4%, 97.4% and 86.6% and 82.6%, respectively. PET-2 was positive in 40 cases (25.1%) and negative in 119 (74.84%). At univariate and multivariate analyses ENS disease and a positive PET-2 were the only variables significantly affecting the EFS rates, with a HR of 3.9 (95% CI:1.62-9.36; P=.002) and 2.9 (95% CI:1.82-4.52; P CONCLUSION: Present findings indicate that (1) PET/CT is mandatory for baseline HL staging while CeCT is of limited value. (2) The combination of ENS and PET-2 in a integrated prognostic model was even more predictive than PET-2 alone on treatment outcome in ABVD-treated HL patients. Disclosures No relevant conflicts of interest to declare.

Perineural tumor spread (PNS) of head and neck malignancies is a well-known form of metastatic disease in which a lesion can migrate away from the primary site along the endoneurium or perineurium. MR imaging is considered the primary method for evaluating patients with symptoms related to the trigeminal nerve in most clinical settings. Both CT and MR imaging can detect perineural spread, but MRI is the modality of choice because of its capability to detect direct signs (nerve enlargement and enhancement) and indirect signs (neuropathic muscular atrophy, obliteration of fat planes). In addition, MRI is more sensitive because of its superior soft-tissue contrast, its multiplanar capability and decreased artifacts from dental hardware. Fat suppression images after contrast injection are mandatory to better detect nerve enhancement. CT is useful in detecting foraminal enlargement or more destructive bone patterns. Nerve function can be perserved until later in the course of the disease: patients with perineural spread demonstrated at radiologic or pathologic examination may have normal or nonspecific nerve function at clinical examination (patients are misdiagnosed with Bell's palsy or trigeminal neuralgia). Hence MRI assessment of perineural tumor location and extension is important.

Purpose Evaluation of clinical and radiological effects of the therapeutic outcome of CT-guided pulsed radiofrequency (PRF) treatment adjacent to the lumbar dorsal root ganglion (DRG) for patients with chronic intractable lumbosacral radicular pain in a single-center prospective longitudinal study in order to evaluate predictive factors of safety and therapeutic success. Materials and methods Thirty patients, from 2016 to 2018, were enrolled (age: 42–80 aa, 66.7% men and 33.3% females) with low back pain, lumbosciatalgia and/or lumbocruralgia, resistant to previous medical and physical treatments for a period not < 3 months, failure of surgical and chemiodiscolysis with ozone oxygen therapy. Each patient was subjected to a clinical evaluation (antalgic walking, sensitive deficit, interviews with specific questionnaires: ODI, RDQ,VAS) and to a radiological evaluation with MRI examination, before and 30 days after the CT-guided PRF treatment. Measurements of the thickness of the involved and not involved DRG were taken using common postprocessing software of MRI examinations in order to have measurement parameters for comparison. We analyzed the clinical course using the paired samples T test in order to evaluate modification for each clinical and radiological parameter (statistical significance p < 0.05). Results Significant improvements of the clinical outcomes with a good resolution of the pain symptoms (VAS evaluation: The score fell from 68.47 to 39.17 with a difference of 29.3 and a reduction of the 42.79% in the perceived pain, p = 0.00000152). The thickness of DRG falls from an average media of 0.586–0.448 cm (p = 0.000085), with a difference of 0.138 cm and a percentage reduction of 22.30%. Conclusions PRF treatment of the DRG may be considered for patients with chronic severe lumbosacral radicular pain refractory to conventional medical management when other noninvasive or surgical procedures fail. It is minimally invasive, inexpensive and simple to perform with no complications.

Epilepsy is the most frequent presenting sign in patients with cavernous angiomas and is the major cause of morbility. Persistence of seizures after surgical treatment prompted many authors to examine the possibility of removing the cavernoma and the surrounding tissue. In our series of 53 cavernous angiomas, all the 35 patients with preoperative seizures underwent surgery by means of lesionectomy alone. One hundred percent of patients with less than five preoperative seizures and/or an history under 12 months was seizure free, while only 62.5% of patients with more than five seizures and/or an history longer than 12 months was seizure free. Number and duration of seizures before surgery seems to be the most important factor in the seizure outcome after surgical treatment.

Severe acute respiratory syndrome coronavirus‐2 (SARS‐CoV‐2) infection has rapidly spread throughout the world since December 2019 to become a global public health emergency for the elevated deaths and hospitalizations in Intensive Care Units. The severity spectrum of SARS‐CoV‐2 pneumonia ranges from mild to severe clinical conditions. The clinical course of SARS‐CoV‐2 disease is correlated with multiple factors including host characteristics (genetics, immune status, age and general health), viral load and, above all, the host distribution of the airways and lungs of the viral receptor cells. In this review, we will briefly summarize the current knowledge on the characteristics and management of COVID‐19‐pneumonia. However, other studies are needed to better understand the pathogenetic mechanisms induced by SARS‐Cov‐2 infection, and to evaluate the long‐term consequences of the virus on the lungs. This article is protected by copyright. All rights reserved.

The actual Coronavirus Disease (COVID 19) pandemic is due to Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a member of the coronavirus family. Besides the respiratory involvement, COVID 19 patients frequently develop a pro-coagulative state caused by virus-induced endothelial dysfunction, cytokine storm and complement cascade hyperactivation. It is common to observe diffuse microvascular thrombi in multiple organs, mostly in pulmonary microvessels. Thrombotic risk seems to be directly related to disease severity and worsens patients’ prognosis. Therefore, the correct understanding of the mechanisms underlying COVID-19 induced prothrombotic state can lead to a thorough assessment of the possible management strategies. Hence, we review the pathogenesis and therapy of COVID 19-related thrombosis disease, focusing on the available evidence on the possible treatment strategies and proposing an algorithm for the anticoagulation strategy based on disease severity.

Trans-bronchial needle aspiration allows lymph node sampling in several thoracic conditions; the ability of Rapid On-Site Evaluation (ROSE) to predict the final diagnosis in this setting has not been well characterized. We performed a retrospective study to establish the utility of ROSE in the diagnosis of thoracic diseases with mediastinal lymph node involvement. We retrospectively reviewed 297 patients with hilar-mediastinal lymph node enlargement detected at CT scan from January 2013 to April 2016. 201 patients underwent conventional TBNA; in 96 patients, TBNA procedure was performed by on-site presence of a team of pathologists and research morphologists. Lung neoplasms, sarcoidosis, infections and lymphoma were the most common diseases diagnosed with TBNA samples. TBNA simultaneously performed in combination with ROSE produced an increase in percentage of appropriate samples compared to single cTBNA (adequate samples cTBNA vs ROSE-TBNA: 73% vs 81%; p

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.

Purpose: We previously showed the positive effects of the new antioxidant molecule bis(1-hydroxy-2,2,6,6-tetramethyl-4-piperidinyl)-decandioate (IAC) in reducing basal hyperglycaemia and relieving glucose intolerance in a diabetes model. However, the chemical properties of IAC did not allow an efficient oral administration, thus representing the main failing of that study. Here, we tested the effect of a new oral delivery system based on solid lipid microparticles (SLMs) in a diabetes mouse model. Methods: The diabetes model was induced in C57B1/6J mice using streptozotocin and nicotinamide. Only the animals that overcame the glycaemic threshold of 180 mg/dL were enrolled in the study. Diabetic animals were then randomly assigned to 4 groups (n = 9) and treated once a day for 5 consecutive weeks with IAC (50, 100, and 150 mg/kg b.w.). The control group was composed of (n = 7) healthy mice that received only the vehicle. Glucose level was weekly monitored during the treatment period and up to 3 weeks after the suspension of the treatment. Glucose tolerance and insulin-resistance test were carried out. Results: Our results showed that SLMs maintained the IAC effect in reducing basal hyperglycaemia as well as improving the insulin sensitivity and glucose tolerance. Conclusion: The present study confirms that SLMs are promising drug carriers, which allow the oral administration of IAC ensuring its therapeutic efficacy. The concrete possibility to administer IAC per os represents a significant breakthrough in the putative consideration of this multi-radical scavenger in the diabetes therapeutic approach.

Cerebral plasticity is the ability of the central nervous system to reorganize itself in response to different injuries. The reshaping of functional areas is a crucial mechanism to compensate for damaged function. It is acknowledged that functional remodeling of cortical areas may occur also in glioma patients. Principal limits of previous investigations on cortical plasticity of motor and language functions included scarce reports of longitudinal evaluations and limited sample sizes. This systematic review is aimed at elucidating cortical brain plasticity for motor and language functions, in adult glioma patients, by means of preoperative and intraoperative mapping techniques. We systematically reviewed the literature for prospective studies, assessing cortical plasticity of motor and language functions in low-grade and high-grade gliomas. Eight longitudinal studies investigated cortical plasticity, evaluated by motor and language task-based functional MRI (fMRI), motor navigated transcranial magnetic stimulation (n-TMS), and intraoperative mapping with cortical direct electrocortical stimulation (DES) of language and motor function. Motor function reorganization appeared relatively limited and mostly characterized by intrahemispheric functional changes, including secondary motor cortices. On the other hand, a high level of functional reshaping was found for language function in DES studies. Occurrence of cortical functional reorganization of language function was described focusing on the intrahemispheric recruitment of perilesional areas. However, the association between these functional patterns and recovery of motor and language deficits still remains partially clear. A number of relevant methodological issues possibly affecting the finding generalization emerged, such as the complexity of plasticity outcome measures and the lack of large longitudinal studies. Future studies are required to further confirm these evidences on cortical plasticity in larger samples, combining both functional imaging and intraoperative mapping techniques in longitudinally evaluations.

Background The purpose of this retrospective study was to identify subcategories in cT3 to cT4a supraglottic/glottic cancers, describing their different spreading patterns, and local and locoregional recurrence modes. Methods Four hundred eighty-nine patients who underwent open partial horizontal laryngectomies (OPHLs) were retrospectively classified as: subcategory I (anterior pT3 with normal arytenoid mobility); subcategory II (posterior pT3 with impaired/absent mobility); subcategory III (anterior pT4 with normal mobility); and subcategory IV (posterior pT4 with impaired/absent mobility). Results Five-year overall survival (OS), disease-specific survival (DSS), disease-free survival (DFS), locoregional, local control, freedom from laryngectomy, and laryngectomy-free survival were significantly better in anterior tumors (subcategories I and III) when compared with the corresponding posterior ones (subcategories II and IV). Conclusion Anterior cT3 tumors are manageable by OPHL, and this approach could also be proposed in the treatment of early anterior cT4aN0. Despite promising results, OPHLs should be considered under investigation in posterior cT3 tumors due to clinical and biological behavior similar to cT4a tumors.

Syringomyelia is a disorder in which a cyst or cavity forms within the spinal cord. This cyst, called syrinx, can expand and elongate over time, destroying the spinal cord. We describe the case of a young patient with partial spontaneous regression of syringomyelia in Chiari I malformation, confirmed by magnetic resonance imaging three years after the diagnosis. During this period the patient did not experience any clinical symptoms. Although described in literature, spontaneous regression is an unusual event and very few cases have been reported. This case report supports the belief that conservative management together with both clinical and imaging periodic controls should be preferred in stable mild-symptomatic patients.

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.

Schwannomas are the most common tumors of the spinal cord. Nevertheless, sporadic intramedullary location of this entity is a very rare condition. We present two cases of solitary thoracic intramedullary schwannoma, located at the T6 and T10 levels respectively, in patients without evidence of Von Recklinghausen's disease. Two males, 71 and 51 years old respectively, presented with signs and symptoms of progressive thoracic myelopathy. In both cases, magnetic resonance imaging disclosed a spinal cord tumor, located at T6 and T10 levels respectively. Both patients were treated surgically. Patient 1 underwent an operation via a posterolateral extrapleural approach. The second patient was submitted to surgical resection of the tumor via a posterior approach. In both patients, total excision of an intramedullary lesion was achieved and histological and immunohistochemical findings were consistent with a typical schwannoma. Postoperative magnetic resonance imaging demonstrated no evidence of residual tumor in either case. The first patient is currently symptom-free and neurologically intact, almost five years after surgery. About one year after operation, patient 2 presents nearly complete resolution of his motor and sensory disturbances. Solitary intramedullary schwannomas are rare. Controversy remains regarding the origin of these tumors. In the majority of the cases, intramedullary schwannomas are benign, non-infiltrating tumors, hence amenable to aggressive surgery. Radical excision remains the gold standard in the treatment of such lesions.

Objective This paper reports four cases of schwannomas of the brachial plexus operated upon with good outcome and discusses the CT and MRI findings and the best surgical treatment of these lesions. Design All patients had large schwannomas (more than 4 cm in diameter) presenting as painless masses in the supraclavear region, explored by CT and MRI. A homogeneous mass, hypo-isointense in T1- and hyperintense in T2-weighted images, with well-defined margins, is in favor of a schwannoma. The nerve of origin, external to the tumor mass, may be defined on MRI. Intervention All patients have been operated upon using microsurgical technique: enucleation of the tumor content, piecemal removal of the capsule, identification and preservation of the neural elements were the main goals of the operation in all cases. Outcome Postoperatively, one patient experienced transient deficit of the deltoid muscle (two weeks). Actually, all four patients are symptom-free with no tumor recurrence, 6 months to 7 years after the operation. Conclusions A correct preoperative diagnosis of schwannoma of the brachial plexus may be obtained by MRI, which shows a rather typical aspect; on the other hand, needle aspiration histology and open biopsies should be avoided. The microsurgical treatment with preservation of the neural structures, as for schwannomas of the cranial and spinal nerves, results in good outcome without recurrences.

Introduction: In Graves' Ophthalmopathy, it is important to distinguish active inflammatory phase, responsive to immunosuppressive treatment, from fibrotic unresponsive inactive one. The purpose of this study is, first, to identify the relevant orbital magnetic resonance imaging signal intensities before treatment, so to classify patients according to their clinical activity score (CAS), discriminating inactive (CAS3) subjects and, second, to follow post-steroid treatment disease. Methods: An observational study was executed on 32 GO consecutive patients in different phases of disease, based on clinical and orbital Magnetic Resonance Imaging parameters, compared to 32 healthy volunteers. Orbital Magnetic Resonance Imaging was performed on a 1.5 tesla Magnetic Resonance Unit by an experienced neuroradiologist blinded to the clinical examinations. Results: In pre-therapy patients, compared to controls, a medial rectus muscle statistically significant signal intensity ratio (SIR) in short-time inversion recovery (STIR) (long TR/TE) sequence was found, as well as when comparing patients before and after treatment, both medial and inferior rectus muscle SIR resulted significantly statistically different in STIR. These increased outcomes explain the inflammation oedematous phase of disease, moreover after steroid administration, compared to controls; patients presented lack of that statistically significant difference, thus suggesting treatment effectiveness. Conclusion: In our study, we proved STIR signal intensities increase in inflammation oedematous phase, confirming STIR sequence to define active phase of disease with more sensibility and reproducibility than CAS alone and to evaluate post-therapy involvement. © 2014 Springer-Verlag.

We report 2 cases of 'de novo' aneurysm formation in a vessel which appeared to be normal at a previous angiography. The first patient developed an anterior communicating artery aneurysm nine years after occlusion of the right internal carotid artery by Gianturco coils for the treatment of a giant intracavernous carotid aneurysm. In the second case a 'de novo' aneurysm of the internal angle A1-A2 segment of the left anterior cerebral artery developed 6 years after successful clipping of another aneurysm of the same location. De novo formation of an aneurysm in a vessel which was found to be normal in a previous angiographic study, may occur as result of hemodynamic changes, such as after internal carotid occlusion or in presence of an arteriovenous malformation or variations of the circle of Willis. However, definite hemodynamic changes may also be absent. We conclude that patients operated on for aneurysm clipping must be periodically explored by magnetic resonance angiography to evaluate the possibility of de novo appearance of another aneurysm.

We report our case and review the literature on reversal DWI lesions, ADC thresholds and correlation between DWI lesion and outcome measured with clinical scales. A 30-years old woman was admitted to our hospital 18 hours after stroke onset. Considering the absence of alterations on CT and the worsening of symptomatology, the patient underwent MRI, which showed a slightly hyperintense signal in FLAIR images in the left portion of the pons and midbrain and a more evident bilateral DWI hyperintensity of the pons. The patient was treated with mechanical and pharmacological intra-arterial thrombolysis. The patient showed a rapid improvement of symptoms. Two weeks after the treatment her clinical conditions were characterized by a residual right hemiparesis and complete recovery of right motility, respiratory and swallowing difficulties. MR examination demonstrated a slight signal alteration of the pons left hemiportion and a disappearance of the mesencephalic signal alteration and of the right portion of the pons. DWI lesions represent irreversibly damaged tissue but new evidence suggests that DWI lesions may be reversible, especially with reperfusion, by now well demonstrated in animal models. Therefore acute DWI lesions probably contain not only irreversibly injured tissue but also parts of the penumbra. The debate on the capability of ADC maps to discriminate irreversibly from reversibly damaged tissue is a matter of controversy. ADC values in human stoke are not an independent indicator of tissue viability. The use of thresholds may improve reproducibility but not validity.

We describe three patients with type A Niemann-Pick disease (NPD-A). NPD-A is an autosomal recessive neuronal storage disease classified among the sphingolipidoses, characterized by accumulation of sphingomyelin in various tissues and in the brain. Magnetic Resonance imaging (MRI) of our three patients showed a marked delay of myelination with frontal atrophy. Few descriptions of this MRI pattern of delayed myelination have been published to date.

This study retrospectively analyzes the CT findings of 50 patients with spontaneous intracerebral haematomas and correlates them with the clinical condition at admission and the outcome. The volume of the haematoma was well correlated with the outcome and was a very useful indicator for surgery. In patients with haematoma volume lower than 25 ml and above 80 ml there was no indication for surgery; in patients with haematoma volume between 25 and 50 ml the operation does not significantly modify the survival rate, but favours a more rapid and complete recovery of the neurological deficits, whereas a haematoma volume between 50 and 80 ml favours the rate of survival.

Hippocampal abnormalities correlated with mesial temporal sclerosis (MTS) are well documented. MTS may be associated with extrahippocampal anomalies involving limbic structures along a known neuroanatomic pathway (Papez circuit). We report a patient with MTS and thalamic changes. Seizure-related thalamic damage could have been caused by abnormal electric discharges from the mamillary body to the anterior thalamus through the mamillothalamic tract. This suggests that MTS is not limited to the temporal lobe but could represent a limbic system pathology.

Summary Background Remission rates of 76, 69·5 and 64·3% have been reported in patients with nontumoural hyperprolactinaemia (NTH), microprolactinoma and macroprolactinoma, respectively, 2‐5 years after cabergoline (CAB) withdrawal. Objective To report the estimated recurrence rate at 24 ‐ 96 months after CAB withdrawal and indicate predictors of disease remission. Design Observational, analytical, prospective. Patients Of 381 previously untreated de novo patients with hyperprolactinaemia, 221 (58%) (173 women, 48 men; 27 with NTH, 115 with micro-, and 79 with macroprolactinoma) were studied. Measurements Using multiple regression analysis the diagnostic accuracy of nadir PRL levels ( t = 7·6, P < 0·0001) and nadir maximal tumour diameter at CAB withdrawal ( t = 3·9, P < 0·001) was analysed using receiver operating characteristic (ROC) curves. Results The recurrence of hyperprolactinaemia was 25·9, 33·9 and 53·1% in patients with NTH, micro- or macroprolactinoma, respectively. To predict the last PRL level after withdrawal, the optimum cut-off of nadir PRL levels at withdrawal was 162 mU/l (5·4 µ g/l) [sensitivity (95% CI) 76% (67 ‐ 84%), specificity 65% (51‐ 77%)] and that of nadir maximal tumour diameter was 3·1 mm [sensitivity 52% (41‐ 63%), specificity 86% (79 ‐ 91%)]. The patients achieving both nadir PRL levels ≤ 162 mU/l and maximal tumour diameter ≤ 3·1 mm ( n = 111) at CAB withdrawal had a significantly lower Kaplan‐Meier estimate of recurrence of hyperprolactinaemia (20%) at 24 ‐ 96 months than those who did not fulfil any of these criteria [( n = 38) 90%; P < 0·0001]. Patients achieving nadir PRL levels ≤ 162 mU/l ( n = 26) or maximal tumour diameter ≤ 3·1 mm during CAB treatment ( n = 46) had an estimated recurrence rate of hyperprolactinaemia of 50 and 56%, respectively. Conclusion Persistent remission of hyperprolactinaemia without any evidence of tumour re-growth after 24 ‐9 6 months of CAB withdrawal occurred in the majority of patients with NTH and microprolactinoma and in about half of those with macroprolactinoma. Nadir PRL levels and maximal tumour diameter at CAB withdrawal of ≤ 162 mU/l and ≤ 3·1 mm predicted remission of hyperprolactinaemia in 80% of patients.

CONTEXT: Primary treatment with depot octreotide and lanreotide induces tumor shrinkage in newly diagnosed patients with acromegaly. OBJECTIVE: The objective of the study was to evaluate clinical predictors of tumor shrinkage. DESIGN: This was an analytical, observational, open, prospective study. SUBJECTS: The study included 99 patients: 13 with microadenoma and 86 with macroadenoma (25 enclosed, 32 extrasellar, 29 invasive). MAIN OUTCOME MEASURES: Age, gender, estimated disease duration, body mass index, GH and IGF-I levels, and tumor volume at diagnosis and after 12 months of treatment were measured. Percentage of GH, IGF-I, and tumor size changes from baseline were also analyzed. Tumor changes were scored as absent (+/- 0-25%), mild (+/- 25.1-50%), moderate (+/- 50.1-75%), or notable (75%). INTERVENTIONS: Sixty patients (60.6%) received depot octreotide im (20-30 mg every 28 d), and 39 patients (39.4%) received lanreotide im (60-90 mg every 28 d). RESULTS: Basal tumor volume and maximal tumor diameter correlated with age, disease duration, and GH levels. After 12 months, GH levels were controlled (

Functional magnetic resonance imaging (fMRI) has become the most widely used approach for studying brain functions in humans. The rapid and widespread diffusion of fMRI has been favoured by the properties this technique presents, and particularly by its sensitivity in analysing brain functional phenomena and by the lack of biological invasiveness, resulting in an unprecedented and unparalleled flexibility of use. These properties of fMRI brought the functional examination of the brain within the reach of the whole neuroscience community and have appreciably stimulated the research on the functional processes of the living brain. Among the main features of fMRI, its spatial and temporal resolution represents clear advantages compared with the other methods of functional neuroimaging. In fact, the high spatial resolution of fMRI permits to produce more precise and better localised information, and its temporal resolution provides the potential of a better understanding of neural dynamics at the level of single functional areas and of the neural constituents of functional patterns. A fundamental possibility of improving spatial and temporal resolution without excessively degrading signal-to-noise ratio consists in the use of high magnetic field intensity fMRI units. Besides, high field units make the use of more demanding fMRI paradigms, like single trial event related studies, much more compatible with the need of a solid statistical evaluation. This has notably promoted the diffusion of high field MRI units for human studies throughout the world, with very high field MRI units, up to 8 T, working in a few research centres, and a larger number of MRI units with field intensity ranging between 3 and 5 T.

The endoscopic endonasal transsphenoidal approach has been proposed in the past decade as a minimally invasive surgical technique for the removal of pituitary tumors. From January 1997 to November 1999, 100 consecutive patients with pituitary tumors underwent endoscopic endonasal surgery, according to Jho's technique. We employed 0 degrees, 30 degrees, 45 degrees, and 70 degrees rigid endoscopes, 18 - 30 cm in length, 4 mm in diameter with an outer sleeve for irrigation and secured to a holder. Among the 87 pituitary adenomas, tumor removal was total in 51, subtotal (> 80 %) in 20 and partial in 16 cases. Four craniopharyngiomas were totally removed and an intra-suprasellar arachnoid cyst was emptied; a biopsy was performed in the two patients with a clivus chordoma. The two cases of sphenoid sinusitis were cured by surgery, the three patients with spontaneous CSF rhinorrhea were successfully treated and the residual nasal meningocele was removed. The endoscopic endonasal transsphenoidal approach appeared to be less traumatic than the traditional microsurgical approach, was very effective, and was characterized by a reduced number of complications. However, the relatively small series together with the short follow-up do not allow us to draw definitive conclusions. The post-operative reduction in hospital stay (two days in 40 of 100), significantly reduced the cost of patient's management.

La malattia di Behçet è una rara vasculite multisistemica ad origine sconosciuta. L'affezione è caratterizzata dalla classica triade di ulcerazioni orali, genitali ed uveite, cui possono associarsi tromboflebiti, artriti, manifestazioni cutanee tipo eritema nodoso ed ulcerazioni intestinali. L'interessamento neurologico, che peggiora la prognosi, è riportato con una frequenza variabile tra il 16 ed il 40% dei casi e può manifestarsi con lesioni cerebrali e meno frequentemente spinali. La Risonanza Magnetica concorre alla diagnosi dell'affezione sia nelle forme acute che nelle fasi di riacutizzazione delle forme croniche, ad andamento intermittente o progressivo. Le localizzazioni più frequenti in fase acuta sono descritte al tronco (soprattutto pontine e mesencefaliche), ai gangli della base, al cervelletto, oltre che alla sostanza bianca sopratentoriale (meno frequentemente periventricolare). Più difficile risulta l'inquadramento diagnostico nelle fasi croniche, in cui prevale un diffuso interessamento della sostanza bianca emisferica. La localizzazione spinale è più rara rispetto a quella cerebrale, con predilezione per il tratto dorsale. Viene riportato un caso di osservazione personale, caratterizzato dalla coesistenza di localizzazioni encefaliche e midollari, studiato con RM.

The authors have examined the relationship between magnetic resonance imaging (MRI) and histopathological features in 35 surgically verified intracranial meningiomas. Tumor signals on T1-weighted images were rather similar regardless of the histologic subtype of the tumors. On T2-weighted images, hypointense meningiomas were mainly fibroblastic and hyperintense tumors were mainly syncytial and angioblastic, and partly transitional. Isointense tumors were mainly transitional and partly fibroblastic and syncytial. The authors conclude that the signal intensity of the MRI may be useful in the preoperative characterization of intracranial meningiomas. T1-weighted images may predict the presence of cysts and intratumoral blood vessels; whereas T2-weighted images can give information about histological subtype, vascularity and consistency. Meningiomas hyperintense to the cortex on T2 are usually soft, more vascular and more frequently of syncytial or angioblastic subtype; tumors hypointense or hypo-isointense on T2 tend to have a more hard consistency and are more often of fibroblastic or transitional subtype.

Cabergoline (CAB), a long-lasting dopamine-agonist, specific for the D2 receptor, is effective in normalizing serum PRL levels in most patients with microprolactinoma or idiopathic hyperprolactinemia. Because few data are presently available on the effects of CAB treatment in macroprolactinomas, the aim of this open-label study was to investigate whether this drug was effective in producing tumor shrinkage, as well as in normalizing PRL levels. Twenty-three patients with macroprolactinoma entered this study: 15 patients had had no treatment, whereas the remaining 8 patients had been previously treated with bromocriptine, which was withdrawn because of intolerance. Three of 23 patients had undergone unsuccessful surgery. Pretreatment serum PRL levels ranged from 100-3860 μg/L. CAB was administered at a dose of 0.5–3 mg once or twice a week for 12–24 months. Magnetic resonance imaging (MRI) scans were performed before and 3, 6, 12, and 24 months after the beginning of treatment, to evaluate tumor shrinkage, defined as a decrease of at least 80% of baseline tumor volume. After 3–6 months of treatment with a low dose (0.5–1 mg/week), serum PRL levels normalized in 18 patients. In the remaining 5 patients, whose serum PRL levels were not normalized, the dose was increased to 2–3 mg/week. This schedule caused the normalization of PRL levels in 1 patient, whereas in the remaining 4 patients, PRL levels were reduced to 30–82 μg/L. A tumor volume reduction greater than 80% at MRI occurred in 14 of 23 patients (61%) after CAB treatment (from 2609.4 ± 534.7 to 530.1 ± 141.3 mm3 at the 12–24th month follow-up, P < 0.001). A volume reduction of 41.8 ± 3.4% was already evident after 3 months (1436 ± 285.9 mm3; P < 0.001). The complete disappearance of the tumor mass at MRI occurred after 6 months of treatment with CAB in 1 patient, and in 5 patients after 1 yr of treatment. An improvement of visual field defects was obtained in 9 of the 10 patients presenting visual impairment before CAB treatment. The drug was tolerated well by all patients. Only 1 patient experienced mild nausea, which disappeared spontaneously after the 2nd day of treatment. Long-term, a low dose of the D2 receptor agonist CAB significantly reduced tumor volume and normalized serum PRL levels in a great majority of patients bearing macroprolactinoma. This treatment met with excellent patient compliance. This study suggests that CAB can be used as a first choice drug treatment in macroprolactinomas, as already shown for microprolactinomas and idiopathic hyperprolactinemia.

We report a case of cutis verticis gyrata-mental deficiency syndrome (CVG-MD) which was associated with drug-resistant epilepsy and bilateral occipital polymicrogyria. Genetic analysis showed an increased number of breaks at the 3p14 and 16q23 sites. We hypothesize that a deleterious factor acting at a critical period of intrauterine development could result in the cerebral malformation and in the development of CVG. Neuroradiological investigation is warranted in cases of CVG-MD.

Fifty patients with intracranial meningiomas have been retrospectively examined, and the cerebral edema on computed tomography scan has been correlated with the clinical evolution, size, location, and histological features of the tumor. The degree of brain edema was found to be related to the clinical evolution and the size of the tumor, whereas the histological features were less significant. These results are discussed after reviewing the data of five other series in the literature of this subject. A correlation between the tumor steroid receptors and edema is suggested.

An exceptional case of lipoma of the sylvian region is described and the other seven reported cases are reviewed. The sylvian fissure is the most rare site of intracranial lipomas. Sylvian lipomas may be asymptomatic or present with epileptic seizures due to irritation of the cortex of the sylvian fissure. Only two previous cases have been diagnosed during life by computerized tomography and operated on; the deep location of these lipomas and their adherences to the sylvian cortex and the branches of the middle cerebral artery make radical removal impossible and dangerous.

Three cases of meningiomas associated with large cysts are reported, and 58 other cases from the literature are reviewed. The etiology of these cysts is analyzed on the basis of surgical and pathological findings. The preoperative diagnosis of large computed tomographic hypodensities associated with meningiomas and the differential features from other cystic tumors are discussed. The surgical aspect of the solid tumor and particularly the absence of a capsule separating the tumor from the cyst are usually decisive in confirming the intratumoral nature of the cyst. In questionable cases a histologic evaluation of the cyst walls may be necessary.

A rare case of craniopharyngioma extending to the cranial base and nasopharynx is reported and eight other cases in the literature are reviewed. The embryology and the clinical features of these tumors are discussed. Tomograms of the skull and computed tomography are the most useful radiological tools of investigation. A nasoseptal or transpalatal approach to surgery, which has been performed in reported cases of cystic nasopharyngeal craniopharyngiomas, was not performed on our patient because of the hard consistency and the diffusely infiltrating aspect of the tumor.

Summary: Purpose: To describe the etiology, characteristics, and clinical evolution of epilepsy in patients with gelastic seizures (GSs). Methods: Nine patients whose seizures were characterized by typical laughing attacks were observed between 1986 and 1997. Patients were selected based on electroencephalogram (EEG) or video-EEG recordings of at least one GS and on magnetic resonance imaging (MRI) study. Results: Five patients were affected by symptomatic localization-related epilepsy (LRE), with four of the patients' disorders related to a hypothalamic hamartoma (HH) and one to tuberous sclerosis (TS) without evident hypothalamic lesions. In four patients (the cryptogenic cases) MRI was negative also in these cases, clinical and EEG data suggested a focal origin of the seizures. The epileptic syndrome in the HH cases was usually drug-resistant, and was surgically treated in two of the patients. The patient with TS became seizure free with vigabatrin. In the cryptogenic cases, the ictal, clinical, and EEG semiology were similar to the symptomatic cases; the clinical evolution was variable, with patients having transient drug resistance or partial response to treatment. No cognitive defects were observed in the cryptogenic patients. None of the nine patients had precocious puberty. Conclusions: We confirm the frequent finding of HHs in GSs and further underline how GSs may also be observed in patients without MRI lesions and with normal neurologic status. In these patients, clinical and EEG seizure semiology is similar to symptomatic cases, but the clinical evolution is usually more benign.