Multiple Sclerosis and Trigeminal Neuralgia: A Well-Noted Association with Unknown Occurrence as a Presenting Symptom: Our Experience in Multi Centric Study

Objective: The association between trigeminal neuralgia (TN) and multiple sclerosis (MS) is well established. In many cases series the frequency of TN is reported in patients with MS but information regarding the frequency of trigeminal pain as a symptom of disease onset is lacking. We present a multi centric analysis of SM-patients affected by TN where we investigated the onset of symptomatology and a current literature analysis. Patients and Methods: We prospectively acquired clinical and radiological information of patients with MS and with TN that were visited at our clinics in two different departments. A pertinent literature review was carried out as well. We investigated the prevalence of TN in patients with MS and we noted whether facial pain was the symptom of MS onset. Results: A total of 718 patients with multiple sclerosis were analyzed, of which 63 presented TN. The neuropathic facial pain was the presenting symptom of MS in 3 patients, that constituted 0.4% of cases. The results reported in the literature confirm a frequency of 0.4-1% of the cases in which the first symptom of MS is TN and are like those observed in our study. Conclusions: Is important to investigate the rate of debut of TN in SM patients to set up the correct diagnostic and therapeutic program. Among the different types of neuropathic pain, TN in MS patients are a difficult to treat with a relevant impact on the quality of life. TN constitutes a clinical picture frequently associated with the MS. Nevertheless, TN as a clinical manifestation of the onset of MS is a rare occurrence and is just noted that is rarely the first symptom of disease

Extradural spinal cavernous angiomas: report of seven cases

Abstract The authors describe seven cases of extradural spinal cavernous angioma. Although cavernoma itself is not rare, the extradural spinal localization is uncommon and makes preoperative differential diagnosis difficult. Routine MRI investigation has aided neurosurgeons in evaluating the true incidence of these vascular malformations, which was understimated in the past. The data published so far have not entirely clarified the treatment of choice for these lesions. Considering their rarity in this site, their presenting symptoms and the difficulties involved in neuroradiological diagnosis, the authors discuss the role of surgery as the principal form of treatment and review the relevant literature. Seven patients (4 male, 3 female) were admitted to our Institute of Neurosurgery between 1992 and 2004, with a 5-6 month history (range=2-365 days) of low back pain or radicular pain, sometimes associated with paresthesia. All patients had a CT scan, as well as MRI with gadolinium when possible, which detected an extradural roundish lesion: differential diagnosis was very difficult, especially between neurinoma and cavernoma. Treatment was always surgical and resection of the lesion radical. Postoperatively, all patients presented complete regression of clinical symptoms. In all cases histological diagnosis was cavernous angioma. Postoperative MRI with gadolinium or CT scan with IV contrast, performed before discharge, confirmed radical removal of the vascular malformation in all cases. Our experience confirms that surgery should be the treatment of choice for these lesions, in view of both their tendency to bleed and their straightforward surgical removal

Development of a Novel Device for Decompressive Craniectomy: An Experimental and Cadaveric Study and Preliminary Clinical Application

Background: Decompressive craniectomy is an intervention of established efficacy in patients with intractable cerebral edema. Objective: To evaluate a new device used in alternative to decompressive craniectomy. This device is designed to perform an augmentative craniotomy by keeping the bone flap elevated using specific cranial suspension titanium plates and giving the brain enough room to swell. Methods: We tested the mechanical characteristics of the cranial brackets on dried skulls, on 3D-printed skull models, and on a preserved cadaver head. The resistance of the device was examined through dynamometric testing, and the feasibility of the surgical technique, including the suspension of the bone flap and the skin closure, was investigated on the cadaveric model. A preliminary clinical series of 2 patients is also reported. Results: The laboratory tests have shown that this system allows an adequate expansion of the intracranial volume and it could withstand a force up to 637 ± 13 N in the synthetic model and up to 658 ± 9 N in the human skull without dislocation or failure of the brackets nor fractures of the bone ridges. Preliminary application in the clinical setting has shown that augmentative craniotomy is effective in the control of intracranial hypertension and could reduce the costs and complications associated with the classical decompressive craniectomy technique. Conclusion: Preliminary laboratory and clinical results show augmentative craniotomy to be a promising, alternative technique to decompressive craniectomy. Further clinical studies will be needed to validate its efficacy

Intramedullary non-specific inflammatory lesion of thoracic spine: A case report

<p>Abstract</p> <p>Background</p> <p>There are several non-neoplastic lesions which mimick intramedullary spinal cord neoplasm in their radiographic and clinical presentation. These can be classified as either infectious (TB, fungal, bacterial, parasytic, syphilis, CMV, HSV) and non-infectious (sarcoid, MS, myelitis, ADEM, SLE) inflammatory lesions, idiopathic necrotizing myelopathy, unusual vascular lesions and radiation myelopathy. Although biopsy may be indicated in many cases, an erroneous diagnosis of intramedullary neoplasm can often be eliminated pre-operatively.</p> <p>Case description</p> <p>the authors report a very rare case of intramedullary non-specific inflammatory lesion of unknown origin, without signs of infection or demyelinization, in a woman who showed no other evidence of systemic disease.</p> <p>Conclusions</p> <p>Intramedullary lesions that mimick a tumor can be various and difficult to interpret. Preoperative MRI does not allow a certain diagnosis because these lesions have a very similar signal intensity pattern. Specific tests for infective pathologies are useful for diagnosis, but histological examination is essential for establishing a certain diagnosis. In our case the final histological examination and the specific tests that we performed have not cleared our doubts regarding the nature of the lesion that remains controversial.</p

ITALIAN CANCER FIGURES - REPORT 2015: The burden of rare cancers in Italy = I TUMORI IN ITALIA - RAPPORTO 2015: I tumori rari in Italia

OBJECTIVES: This collaborative study, based on data collected by the network of Italian Cancer Registries (AIRTUM), describes the burden of rare cancers in Italy. Estimated number of new rare cancer cases yearly diagnosed (incidence), proportion of patients alive after diagnosis (survival), and estimated number of people still alive after a new cancer diagnosis (prevalence) are provided for about 200 different cancer entities. MATERIALS AND METHODS: Data herein presented were provided by AIRTUM population- based cancer registries (CRs), covering nowadays 52% of the Italian population. This monograph uses the AIRTUM database (January 2015), which includes all malignant cancer cases diagnosed between 1976 and 2010. All cases are coded according to the International Classification of Diseases for Oncology (ICD-O-3). Data underwent standard quality checks (described in the AIRTUM data management protocol) and were checked against rare-cancer specific quality indicators proposed and published by RARECARE and HAEMACARE (www.rarecarenet.eu; www.haemacare.eu). The definition and list of rare cancers proposed by the RARECAREnet "Information Network on Rare Cancers" project were adopted: rare cancers are entities (defined as a combination of topographical and morphological codes of the ICD-O-3) having an incidence rate of less than 6 per 100,000 per year in the European population. This monograph presents 198 rare cancers grouped in 14 major groups. Crude incidence rates were estimated as the number of all new cancers occurring in 2000-2010 divided by the overall population at risk, for males and females (also for gender-specific tumours).The proportion of rare cancers out of the total cancers (rare and common) by site was also calculated. Incidence rates by sex and age are reported. The expected number of new cases in 2015 in Italy was estimated assuming the incidence in Italy to be the same as in the AIRTUM area. One- and 5-year relative survival estimates of cases aged 0-99 years diagnosed between 2000 and 2008 in the AIRTUM database, and followed up to 31 December 2009, were calculated using complete cohort survival analysis. To estimate the observed prevalence in Italy, incidence and follow-up data from 11 CRs for the period 1992-2006 were used, with a prevalence index date of 1 January 2007. Observed prevalence in the general population was disentangled by time prior to the reference date (≤2 years, 2-5 years, ≤15 years). To calculate the complete prevalence proportion at 1 January 2007 in Italy, the 15-year observed prevalence was corrected by the completeness index, in order to account for those cancer survivors diagnosed before the cancer registry activity started. The completeness index by cancer and age was obtained by means of statistical regression models, using incidence and survival data available in the European RARECAREnet data. RESULTS: In total, 339,403 tumours were included in the incidence analysis. The annual incidence rate (IR) of all 198 rare cancers in the period 2000-2010 was 147 per 100,000 per year, corresponding to about 89,000 new diagnoses in Italy each year, accounting for 25% of all cancer. Five cancers, rare at European level, were not rare in Italy because their IR was higher than 6 per 100,000; these tumours were: diffuse large B-cell lymphoma and squamous cell carcinoma of larynx (whose IRs in Italy were 7 per 100,000), multiple myeloma (IR: 8 per 100,000), hepatocellular carcinoma (IR: 9 per 100,000) and carcinoma of thyroid gland (IR: 14 per 100,000). Among the remaining 193 rare cancers, more than two thirds (No. 139) had an annual IR &lt;0.5 per 100,000, accounting for about 7,100 new cancers cases; for 25 cancer types, the IR ranged between 0.5 and 1 per 100,000, accounting for about 10,000 new diagnoses; while for 29 cancer types the IR was between 1 and 6 per 100,000, accounting for about 41,000 new cancer cases. Among all rare cancers diagnosed in Italy, 7% were rare haematological diseases (IR: 41 per 100,000), 18% were solid rare cancers. Among the latter, the rare epithelial tumours of the digestive system were the most common (23%, IR: 26 per 100,000), followed by epithelial tumours of head and neck (17%, IR: 19) and rare cancers of the female genital system (17%, IR: 17), endocrine tumours (13% including thyroid carcinomas and less than 1% with an IR of 0.4 excluding thyroid carcinomas), sarcomas (8%, IR: 9 per 100,000), central nervous system tumours and rare epithelial tumours of the thoracic cavity (5%with an IR equal to 6 and 5 per 100,000, respectively). The remaining (rare male genital tumours, IR: 4 per 100,000; tumours of eye, IR: 0.7 per 100,000; neuroendocrine tumours, IR: 4 per 100,000; embryonal tumours, IR: 0.4 per 100,000; rare skin tumours and malignant melanoma of mucosae, IR: 0.8 per 100,000) each constituted &lt;4% of all solid rare cancers. Patients with rare cancers were on average younger than those with common cancers. Essentially, all childhood cancers were rare, while after age 40 years, the common cancers (breast, prostate, colon, rectum, and lung) became increasingly more frequent. For 254,821 rare cancers diagnosed in 2000-2008, 5-year RS was on average 55%, lower than the corresponding figures for patients with common cancers (68%). RS was lower for rare cancers than for common cancers at 1 year and continued to diverge up to 3 years, while the gap remained constant from 3 to 5 years after diagnosis. For rare and common cancers, survival decreased with increasing age. Five-year RS was similar and high for both rare and common cancers up to 54 years; it decreased with age, especially after 54 years, with the elderly (75+ years) having a 37% and 20% lower survival than those aged 55-64 years for rare and common cancers, respectively. We estimated that about 900,000 people were alive in Italy with a previous diagnosis of a rare cancer in 2010 (prevalence). The highest prevalence was observed for rare haematological diseases (278 per 100,000) and rare tumours of the female genital system (265 per 100,000). Very low prevalence (&lt;10 prt 100,000) was observed for rare epithelial skin cancers, for rare epithelial tumours of the digestive system and rare epithelial tumours of the thoracic cavity. COMMENTS: One in four cancers cases diagnosed in Italy is a rare cancer, in agreement with estimates of 24% calculated in Europe overall. In Italy, the group of all rare cancers combined, include 5 cancer types with an IR&gt;6 per 100,000 in Italy, in particular thyroid cancer (IR: 14 per 100,000).The exclusion of thyroid carcinoma from rare cancers reduces the proportion of them in Italy in 2010 to 22%. Differences in incidence across population can be due to the different distribution of risk factors (whether environmental, lifestyle, occupational, or genetic), heterogeneous diagnostic intensity activity, as well as different diagnostic capacity; moreover heterogeneity in accuracy of registration may determine some minor differences in the account of rare cancers. Rare cancers had worse prognosis than common cancers at 1, 3, and 5 years from diagnosis. Differences between rare and common cancers were small 1 year after diagnosis, but survival for rare cancers declined more markedly thereafter, consistent with the idea that treatments for rare cancers are less effective than those for common cancers. However, differences in stage at diagnosis could not be excluded, as 1- and 3-year RS for rare cancers was lower than the corresponding figures for common cancers. Moreover, rare cancers include many cancer entities with a bad prognosis (5-year RS &lt;50%): cancer of head and neck, oesophagus, small intestine, ovary, brain, biliary tract, liver, pleura, multiple myeloma, acute myeloid and lymphatic leukaemia; in contrast, most common cancer cases are breast, prostate, and colorectal cancers, which have a good prognosis. The high prevalence observed for rare haematological diseases and rare tumours of the female genital system is due to their high incidence (the majority of haematological diseases are rare and gynaecological cancers added up to fairly high incidence rates) and relatively good prognosis. The low prevalence of rare epithelial tumours of the digestive system was due to the low survival rates of the majority of tumours included in this group (oesophagus, stomach, small intestine, pancreas, and liver), regardless of the high incidence rate of rare epithelial cancers of these sites. This AIRTUM study confirms that rare cancers are a major public health problem in Italy and provides quantitative estimations, for the first time in Italy, to a problem long known to exist. This monograph provides detailed epidemiologic indicators for almost 200 rare cancers, the majority of which (72%) are very rare (IR&lt;0.5 per 100,000). These data are of major interest for different stakeholders. Health care planners can find useful information herein to properly plan and think of how to reorganise health care services. Researchers now have numbers to design clinical trials considering alternative study designs and statistical approaches. Population-based cancer registries with good quality data are the best source of information to describe the rare cancer burden in a population

LA NEUROCHIRURGIA ENDOSCOPICA NAVIGATA

Studio prospettico su una serie di 19 pazienti affetti da patologie intraventricolari o del basicranio approcciabili con metodica endoscopica. Scopo della trattazione è verificare quale tecnica di neuronavigazione, ottica od elettromagnetica, sia più adatta all’endoscopia trasfenoidale-transnasale e quale all’endoscopia transventricolare. Vengono, inoltre, valutati altri parametri quali gli eventuali allungamenti dei tempi operatori, l’ingombro sterico in sala operatoria ed i costi di gestione delle metodiche. Tramite la descrizione di alcuni casi clinici esplicativi vengono, infine, delucidate le tecniche impiegate, il layout di sala operatoria e l’utilità delle metodiche di navigazione nei casi specifici. Al termine dello studio si evince come la neuronavigazione di tipo ottico sia più idonea all’endoscopia transnasale, mentre quella di tipo magnetico si adatti meglio all’endoscopia transventricolare sfruttandone appieno le migliorie introdotte negli ultimi anni

Gliosarcomas in the elderly: analysis of 7 cases and clinico-pathological remarks.

AIMS AND BACKGROUND: Gliosarcomas are rare malignant primary brain tumors that usually affect the fifth or sixth decades of life. The purpose of this study was to describe our experience with such lesions in elderly patients and to establish their prognosis factors. METHODS: Between 1993 and 2001, 7 patients over 60 years of age were treated at our institute for cerebral gliosarcomas. All patients underwent surgery for total or at least sub-total removal of a neoplastic mass. RESULTS: Owing to poor clinical conditions (Karnofsky performance score = 40), one patient was not treated postoperatively. Remaining patients were treated with whole-brain radiotherapy, whereas concomitant chemotherapy (temozolomide) was administrated only to 4 patients. Histological examination showed the prevalence of sarcomatous aspects in 3 patients; the gliomatous aspect prevailed in 4 patients. CONCLUSIONS: Sarcomatous aspects and multimodality treatment (surgery, radiotherapy and chemotherapy) were associated with a better prognosis and showed in these elderly patients a trend similar to that of young people

Surgical management of falcine meningiomas: Experience of 95 patients

Falcine meningiomas, defined as a meningiomas arising from the falx not involving the superior sagittal sinus, account for 9% of all intracranial meningiomas. We analyzed 95 patients with falcine meningiomas who underwent surgical removal of their lesion at our institution between 2001 and 2014. Surgical management of these patients, focusing on anatomical and clinical features is described. Thus, based on our series, a surgical algorithm, classifying the falcine meningioma into four types, according to location at the falx, and using an ipsilateral interhemispheric approach in supine or prone position, is described. The median length of follow-up was 7.1years (range 1.6-12.3years). Approximately one-third of all patients was asymptomatic, headaches occurred in 27 patients, seizures in 14 cases, and lower-extremity weakness in 9 cases. In this series, the middle third of the falx was the most frequently involved site (55,78%), while the anterior third (26,31%) and the posterior type (17,89%) were less common. The transitional and meningothelial types occurred in 69 of patients and a high grade in only two patients. Compared with previous series in literature, there was no mortality and Gross Total Resection was obtained in 83 (87,5%) cases. Three of 95 patients experienced new or worsened neurological deficits after surgery while other complications were relatively in only 6 cases. This study presents our good results about removal of the tumor while preserving major cortical veins and the sinus using advanced microsurgical tools

Minimally Invasive Transforaminal Lumbar Interbody Fusion with Percutaneous Bilateral Pedicle Screw Fixation for Lumbosacral Spine Degenerative Diseases. A retrospective database of 40 consecutive treated cases and literature review

To report our results about minimally invasive transforaminal lumbar interbody fusion (MI-TLIF) with bilateral pedicle screw fixation, in patients with degenerative lumbosacral spine disease. To describe the indications, surgical technique and results of a consecutive series of 40 patients undergone MI-TLIF. Despite the limited number of clinical studies, published data suggest tremendous potential advantages of this technique

Trigeminal neuralgia secondary to intracranial neoplastic lesions: a case series and comprehensive review

It is known that intracranial tumors may trigger trigeminal neuralgia (TN) in some patients although the exact prevalence and occurrence is not completely defined yet. In the present study, we present a case series of patients with brain tumor and a clinical diagnosis of TN as the first and main manifestation of the disease. A retrospective analysis was performed involving patients diagnosed with brain tumor whose exclusive clinical feature our department focused on was TN. In addition, a review of all published cases was performed. From January 2017 to November 2018, 718 patients with brain tumor were admitted to our department, 17 of which suffered of TN, of which 8 patients presented with at least another neurologic symptom and 9 patients presented with TN alone, with typical symptoms of stubbing electric pain in 6 cases. In our series, we found that 2.3% of patients admitted for brain tumors had TN. In 0.8% of cases, TN was the main clinical symptom. The prevalence of tumor lesion in patients with facial neuropathic pain is not defined, but it is a well-known recognized initial symptom; however, early cerebral magnetic resonance imaging (MRI) is not yet strongly recommended in patients with newly diagnosed trigeminal neuralgia. The purpose of this article is, especially in unusual cases, to show that the application of such MR techniques and preoperative evaluation may contribute to diagnosis, indication, and surgery planning