Sequential Evaluation of Hematology Markers as a Prognostic Factor in Glioblastoma Patients.

In our study, we investigated the prognostic significance of hematological markers-NLR (Neutrophil-to-Lymphocyte Ratio), PLR (Platelet-to-Lymphocyte Ratio), and RDW-CV (Red Blood Cell Distribution Width-Coefficient of Variation)-in 117 glioblastoma patients. The data collected from January 2016 to December 2018 included demographics, clinical scores, and treatment regimens. Unlike previous research, which often examined these markers solely before surgery, our unique approach analyzed them at multiple stages: preoperative, postoperative, and before adjuvant therapies. We correlated these markers with the overall survival (OS) and progression-free survival (PFS) using statistical tools, including ANOVA, Cox regression, and Kaplan-Meier survival analyses, employing SPSS version 29.0. Our findings revealed notable variations in the NLR, PLR, and RDW-CV across different treatment stages. The NLR and PLR decreased after surgery, with some stabilization post-STUPP phase (NLR: p = 0.007, η2p = 0.06; PLR: p = 0.001, η2p = 0.23), while the RDW-CV increased post-surgery and during subsequent treatments (RDW-CV: p < 0.001, η2p = 0.67). Importantly, we observed significant differences between the preoperative phase and other treatment phases. Additionally, a higher NLR and RDW-CV at the second-line treatment and disease progression were associated with an increased risk of death (NLR at 2nd line: HR = 1.03, p = 0.029; RDW-CV at progression: HR = 1.14, p = 0.004). We proposed specific marker cut-offs that demonstrated significant associations with survival outcomes when applied to Kaplan-Meier survival curves (NLR at 2nd line < 5: p < 0.017; RDW-CV at progression < 15: p = 0.007). An elevated NLR and RDW-CV at later treatment stages correlated with poorer OS and PFS. No significant preoperative differences were detected. These biomarkers may serve as non-invasive tools for glioblastoma management.

Metabolic Syndrome and Survival in Glioblastoma Patients: Retrospective Cohort Study and Review of the Literature.

Background Several studies point to metabolic syndrome as a risk factor for the development and progression of several types of cancer. Its association with glioblastoma has yet to be determined, and only two studies investigate the impact of metabolic syndrome on the survival of glioblastoma patients, indicating a trend toward decreased survival in patients with metabolic syndrome. The aim of this study was to determine whether patients with glioblastoma and metabolic syndrome had a worse clinical outcome. Methods We retrospectively reviewed the clinical records of 180 patients diagnosed with glioblastoma. Metabolic syndrome was defined according to the American Heart Association, as the presence of at least three of the following criteria: diabetes, hypertension, hyperlipidemia, and obesity. We analyzed the overall survival and progression-free survival of patients with and without metabolic syndrome. Results Of 180 patients, 20 (11.1%) met the diagnostic criteria for metabolic syndrome. The overall survival of patients with metabolic syndrome was 19.8 months, and without metabolic syndrome was 17.7 months (p-value=0.085). The progression-free survival of patients with metabolic syndrome was 9.9 months, and without metabolic syndrome was 7.9 months (p-value=0.076). Conclusion Our results showed no prognostic relevance of metabolic syndrome in patients with glioblastoma, although there was a trend towards increased overall survival and progression-free survival in patients with metabolic syndrome.

Deep Brain Stimulation of the Anterior Nucleus of the Thalamus in Drug-Resistant Epilepsy in the MORE Multicenter Patient Registry.

BACKGROUND AND OBJECTIVES: The efficacy of deep brain stimulation of the anterior nucleus of the thalamus (ANT DBS) in patients with drug-resistant epilepsy (DRE) was demonstrated in the double-blind Stimulation of the Anterior Nucleus of the Thalamus for Epilepsy randomized controlled trial. The Medtronic Registry for Epilepsy (MORE) aims to understand the safety and longer-term effectiveness of ANT DBS therapy in routine clinical practice. METHODS: MORE is an observational registry collecting prospective and retrospective clinical data. Participants were at least 18 years old, with focal DRE recruited across 25 centers from 13 countries. They were followed for at least 2 years in terms of seizure frequency (SF), responder rate (RR), health-related quality of life (Quality of Life in Epilepsy Inventory 31), depression, and safety outcomes. RESULTS: Of the 191 patients recruited, 170 (mean [SD] age of 35.6 [10.7] years, 43% female) were implanted with DBS therapy and met all eligibility criteria. At baseline, 38% of patients reported cognitive impairment. The median monthly SF decreased by 33.1% from 15.8 at baseline to 8.8 at 2 years (p < 0.0001) with 32.3% RR. In the subgroup of 47 patients who completed 5 years of follow-up, the median monthly SF decreased by 55.1% from 16 at baseline to 7.9 at 5 years (p < 0.0001) with 53.2% RR. High-volume centers (>10 implantations) had 42.8% reduction in median monthly SF by 2 years in comparison with 25.8% in low-volume center. In patients with cognitive impairment, the reduction in median monthly SF was 26.0% by 2 years compared with 36.1% in patients without cognitive impairment. The most frequently reported adverse events were changes (e.g., increased frequency/severity) in seizure (16%), memory impairment (patient-reported complaint, 15%), depressive mood (patient-reported complaint, 13%), and epilepsy (12%). One definite sudden unexpected death in epilepsy case was reported. DISCUSSION: The MORE registry supports the effectiveness and safety of ANT DBS therapy in a real-world setting in the 2 years following implantation. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that ANT DBS reduces the frequency of seizures in patients with drug-resistant focal epilepsy. TRIAL REGISTRATION INFORMATION: MORE ClinicalTrials.gov Identifier: NCT01521754, first posted on January 31, 2012.

Endoglin (CD105) and proliferation index in recurrent glioblastoma treated with anti-angiogenic therapy.

Introduction: CD105 is an angiogenic biomarker that is useful to determine the microvessel density (MVD) within a tumor, namely, in highly vascularized tumors like glioblastoma (GBM). However, its expression has shown inconsistent associations with the prognosis of GBM patients. The aim of this study was to evaluate the value of MVD-CD105 (microvessel density assessed with anti-CD105 antibody) and Ki-67 (proliferation index marker) as prognostic and therapy response biomarkers, specifically in primary tumors and in recurrent tumoral specimens of a cohort of GBM patients treated with bevacizumab upon recurrence. Materials and methods: We conducted a retrospective study of 102 consecutive GBM patients treated with bevacizumab upon recurrence at CHUSJ between 2010 and 2017. Demographic, clinical, and survival data of all patients were collected and analyzed. The tissue expression of MVD-CD105 and Ki-67 in primary and recurrent specimens was correlated with progression-free survival after temozolomide (PFS-1), progression-free survival after bevacizumab (PFS-2), and overall survival (OS). Results: The immunohistochemical expression score for MVD-CD105 was similar in primary and recurrent tumoral specimens (mean scores of 15 and 16, respectively). Likewise, the mean Ki-67 expression was similar in primary (mean of 31% of tumor cells) and recurrent tumoral specimens (mean of 29% of tumor cells). MVD-CD105 expression in primary tumors had no impact on PFS-1, PFS-2, or OS. At recurrence, patients whose tumors showed increased MVD-CD105 had worse median PFS-2 (2 vs. 8 months, p = 0.045) and OS (17 vs. 26 months, p = 0.007) compared to those whose tumors showed lower MVD-CD105. CD105 tumoral pattern and localization had no impact on prognosis. Ki-67 expression was not associated with differences in survival outcomes. Conclusion: In this study, higher MVD-CD105 expression in recurrent GBM patients seems to be associated with a worse PFS-2 and OS while portending no prognostic significance in the primary tumors. This highlights the importance of keeping track of the molecular evolution of the tumor over the course of the disease.

Perineal and foot muscle synkinesis following trauma to the sacrum in an adult male - A case of motor root ephaptic transmission?

Ephaptic transmission has been proven as an alternative to chemical synaptic neural transmission and occurs in pathological situations, such as epilepsy and demyelination. Hereby, we report the case of an adult male that in 2012 was involved in a low-speed motorcycle accident with sacrum impact that from day three onwards reported unwanted penile movement when performing hallux and toe plantar flexion of the right foot. Urologic studies and perineal MRI were unremarkable but sacral MRI showed a significantly stenotic canal at the S1-S2 level while EMG displayed chronic moderate right S2 radiculopathy. Nine years later the patient underwent surgical decompression of the sacral canal with complete resolution of the synkinesis. We hypothesize ephaptic transmission between adjacent motor nerve fibres at the S2 sacral nerve root to be the likely mechanism explaining this phenomenon.

Early atypical malignant transformation of diffuse low-grade astrocytoma: The importance of genotyping.

Diffuse astrocytoma (WHO grade II) has classically been considered a slow growing tumour, typically affecting young adults, with tendency for late malignant conversion. We describe a case of early atypical malignant transformation of diffuse astrocytoma seventeen months after complete surgical removal, as an intraventricular high-grade glioma (HGG). Retrospective laboratory findings for the presence of IDH 1/2 (isocitrate dehydrogenase) mutations were negative. There is growing evidence that IDH-wildtype (wt) astrocytomas behave more aggressively, therefore identifying IDH-mutation status should be mandatory in order to determine disease prognosis and guide treatment course.

A rare case of meningioma recurrence on a titanium mesh cranioplasty.

Cranioplasty with titanium mesh provides a stable and cosmetically sound option for the correction of extensive skull bone defects following trauma or tumour surgery with osseous involvement. Meningiomas are for the most part benign lesions that are amenable to surgical cure, however lesions with extradural extension pose additional challenges not only due to increased technical difficulty in achieving gross total resection but also because of distinct biological behaviour. We describe the case of a 43-years-old woman that had been submitted to gross total resection of a WHO grade I falcine and superior sagittal sinus secretory meningioma with extradural and bone extension and cranioplasty with a titanium mesh who had a recurrence 4 years later as two tumour masses on top of the titanium mesh with no adjacent soft tissue invasion, and without dural involvement. To our knowledge, this is the first reported case of meningioma growth on top of titanium cranioplasty material. Seeded or incompletely removed tumoral cells might have exploited the biocompatibility of titanium to promote tumour regrowth.

Hyperbaric oxygen treatment: Results in seven patients with severe bacterial postoperative central nervous system infections and refractory mucormycosis.

INTRODUCTION: Resistant bacterial infections following brain and spine surgery and spontaneous mucormycosis with central nervous system (CNS) involvement represent a serious treatment challenge and more efficient therapeutic approaches ought to be considered. Hyperbaric oxygen treatment (HBOT) has shown promise as a complementary therapy. This case series evaluated whether HBOT contributed to infection resolution in seven patients with refractory CNS infectious conditions. METHODS: Clinical results for seven patients referred for HBOT between 2010 to 2018 to treat refractory postoperative brain and spine infections or spontaneously developing mucormycosis were retrospectively analysed. The patients' clinical files and follow-up consultations were reviewed to assess evolution and outcome. RESULTS: Seven patients were referred with a median age of 56 years. The median follow-up was 20 months. Four patients had postoperative infections and three had rhino-orbital-cerebral mucormycosis (ROCM). HBOT was used as an adjunctive treatment to antimicrobial therapy in all patients. Prior to HBOT, all patients had undergone an average of four operations due to infection refractoriness and had completed an average of five months of antimicrobial therapy. After HBOT, infection resolution was obtained in six patients without additional operations, while one patient with ROCM stopped HBOT after the third session due to intolerance. Three patients stopped antimicrobial therapy while four were maintained on prophylactic treatment. CONCLUSIONS: Infection resolution was reached in the six patients that completed HBOT as prescribed. HBOT may serve as an effective complementary treatment in CNS refractory postoperative and spontaneous infections.

The role of c-Met and VEGFR2 in glioblastoma resistance to bevacizumab.

Dismal prognosis of glioblastoma (GBM) prompts for the identification of response predictors and therapeutic resistance mechanisms of current therapies. The authors investigated the impact of c-Met, HGF, VEGFR2 expression and microvessel density (MVD) in GBM patients submitted to second-line chemotherapy with bevacizumab. Immunohistochemical expression of c-Met, HGF, VEGFR2, and MVD was assessed in tumor specimens of GBM patients treated with bevacizumab, after progression under temozolomide. Survival analysis was evaluated according to the expression of the aforementioned biomarkers. c-Met overexpression was associated with a time-to-progression (TTP) after bevacizumab of 3 months (95% CI, 1.5-4.5) compared with a TTP of 7 months (95% CI, 4.6-9.4) in patients with low or no expression of c-Met (p = 0.05). VEGFR2 expression was associated with a TTP after bevacizumab of 3 months (95% CI, 1.8-4.2) compared with a TTP of 7 months (95% CI, 5.7-8.3) in patients with no tumoral expression of VEGFR2 (p = 0.009). Concomitant c-Met/VEGFR2 overexpression was associated with worse overall survival (13 months) compared with concomitant c-Met/VEGFR2 negative expression (19 months; p = 0.025). Our data support the hypothesis that c-Met and VEGFR2 overexpression have a role in the development of glioblastoma early resistance and might predict poorer responses to anti-angiogenic therapies.

Body mass index as an independent prognostic factor in glioblastoma.

PURPOSE: Glioblastoma prognosis remains dismal despite gross total removal (GTR) followed by chemoradiotherapy. Other known prognostic factors include functional status, age and IDH mutation status. However, to improve patient outcome, a search for other features with impact on survival is needed. We aimed to analyse the impact of body mass index (BMI) on overall survival (OS) and progression-free survival (PFS) of surgically resected primary glioblastoma and evaluate if BMI constitutes an independent prognostic factor. METHODS: We analysed all adult glioblastoma patients who underwent surgery and chemoradiotherapy between 2011 and 2017 at our institution. Overall survival was the study-primary endpoint, and progression-free survival-the secondary endpoint. We assayed age, gender, histology, extent of resection, IDH, functional and smoking status, cardiovascular risk factors, BMI, OS and PFS. Univariate analysis was conducted followed by multivariate analysis to establish independent prognostic factors. In accordance with the World Health Organization (WHO) BMI stratification, survival curves were obtained for normal-weight (18.5-24.9 kg/m2), overweight (25-29.9 kg/m2) and obese (≥ 30 kg/m2) patient subgroups in addition to the non-obese (18.5-29.9 kg/m2) population. RESULTS: 193 patients were evaluated, with a median follow-up time of 17.3 months. Median OS was 21.3 months in obese patients vs 16.2 months in the non-obese (p = 0.017) and 16 months in the normal weight (p = 0.007). Higher median OS was also observed in patients under 60 and those in which GTR was obtained. Median PFS in obese individuals was 9 months in comparison to 6 months in the normal-weight subgroup (p = 0.04) and 7 months in the non-obese (p = 0.050). Multivariate analysis identified age < 60 (p = 0.044), GTR (p = 0.004) and BMI ≥ 30 (p = 0.009) as independent prognostic factors for increased overall survival. CONCLUSION: Higher BMI was associated with longer OS and PFS. Prospective studies are needed to validate these findings.

Myxoid mesenchymal intraventricular brain tumour with EWSR1-CREB1 gene fusion in an adult woman.

Myxoid mesenchymal tumours harbouring fusions between EWSR1 and the CREB family transcription factors have recently been described. Whether they represent a novel entity or a myxoid variant of angiomatoid fibrous histiocytoma (AFH) remains a matter of debate. We describe the case of a 58-year-old woman with a previous history of breast cancer that developed progressive neurological decline due to a large mass located in the left lateral ventricle of the brain. Histology revealed a mesenchymal tumour with multinodular growth, variable cellularity, prominent myxoid stroma and numerous amianthoid fibres. No evidence of pseudo-capsule or lymphoid cuffing was identified. RNA sequencing disclosed EWSR1-CREB1 gene fusion. Only 20 cases of intracranial mesenchymal tumours harbouring these translocations have been described, mostly in adolescents and young adults and with dural attachment. Occurrence in this age group and with intraventricular location has been even more rarely reported. A better understanding of tumour behaviour is needed to establish treatment guidelines and improve patient outcome.

Early atypical malignant transformation of diffuse low-grade astrocytoma: The importance of genotyping.

Diffuse astrocytoma (WHO grade II) has classically been considered a slow growing tumour, typically affecting young adults, with tendency for late malignant conversion. We describe a case of early atypical malignant transformation of diffuse astrocytoma seventeen months after complete surgical removal, as an intraventricular high-grade glioma (HGG). Retrospective laboratory findings for the presence of IDH 1/2 (isocitrate dehydrogenase) mutations were negative. There is growing evidence that IDH-wildtype (wt) astrocytomas behave more aggressively, therefore identifying IDH-mutation status should be mandatory in order to determine disease prognosis and guide treatment course.

Glioblastoma: Is There Any Blood Biomarker with True Clinical Relevance?

Glioblastoma (GBM) is the most frequent malignant primary brain tumor in adults, characterized by a highly aggressive, inflammatory and angiogenic phenotype. It is a remarkably heterogeneous tumor at several levels, including histopathologically, radiographically and genetically. The 2016 update of the WHO Classification of Tumours of the Central Nervous System highlighted molecular parameters as paramount features for the diagnosis, namely IDH1/2 mutations that distinguish primary and secondary GBM. An ideal biomarker is a molecule that can be detected/quantified through simple non- or minimally invasive methods with the potential to assess cancer risk; promote early diagnosis; increase grading accuracy; and monitor disease evolution and treatment response, as well as fundamentally being restricted to one aspect. Blood-based biomarkers are particularly attractive due to their easy access and have been widely used for various cancer types. A number of serum biomarkers with multiple utilities for glioma have been reported that could classify glioma grades more precisely and provide prognostic value among these patients. At present, screening for gliomas has no clinical relevance. This is because of the low incidence, the lack of sensitive biomarkers in plasma, and the observation that gliomas may develop apparently de novo within few weeks or months. To the best of our knowledge, there is no routine use of a serum biomarker for clinical follow-up. The purpose of this paper is to review the serum biomarkers described in the literature related to glioblastoma and their possible relationship with clinical features.

Hypertension and proteinuria as clinical biomarkers of response to bevacizumab in glioblastoma patients.

INTRODUCTION: Arterial hypertension and proteinuria are common side effects of antiangiogenic treatment and might represent a biomarker of response in patients with glioblastoma. The aim of this study was to assess the impact of these side effects in predicting therapeutic response to second line chemotherapy with bevacizumab. METHODS: We evaluated clinical and survival data of glioblastoma patients who underwent treatment with bevacizumab after progression under temozolomide, at CHUSJ between 2010 and 2017. We analysed treatment-related arterial hypertension, proteinuria grade, thrombotic and haemorrhagic events during treatment. Overall survival (OS) and progression free survival (PFS) under bevacizumab were calculated according to the Kaplan-Meier method. Multivariate analysis was performed using Cox proportional hazards method. RESULTS: We evaluated 140 patients. Arterial hypertension and proteinuria occurred in 23 (16.3%) and 17 (12.1%) patients, respectively. PFS during treatment with bevacizumab was 12 months (95% CI 7.9-16.1) in the hypertensive group and 4 months (95% CI 3.2-4.8) in the normotensive group (p = 0.005). Patients with proteinuria had a PFS of 10 months (95% CI 4.9-15.0) versus 4 months (95% CI 3.4-4.8) in patients without proteinuria (p = 0.002). Multivariate analysis revealed hypertension and proteinuria as independent prognostic factors of PFS and OS. CONCLUSION: Our data suggest that hypertension and proteinuria can be effective predictors of response to antiangiogenic therapy in recurrent glioblastoma and are associated with longer disease control.

Replication of GWAS identifies RTEL1, CDKN2A/B, and PHLDB1 SNPs as risk factors in Portuguese gliomas patients.

Diffuse gliomas are the most common malignant primary brain tumors and remain incurable. A better knowledge of the tumor etiology is required. Specific single nucleotides polymorphisms (SNPs) rs4977756 (CDKN2A/B), rs6010620 (RTEL1), rs498872 (PHLDB1), rs2736100 (TERT), and rs4295627 (CCDC26) have been associated with glioma susceptibility and are potential risk biomarkers. This study aimed to analyze five SNPs associated with glioma susceptibility, in the Portuguese population. SNPs were genotyped using the Sequenom MassARRAY platform in 127 gliomas and 180 controls. Unconditional logistic regression models were used to calculate odds ratio (OR) and 95% confidence intervals. The false-positive report probability was also assessed. The associations between polymorphisms and survival were evaluated using the log-rank test. It was found that the AG and GG genotypes of the rs4977756 (CDKN2A/B) were associated with an increased risk of gliomas (OR 1.85 and OR 2.38) and glioblastomas (OR 2.77 and OR 3.94). The GA genotype of the rs6010620 (RTEL1) was associated with a decreased risk of glioblastomas (OR 0.45). We also observed that the GA genotype of the rs498872 (PHLDB1) was associated with an increased risk of gliomas (OR 2.92) and glioblastomas (OR 2.39). No significant risk associations were found for the rs2736100 (TERT) and rs4295627 (CCDC26). In addition, the genotype AA of the rs498872 (PHLDB1) was associated with poor overall survival of gliomas patients (AA vs. GA, p = 0.037). The rs6010620 (RTEL1), rs4977756 (CDKN2A/B), and rs498872 (PHLDB1) are associated with glioma risk in the Portuguese population and these data may contribute to understanding gliomas etiology.

Inovação institucional na coordenação federativa para o enfrentamento e saída do surto de Covid-19 / Nota Técnica n. 41 (Diest) : Inovação institucional na coordenação federativa para o enfrentamento e saída do surto de Covid-19

A emergência da pandemia causada pela Covid-19 exigiu uma resposta rápida e abrangente dos governos e das sociedades civis em todo o mundo. Velocidade, multiplicidade e diversidade de ações e de atores envolvidos colocaram em relevo problemas de coordenação. Esta nota técnica tem por objetivo identificar as medidas institucionais adotadas pelos governos estaduais brasileiros que visam à criação de instituições para a promoção da coordenação de ações entre os órgãos estaduais, municipais e os representantes da sociedade civil diante da emergência da pandemia no âmbito de cada estado. Embora sejam registradas aqui medidas adotadas até 9 de junho, o período de observação concentra-se mais detidamente nos meses de março e abril de 2020, quando os governos estaduais estabeleceram os marcos institucionais sobre os quais cada um iria atuar na gestão da crise que se vislumbrava à frente. Percorreremos a sequência inicial de medidas formais dos governos estaduais e, ao fazê-lo, buscaremos sugerir aprimoramentos ainda úteis ao momento de enfrentamento da pandemia, além de, sobretudo, propor melhorias aos arranjos já instituídos – no sentido de aprimorar a coordenação entre os entes da Federação brasileira – para a fase de retomada das atividades após o pico de contaminação da população pelo vírus da Covid-19.

Serum lactate levels are associated with glioma malignancy grade.

OBJECTIVE: Recent studies have suggested that high grade gliomas are associated with elevated serum lactate concentrations. The aim of the present study is to assess these findings in a sample of patients. PATIENTS AND METHODS: We reviewed the anesthetic charts of patients with low-grade and high-grade glioma who underwent resection surgery and collected serum lactate concentration before tumor resection, as well as other demographic and tumor-related data (age, gender, WHO grade, and size of the tumor). A statistical comparison between patients with normal (<2 mmol/L) and elevated (≥ 2 mmol/L) serum lactate was performed. RESULTS: We included a total of 152 patients (mean age 49.07 years). 62.5% of patients (n = 95) had a high-grade glioma and 37.5% (n = 67) a low-grade glioma. The multivariate regression showed that high grade gliomas had significantly higher lactate concentration (p < 0.01). The OR for elevated pre-resection serum lactate increased from 4.94 to 14.33 after adjusting for age and pre-surgical corticosteroid use, and the AUC for the final regression model was 0.98. CONCLUSION: This study reinforces the role of serum lactate as a potential biomarker of brain tumors malignancy, and its results encourage further research on this subject, in order to improve the understanding of this phenomenon and to assess its potential as prognostic and therapeutic monitoring tool.

Automated and objective measures of gait dynamics in camptocormia Parkinson's Disease subthalamic deep brain stimulation.

OBJECTIVE: Axial motor features are common in Parkinson's disease (PD). These include gait impairment and postural abnormalities, such as camptocormia. The response of these symptoms to deep brain stimulation (DBS) is variable and difficult to assess objectively. For the first time, this study analyzes the treatment outcomes of two PD patients with camptocormia that underwent bilateral subthalamic nucleus (STN)-DBS evaluated with disruptive technologies. PATIENTS AND METHODS: Two patients with PD and camptocormia who underwent STN-DBS were included. Gait parameters were quantitatively assessed before and after surgery by using the NeuroKinect system and the camptocormia angle was measured using the camptoapp. RESULTS: After surgery, patient 1 improved 29 points in the UPDRS-III. His camptocormia angle was 68° before and 38° after surgery. Arm and knee angular amplitudes (117.32 ±â€¯7.47 vs 134.77 ±â€¯2.70°; 144.51 ±â€¯7.47 vs 169.08 ±â€¯3.27°) and arm swing (3.59 ±â€¯2.66 vs 5.40 ±â€¯1.76 cm) improved when compared with his preoperative measurements. Patient 2 improved 22 points in the UPDRS-III after surgery. Her camptocormia mostly resolved (47° before to 9° after surgery). Gait analysis revealed improvement of stride length (0.29 ±â€¯0.03 vs 0.35 ±â€¯0.03 m), stride width (18.25 ±â€¯1.16 vs 17.9 ±â€¯0.84 cm), step velocity (0.91 ±â€¯0.57 vs 1.33 ±â€¯0.48 m/s), arm swing (4.51 ±â€¯1.01 vs 7.38 ±â€¯2.71 cm) and arm and hip angular amplitudes (131.57 ±â€¯2.45° vs 137.75 ±â€¯3.18; 100.51 ±â€¯1.56 vs 102.18 ±â€¯1.77°) compared with her preoperative results. CONCLUSION: The gait parameters and camptocormia of both patients objectively improved after surgery, as assessed by the two quantitative measurement systems. STN-DBS might have a beneficial effect on controlling axial posturing and gait, being a potential surgical treatment for camptocormia in patients with PD. However, further studies are needed to derive adequate selection criteria for this patient population.

The impact of deep brain stimulation on health related quality of life and disease-specific disability in Meige Syndrome (MS).

Meige Syndrome (MS) is a disabling movement disorder which impairs daily routines such as eating and speaking and, when not responsive to best medical treatment, deep brain stimulation (DBS) of the globus pallidus interna (GPi) has been considered. Previous evidence has shown a significant improvement in motor dysfunction with DBS, however its benefit on disease-specific disability and quality of life has not been thoroughly studied. We describe two patients with severe MS submitted to GPi-DBS. Patient improvement was assessed preoperatively and 24 months after the surgery by applying the movement subscore of the Burke-Fahn-Marsden Dystonia Rating Scale (BFMRS) and Jankovic Rating Scale (JRS) for motor function and the BFMRS disability subscore and Blepharospasm Disability Scale (BDS) for disability. At 24-month follow-up, dystonia improved 68% in Patient 1 and 96% in Patient 2, while disability improved 77%-92% respectively. No major adverse effects were observed. Improvement in motor function is in agreement with previous findings, but we emphasise the important improvement in disability and consequently in quality of life. Therefore, we suggest that DBS should be a therapeutic tool in refractory cases of MS.