Long-term quality of life and functional outcomes in adults surgically treated for intramedullary spinal cord tumor.

Introduction: Intramedullary spinal cord tumors (IMSCTs) are rare but clinically significant entities that may cause severe neurological decline with progressive pain and motor or sensory deterioration. Beyond the beneficial effects of surgical treatment and the long-term progression-free survival, neurological deficits may still persist after surgery, and information about the long-term patients' health-related quality of life (QoL) is still lacking. In this study, we investigate the patients' health perception 15 years after the surgery in an overall patients' wellbeing framework. Methods: Patients surgically treated for IMSCT over a period from 1996 to 2011 were selected. After a mean of 15 years from the surgery, patient's self-administered questionnaire on disability, pain, sleep quality, and QoL was collected and neurological postoperative evaluation at the chronic stage was reexamined. Results: Neurological deficits are reported in half of the patients in the postoperative chronic phase. After 15 years of surgery, half of the patients still report mild or severe disability grades associated with significantly higher pain and poor sleep and QoL. In accordance, the neurological condition measured at the chronic stage is significantly related not only to disease-specific symptoms (i.e., pain) but even to sleep quality complaints and poor QoL, measured at 15 years follow-up. Conclusions: Health-related QoL is an important secondary outcome in patients. Although the progression-free survival, worse postoperative neurological conditions could predict long-term sequelae reflecting patients' poor health perception. It suggests the importance of preserving patients' functional status and globally evaluating patients' wellbeing to handle disease-specific symptoms but even more general aspects of QoL.

Extradural Motor Cortex Stimulation in Parkinson's Disease: Long-Term Clinical Outcome.

Previous investigations have reported on the motor benefits and safety of chronic extradural motor cortex stimulation (EMCS) for patients with Parkinson's disease (PD), but studies addressing the long-term clinical outcome are still lacking. In this study, nine consecutive PD patients who underwent EMCS were prospectively recruited, with a mean follow-up time of 5.1 ± 2.5 years. As compared to the preoperatory baseline, the Unified Parkinson's Disease Rating Scale (UPDRS)-III in the off-medication condition significantly decreased by 13.8% at 12 months, 16.1% at 18 months, 18.4% at 24 months, 21% at 36 months, 15.6% at 60 months, and 8.6% at 72 months. The UPDRS-IV decreased by 30.8% at 12 months, 22.1% at 24 months, 25% at 60 months, and 36.5% at 72 months. Dopaminergic therapy showed a progressive reduction, significant at 60 months (11.8%). Quality of life improved by 18.0% at 12 months, and 22.4% at 60 months. No surgical complication, cognitive or behavioral change occurred. The only adverse event reported was an infection of the implantable pulse generator pocket. Even in the long-term follow-up, EMCS was shown to be a safe and effective treatment option in PD patients, resulting in improvements in motor symptoms and quality of life, and reductions in motor complications and dopaminergic therapy.

Frameless stereotaxy in subthalamic deep brain stimulation: 3-year clinical outcome.

BACKGROUND: In most centers, the surgery of deep brain stimulation (DBS) is performed using a stereotactic frame. Compared with frame-based technique, frameless stereotaxy reduces the duration of surgical procedure and patient's discomfort, with lead placing accuracy equivalent after the learning curve. Although several studies have investigated the targeting accuracy of this technique, only a few studies reported clinical outcomes, with data of short-term follow-up. OBJECTIVE: To assess clinical efficacy and safety of frameless bilateral subthalamic nucleus (STN) DBS in Parkinson's disease (PD) patients at 1- and 3-year follow-up. METHODS: Consecutive PD patients who underwent bilateral STN-DBS with a manual adjustable frameless system were included in the study. The data were collected retrospectively. RESULTS: Eighteen PD patients underwent bilateral STN-DBS implant and were included in the study. All patients completed 1-year observation and ten of them completed 3-year observation. At 1-year follow-up, motor efficacy of STN stimulation in off-med condition was of 30.1% (P = 0.003) and at 3-year follow-up was of 36.3%, compared with off-stim condition at 3-year follow-up (P = 0.005). Dopaminergic drugs were significantly reduced by 31.2% 1 year after the intervention (P = 0.003) and 31.7% 3 years after the intervention (P = 0.04). No serious adverse events occurred during surgery. CONCLUSIONS: Frameless stereotaxy is an effective and safe technique for DBS surgery at 1- and 3-year follow-up, with great advantages for patients' discomfort during surgery.

Extradural Motor Cortex Stimulation might improve episodic and working memory in patients with Parkinson's disease.

Electric Extradural Motor Cortex Stimulation (EMCS) is a neurosurgical procedure suggested for treatment of patients with advanced Parkinson's disease (PD). We report two PD patients treated by EMCS, who experienced worsening of motor symptoms and cognition 5 years after surgery, when EMCS batteries became discharged. One month after EMCS restoration, they experienced a subjective improvement of motor symptoms and cognition. Neuropsychological assessments were carried out before replacement of batteries (off-EMCS condition) and 6 months afterward (on-EMCS condition). As compared to off-EMCS condition, in on-EMCS condition both patients showed an improvement on tasks of verbal episodic memory and backward spatial short-term/working memory task, and a decline on tasks of selective visual attention and forward spatial short-term memory. These findings suggest that in PD patients EMCS may induce slight beneficial effects on motor symptoms and cognitive processes involved in verbal episodic memory and in active manipulation of information stored in working memory.

Pre-seizure architecture of the local connections of the epileptic focus examined via graph-theory.

OBJECTIVE: Epilepsy is characterized by unpredictable and sudden paroxysmal neuronal firing occurrences and sometimes evolving in clinically evident seizure. To predict seizure event, small-world characteristic in nine minutes before seizure, divided in three 3-min periods (T0, T1, T2) were investigated. METHODS: Intracerebral recordings were obtained from 10 patients with drug resistant focal epilepsy examined by means of stereotactically implanted electrodes; analysis was focused in a period of low spiking (Baseline) and during two seizures. Networks' architecture is undirected and weighted. Electrodes' contacts close to epileptic focus are the vertices, edges are weighted by mscohere (=magnitude squared coherence). RESULTS: Differences were observed between Baseline and T1 and between Baseline and T2 in theta band; and between Baseline and T1, Baseline and T2, and near-significant difference between T0 and T2 in Alpha 2 band. Moreover, an intra-band index was computed for small worldness as difference between Theta and Alpha 2. It was found a growing index trend from Baseline to T2. CONCLUSIONS: Cortical network features a specific pre-seizure architecture which could predict the incoming epileptic seizure. SIGNIFICANCE: Through this study future researches could investigate brain connectivity modifications approximating a clinical seizure also in order to address a preventive therapy.

Motor cortex stimulation for movement disorders.

INTRODUCTION: Motor cortex stimulation (MCS) was introduced by Tsubokawa in 1991 1 for the treatment of thalamic pain, after coming to the conclusion that the hyperactivity of thalamic neurons after spino-thalamic tractotomy was inhibited by stimulation of the motor cortex. MCS has been reported not only to be effective on pain, but also to improve movement disorders such as Parkinson's disease, tremor, dystonia, poststroke movement disorders and hemiparesis. Most of these publications are case reports or small series, and the real impact of MCS on movement disorders remains to be determined. EVIDENCE ACQUISITION: In order to clarify this point, we conducted a PubMed search from 1991 to 2016 using established MeSH words. A total of 40 papers were selected and examined. Furthermore, personal experience with MCS for Parkinson's disease and akinesia, is reported. EVIDENCE SYNTHESIS: Only four studies were randomized controlled clinical trials: three out of four failed to demonstrate the efficacy of MCS at short term. CONCLUSIONS: At long term, MCS seems to show a clinical positive effect in the studies prolonged in an open observational trial.

The role of laminoplasty in preventing spinal deformity in adult patients submitted to resection of an intradural spinal tumor. Case series and literature review.

OBJECTIVE: Laminectomy has normally been used as a standard approach for intradural spinal tumors but this procedure is associated with spinal instability and deformity. Laminoplasty was developed to overcome these limitations. Controversies still exist regarding its actual role in preventing spinal deformity in adults. The aim of our study was to determine the impact of laminoplasty on the prevention of spinal deformity's onset or worsening in adult patients submitted to intradural spinal tumors resection. METHODS: We retrospectively reviewed the data of 43 consecutive adult patients, who underwent either laminectomy or laminoplasty for spinal intradural tumor resection, between January 2006 and May 2011. We evaluated the role of sex, spinal segment (cervical, thoracic, lumbar), tumor location (intra- or extra-medullary), procedure (laminoplasty or laminectomy), number of treated levels (≤2 vs >2), presence of pre-operative deformity and pre-operative Modified McCormick Scale (≤2 vs >2) in the development or worsening of spinal deformity, using Fisher's exact test and multivariate logistic regression analysis. RESULTS: Nine patients developed deformity or experienced a worsening of pre-operative deformity at latest follow-up. Among the considered potential prognostic factors, laminectomy (p=0.03) and evidence of pre-operative spinal deformity (p=0.009) were significantly associated with new-onset or worsening of spinal deformity. At logistic regression analysis, only the performed surgical procedure emerged as independent prognostic factor (p=0.044). No CSF leak was recorded in the laminoplasty cohort. CONCLUSIONS: No new-onset spinal deformities, no CSF leaks and a lower rate of spinal deformity progression were observed after laminoplasty for intradural intra- or extra-medullary tumor resection.

The role of percutaneous balloon compression in the treatment of trigeminal neuralgia recurring after other surgical procedures.

Trigeminal neuralgia (TN) recurring after surgery can be difficult to treat. Treatment algorithms have not been standardized or universally accepted. Here we investigated the effectiveness of percutaneous balloon compression (PBC) in the treatment of patients with TN recurrence after other surgical techniques and analyzed the role of some clinical and operative factors in determining the prognosis. The records of 22 patients (13 M and 9 F) suffering recurrent TN after one (2 gamma knife surgery, 5 percutaneous radiofrequency rhizotomy, 6 percutaneous retrogasserian glycerol rhizotomy, 3 microvascular decompression) or more (6 patients) procedures and submitted to PBC at our institution from January 2003 to February 2012 were reviewed. Seven patients had TN related to multiple sclerosis (MS). Mean follow-up was 51.81 ± 26.63 months. 81.81 % of patients reported an acute pain relief. No major complication was observed after PBC. Eight patients (36.36 %) experienced pain recurrence and underwent one (five patients) or more (three patients) PBC. At the last follow-up, we obtained an excellent outcome (BNI I-II) in 16 patients out of 22 (72.72 %) and a good outcome (BNI III) in the remaining six. No patients had an uncontrolled pain. The lack of history of MS (p = 0.0174), the pear-like shape of the balloon at the operation (p = 0.0234) and a compression time <5 min (p < 0.05) were associated to higher pain-free survival. Considering these results PBC could be considered a useful technique for patients whose pain recurs after other procedures.

Primary multicentric anaplastic pleomorphic xanthoastrocytoma with atypical features.

Pleomorphic xanthoastrocytoma (PXA) is a rare tumor with good prognosis after surgery. Few cases of anaplastic PXA (either de novo or secondary to transformation of a recurrent low grade PXA) have been reported. Moreover, primary anaplastic PXA with dissemination at diagnosis has been described only in two patients, to our knowledge. We report the first case of primary multicentric anaplastic PXA and discuss its atypical features and the pertinent literature.

What is the best treatment of drug-resistant trigeminal neuralgia in patients affected by multiple sclerosis? A literature analysis of surgical procedures.

OBJECTIVE: Drug-resistant trigeminal neuralgia (TN) can complicate the clinical course of patients affected by multiple sclerosis (MS). Various surgical procedures have been reported for the treatment of this condition, but there is no agreement on the best management of these patients. To our knowledge, there is no critical literature analysis focusing on this particular topic. The aim of this study was to evaluate the clinical outcome of different surgical procedures utilized for drug-resistant TN in MS patients. METHODS: We reviewed the literature about the studies reporting on surgical treatment of drug-resistant TN in MS patients. Case reports and case series less than 4 patients were excluded from the analysis. Nineteen studies were selected for the statistical analysis. To reduce the variability of the data, the selected studies were evaluated for the following outcome parameters: acute pain relief rate (APR), rate of recurrence (RR), pain free at follow-up rate (PF at FU) and complication rate (CR). For the statistical analysis, chi-square statistic, using the Fisher's exact test was utilized. RESULTS: There was no procedure statistically superior in terms of APR rate in MS patients following the surgical treatment of TN. The highest RR was observed for percutaneous balloon compression (PBC) (60.2±14.4%). This result was statistically significant when compared to gamma knife surgery (GKS) (p=0.0129) and microvascular decompression (MVD) (p=0.0281). MVD together with percutaneous radiofrequency rhizothomy (PRR) was associated with a statistically better PF at FU rate (56.5±16.8% and 73.5±14.2%, respectively). However PBC and MVD showed statistical significant minor CR compared to other techniques (no complications and 18.7±17.4%, respectively). CONCLUSION: Our study shows no differences in the short term results among different procedures for TN in MS patients. Each technique demonstrate advantages and limits in terms of long term pain, recurrence rate and complication rate. Each patient should be accurately informed on pros and cons of each procedure in order to be involved in the most appropriate choice.

Unilateral extradural motor cortex stimulation is safe and improves Parkinson disease at 1 year.

BACKGROUND: The primary motor cortex, which is part of the corticobasal ganglia loops, may be an alternative option for the surgical treatment of Parkinson disease. OBJECTIVE: To report on the 1-year safety and efficacy of unilateral extradural motor cortex stimulation in Parkinson disease. METHODS: A quadripolar electrode strip was extradurally implanted over the motor cortex. Stimulation was continuously delivered through the electrode paddle contralateral to the most affected clinical side. Subjects were prospectively evaluated by the Unified Parkinson's Disease Rating Scale (UPDRS) and the Parkinson's Disease Quality of Life Questionnaire. In addition, an extensive cognitive and behavioral assessment and electroencephalogram recording were performed. RESULTS: Nine patients were included in this study. No surgical complications or adverse events occurred. Moreover, no cognitive or behavioral changes were observed. Under the off-medication condition, the UPDRS III at baseline was decreased by 14.1%, 23.3%, 19.9%, and 13.2%, at 1, 3, 6, and 12 months, respectively. The motor effects were bilateral, appeared after 3 to 4 weeks of stimulation, and outlasted the stimulation itself for 3 to 4 weeks in 1 case of stimulator accidental switching off. The UPDRS IV was decreased by 40.8%, 42.1%, and 35.5% at 1, 3, and 12 months, respectively. The scores on the Parkinson's Disease Quality of Life Questionnaire were increased at months 3, 6, and 12. CONCLUSION: Extradural motor cortex stimulation is a safe procedure. After 12 months, the patients demonstrated a moderate improvement of motor symptoms (particularly axial symptoms) and quality of life.

Chronic motor cortex stimulation in patients with advanced Parkinson's disease and effects on striatal dopaminergic transmission as assessed by 123I-FP-CIT SPECT: a preliminary report.

OBJECTIVE: The objective of this study was to assess striatal dopamine transporter availability in patients with advanced Parkinson's disease (PD) before and after 13 months of unilateral extradural motor cortex stimulation (EMCS) with [123I]N-ω-fluoropropyl-2-ß-carbo-methoxy-3-ß-(4-iodophenyl)nortropane single photon emission computed tomography (123I-FP-CIT SPECT). METHODS: Six PD patients (five women and one man, aged 63.2 ± 5.6 years) underwent 123I-FP-CIT SPECT and clinical evaluation [Unified Parkinson's Disease Rating Scale (UPDRS) and Parkinson's Disease Quality of Life Scale (PDQL)] preoperatively, 8 and 13 months after EMCS. Striatum-to-occipital cortex, caudate-to-occipital cortex and putamen-to-occipital cortex 123I-FP-CIT uptake ratios were calculated using the region of interest method. RESULTS: Total and part III UPDRS scores significantly decreased at 8 and 13 months after stimulation (P=0.02 and 0.04, respectively); UPDRS part II and PDQL scores improved after 13 months (P=0.02 and 0.04, respectively). No significant differences in 123I-FP-CIT uptake ratios between baseline and follow-up were found in the examined regions. However, a progressive reduction in 123I-FP-CIT uptake ratios in the striatum contralateral to the implant was found. In contrast, no further decrease in 123I-FP-CIT uptake ratios was detected in the striatum ipsilateral to the implant. There were no correlations between changes in 123I-FP-CIT uptake ratios with disease duration, changes in medication dosage and motor UPDRS scores. CONCLUSION: Despite a small but highly selected sample of advanced PD patients, our results showed that no further dopamine transporter reduction occurred in the striatum ipsilateral to the implant side. This finding could lead to the hypothesis that EMCS might elicit a 'neuroprotective' effect, as suggested by significant clinical benefits.

Percutaneous balloon compression for the treatment of trigeminal neuralgia in patients with multiple sclerosis. Analysis of the potentially prognostic factors.

BACKGROUND: The role of different procedures for the treatment of drug-resistant trigeminal neuralgia (TN) in patients affected by multiple sclerosis (MS) is under discussion and there are no clear indications in the literature. In particular, the role of percutaneous balloon compression (PBC) has been poorly addressed so far. Moreover, to the best of our knowledge, there are no reports analysing the factors potentially related to the prognosis in these patients. We examined the role of PBC for the treatment of TN in MS patients and investigated the role of some clinical and procedure-related factors in determining the prognosis of these patients. METHOD: We retrospectively reviewed clinical and outcome data of 21 MS patients submitted to PBC (eight of them had already been treated with different procedures). We analysed the impact of the sex, number of affected trigeminal divisions, pre-operative deficit, previous operations, compression time and balloon shape at the operation, on acute pain relief (APR) by the chi-squared statistic and on pain-free survival (PFS) by the Kaplan-Meier method. FINDINGS: An excellent (BNI I-II)-good (BNI III) outcome was obtained in all patients with a single or repeated procedure. The presence of a single affected trigeminal division (p = 0.042), the absence of previous operations (p = 0.048), the compression time ≤5 min (p = 0.0067) and the pear-like shape of the balloon at the operation (p < 0.05) were associated to higher pain-free survival. CONCLUSIONS: PBC is a safe and effective technique to treat drug-resistant TN in MS patients. Some clinical and procedure-related factors may play a role in the prognosis of these patients.

Spinal cord stimulation for the treatment of refractory angina pectoris: a multicenter randomized single-blind study (the SCS-ITA trial).

Spinal cord stimulation (SCS) is believed to be effective in treating refractory angina. The need for SCS-related chest paresthesia, however, has hitherto made impossible placebo-controlled trials. Subliminal (non paresthesic) SCS, however, might be also effective on anginal pain. In this trial we compared subliminal SCS with paresthesic SCS and with sham SCS. Twenty-five refractory angina patients, who had never received SCS before, underwent SCS device implantation and were randomized to three groups: paresthesic SCS (group PS; n=10), subliminal SCS (group SS; n=7) or "sham" SCS (group NS; n=8). After 1month group NS patients were randomized to either group PS or SS. After 1month, changes in angina episodes (p=0.016), nitroglycerin use (p=0.015), angina class (p=0.02), quality of life score (p=0.05), and items 2 (p=0.008) and 3 (p=0.009) of Seattle angina questionnaire differed significantly among groups. Group PS showed significant improvement in outcomes compared to group NS, whereas there were no significant differences between groups SS and NS; furthermore, only nitroglycerin use differed significantly between groups PS and SS. At 3months, a significant difference between groups PS and SS was observed in angina attacks (p=0.002), but not in other variables. Thus, in this study, paresthesic, but not subliminal SCS was superior to sham SCS in improving clinical status in refractory angina patients. The lack of significant differences between PS and SS groups in this small study suggests that a possible role for subliminal SCS in individual patients deserves to be assessed in larger trials with appropriate statistical power.

Long-term motor cortex stimulation for amyotrophic lateral sclerosis.

BACKGROUND: Motor cortex stimulation has been proposed for treatment of amyotrophic lateral sclerosis (ALS) and preliminary studies have reported a slight reduction of disease progression using both invasive and noninvasive repetitive stimulation of the motor cortex. OBJECTIVE: The aim of this proof of principle study was to investigate the effects of motor cortex stimulation performed for a prolonged period (about 2 years) on ALS progression. METHODS: Two patients were included in the study; the first patient was treated with monthly cycles of repetitive transcranial magnetic stimulation (rTMS) and the second one was treated with chronic epidural motor cortex stimulation. The rate of progression of the disease before and during treatment was compared. RESULTS: The treatments were well tolerated by the patients. Both patients deteriorated during treatment; however, the patient treated with rTMS showed a slight reduction in deterioration rate. CONCLUSIONS: Although we cannot be sure whether the effects observed in the patient treated with rTMS can be attributed to this form of stimulation, our study set the groundwork for possible future studies investigating the effects of rTMS, for a prolonged period, on a larger group of ALS patients.