Clinical and magnetic resonance study of a case of subacute sclerosing panencephalitis treated with ketogenic diet

Background Subacute sclerosing panencephalitis is a progressive neurodegenerative disorder caused by a latent and mutant measles virus which is extremely rare in developed countries. The lack of effective treatments leads to the research of other anti-inflammatory and neuroprotective treatments. Case Here we present a case of a 17-year-old patient affected by subacute sclerosing panencephalitis who manifest a dramatic improvement in neurological and general clinical conditions, as well as an arrest in the progression of demyelinating process in the central nervous system, after the beginning of a high ratio ketogenic diet. Conclusions Given its anti-inflammatory, antioxidant and metabolic effects, we believe that ketogenic diet utilisation could be a rational approach, can be considered a safe add-on therapy, carrying on with only a minimal risk of adverse effects or interactions

Imaging practice in low-grade gliomas among European specialized centers and proposal for a minimum core of imaging

Objective: Imaging studies in diffuse low-grade gliomas (DLGG) vary across centers. In order to establish a minimal core of imaging necessary for further investigations and clinical trials in the field of DLGG, we aimed to establish the status quo within specialized European centers. Methods: An online survey composed of 46 items was sent out to members of the European Low-Grade Glioma Network, the European Association of Neurosurgical Societies, the German Society of Neurosurgery and the Austrian Society of Neurosurgery. Results: A total of 128 fully completed surveys were received and analyzed. Most centers (n=96, 75%) were academic and half of the centers (n=64, 50%) adhered to a dedicated treatment program for DLGG. There were national differences regarding the sequences enclosed in MRI imaging and use of PET, however most included T1 (without and with contrast, 100%), T2 (100%) and TIRM or FLAIR (20, 98%). DWI is performed by 80% of centers and 61% of centers regularly performed PWI.ConclusionA minimal core of imaging composed of T1 (w/wo contrast), T2, TIRM/FLAIR, PWI and DWI could be identified. All morphologic images should be obtained in a slice thickness of 3mm. No common standard could be obtained regarding advanced MRI protocols and PET. Importance of the study: We believe that our study makes a significant contribution to the literature because we were able to determine similarities in numerous aspects of LGG imaging. Using the proposed minimal core of imaging in clinical routine will facilitate future cooperative studies

Imaging practice in low-grade gliomas among European specialized centers and proposal for a minimum core of imaging.

OBJECTIVE: Imaging studies in diffuse low-grade gliomas (DLGG) vary across centers. In order to establish a minimal core of imaging necessary for further investigations and clinical trials in the field of DLGG, we aimed to establish the status quo within specialized European centers. METHODS: An online survey composed of 46 items was sent out to members of the European Low-Grade Glioma Network, the European Association of Neurosurgical Societies, the German Society of Neurosurgery and the Austrian Society of Neurosurgery. RESULTS: A total of 128 fully completed surveys were received and analyzed. Most centers (n = 96, 75%) were academic and half of the centers (n = 64, 50%) adhered to a dedicated treatment program for DLGG. There were national differences regarding the sequences enclosed in MRI imaging and use of PET, however most included T1 (without and with contrast, 100%), T2 (100%) and TIRM or FLAIR (20, 98%). DWI is performed by 80% of centers and 61% of centers regularly performed PWI. CONCLUSION: A minimal core of imaging composed of T1 (w/wo contrast), T2, TIRM/FLAIR, PWI and DWI could be identified. All morphologic images should be obtained in a slice thickness of ≤ 3 mm. No common standard could be obtained regarding advanced MRI protocols and PET. IMPORTANCE OF THE STUDY: We believe that our study makes a significant contribution to the literature because we were able to determine similarities in numerous aspects of LGG imaging. Using the proposed "minimal core of imaging" in clinical routine will facilitate future cooperative studies

Opportunities and Obstacles Associated With Sequential Immune Reconstitution Therapy for Multiple Sclerosis: A Case Report

Cladribine and alemtuzumab have proven to be effective treatments for relapsing-remitting multiple sclerosis (RRMS), and both act as immune reconstitution therapies administered in a pulsed manner (1–3). Disease activity may occur early after the first course of treatment. However, this does not necessarily imply a treatment failure that requires further modifications to the treatment strategy. For this reason, drug response evaluation is generally performed at least a few months after the second drug course (4). Nevertheless, relevant disease activity early after a treatment course of one of these drugs may sometimes represent a serious clinical problem, potentially leading to permanent disability. In the CLARITY trial, interferon beta-1a rescue therapy was used (1). However, evidence of managing such a problem is scarce, subsequently leading to different clinical choices in a real-world setting (5). Here, we report a case of considerable ongoing disease activity after the first course of cladribine treatment, which was managed with alemtuzumab administration. Data regarding this sequence of therapies, which act through immune system depletion and reconstitution, are lacking, and real-world observations are, therefore, of interest. After alemtuzumab treatment, the patient achieved disease stability; however, several infectious complications were observed. This suggests that this sequential treatment strategy can be applied but warrants caution and careful monitoring

Double osseous flaps for simultaneous midfacial and mandible reconstruction: Automation in surgical complexity within an entirely computerized workflow

IntroductionBroad maxillofacial surgical resections involving both the midface and the mandible represent a challenge in terms of reconstruction. Although several papers have explored the possibility of simultaneously using two microsurgical flaps, reports on the implementation of a dual osseous flap strategy are limited, and mainly addressed to static anatomical reconstruction, regardless of functional implications. In particular, there is a lack in the literature of a unifying protocol which illustrates how technology including virtual planning, statistical shape modeling, virtual occlusion, 3D-printing and patient-specific implants can address the functional and accuracy needs required for an optimal reconstruction. Materials and methodsIn this paper, the Authors present their preliminary experience in a two-center study, showing how broad maxillofacial defects, requiring a simultaneous reconstruction in both the mandible and the midface, can be successfully reconstructed using the combination of two osseous flaps in an automated sequence in which all steps are anticipately defined in a virtual plan, accounting for the optimal alignment of temporomandibular joint, predicting the final occlusion and defining a mandibular shape according to a statistical shape model. ResultsAverage RMSE for the iliac bone crest flap was of 3.2 +/- 0.36 mm; for the fibula flap, RMSE value was of 2.3 +/- 0.65 mm, for patient-specific implants, for mandibular prostheses the average RMSE was 2.46 mm with 0.76 mm standard deviation. Temporomandibular joint function increased when a TMJ prosthesis was placed. ConclusionsDouble bone free flap is a valuable resource to reconstruct wide defects that simultaneously involve two thirds of the cranio-maxillo-facial skeleton, but a careful virtual planning study should be always performed before approaching this surgical option

Anatomical Computerized Exploration to Excise Malignancies in Deep Facial Compartments: An Advanced Virtual Reality Protocol for a Tailored Surgical Approach

Objective/Hypothesis: This study describes the design and application of a novel advanced protocol for virtual three-dimensional anatomical reconstruction of the deep facial compartments, aiming to improve the preoperative understanding and the intraoperative assistance in complex resective surgeries performed for malignant diseases which extend in complex spaces, including the pterygomaxillopalatine fossa, the masticator space, and the infratemporal fossa. Methods: This study is a non-profit, retrospective, and single-institution case series. The authors clearly describe in detail imaging acquisition protocols which are suitable to segment each target, and a multilayer reconstruction technique is presented to simulate anatomical structures, with particular focus on vascular networks. Virtual surgical planning techniques are individually designed for each case to provide the most effective access to the deep facial compartments. Intraoperative guidance systems, including navigation and virtual endoscopy, are presented, and their role is analyzed. Results: The study included seven patients with malignant disease located in the deep facial compartments requiring radical resection, and all patients underwent successful application of the protocol. All lesions, except one, were subject to macroscopically radical resection. Vascular structures were identified with overall reconstruction rates superior to 90% for major caliber vessels. Prominent landmarks for virtual endoscopy were identified for each case. Conclusions: Virtual surgical planning and multilayer anatomical reconstruction are valuable methods to implement for surgeries in deep facial compartments, providing the surgeon with improved understanding of the preoperative condition and intraoperative guidance in critical phases for both open and endoscopic phases. Such techniques allow to tailor each surgical access, limiting morbidity to strictly necessary approaches to reach the disease target

A novel approach to skull-base and orbital osteotomies through virtual planning and navigation

ObjectiveComputer-assisted planning of osteotomy lines, coupled with navigation-guided performance of planned osteotomies, is a highly innovative approach to skull-base and orbital surgery. The aim of this pilot study is to provide an assessment of the accuracy of this novel approach in guiding the correct positioning of osteotomy lines in frontal, temporal, and orbital regions, defining the agreement between the spatial position of the planned and performed osteotomies.MethodsFifteen patients with orbital, frontal sinus, and lateral skull-base diseases underwent virtual surgical planning. Osteotomies to access the orbit, frontal sinus, and lateral skull base were planned on computer tomography-based three-dimensional models. The planned osteotomies were reproduced on the operating field using a navigation system. The positions of the performed and planned osteotomies were compared. The results were described as the mean positional difference between planned and performed osteotomies and as Lin's concordance coefficient, and Bland-Altman limits of agreement were also defined.ResultsThe overall mean difference was 0.719mm (95% confidence interval [CI]: 0.472 to 0.965mm). Overall, Lin's concordance coefficient was 0.997 (95% CI: 0.996 to 0.998), and overall Bland-Altman limits of agreement ranged from -1.407 to 2.844mm. The smallest mean difference (0.587mm, 95% CI: 0.244 to 0.931mm) was calculated in the orbit group, whereas the highest mean difference (0.904mm, 95% CI: 0.428 to 1.379mm) was described in the lateral skull-base group.ConclusionThis study's results support the use of this novel planning and navigation protocol for guiding osteotomy in anterior and lateral skull-base surgery, providing a clinical validation of this technique.Level of Evidence4 Laryngoscope, 00:1-9, 2018 Laryngoscope, 129:823-831, 201

Chest multidetector computed tomography (MDCT) in patients with suspected acute pulmonary embolism: diagnostic yield and proportion of other clinically relevant findings

PURPOSE: The authors evaluated the diagnostic yield of chest multidetector computed tomography (MDCT) in acute pulmonary embolism (PE) and the proportion of other clinically relevant findings in a large cohort of consecutive inpatients and patients referred from the emergency department (outpatients). MATERIALS AND METHODS: A total of 327 radiological reports of chest MDCT scans performed for suspected acute PE in 327 patients (158 men, 169 women; mean age 69 years, standard deviation 17.33 years; 233 inpatients, 94 outpatients) were retrospectively evaluated and classified into four categories: 1, positive for PE; 2, negative for PE but positive for other findings requiring specific and immediate intervention; 3, completely negative or positive for findings with a potential for significant morbidity requiring specific action on follow-up; 4, indeterminate. The distribution of findings by categories among the entire population and inpatients and outpatients separately was calculated (chi-square test, \u3b1=0.05). RESULTS: In the entire population, the diagnostic yield (i.e. proportion of cases classified as category 1) was 20.2% (66/327). Proportions of cases classified as categories 2, 3 and 4 were 27.5% (90/327), 44.3% (145/327) and 7.9% (26/327), respectively. No statistically significant difference was found between inpatients and outpatients (p=0.193). CONCLUSIONS: In patients with suspected acute PE, chest MDCT provides evidence of conditions requiring immediate and specific intervention (i.e. categories 1 and 2) in nearly 50% of cases, without differences between inpatients and outpatients

A Novel Protocol for Planning and Navigation in Craniofacial Surgery: A Preclinical Surgical Study

Purpose: In modern craniofacial surgery, the accuracy of osteotomies plays a central role in surgical technique. To reach a higher level of accuracy, many centers use virtual presurgical planning. In the past decade, some surgeons also have applied navigational approaches to craniofacial procedures. In this work, a novel protocol for surgical planning and intraoperative navigation is described and validated in a preclinical setting to determine its accuracy in guiding osteotomies. Materials and Methods: This study was based on planning a set of osteotomies using 3-dimensional models of computed tomographic images of human skulls. The planned osteotomies were reproduced on real skulls using an optical infrared navigation system. Positions of the performed osteotomies and planned osteotomies were compared. Results were described as the mean positional error and as a Lin concordance coefficient. The Bland-Altman interval of agreement also was defined to assess a range that could include 95% of possible errors. Results: The mean error was 0.044 mm (95% confidence interval [CI], -0.128 to +0.216), the Lin concordance interval was 0.999 (95% CI, 0.999-1.000), and the Bland-Altman limit of agreement ranged from -1.500 to +1.589 mm. Conclusions: These results show a submillimetric mean error and a very narrow interval of agreement, providing preclinical validation of this new protocol and suggesting that it could be applied in a clinical setting