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Background The enlargement of the posterior cranial fossa volume is considered one of the main steps of the surgical management of children with multiple sutures craniosynostosis. Different management options have been proposed including fixed expansive craniotomy, free bone flap craniotomy, and distraction osteogenesis. Objectives To review indications to “free bone flap” craniotomy for the posterior fossa expansion, detailing advantages, disadvantages, and complications related to the technique. Results and conclusions A review of the literature shows that “free bone flap” posterior expansion cranioplasty still has a role, particularly in infants with thin and “honeycomb” structure of the bone, allowing to gain adequate intracranial volume increases and to postpone to a more adequate time surgery aimed at anterior cranial fossa expansion.

Raising a child with autism spectrum disorder (ASD) can be a very stressful experience, impacting parents and their ability to take care of their child. We examined the relationship between parenting stress and child, and parent and contextual factors in a sample of 61 families of children with ASD recruited in a centre for ASD assessment and treatment. Results showed that mothers had a higher level of stress related to their parental role when compared to fathers. Data also showed the importance of considering the specific role of parental emotion regulation, family functioning and educational level in influencing maternal stress and the specific role of couple adjustment in influencing fathers’ parental distress. This study pointed out the importance of employing a vision that embraces different domains of family life in cases of children with ASD and in parents’ intervention programmes because these factors may contribute to parental stress.

Background Gelfoam is a simple and effective hemostatic agent, which is utilized to seal brain corticotomies or skull burr holes too. Thanks also to its cheap costs, it is one of the most widely used tool in the neurosurgical daily practice. However, the migration of Gelfoam fragments can account for the occlusion of endoscopic third ventriculostomy (ETV) or shunt, leading to hydrocephalus recurrence. Case description The authors report on two cases of Galfoam migration causing recurrent hydrocephalus: a young girl, operated on by posterior fossa tumor removal and ETV for associated hydrocephalus, where part of the Gelfoam (used to seal the burr hole) migrated up to close the ETV; and a preterm boy, treated by endoscopic brain wash and, afterwards, by ventriculo-peritoneal shunt for post-hemorrhagic hydrocephalus, where all the Gelfoam used to close the corticotomy migrated into the lateral ventricle, thus reopening the corticotomy and releasing small fragments which finally obstructed the shunt. Both patients needed a new endoscopic procedure (the second one also a shunt revision). Conclusions The reviewed pertinent literature discloses other complications of Gelfoam migration (mass effect, granulomatous reaction) as well as other causes of uncommon ETV/shunt obstruction. Anyway, Gelfoam will remain an irreplaceable tool for neurosurgeons. The present paper just emphasizes the need of its correct use to avoid complications.

In spite of literature data stating that children with single-suture craniosynostosis have an increased risk for neuropsychological deficits, no data are present clarifying the potential risk factors. All children with non-syndromic single-suture craniosynostosis operated on from January 2014 to January 2017 were enrolled. A comprehensive neurocognitive and neuro-ophthalmological evaluation was performed before surgery and 6 months after surgery. A further neurocognitive evaluation was performed 12 months after surgery. All children had a preoperative CT/MR study. One hundred forty-two patients were enrolled; 87 are affected by sagittal craniosynostosis, 38 by trigonocephaly, and 17 by plagiocephaly. A global neurocognitive impairment was documented in 22/87 children with scaphocephaly, 5/38 children with trigonocephaly, and 6/17 children with anterior plagiocephaly. There was a significant relationship between results of the ophthalmological evaluation, global IQ, and CT findings at diagnosis (r = 0.296, p

Background Causes of the nonaneurysmal subarachnoid hemorrhage (na-SAH) are still debated. Many studies confirmed the possible involvement of the intracranial venous system, in particular variants of the basal vein of Rosenthal. Study Object The aim of this study is to investigate the role of extracranial venous system, in particular the jugular drainage, in the na-SAH pathophysiology. Materials and Methods This is a multicenter retrospective study including patients suffering from na-SAH who were radiologically screened to exclude vascular malformations. The course of the internal jugular veins was evaluated to reveal any stenosis (caliber reduction >80%). Particular attention was paid at the passage between the styloid process and the arch of C1 to detect possible compression. As a control group, we enrolled patients who underwent CT angiograms and/or cerebral DSAs in the past 2 years. Results We included 194 patients. The na-SAH group consisted of 94 patients, whereas the control group consisted of 100 patients. Fifty patients of the control group underwent a CT angiography for an ischemic cerebrovascular disease or trauma and 50 patients for an SAH due to a ruptured aneurysm. A significant jugular stenosis was found in 49 (52.1%) patients in the na-SAH group and in 18 (18%) patients in the control group. At univariate and multivariate analysis, the difference was statistically significant (p Conclusions This is the first study investigating the correlation between jugular stenosis and the occurrence of na-SAH. The impaired venous outflow due to reduced venous caliber could result in an engorgement of the upstream intracranial veins with transient hypertensive phases facilitating ruptures. Further larger prospective studies are necessary to confirm these data.

Background Autism spectrum disorder is a complex condition with wide variation in type and severity that involves persistent challenges in social interaction, speech and nonverbal communication, restricted/repetitive behaviours and adaptive behaviours. In recent years, research has deepened the study of the predictive factors of optimal outcome, intended as indicators of positive trajectory in children with a previous diagnosis of autism who, after a therapeutic path, show a significant reduction in the “core” symptoms of autism and a positive evolution in social, adaptive, affective, and relational skills. Methods The study included 40 children aged 21 to 66 months, enrolled between 2015 and 2016 for an autism spectrum disorder clinical suspicious. Children were re-evaluated after at least 2 years of therapy and they were divided into two groups: the ASD-ASD group included children with a confirmed diagnosis of ASD, and the ASD-OO comparison group included children who no longer met the criteria for an autism classification. The aim of this retrospective study was to investigate the presence of cognitive, emotional and relational predictors capable of predicting the presence of optimal outcome in with a diagnosis of autism; the predictors taken into consideration were the intelligence quotient, the play, the emotional contagion and the understanding of other’s intentions. In this way, it is possible to support clinicians in defining a more complete diagnostic framework of autism, using assessment tools that can be administered quickly and therefore suitable for short observation sessions in paediatric patients. Results The findings showed that 15 out of 40 children, after at least for 2 years, no longer fell into the diagnostic ASD category based on the ADOS-2, DSM-5 and clinical criteria. The children in the ASD-OO group initially had a higher IQ than those in the ASD-ASD group, lower severity of autistic symptoms, greater understanding of intentions, more emotional contagion, and better quality of play. The results suggest that the initial coexistence of skills in these areas at the time of the first diagnostic assessment may allow us to predict the possibility of achieving optimal outcome after 2 years of therapy. Conclusions The data of this study highlight the importance of considering, during assessment, intelligence quotient, play, emotional contagion, and understanding of the intentions of others as potential prognostic predictors that can become useful tools for clinicians and paediatricians. This allows us to focus attention, in both the diagnostic and prognostic phases, on emotional-relational variables that can support the clinician in defining a more complete diagnostic framework and in planning a more personalized therapeutic path.

Objective: The objective of the present study was to selectively evaluate the long-term impact of posterior fossa pilocytic astrocytomas, which are known to be among the most benign forms of paediatric brain tumours on neurocognitive and behavioural functions. Methods: Children that were operated on for a posterior fossa pilocytic astrocytoma in the Pediatric Neurosurgery Department of the Catholic University Medical School were selected according to the following criteria: (a) age &gt, 5 years (in order to have a complete set of neurocognitive evaluations data), (b) ability to perform a complete set of tests before and after surgery, and (c) children that had a regular follow-up up to 10 years from the surgical treatment. Results: Forty-three percent of the children selected for the present study showed a borderline IQ before surgery, which is a result corresponding to those previously reported in the literature for children affected by posterior fossa pilocytic astrocytomas, praxis and visual perception were the selective functions that were more frequently affected. Language performance tests scores were below average in 40% of the cases but tended to improve in terms of expressive and receptive skills even at the 1-year follow-up, the improvements became significant at the 5-year and 10-year follow-ups. Conclusions: Recognising and measuring the short- and long-term effects of cerebellar tumours in children and their treatment are the first step towards improving their clinical course and quality of life. Early interventions should be offered to all of them, with specific attention bestowed on visual-spatial stimulation, speech and occupational therapies in order to act on praxic and visuo-perceptive skills, as well as on emotion and behaviour tracts of the neurocognitive profile, which more commonly tend to persist in the long term.

Purpose The assessment of Autism Spectrum Disorders (ASD) in childhood has two essential aspects: the identification of the risk (under 30 months of age) and the definition of a diagnosis that takes into account its core areas as well as further non-specific aspects. The purpose of this paper is to present an approach that considers the combination of clinical evaluation with the use of tools that analyse the various levels of the child’s functioning as fundamental. Design/methodology/approach The comprehensive assessment at the Institute of Ortofonologia in Rome provides the ADOS-2 and the Leiter-R for the evaluation of the symptomatology, the severity level, the non-verbal cognitive functioning and the fluid reasoning; the TCE and the UOI are used to identify, respectively, the child’s emotional skills and the ability to understand the intentions of others, as precursors of the theory of mind. Within this assessment, the Brief-P, the Short Sensory Profile and the RBS are also included for the evaluation of executive functions, sensory pattern and of restricted and repetitive behaviours, as observed by parents. Findings How to define a reliable development profile, which allows to plan a specific intervention calibrated on the potential of the child and on his development trajectory, is described. Two clinical cases are also presented. Originality/value The entire process is aimed both at a detailed assessment of the child’s functioning and at identifying a specific therapeutic project and predictive factors for achieving an optimal outcome.

Background Piezosurgery (PS) has gained increasing dispersion in neurosurgery. In pediatric neurosurgery, the experience is limited to craniosynostosis surgery. The present study assesses PS in the pediatric population, also considering outcomes and complications in cranial and spinal procedures. Methods All consecutive craniotomies and laminotomies, performed with PS (group A) or conventional osteotomes (group B) in the 2014–2017 period were reviewed. The following variables were analyzed: dural tears, estimated blood loss and need of transfusion, cosmetic outcome (Sloan score), and operative times. A review of the pertinent literature is included. Results 172 children were enrolled, 90 in group A and 82 in group B. The mean follow-up time was 2.1 years. A statistically significant difference in favor of group A was found about EBL (105 vs. 113 ml) and late outcome (Sloan class A 98.5% vs. 91.5%). PS also reduced the risk of dural tears (1 vs. 7 cases in groups A and B, respectively) and blood transfusion (52% vs. 55.5%) but without statistical significance. The operative times were significantly shorter in group B (13 vs. 23 minutes), although the newer PS plus (PSP) was demonstrated to significantly shorten these times compared with the traditional PS (3.5 vs. 6.5 minutes for orbitotomy and 7.5 vs. 9.5 minutes for hemicraniotomy). Conclusions PS is a safe and effective tool that can be specifically recommended for bone splitting and graft, laminotomy, and craniotomy in cosmetically eloquent areas. The limit of operation times can be overcome by a learning curve in neurosurgery and PSP.

Background The optimal management of neonatal post-hemorrhagic hydrocephalus (PHH) is still debated, though several treatment options have been proposed. In the last years, ventriculosubgaleal shunt (VSgS) and neuroendosdcopic lavage (NEL) have been proposed to overcome the drawbacks of more traditional options, such as external ventricular drainage and ventricular access device. Methods We retrospectively reviewed neonates affected by PHH treated at our institution since September 2012 to September 2020. Until 2017 patients received VSgS as initial treatment. After the introduction of NEL, this treatment option was offered to patients with large intraventricular clots. After NEL, VSgS was always placed. Primary VSgS was reserved to patients without significant intraventricular clots and critically ill patients that could not be transferred to the operating room and undergo a longer surgery. Results We collected 63 babies (38 males and 25 females) with mean gestational age of 27.8 ± 3.8SD weeks (range 23–38.5 weeks) and mean birthweight of 1199.7 ± 690.6 SD grams (range 500–3320 g). In 6 patients, hemorrhage occurred in the third trimester of gestation, while in the remaining cases hemorrhage complicated prematurity. This group included 37 inborn and 26 outborn babies. Intraventricular hemorrhage was classified as low grade (I–II according to modified Papile grading scale) in 7 cases, while in the remaining cases the grade of hemorrhage was III to IV. Mean age at first neurosurgical procedure was 32.2 ± 3.6SD weeks (range 25.4–40 weeks). Death due to prematurity occurred in 5 patients. First-line treatment was VSgS in 49 patients and NEL in the remaining 14 cases. Mean longevity of VSgS was 30.3 days (range 10–97 days) in patients finally requiring an additional treatment of hydrocephalus. Thirty-two patients required one to three redo VSgS. Interval from initial treatment to permanent shunt ranged from 14 to 312 days (mean 70.9 days). CSF infection was observed in 5 patients (7.9%). Shunt dependency was observed in 51 out of 58 surviving patients, while 7 cases remained shunt-free at the last follow-up. Multiloculated hydrocephalus was observed in 14 cases. Among these, only one patient initially received NEL and was complicated by isolated trapped temporal horn. Conclusions VSgS and NEL are two effective treatment options in the management of PHH. Both procedures should be part of the neurosurgical armamentarium to deal with PHH, since they offer specific advantages in selected patients. A treatment algorithm combining these two options may reduce the infectious risk and the risk of multiloculated hydrocephalus.

Parents of children with autism spectrum disorder (ASD) have to address various challenges mainly due to their children’s atypia related to communication, emotion regulation and behaviors, arising also within the relationship with their caregivers. Several studies have pointed out that children with ASD can exhibit many difficulties regarding initiating and maintaining meaningful relationships with others. To date, little research has explored the interactions between children with ASD and their fathers, focusing more on mothers. In the context of parent–child interactions in the presence of autism, recent studies have highlighted the importance of parental attunement but there is little research considering solely the affective-bodily dimension. Therefore, the aim of the present study was to explore the parental attunement in fathers of children with ASD observed during play interactions and to investigate the relationship between paternal attunement and the perception of their psychological characteristics related to care relationships. The results highlight that fathers who describe themselves as better in affective care and sensitivity toward others more likely have an absence of paternal attunement during play interactions. The data presented are discussed in the light of intervention hypotheses, aimed at improving the relationship between fathers and children with autism.

Introduction Electrical source imaging (ESI) and especially hdEEG represent a noninvasive, low cost and accurate method of localizing epileptic zone (EZ). Such capability can greatly increase seizure freedom rate in surgically treated drug resistant epilepsy cases. Furthermore, ESI might be important in intracranial record planning. Case report We report the case of a 15 years old boy suffering from drug resistant epilepsy with a previous history of DNET removal. The patient suffered from heterogeneous seizure semiology characterized by anesthesia and loss of tone in the left arm, twisting of the jaw to the left and dysarthria accompanied by daze; lightheadedness sometimes associated with headache and dizziness and at a relatively short time distance negative myoclonus involving the left hand. Clinical evidence poorly match scalp and video EEG monitoring thus requiring hdEEG recording followed by SEEG to define surgical target. Surgery was also guided by ECoG and obtained seizure freedom. Discussion ESI offers an excellent estimate of EZ, being hdEEG and intracranial recordings especially important in defining it. We analyzed our results together with the data from the literature showing how in children hdEEG might be even more crucial than in adults due to the heterogeneity in seizures phenomenology. The complexity of each case and the technical difficulties in dealing with children, stress even more the importance of a noninvasive tool for diagnosis. In fact, hdEEG not only guided in the presented case SEEG planning but may also in the future offer the possibility to replace it.

The space existing between the external and internal dural layer has been anatomically described as a virtual space; no clear clinical significance has been given to it to date. We hereby describe a case of a child with what was expected to be a purely epidural hematoma, at surgery, was found to be composed of two equally coexistent components, one in the epidural space and the second one between the two dural layers. The recognition of a possible involvement of the transdural space in the case of post-traumatic epidural hematomas is relevant for a correct conclusion of the surgical treatment of epidural hematomas, extensively considered basic practice among neurosurgical procedures.

Early parent-child relationships are an important factor influencing many domains of child development, even in the presence of autism. In this study, we investigated the associations between parent-child attunement during play, parental insightfulness, and parental acceptance of their child's diagnosis of an autism spectrum disorder. A sample of 50 parents (26 mothers and 24 fathers) of 26 children aged between 24 and 58 months were videotaped during parent-child play interactions and then interviewed about what they thought had gone through their child's head during the play interaction, and about their feelings and thoughts about their child's diagnosis. Play interactions were evaluated using a coding protocol to assess parental attunement. The results showed that parents who were more able to accept their child's diagnosis and to see things from their child's perspective were more likely to also be attuned during play interactions with their children. These findings highlight the importance of studying the parental ability of insightfulness and acceptance of their child diagnosis of ASD for the implementation of intervention programs for supporting parental attunement and improving the interactions between the parents and the children with autism spectrum disorders.

Aim To analyze the current literature on neurocutaneous melanosis (NCM), as well as, our cases, in order to better define the prognosis and the presence of risk factors affecting it, thus, offering better information to the parents. Material and methods Two cases observed at the Pediatric Neurosurgery Unit of the Catholic University Medical School in Rome are described. Both of them had cutaneous stigmata and cerebral MR evidence of intracranial melanin deposits. These two children showed a very different clinical course. Results The present study enlighten the differences among the two cases and review the literature on the subject, with the attempt to understand which are clinical and disease related factors that might influence the prognosis. Conclusion Beside malignant features of cutaneous melanotic lesions, the presence of hydrocephalus at diagnosis and the early appearance of clinical symptoms, when appearing contemporarily, are predicting the rapid progression of the disease and a worse prognosis.

Purpose Sturge-Weber syndrome (SWS) is a neurocutaneous facomatosis characterized by facial and leptomeningeal angioma, glaucoma, seizures, and neurological disability. Therefore, a challenging multidisciplinary interaction is required for its management. The goal of this paper is to review the main aspects of SWS and to present an illustrative pediatric series. Methods The pertinent literature has been analyzed, focused mainly on etiopathogenesis, pathology, clinical features, diagnostic tools, management, and outcome of the disease. Moreover, a series of 11 children operated on for refractory epilepsy between 2005 and 2015 (minimum follow-up 5 years, mean follow-up 9.6 years) is reported. The series consists of six boys and five girls with 6.5-month and 16.2-month mean age at seizure onset and at surgery, respectively. Seizures affected all children, followed by hemiparesis and psychomotor delay (81%), glaucoma (54%), and other neurological deficits (45%). Results All children underwent hemispherectomy (anatomical in three cases, functional in two cases, hemispherotomy in six cases); one patient needed a redo hemispherotomy. Mortality was nil; disseminated intravascular coagulation and interstitial pneumonia occurred in one patient each; three children had subdural fluid collection. Eight patients (72%) are in the ILAE Class 1 (completely seizure and aura free), two in Class 2 (only auras, no seizure), and one in Class 3 (1-3 seizure days per year). AEDs discontinuation was possible in 73% of cases. The most important news from the literature concerned the pathogenesis (role of the mutation of the GNAQ gene in the abnormal SWS vasculogenesis), the clinical findings (the features and pathogenesis of the stroke-like episodes are being understood), the diagnostic tools (quantitative MRI and EEG), and both the medical (migraine, seizures) and surgical management (epilepsy). The epileptic outcome of SWS patients is very good (80% are seizure-free), if compared with other hemispheric syndromes. The quality of life is affected by the neurological and cognitive deficits. Conclusions SWS still is an etiological and clinical challenge. However, the improvements over the time are consistent. In particular, the neurosurgical treatment of refractory epilepsy provides very good results as long as the indication to treatment is correct.

Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs. A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus. Follow-up brain MRI 8 months later showed a huge growth of the tumor (90 × 80 × 65 mm) with polycyclic and apparently defined margins, cystic components, and diffuse contrast enhancement. Complete tumor removal was performed in two planned surgical steps. Histological diagnosis was CNS neuroblastoma. At a follow-up of 8 months, the child is in good clinical and neurological condition and is completing chemotherapy treatment according to the SIOP PNET 4 protocol. A thorough review of the literature confirms that primary CNS neuroblastoma has to be considered a distinct entity. The disease related mortality is 12.5%, lower than the one usually reported for other previously described as PNETs tumors. The most relevant factors influencing prognosis are the possibility of obtaining a complete tumor removal and age more than 3 years, which allows to include radiotherapy among treatment options.

Residual and recurrent intracranial aneurysms after surgical clipping present a persistent risk of bleeding. Secondary coiling after incomplete clipping represents a strategy to occlude the residual sac: feasibility, bleeding risk and outcome were evaluated through a systematic review of literature along with the series of two tertiary referral neurovascular centres. Demographics, ruptured status, aneurysm morphology, topography, exclusion at surgery, timing of secondary coiling, complications, occlusion rate and outcome were analysed. Percentage of incidence and 95% CI were calculated for all variables. T test was used for continue variables, whereas Fisher's test (two-sided) is for categorical ones. Overall, 102 patients (92 cases from literature and 10 cases from institutional series) were included. Mean age at diagnosis was 52.94 ± 12.17 years, and male/female ratio 0.5; 3/4 of aneurysms involved the anterior circulation, whereas ¼ the posterior circulation. An aneurysmal neck remnant was described in 58.43% of cases, an aneurysmal sac remnant in 29.21% and a regrowth in 12.36%. Residual aneurysm rupture was reported in 22% of cases. Complete/near-complete occlusion after secondary coiling was observed in 70% of cases, a partial in 25.56% and a failure in 4.44%. Only one case of perforation was reported. Complications were comparable to standard endovascular procedures. Aneurysms remnants after clipping are often observed in cases difficult anatomical locations. Their bleeding risk is not negligible. Secondary coiling is a rescue strategy to effectively and safely secure the aneurysm remnant. Only in a minority of cases, it is a staged treatment after 'remodelling' of the aneurysm neck.

Background Postoperative seizures (PSs) after neurosurgical operations are common but little is known about the role of surgical brain incision on their genesis. This topic has not been addressed so far. Objective To verify if the corticotomy affects the risk of PSs and postoperative epilepsy (PE) in children. Methods One hundred forty-three consecutive pediatric cases operated on for supratentorial lesions at the same institution in the last 15 yr have been retrospectively reviewed by dividing them into group A, 68 children who required brain corticotomy mainly for hemispheric tumors, and group B, 75 children treated through extracortical approaches mainly for suprasellar and optic tumors. Patients with possible "epileptic" biases, like preoperative seizures, were excluded. Results No significant differences have been found between group A and B as far as incidence of PSs (11.7% vs 14.5%) and PE (4.5% vs 6.5%), timing, and type of seizures are concerned after a mean 6.8 yr follow-up. The size of corticotomy in group A ( 3 cm2) had no impact on epileptogenesis as well as the other variables considered in both groups (age, sex, extent of lesion resection). Conclusion This study shows that the surgical cortical "trauma" would not represent a risk factor for PSs and PE. According to the present analysis and the literature, other causes seem to be involved (namely, electrolytic imbalance and brain gliosis). This information is important for preoperative surgical planning and postoperative management. A validation by both adult series and prospective studies is needed.

Cranioplasty aims at restoring the physiological integrity and volume of the skull. Any disproportion between the intracranial content and the volume of the container may favor the occurrence of complications. A classification of volume mismatches is proposed. A negative mismatch, consisting of intracranial content minor to skull volume, is well represented by the sinking flap. On the other side, a positive mismatch, consisting of intracranial content higher than skull volume, usually depends on CSF collection or hydrocephalus once the brain edema is regressed. Though, in children, this condition may result from physiological brain growth after decompressive craniectomy. Treatment algorithm based on this classification is presented. A 1-year-old boy with a severe traumatic brain injury underwent right decompressive craniectomy, evacuation of subdural hematoma, and dural expansion at another institution. After failure of autologous bone-assisted cranioplasty for infection, a helmet was recommended in order to postpone the cranial repair. Patient was admitted to our institution 3 years later. CT scan showed brain herniation through the cranial defect, associated to a condition of acquired craniocerebral disproportion, due to the condition of “open skull”. Augmented hydroxyapatite cranioplasty (CustomBone, Finceramica, Faenza, Italy) was performed in order to manage this rare condition of positive volume mismatch. Subsequent course was uneventful and no complication was recorded at 30-month follow-up. This illustrative case highlights the possible occurrence of a positive structural mismatch between the skull and the intracranial content after decompressive craniectomy, thus configuring a condition of acquired craniocerebral disproportion, aside of other brain or CSF complications. We firstly recognize this condition in the literature and propose it as a possible factor affecting the outcome of cranioplasty in infants and young children.

Treatment of intraosseous meningiomas is a two-staged procedure including tumor resection and cranial reconstruction. Several are the potential pitfall of this kind of surgery such as the conspicuous dimensions usually reached by the tumor and the peculiar involvement of irregular and deep bony structures. For these reasons, a surgical accurate virtual planning and a careful removal followed by tailored reconstruction are mandatory to achieve satisfactory results. We analyzed six patients operated on for intraosseous meningiomas between September 2014 and June 2018. Resection strategy was planned beforehand and shared with the manufacturer who provided the PEEK cranioplasty used in the reconstructive phase. Between September 2014 and April 2018, six patients affected by intraosseous meningioma were operated on. Female/male ratio was 5:1 and mean age was 54 ± 10.8 years. Mean FU was 20.3 ± 16.4 months. Mean dimension was 73.9 ± 24.8 mm × 69.2 ± 16.2 mm. Mean surgical time was 5.1 ± 1.1 h. The resection of intraosseous meningiomas requires the earliest and finest reconstructive phase. Custom-made implants should be considered the gold-standard for cranioplasty, especially in large skull and in frontal or hairless areas of the skull. The described technique is simple, accurate, and effective in achieving good results in disease control as well as cosmetic and functional restoration.

Desmoplastic infantile gangliogliomas (DIGs) and desmoplastic infantile astrocytomas (DIAs) are tumors typical of the infantile age. A large size, with a mixed solid and cystic component, clinical presentation with progressing signs of increased intracranial pressure, a prominent benign desmoplastic structure at histological examination, and a favorable clinical course in the majority of cases are the prominent features of these tumors. The objective of the present paper was to review the pertinent literature on the topic together with our personal experience, with the aim of an updated review of the subject. Only 28 papers are present in the literature devoted to DIGs and DIAs, most of them reporting on single cases or small series, with a total of 107 patients aged from 5 days to 48 months with a slight male prevalence. Most of the reported cases refer to supratentorial and hemispheric locations, a few cases involving the hypothalamic region, the posterior fossa, and the spinal cord. The typical MRI appearance is of large mixed solid and cystic tumors with a spontaneous hyperintense T2 appearance of the solid part which also shows a strong contrast enhancement. Mixed ganglionic and astrocytic cells are identifiable in DIGs, whereas DIAs are typically featured by the exclusive presence of glial cells. In both cases, more primitive cells may be observed, which present a higher number of mitoses and these areas can mimic the features of malignant astrocytomas. Surgery represents the treatment of choice; however, radical removal has been reported as possible only in around 30 % of the cases: the low age of the patients together with their low weight and the large size of and the hyper-vascularized structure of the tumors represent the main factors limiting surgery. Pure observation is considered as first choice in children undergoing a partial/subtotal tumor resection, chemotherapic regimens being considered in cases of recurrences after a second look surgery. Long-term prognosis is favorable with mortality being related mostly to the rare midline (i.e., hypothalamic) locations, which beyond the functionally relevant site, tend to have an unusually more aggressive histological behavior.

Background and aims The daily challenges of caring for a child with autism spectrum disorder affect many areas of everyday life and parental well-being, as well as parents’ ability to manage the needs of the family and the child concerned. A better understanding of parents’ perception of their child’s characteristics can allow better support for them and individualize intervention protocols in a more accurate way. The main objective of this study is the evaluation of the perception of stress by parents of children with autism compared to parents of children with specific language impairment. Methods The parents of 87 children aged between 2 and 6 years were included in this study, 34 children with a specific language impairment diagnosis and 53 children with an autism spectrum disorder diagnosis (ASD) or at risk of developing it. They were asked to complete a self-report on perceived stress and rating scales on adaptive/problematic behaviours, executive functions and sensory profile of the child. Results The results reveal that parents of ASD children, compared to the control group, showed significantly higher levels of stress, mainly due to the difficulty of managing unexpected events, the feeling of loss of control over one's life and the fear of not being able to cope with the adversities they were experiences. The most critical area, both for ASD and control group, concern the executive function related to emotional reactions. Conclusions Thus, we argue that the difficulties in self-control, sensory modulation and emotional regulation, represent an element of stress for parents of children with developmental disorders. Implications: Regarding the difficulties of children with ASD, supporting the ways in which caregivers adapt to the signals of children is an important strategy, which has now become a key element of treatments for autism mediated by parents.

The aim of this paper is to review the current knowledge on the management of local infections in posterior cranial fossa (PCF) and spinal surgery; the center of our paper will be the diagnosis and management of infections of the wound, the subcutaneous tissue, and muscle strata. Very few papers address the problem of local infections in posterior cranial fossa and spinal surgery. The largest amount of the literature data is indeed centered on how to deal with deep infections, CSF leak, and secondary meningitis or hardware infection. Concerning PCF surgery, a higher rate of local infections has been reported, compared with supratentorial surgery. To prevent such complications, prophylaxis remains the most important tool in dealing with local infections; a careful surgical planning and patient selection are also important. On the other hand, in dealing with local infection in spinal setting, there are several evidences that local vancomycin might help reducing them in adults as well as in children. The treatment protocol consists in antibiotic therapy and, whenever needed, wound revision to remove infectious material or to obtain a microbiological diagnosis. Prognosis has overall to be considered favorable both in the short term as well as in the long term.

We have simplified the published procedure (5) for measuring sweat rates in individual human sweat glands. Sweat secretion rates were obtained from sweat drops secreted on the forearm by multiple individual glands. We computed a ratio between CFTR-dependent (by intradermal microinjection of a β adrenergic cocktail) and CFTR-independent (by methacoline as cholinergic stimulus) sweat secretion rates. We obtained a reproducible, approximately linear readout of CFTR function with measurements performed by two different independent teams. We considered three groups (CF subjects, CF carriers and non-CF controls, n=22 in each group); their mean ratios was respectively 0.000, 0.104 and 0.205 The average ratio of CF subjects was consistent with diagnosis in 3 additional cases clinically resembling CF. All groups were clearly discriminated, with sensibility and specificity ranging from 82% to 100%. A software was developed for detecting sweat droplets. This bioassay is suitabile for multicentre studies focusing on CFTR targeted therapies, controversial diagnosis and functional relevance of rare CFTR mutations.

The management of obstructive hydrocephalus associated to posterior cranial fossa tumor remains a controversial issue. In this context, the role of endoscopic third ventriculostomy (ETV) performed before tumor removal (the so-called preoperative ETV) is debated and its success rate is reported as being largely variable in the literature. We describe two children who experienced the closure of a preoperatively performed ETV after the surgical resection of their posterior fossa tumor. In both cases tumor removal was performed in the prone position. Radiological investigations and intraoperative evidence documented that the failure of the ETV was due to a blood clot which directly occluded the stoma. We speculate that the prone position favored the migration of blood, through the aqueduct, thereafter, clotting on the most declivous part of the third ventricle once the patient assumed the upright position, with the consequent occlusion of the stoma. Based on a gravitational hypothesis, we named this mechanism the "snow globe effect."

Shunt malfunction represents one of the most common problem to deal with in the daily neurosurgical practice. Among the variety of possible presentations, the cyst-like dilatation of the brain around the proximal catheter is one of the most rare and misleading type. The case of a boy with a 'tumour-like' proximal shunt malfunction is described and the pertinent literature is analysed.

The authors reported on a 63-year-old diabetic male who developed a strange wandering hemiparesis affecting first left side and then right side, not consistent with a right parasagittal meningioma nor with ischemic stroke. The subsequent rapid worsening of clinical picture, with occurrence of paraparesis, urinary incontinence and midthoracic sensory level, together with the evidence of leucocytosis, led to the diagnosis of a T6-T7 spondylodiscitis with spinal cord compression. The authors underlied the difficulties in diagnosing this condition in the setting of general hospital practice and the importance of clinical and neurological examination.

Autism is a neurodevelopmental disorder characterized by impairments in social communication and presence of restricted and repetitive patterns of behavior and interests, including abnormal reactions to sensory stimuli. Atypical responses to sensory input are increasingly recognized as a common feature of ASDs. In addition to repetitive patterns of behavior, the inclusion of sensory aspects in the diagnosis of autism in the latest Diagnostic and Statistical Manual of Mental Disorders (DSM-5) is evidence of the prevalence of sensory dysfunctions in this population. There are several tools investigating these areas, mostly rating scales that, far from wishing to replace other methods, allow a better understanding of the alterations presents and help in defining clinical picture and individual trajectory so improving diagnosis and intervention. The purpose of this study was to investigate the relation between sensory and repetitive behavioral characteristics of a sample of 50 preschool children with ASD measured with the Short Sensory Profile and the Repetitive Behavior Scales-Revised. Results are consistent with previous researches describing the co-occurrence of repetitive behaviors and sensory response abnormalities in the ASD population. We found evidence to support that the most frequently dysfunctional sensory areas in ASD children regard tactile hypersensitivity, auditory filtering, hyporeactivity and low energy/weak; while among repetitive behaviors the most common are those stereotyped. Furthermore it emerged that direct assessment with ADOS-2, a gold standard test for ASDs, and indirect assessment with questionnaires converged on the same profiles, so suggesting the usefulness to hold together both ecological and structured observations.

Objective: In developmental age the drawing process represents one of the best tools to assess the level of maturity reached by the child in his various areas of functioning. This study considers a sample of 84 children aged between 2.5 and 15 years, all diagnosed with autism, evaluated by an expert team of professionals, in order to highlight the drawing stage reached and the correlation with the severity of autistic symptoms assessed by the ADOS and cognitively evaluated with the Leiter-R nonverbal scale. Methods: The drawing level was observed in spontaneous production by establishing a scale ranging from the presence of the disordered scribbling to the presence of the body schema, taking into account the stereotypes that can lead to some echo-graphic representations that are similar to the echo-lalic productions of the verbal language. Results: The results showed a significant correlation between the drawing production and the Social-Affection component of the ADOS, so demonstrating that the delay in the drawing is heavily influenced by an emotional as well as intellectual inhibition. Conclusion: Through some examples of drawings it is also emphasized the relationship between drawing and chronological age and the difference that emerges between drawings made in the evaluation context and those that come to life during the therapeutic process.

Unfortunately in the original publication, the affiliation provided for the author G. Tamburrini was incorrect. The correct affiliation for G. Tamburrini should read as follows.

PURPOSE: The provirus integration site for Moloney murine leukemia virus 1 (Pim-1) kinase is overexpressed in various tumors and has been linked to poor prognosis. Its role as proto-oncogene is based on several Pim-1 target proteins involved in pivotal cellular processes. Here, we explore the functional relevance of Pim-1 in colon carcinoma. EXPERIMENTAL DESIGN: RNAi-based knockdown approaches, as well as a specific small molecule inhibitor, were used to inhibit Pim-1 in colon carcinoma cells. The effects were analyzed regarding proliferation, apoptosis, sensitization toward cytostatic treatment, and overall antitumor effect in vitro and in mouse tumor models in vivo . RESULTS: We demonstrate antiproliferative, proapoptotic, and overall antitumor effects of Pim-1 inhibition. The sensitization to 5-fluorouracil (5-FU) treatment upon Pim-1 knockdown offers new possibilities for combinatorial treatment approaches. Importantly, this also antagonizes a 5-FU-triggered Pim-1 up-regulation, which is mediated by decreased levels of miR-15b, a microRNA we newly identify to regulate Pim-1. The analysis of the molecular effects of Pim-1 inhibition reveals a complex regulatory network, with therapeutic Pim-1 repression leading to major changes in oncogenic signal transduction with regard to p21 Cip1/WAF1 , STAT3, c-jun-N-terminal kinase (JNK), c-Myc, and survivin and in the levels of apoptosis-related proteins Puma, Bax, and Bcl-xL. CONCLUSIONS: We demonstrate that Pim-1 plays a pivotal role in several tumor-relevant signaling pathways and establish the functional relevance of Pim-1 in colon carcinoma. Our results also substantiate the RNAi-mediated Pim-1 knockdown based on polymeric polyethylenimine/ small interfering RNA nanoparticles as a promising therapeutic approach.

The article aims to highlight some predictive indicators of improvement in ADOS scores in a group of 49 children with Autism Spectrum Disorders. For this purpose we created a specific protocol named T.U.L.I.P. that, using the Fluid Reasoning of the Leiter-R, the presence of Emotional Contagion (TCE) and the ability to understand the intentions of the others (UOI) as predictive indicators, can identify a category of autistic children who positively respond to treatment and improves the autistic symptomatology. The children who at the intake had predictive indicators of Emerging or Present UOI and TCE improved their autistic symptomatology with therapy and some of them (those with Present indicators) positively changed their ADOS diagnosis. In pre-school children, or in the first year of their schooling, the assessment of cognitive and social components shows that relational skills have a greater importance in predicting the decrease of the ADOS scores. The presence of predictive indicators, especially on the emotional response and the ability to understand the intentions of the others, also helps to work through a developmental- relational approach that activates in children their existing potentialities to get improvements even in the cognitive functioning. The Social Affection component of the ADOS-2 correlates with predictive indicators and accounts for an intervention targeted to the emotional dimension.

This study examines the context of executive functioning in preschool children with Autism Spectrum Disorders (ASDs) and verifies the degree of agreement between the compilation of the Behavior Rating Inventory of Executive Functions-Preschool Version (BRIEF-P) by parents and therapists. To this end, parents and professionals of 46 ASD children aged between 24 and 71 months completed the BRIEF-P in order to highlight the level of agreement between these two different evaluators and then investigate the correlations between executive functions impairment, the degree of autistic symptomatology, the level of cognitive development, the chronological age and the therapy duration. The results showed that parents tend to underestimate the impairment of executive functions in ASD children compared to what estimated by specialized operators. A low level of agreement was found between the two evaluators. Moreover, both for parents and professionals, the EF impairment was not related to the severity of autistic symptoms, assessed with the ADOS-2, while it was correlated to cognitive development in professional evaluations, and to chronological age and therapy duration in parental evaluations. These results indicate that, even if among the pathogenic assumptions about ASDs there has been included also that of the EF impairment, this, if present, does not correlate with the severity of autistic symptoms. With regard to cognitive development, the greater correlation between cognitive and executive impairment seems to be rather obvious. The use of the BRIEF-P completed by parents allows them a greater awareness but does not add clinical data to the diagnosis.

Background: Children with autism spectrum disorder show a deficit in neurobiological processes. This deficit hinders the development of intentional behavior and appropriate problem-solving, leading the child to implement repetitive and stereotyped behaviors and to have difficulties in reciprocal interactions, empathy and in the development of a theory of mind. The objective of this research is to verify the effectiveness of a relationship-based approach on the positive evolution of autistic symptoms. Method: A sample of 80 children with autism spectrum disorder was monitored during the first four years of therapy, through a clinical diagnostic assessment at the time of intake and then in two follow-up. Results: The results showed that through the Autism Diagnostic Observation Schedule it is possible to assess the socio-relational key elements on which the therapy is based. There was evidence, in fact, of significant improvements after two and four years of therapy, both for children with severe autistic symptoms and for those in autistic spectrum. Conclusions: Socio-relational aspects represent the primary element on which work in therapy with autistic children and can be considered as indicators of a positive evolution and prognosis that will produce improvements even in the cognitive area.

Although the c-Myc oncogene is frequently deregulated in human cancer, its involvement in the pathogenesis of glioblastoma is not clear. We conducted immunohistochemical analysis of the expression of c-Myc, polycomb ring finger oncogene (BMI1), and acetylation of the lysine 9 (H3K9Ac) of histone 3 in 48 patients with glioblastoma who underwent surgery followed by radiotherapy and temozolomide treatment. The expression of c-Myc, BMI1, and H3K9ac was correlated with clinical characteristics and outcome. We found that overexpression of c-Myc was significantly associated with that of BMI1 (P = .009), and that patients who harbored glioblastomas overexpressing c-Myc and BMI1 showed significantly longer overall survival (P < .0001 and P = .0009, respectively). Our results provide the first evidence of the prognostic value of c-Myc and associated genes in patients with glioblastoma. The favorable effect of c-Myc and BMI1 expression on survival is likely mediated by the sensitization of cancer cells to radiotherapy and temozolomide through the activation of apoptotic pathways.Although the c-Myc oncogene is frequently deregulated in human cancer, its involvement in the pathogenesis of glioblastoma is not clear. We conducted immunohistochemical analysis of the expression of c-Myc, polycomb ring finger oncogene (BMI1), and acetylation of the lysine 9 (H3K9Ac) of histone 3 in 48 patients with glioblastoma who underwent surgery followed by radiotherapy and temozolomide treatment. The expression of c-Myc, BMI1, and H3K9ac was correlated with clinical characteristics and outcome. We found that overexpression of c-Myc was significantly associated with that of BMI1 (P = .009), and that patients who harbored glioblastomas overexpressing c-Myc and BMI1 showed significantly longer overall survival (P < .0001 and P = .0009, respectively). Our results provide the first evidence of the prognostic value of c-Myc and associated genes in patients with glioblastoma. The favorable effect of c-Myc and BMI1 expression on survival is likely mediated by the sensitization of cancer cells to radiotherapy and temozolomide through the activation of apoptotic pathways.

Polycythemia associated with acromegaly is usually caused by the systemic manifestations of the disease, such as sleep-apnea or concomitant erythropoietin-secreting kidney tumors. The recognition of underlying pathologies requires a thorough diagnostic process. We report a unique case of acromegaly with polycythemia, not caused by commonly described manifestations of the disease, and receding with octreotide therapy. The medical history of 141 acromegalic patients followed by the Endocrinology Unit of the San Martino University Hospital in Genoa has been also reviewed, together with the literature evidence for similar cases. The diagnostic workflow and 2-years follow-up of a 43-years old acromegalic, polycythemic man with a history of past smoking, moderate hypertension, and mental retardation are described. The hematological parameters of our cohort was retrospectively compared with those of a healthy, age/gender-related control group as well. Therapy with octreotide LAR, 20 mg i.m. q28d was begun soon after diagnosis of acromegaly in the polycythemic patient. Haematocrit level, hormonal setting, as well as pituitary tumor size and visual perimetry during treatment were recorded. Octreotide LAR treatment normalized hormonal alterations, as well as hematological parameters. Polycythemia has not recurred after 2 years of therapy. The median hemoglobin and hematocrit levels of the retrospectively analyzed cohort of acromegalic were significantly lower than normal ranges of a healthy, age/sex- related control population. In conclusions, polycythemia can be a direct, albeit rare, secondary manifestation of acromegaly, that must be considered during the diagnostic work-up of acromegalic patients presenting with such disorder.

90 Italian children (72 boys, 18 girls) with a diagnosis of infantile autism (age range = 2.5–16.5 yr.) were assessed with a non-verbal intelligence test (Leiter–R). The test was repeated 3 times in four years. The measures used were IQ and Fluid Reasoning (FR), as evaluation of inductive and deductive reasoning, and the Autism Diagnostic Observation Schedule (ADOS), indicative of the severity of autistic symptoms at the beginning and at the end of therapy. The increase in the average IQ and FR scores at retests demonstrates the effectiveness of a treatment that emphasizes the centrality of the relationship-based approach. Moreover, the FR score at intake was predictive of a significant decrease of ADOS scores after four years of treatment, and of the increase in IQ observed in later evaluations. The data support the hypothesis that a relationship-based intervention allows cognitive improvement regardless of the autism severity expressed in the ADOS score.

Background In the large-scale trial, Gruppo Italiano per lo Studio della Sopravvivenza nell'Infarto Miocardico-3 (GISSI-3), patients receiving the combination of lisinopril and glyceryl trinitrate benefited most from experimental therapy. Therefore, a multicenter, randomized, double-blind study, Delapril Remodeling After Acute Myocardial Infarction (DRAMI), was designed to assess (1) the possible additive beneficial effect on left ventricular remodeling of nitrates when combined with an angiotensin-converting enzyme inhibitor (ACEI), and (2) the tolerability of a new ACEI, delapril, in respect to lisinopril in patients with large myocardial infarction (MI). Methods A total of 177 patients were randomized to receive delapril plus isosorbide-5-mononitrate (IS5MN) placebo, delapril plus IS5MN, lisinopril plus IS5MN placebo, or lisinopril plus IS5MN starting within the first 36 hours after the onset of symptoms and continuing for 3 months. Results More than 80% of the patients showed extensive ST-segment changes and 36.7% had signs or symptoms of heart failure during the first 36 hours. Over 3 months, IS5MN reduced, by 76%, the increase in LVEDV (17.4 ± 5.0 mL placebo vs 4.2 ± 4.4 mL IS5MN, P = .0439), reversed the increase in LVESV (7.5 ± 3.9 mL placebo vs −5.5 ± 2.9 mL IS5MN, P = .0052), and increased the recovery of LVEF (1.9% ± 1.3% placebo vs 6.7% ± 1.2% IS5MN, P = .0119). Overall, 3-month mortality was 10.2%; the most frequent clinical events were new episodes of severe heart failure (18.1%), persistent hypotension (10.7%), and post-MI angina (18.1%), with no differences between treatment groups. Conclusions Administration for 3 months of IS5MN combined with an ACEI, both started within 36 hours from the onset of symptoms, was safe and effective in reducing LV dilation and dysfunction after MI. The 2 ACEIs, delapril and lisinopril, appeared to be equally well tolerated.

Objective Duraplasty can be performed both by means of autologous tissues (such as galea-pericranium, temporal muscle, fascia lata) or by commercially available dural patches. Nowadays many neurosurgeons consider galea-pericranium duraplasty time-consuming, technically demanding or not adequate, thus dural surrogates are increasingly popular. In this prospective research we compared duraplasty using autologous galea-pericranium vs. dural patches in terms of postoperative long term results, ease/time required and costs. Patients and methods Research has been designed as prospective cohort study, that included 185 patients undergoing supratentorial elective neurosurgery with galea-pericranium or non-autologous duraplasty (minimum follow-up 12 months). Variables taken into account were: wound infection, CSF fistula, subcutaneous CSF collection, bone flap osteitis, brain abscess, empyema and tardive wound dehiscence (particularly after postoperative radiotherapy). Time for galea-pericranium collection, size of galea-pericranium harvest and dural defects were recorded in each case. Costs for non-autologous duroplasty were calculated. Results No statistically significant differences were evident in long term postoperative results between the two groups. Mean time of galea-pericranium collection is less than 2 min and enough galea-pericranium can be harvested in supratentorial approach to cover almost any dural defect. The only difference between the two groups is costs: an average of 268.7€/patient spent just for duraplasty. This figure is surely substantial if considered for the entire amount of surgeries performed in a department. Conclusions Considering that in our study long term results are equivalent, that galea-pericranium duraplasty is feasible and rapid, our indications are in favor of saving a considerable amount of money since an ideal autologous dural substitute is available and “free”.

Objectives. This study sought to assess the value of dipyridamole echocardiography in predicting reinfarction in patients evaluated early after uncomplicated acute myocardial infarction. Background. The identification of future nonfatal reinfarction seems an elusive target for physiologic testing. However, a large sample population is needed to detect minor differences in phenomena with a low event rate. Methods. We assessed the value of dipyridamole echocardiography in predicting reinfarction in 1,080 patients (mean [±SD] age 56 ± 9 years; 926 men, 154 women) evaluated early (10 ± 5 days) after uncomplicated acute myocardial infarction and followed up for 14 ± 10 months. Results. Submaximal studies due to limiting side effects occurred in 14 patients (1.3%); these test results were included in the analysis. Results of dipyridamole echocardiography were positive in 475 patients (44%). During follow-up, there were 50 reinfarctions: 45 nonfatal, 5 fatal (followed by cardiac death ≤4 days after reinfarction). Reinfarction (either nonfatal or fatal) occurred in 30 patients with positive and 20 with negative results (6.3% vs. 3.3%, p Conclusions. Dipyridamole echocardiographic positivity identifies patients evaluated early after uncomplicated acute myocardial infarction at higher risk of reinfarction, especially fatal reinfarction.

Decompressive hemicraniectomy (DHC) is performed to treat a variety of conditions, such as traumatic brain injury, subarachnoid hemorrhage, and brain swelling following middle cerebral artery (MCA) infarction. The rationale for this treatment is to remove a portion of bone and open the dura mater in order to decrease intracranial pressure (ICP) and prevent brain shift. However, the usefulness of routine hemicraniectomy for MCA infarction is still unclear because some patients may not progress to cerebral herniation [1]. We report on a man submitted to DHC for brain swelling and midline shift following right MCA infarction, whose clinical conditions rapidly ameliorated, despite an increase of cerebral herniation on post-operative CT scan. We, also, review the pertinent literature.

Primary empty sella syndrome (ESS) shares with idiopathic intracranial hypertension (IIH) high intracranial pressure (ICP) and association with obesity. Bariatric surgery is effective in IIH treatment; no data, however, exist about ESS. We report two overweight ESS patients in whom bariatric surgery resolved obesity but failed in curing ESS, requiring cerebrospinal fluid (CSF) shunt placement.

Object Communicating hydrocephalus is an uncommon complication in patients treated for glioblastoma multiforme (GBM). Its pathogenesis remains unclear. The authors evaluated the clinical and radiological factors associated with the onset of communicating hydrocephalus and the impact of ventriculoperitoneal (VP) shunt surgery on the outcome of these patients. Methods One hundred twenty-four patients harboring GBM, who had undergone craniotomy for tumor resection and adjuvant radiochemotherapy, were retrospectively assessed. Seven of them developed communicating hydrocephalus and were treated with VP shunt surgery. Clinical and radiological estimates included Karnofsky Performance Scale (KPS) score, previous surgery, overall survival (OS), CSF pressure and components, tumor location, and leptomeningeal dissemination. Results All 7 patients who developed communicating hydrocephalus had undergone at least 2 craniotomies for tumor resection before the onset of hydrocephalus (p = 0.0006; Fisher exact test). Six cases showed high levels of CSF proteins. There was a highly significant relationship between ventricular opening at surgery for tumor recurrence and onset of hydrocephalus (p = 0.0002; Fisher exact test). In these patients, VP shunt surgery was followed by a significant improvement of KPS score (p = 0.0180; Wilcoxon signed-rank test). The median OS after VP shunt insertion was 5 ± 2.9 months. Conclusions Ventricular opening after radiochemotherapy and high CSF protein levels are significant predictors of communicating hydrocephalus in patients with GBM. The VP shunt surgery improves quality of life in these patients.

Objectives. This study was conducted to assess the feasibility, safety and prognostic value of dipyridamole echocardiography in elderly patients recovering from an uncomplicated acute nyocardial infarction in a subset analysis performed on the patients entered in the subproject “residual ischemia” of the Echo Persantine Italian Cooperative Study (EPIC). Background. Coronary heart disease accounts for two thirds of all deaths in the age group >65 years, and >50% of all patients admitted to the hospital with acute myocardial infarction are >65 years old. The prognostic value of dipyridamole-induced left ventricular dysfunction was clearly established in patients evaluated early after acute infarction. Methods. In a subgroup analysis of the Echo Persantine Italian Cooperative Study (EPIC), we assessed the value of dipyridamole echocardiography in predicting cardiac events in 190 elderly (≥65 years) patients (age 68.4 ± 3.3 years, range 65 to 78; 147 men and 43 women) evaluated early (mean 10 days) after uncomplicated acute myocardial infarction and followed up for 14 ± 9.8 months. Results. There was no major side effect during dipyridamole echocardiography. A positive test result occurred in 85 patients (44.7%). During follow-up, there were 62 events (14 cardiac deaths, 7 nonfatal reinfarctions, 21 cases of class III or IV angina and 20 revascularization procedures). Of these 62 events, 44 occurred among 85 patients with positive dipyridamole echocardiography and 18 among 105 patients with negative dipyridamole echocardiography (52% vs. 17%, p < 0.001). Spontaneous events (death, reinfarction, angina) occurred in 31 patints with positive and in 11 with negative dipyridamole echocardiography (36% vs. 10%, p < 0.001). Hard events (myocardial infarction or death) occurred in 14 patients with positive and 7 with negative dipyridamole echocardiography (16% vs. 6%, p < 0.05). Death occurred in 11 patients with positive and in 3 with negative dipyridamole echocardiography (13% vs. 3%, p < 0.01). The positive predictive value of positive dipyridamole echocardiography and negative predictive value of negative dipyridamole echocardiography as related to the occurrence of all events in the follow-up period (death, reinfarction, angina, revascularization procedures) were 52% and 83%, respectively. The relative risk (that is, the relative risk of occurrence of future cardiac events in the group with positive dipyridamole echocardiography compared with that in those with negative dipyridamole echocardiography) was 3 for all events and 4.4 for death. Conclusions. Dipyridamole echocardiography was well tolerated by elderly patients and proved to be very effective in prognostic stratification early after uncomplicated acute myocardial infarction, even when only survival was considered.

purpose: To determine the prognostic capability of the dipyridamole echocardiography test (DET) early after an acute myocardial infarction. patients and methods: On the basis of 11 different echocardiographic laboratories, all with established experience in stress echocardiography and fulfilling quality-control requirements for stress echocardiographic readings, 925 patients were evaluated after a mean of 10 days from an acute myocardial infarction and followed up for a mean of 14 months. results: During the follow-up, there were 34 deaths and 37 nonfatal myocardial infarctions; 104 patients developed class III or IV angina and 149 had coronary revascularization procedures (bypass or angioplasty). Considering all spontaneous events (angina, reinfarction, and death), the most important univariate predictor was the presence of an inducible wall motion abnormality after dipyridamole administration (χ2 = 45.8). With a Cox analysis, echocardiographic positivity, age, and male gender were found to have an independent and additive value. Considering survival (and, therefore, death as the only event), age was the most meaningful parameter, followed by the wall motion score index during dipyridamole administration (χ2 = 12.1). Among other parameters, the resting wall motion score index was a significant predictor of death. In a multivariate analysis, the prognostic contributions of age (relative risk estimate = 1.08) and wall motion score index during dipyridamole administration (relative risk estimate = 4.1) were independent and additive. In particular, considering death only, the event rate was 2 % in patients with negative DET results, 4% in patients with positive high-dose DET results, and 7% in patients with positive low-dose DET results. conclusions: DET is feasible and safe early after uncomplicated myocardial infarction and allows effective risk stratification on the basis of the presence, severity, extent, and timing of the induced dyssynergy.

We have previously demonstrated peripheral nerve enlargement in acromegaly.The aim of this study was to use ultrasound (US) to assess any changes in the peripheral nerves of patients with acromegaly 1 year after the first evaluation.We prospectively examined the median and ulnar nerve cross-sectional area (CSA) in 34 non-diabetic, patients with acromegaly (18 females and 16 males; 18-79 years) and 34 age-, sex-, BMI-matched controls, using a 17-5 MHz US probe.The median nerve was examined at the mid-forearm (MN-f) and at the carpal tunnel (MN-Ct) levels; the ulnar nerve at mid-forearm (UN-f) and at distal arm (UN-a). Patients were grouped according to the clinical control of the disease: 'improved'; 'always controlled'; 'always uncontrolled'; and 'worsened'.The median nerve at mid-forearm (MN-f), the ulnar nerve at mid-forearm (UN-f) and at distal arm (UN-a) were significantly reduced after 1-year follow-up in all patients (P0.001, P0.008, P0.012, respectively). In the 'improved' group, there was a significant reduction of median nerve CSA examined at mid-forearm (MN-f) (P = 0.02), and distal arm ulnar nerve CSA (UN-a) (P = 0.002). In the other groups no statistically significant differences in ultrasound parameters were recorded. However, UN-a, UN-f, MN-f, MN-ct were still significantly higher in all groups compared with controls (P0.001).These data demonstrate that median and ulnar nerves CSA are reduced after 1 year follow-up, in line with the reduction of GH/IGF-I levels. However, as the control of the disease incompletely reverts nerve enlargement, this phenomenon could be only partially reversible.

Acromegalic patients may complain of sensory disturbances in their hands.Our objective was to examine median (MN) and ulnar nerves (UN) of acromegalic patients with ultrasound (US) and to determine whether nerve abnormalities correlate with clinical parameters and nerve conduction studies (NCS).We prospectively examined the MN and UN in 34 nondiabetic, acromegalic patients (18 females and 16 males; age range 18-79 yr) and 34 sex-, age-, and body mass index-matched controls with 17-5 MHz US.The MN was examined at the carpal tunnel (MN-Ct) and at mid-forearm (MN-f) levels; the UN at the mid-forearm (UN-f) and distal arm (UN-a). A total of 272 nerve cross-sectional areas (CSA) were recorded from both patients and controls. In addition, 22 patients underwent NCS.Nerves of acromegalic patients (MN-Ct=16.5+/-4.4 mm2; MN-f=10.5+/-2.4 mm2; UN-f=9.5+/-3.0 mm2; UN-a=13.1+/-3.7 mm2) had significantly (P0.0001) greater CSA compared with controls (MN-Ct=7.4+/-1.7 mm2; MN-f=5.5+/-1.4 mm2; UN-f=5.3+/-1.4 mm2; UN-a=6.6+/-1.7 mm2). NCS displayed at least one abnormality in 59% of patients. Acromegalic patients, grouped according to disease activity (14 controlled, 8 partially controlled, 12 uncontrolled), had significantly (P0.0001) greater CSA compared with controls. Nerve CSA were significantly greater in uncontrolled patients compared to controlled, both at MN-Ct and at UN-f levels (P0.01). Abnormal NCS were observed in five of seven uncontrolled patients and four of nine controlled patients. IGF-I levels, but not GH levels, were correlated with CSA (r=0.34), whereas disease duration correlated with both nerve CSA and NCS (r=0.33 and r=0.31).US identified a significantly increased volume of MN and UN in acromegalic patients. Peripheral nerve enlargement in acromegaly seems to be an intrinsic feature of the disease related to clinical control, disease duration, and IGF-I levels.

Criteria to define the response to somatostatin (SS) analogs (SSA) in acromegaly are based on biochemical control of the disease. However, the mechanisms of action of SSAs in inhibiting tumor growth and hormonal secretion are only partially understood, and the two effects may occur independently.The objective of the study was to investigate the dissociation between antiproliferative and antisecretive effects of SSA in an octreotide-resistant patient displaying dramatic tumor shrinkage during primary therapy with octreotide LAR.We characterized somatostatin and dopamine D(2) receptor expression by immunohistochemistry and real-time RT-PCR. The effects of different receptor-selective, bispecific analogs, and chimeric somatostatin/dopamine compounds on GH secretion and cell proliferation in primary cell cultures of the tumor were assessed.The expression of SS receptor subtypes (sst)(5) and D(2) receptor was higher, compared with the other receptor subtypes. GH inhibition by SS-14 and the two chimeric somatostatin/dopamine compounds was scant but greater than subtype-selective and sst(2)/sst(5) bispecific agonists. Conversely, cell growth was potently inhibited by all test substances. However, SS-14, sst(2)/sst(5) bispecific agonist, and chimeric molecules were more potent than the other compounds.The significant antiproliferative effect of octreotide seems to be related to the higher expression of sst(5) and the negligible antihormonal effect to the lower expression of sst(2). However, activation of multiple receptors by new analogs may produce better control of tumor cell activities. The dissociation between antisecretive and antiproliferative effects observed in vivo and in vitro confirms that SSAs may induce tumor shrinkage despite the lack of effect on GH secretion.