Could Cerebral Inflammatory Lesions be the Cellular Origin of Primary Central Nervous System Lymphoma?

INTRODUCTION: Primary central nervous system lymphoma (PCNSL) presents a diagnostic enigma due to the inherent absence of lymphoid tissue in the central nervous system (CNS). The hypothesis posits that lymphocytes infiltrating the CNS during inflammatory responses could represent a cellular source for PCNSL, challenging traditional understandings of its etiology. PATIENT CONCERNS: In 2 illustrative cases, patients presented with neurological symptoms initially misdiagnosed as encephalitis and demyelinating disease, respectively. These diagnoses were established based on clinical assessments and initial biopsy findings. DIAGNOSIS: Subsequent biopsies, conducted months after the first signs of disease, confirmed the diagnosis of PCNSL in both patients. Identifying CD20-positive tumor cells was pivotal, indicating a B-cell lymphoma origin. INTERVENTIONS: Treatment strategies included high-dose methotrexate chemotherapy for both patients. In addition, the second patient underwent adjuvant whole-brain radiotherapy after the chemotherapy regimen. OUTCOMES: The therapeutic approach significantly reduced tumor size in both cases, with no evidence of recurrence observed during the follow-up period. This outcome underscores the potential efficacy of the chosen interventions. CONCLUSION: In response to inflammatory lesions, lymphocyte infiltration into the CNS may serve as a pivotal origin for tumor cells in PCNSL. These cases highlight the complexity of diagnosing CNS disorders and suggest that various forms of encephalitis in the early stages could influence the prognosis of lymphoma. This insight into the cellular origins and treatment responses of PCNSL contributes to a broader understanding of its pathophysiology and management.

Volume of tissue activated within subthalamic nucleus and clinical efficacy of deep brain stimulation in Meige syndrome.

OBJECTIVE: The clinical efficacy of deep brain stimulation (DBS) relies on the optimal electrode placement in a large extent. Subthalamic nucleus (STN) DBS was recognized as clinically effective for Meige syndrome. This study identified the correlations of volume of tissue activated (VTA) within the motor STN and the final efficacy of the surgical procedure. METHODS: Clinical outcomes of the patients (n=25) were evaluated with the percentage improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) scores at the last follow-up (LFU) visit. Pearson's correlation coefficients were calculated to identify the relationship of the final clinical outcomes with the VTA within the STN, VTA within the different STN territories, and other clinical variables. RESULTS: On the whole, the patients showed an average of 59.21% improvement at the LFU visit relative to the baseline (5.72 ± 7.31 vs. 13.70 ± 7.36, P ˂ 0.001). Active electrode contacts mainly clustered in the STN motor territories. There were significant positive correlations between the BFMDRS-M percentage improvement and VTA within the STN (Pearson r = 0.434, P = 0.039) and the STN motor territories (r = 0.430, P = 0.041), but not associative or limbic STN. Other basic clinical characteristics including age, disease duration, and preoperative scores were not significantly correlated with the final outcomes. CONCLUSIONS: Our study further validated the efficacy of STN-DBS in even the cases with intractable Meige syndrome. Furthermore, VTA within the motor STN could serve as a potential prognostic factor for the final clinical outcomes.

Intracranial brain-computer interface spelling using localized visual motion response.

Intracranial brain-computer interfaces (BCIs) can assist severely disabled persons in text communication and environmental control with high precision and speed. Nevertheless, sustainable BCI implants require minimal invasiveness. One of the implantation strategies is to adopt localized and robust cortical activities to drive BCI communication and to make a precise presurgical planning. The visual motion response is a good candidate for inclusion in this strategy because of its focal activity over the middle temporal visual area (MT). Here, we developed an intracranial BCI for spelling, utilizing only three electrodes over the MT area. The best recording electrodes were decided by preoperative functional magnetic resonance imaging (MRI) localization of the MT, and local neural activities were further enhanced by differential rereferencing of these electrodes. The BCI spelling system was validated both offline and online by five epilepsy patients, achieving the fastest speed of 62 bits/min, i.e., 12 characters/min. Moreover, the response patterns of dual-directional visual motion stimuli provided an additional dimension of BCI target encoding and paved the way for a higher information transfer rate of intracranial BCI spelling.

Can C1 lateral mass and C3 pedicle screw fixation be used as an option for atlantoaxial reduction and stabilization in Klippel-Feil patients? A study of its morphological feasibility, technical nuances, and clinical efficiency.

In Klippel-Feil patients with atlantoaxial dislocation, narrow C2 pedicles are often encountered preventing pedicle screw placement. Alternative techniques, including translaminar screws, pars screws, and inferior process screws could not achieve 3-column rigid fixation, and have shown inferior biomechanical stability. The present study aimed to evaluate the feasibility, safety, and efficacy of C3 pedicle screws (C3PSs) as an option for atlantoaxial stabilization in Klippel-Feil patients, and to introduce a freehand technique, the "medial sliding technique," for safe and accurate C3PS insertion. Thirty-seven Klippel-Feil patients with congenital C2-3 fusion who have received atlantoaxial fixation were reviewed. Preoperative CT and CT angiography were acquired to evaluate the feasibility of C3PS placement. C1 lateral mass and C3PS constructs were used for atlantoaxial stabilization. The "medial sliding technique" was introduced to facilitate C3PS insertion. Clinical outcomes and complications were evaluated, and screw accuracy was graded on postoperative CT scans. Morphological measurements showed that more than 80% C3 pedicles could accommodate a 3.5-mm screw. Fifty-eight C3PSs were placed in 33/37 patients using the medial sliding technique. Overall, 96.7% screws were considered safe and there was no related neurovascular complications; 27/33 patients exhibited neurological improvement and 30/33 patients had a solid bone fusion at an average 19.3-month follow-up. Therefore, the C3PS was a feasible option for atlantoaxial fixation in Klippel-Feil patients. The clinically efficiency of C3PS was satisfied with high fusion rates and low complications. The medial sliding technique we used could facilitate safe and accurate placement of C3PSs in Klippel-Feil patients with fused C2-3 vertebra.

Intraoperative detection of human meningioma using a handheld visible resonance Raman analyzer.

To report for the first time the preliminary results for the evaluation of a VRR-LRR™ analyzer based on visible resonance Raman technique to identify human meningioma grades and margins intraoperatively. Unprocessed primary and recurrent solid human meningeal tissues were collected from 33 patients and underwent Raman analysis during surgeries. A total of 1180 VRR spectra were acquired from fresh solid tissues using a VRR-LRR™ analyzer. A confocal HR Evolution (HORIBA, France SAS) Raman system with 532-nm excitation wavelength was also used to collect data for part of the ex vivo samples after they were thawed from - 80 °C for comparison. The preliminary analysis led to the following observations. (1) The intensity ratio of VRR peaks of protein to fatty acid (I2934/I2888) decreased with the increase of meningioma grade. (2) The ratio of VRR peaks of phosphorylated protein to amid I (I1588/I1639) decreased for the higher grade of meningioma. (3) Three RR vibration modes at 1378, 3174, and 3224 cm-1 which were related to the molecular vibrational bands of oxy-hemeprotein, amide B, and amide A protein significantly changed in peak intensities in the two types of meningioma tissues compared to normal tissue. (4) The changes in the intensities of VRR modes of carotenoids at 1156 and 1524 cm-1 were also found in the meningioma boundary. The VRR-LRR™ analyzer demonstrates a new approach for label-free, rapid, and objective identification of primary human meningioma in quasi-clinical settings. The accuracy for detecting meningioma tissues using support vector machines (SVMs) was over 70% based on Raman peaks of key biomolecules and up to 100% using principal component analysis (PCA).

Convergent structural network and gene signatures for MRgFUS thalamotomy in patients with Parkinson's disease.

MRgFUS has just been made available for the 1.7 million Parkinson's disease patients in China. Despite its non-invasive and rapid therapeutic advantages for involuntary tremor, some concerns have emerged about outcomes variability, non-specificity, and side-effects, as little is known about its impact on the long-term plasticity of brain structure. We sought to dissect the characteristics of long-term changes in brain structure caused by MRgFUS lesion and explored potential biological mechanisms. One-year multimodal imaging follow-ups were conducted for nine tremor-dominant Parkinson's disease patients undergoing unilateral MRgFUS thalamotomy. A structural connectivity map was generated for each patient to analyze dynamic changes in brain structure. The human brain transcriptome was extracted and spatially registered for connectivity vulnerability. Genetic functional enrichment analysis was performed and further clarified using in vivo emission computed tomography data. MRgFUS not only abolished tremors but also significantly disrupted the brain network topology. Network-based statistics identified a U-shape MRgFUS-sensitive subnetwork reflective of hand tremor recovery and surgical process, accompanied by relevant cerebral blood flow and gray matter alteration. Using human brain gene expression data, we observed that dopaminergic signatures were responsible for the preferential vulnerability associated with these architectural alterations. Additional PET/SPECT data not only validated these gene signatures, but also suggested that structural alteration was significantly correlated with D1 and D2 receptors, DAT, and F-DOPA measures. There was a long-term dynamic loop between structural alteration and dopaminergic signature for MRgFUS thalamotomy, which may be closely related to the long-term improvements in clinical tremor.

New imaging features of tuberous sclerosis complex: A 7 T MRI study.

Few in vivo studies have focused on the perivenous association of tubers and iron deposition in the deep gray nuclei in patients with tuberous sclerosis complex (TSC). We investigated this possible relationship in TSC patients using susceptibility weighted imaging (SWI) at 7 T. SWI with high spatial resolution and enhanced sensitivity was performed on 11 TSC patients in comparison with 15 age- and sex-matched healthy controls. The relationship between tubers and veins was evaluated. In addition, the phase images of SWI were processed to produce local field shift (LFS) maps to quantify iron deposition. The mean LFS in the deep gray nuclei was compared between the TSC patients and healthy controls using a covariance analysis. Venous involvement was observed in 211 of the 231 (91.3%) cortical tubers on SWI. The slender tubers often oriented around the long axis of penetrating veins, possibly because cortical tubers typically developed and/or migrated along venous vasculatures. A significant difference in LFS of the thalamus was detected between the TSC patients and healthy controls (3.36 ± 0.50 versus 3.01 ± 0.39, p < 0.01). The new in vivo imaging features observed at 7 T provide valuable insights into the possible venous association of TSC lesions and iron accumulation in the deep gray nuclei. Our results may lead to a better understanding of the pathological changes involved in TSC under in vivo conditions.

Clinical features and long-term outcomes of pediatric spinal cord cavernous malformation-a report of 18 cases and literature review.

PURPOSE: Pediatric intramedullary spinal cord cavernous malformation (ISCM) is a rare vascular disease with unclear natural history and long-term outcomes. We aim to determine the demographics, hemorrhagic risk, and long-term outcomes of this rare entity. METHODS: A retrospective review of clinical data and treatment outcomes of pediatric patients treated with ISCM in our institution from 3/2000 to 3/2017 was conducted. In addition, we performed a systematic review of the literature on pediatric ISCM. RESULTS: Eighteen consecutive pediatric patients were included, with an average age of 12.9 ± 4.7 years (range: 4-18 years) and 66.7% being male. Locations were equally distributed in cervical and thoracic segments, with mean extension of 1.3 ± 0.7 segments. Clinical manifestation included extremity weakness (n = 15, 83.3%), pain (n = 10, 55.6%), sensory disorders (n = 8, 44.4%), sphincter disturbance (n = 6, 33.3%), muscular atrophy (n = 3, 16.7%), and spinal deformity (n = 1, 5.6%). Most patients presented with acute symptoms (n = 11, 61.1%), and 7 (38.9%) of them had severe neurological deficits. The annual retrospective hemorrhagic risk was 7.7 per patient-year. Two patients received conservative management, with one improved neurologically and the other remained unchanged. Total resection was achieved in 12 (75%) of the 16 surgical cases, with 8 patients (50%) improved their clinical outcomes, 7 patients (43.8%) remained unchanged, and 1 (6.3%) worsened. During follow-up, one patient had relapse of ISCM. CONCLUSION: Pediatric ISCM appears to have higher hemorrhage risk than their adult counterparts, and they can benefit from surgery whether in the acute phase of neurological deterioration or after clinical recuperation.

Deep brain stimulation for myoclonus dystonia syndrome: a meta-analysis with individual patient data.

Good outcomes have been reported in deep brain stimulation (DBS) for myoclonus-dystonia syndrome (M-D), a heritable disease characterized by childhood-onset myoclonic jerks and dystonia in the upper body. This meta-analysis was to evaluate the clinical outcomes consecutively, compare the stimulation targets, and identify potential prognostic factors. A systematic literature search was performed on PubMed, Web of Science, and Embase. The primary outcome was the percent improvement in Burke-Fahn-Marsden Dystonia Rating Scale movement (BFMDRS-M) scores for dystonia and Unified Myoclonus Rating Scale (UMRS) scores for myoclonus at the last follow-up visit. BFMDRS-disability scores of the patients were also summarized. Pearson correlation analyses were performed to identify the myoclonus and dystonia outcome predictors. Thirty-one studies reporting 71 patients were included. There were significant improvements in BFMDRS-M and BFMDRS-disability scores in each time category and at the last follow-up visit. Mean improvement (%) in UMRS was 79.5 ± 18.2, and 94.1% of the patients showed > 50% improvement in UMRS scores at the last follow-up visit. There was a significant trend toward improved myoclonus outcome with older age at onset and shorter disease duration. Most of the adverse events were mild and transient, and pallidal stimulation seemed to be better with respect to fewer stimulation-induced events. Based on the current data, DBS is effective for even the severe M-D. Surgery at an early stage may predict a better outcome. Although targets do not serve as the outcome predictors, pallidal stimulation may be preferred due to fewer stimulation-induced events.

A novel surgical protocol for safe and accurate placement of C1 lateral mass screws in patients with atlas assimilation, basilar invagination and atlantoaxial instability: technical details, accuracy assessment and perioperative complications.

PURPOSE: To introduce a novel surgical protocol for safe and accurate placement of C1 lateral mass screws in patients with atlas assimilation, basilar invagination and atlantoaxial instability, and to categorize the screw accuracy and perioperative complications regarding this technique in a large case series. METHODS: Between January 2015 and January 2020, patients who had atlas assimilation, basilar invagination and atlantoaxial instability, and underwent atlantoaxial fixation using C1 lateral mass screws were reviewed. C1 lateral mass screws were placed with a novel surgical protocol following a series key steps, including posterior para-odontoid ligament release, panoramic exposure of the invaginated lateral mass, and diligent protection of the abnormal VA. Screw accuracy and related complications were specifically evaluated. RESULTS: A total of 434 C1 lateral mass screws were placed. Fifteen screws (3.5%) were classified as unacceptable, 54 screws (12.4%) were classified as acceptable, and 365 screws (84.1%) were classified as ideal. Overall, 96.5% of screws were deemed safe. There were no cases of vascular injury or permanent neurological defects. One patient with an unacceptable screw presented with hypoglossal nerve paralysis and recovered after an immediate revision surgery. Thirty-seven patients complained about occipital neuralgia and were successfully managed with medication. CONCLUSION: Placement of C1 lateral mass screws in patients with atlas assimilation, basilar invagination and atlantoaxial instability following this surgical protocol is safe and accurate. Thorough para-odontoid ligamental release, wide exposure of the invaginated lateral mass, and diligent protection of the vertebral artery are critical to maximize the chances of successful screw placement.

Clinical characteristics and long-term surgical outcomes of spinal pilocytic astrocytoma: a report of twenty cases.

BACKGROUND: Primary spinal pilocytic astrocytoma (PA) is an extremely rare low-grade astrocytoma with unclear natural history. The demographic characteristics, imaging features, and long-term surgical outcomes have not been clarified due to low prevalence and limited reports. METHODS: A retrospective review within a single institution between 2004 and 2018 of all patients with pathologically proven PA was conducted. Patient data including demographics, radiographic features, treatment modalities, and long-term outcomes were evaluated. RESULTS: Twenty consecutive patients were identified, and 16 (80%) were male patients, with a mean age at presentation of 29 ± 13 years. The lesion was primarily located in cervical (n = 10, 50%), thoracic (n = 7, 35%), cervico-thoracic junction (n = 2), and lumbar level (n = 1, 5%). The tumor had a mean extension of 4 ± 2 (1-7) vertebral segments. Most PAs were located eccentrically (n = 16, 80%), with most being heterogeneous in appearance (cystic and solid) or purely cystic (n = 14, 70%), and had unclear margins (n = 16, 80%). Eleven patients (55%) had associated syringomyelia. Gross total resection (GTR) was achieved in 11 (55%) patients, and subtotal resection (STR) in 9 (45%). During a mean follow-up of 104 ± 56 months, 2 patients died and recurrence was found in 4 patients (20%), translating to a mean progression-free survival of 21 ± 11 months. CONCLUSION: Primary spinal PA is a rare entity with acceptable progression-free survival if treated appropriately. Surgical resection may provide reasonable prolongation of survival, and GTR should be achieved if possible. A close follow-up is recommended especially for residual lesions, and a further in-depth investigation of molecular biomarkers is needed to stratify risk and prognostic factors.

Feasibility of occipital condyle screw placement in patients with Chiari malformation type I: a computed tomography-based morphometric study.

BACKGROUND: The occipital condyle (OC) screw is an alternative technique for occipitocervical fixation that is especially suitable for revision surgery in patients with Chiari malformation type I (CMI). This study aimed to investigate the feasibility and safety of this technique in patients with CMI. METHODS: The CT data of 73 CMI patients and 73 healthy controls were retrospectively analyzed. The dimensions of OCs, including length, width, height, sagittal angle, and screw length, were measured in the axial, sagittal, and coronal planes using CT images. The OC available height was measured in the reconstructed oblique parasagittal plane of the trajectory. RESULTS: The mean length, width, and height of OCs in CMI patients were 17.79 ± 2.31 mm, 11.20 ± 1.28 mm, and 5.87 ± 1.29 mm, respectively. All OC dimensions were significantly smaller in CMI patients compared with healthy controls. The mean screw length and sagittal angle were 19.13 ± 1.97 mm and 33.94° ± 5.43°, respectively. The mean OC available height was 6.36 ± 1.59 mm. According to criteria based on OC available height and width, 52.1% (76/146) of OCs in CMI patients could safely accommodate a 3.5-mm-diameter screw. CONCLUSIONS: The OC screw is feasible in approximately half of OCs in CMI patients. Careful morphometric analyses and personalized surgical plans are necessary for the success of this operation in CMI patients.

Applications of cerebrospinal fluid circulating tumor DNA in the diagnosis of gliomas.

OBJECTIVE: The 2016 World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS) was revised to include molecular biomarkers as diagnostic criteria. However, conventional biopsies of gliomas were spatially and temporally limited. This study aimed to determine whether circulating tumor DNA (ctDNA) from cerebrospinal fluid (CSF) could provide more comprehensive diagnostic information to gliomas. METHODS: Combined with clinical data, we analyzed gene alterations from CSF and tumor tissues of newly diagnosed patients, and detected mutations of ctDNA in recurrent patients. We simultaneously analyzed mutations of ctDNA in different glioma subtypes, and in lower-grade gliomas (LrGG) versus glioblastoma multiforme (GBM). RESULTS: CSF ctDNA mutations had high concordance rates with tumor DNA (tDNA). CSF ctDNA mutations of PTEN and TP53 were commonly detected in recurrent gliomas patients. IDH mutation was detected in most of CSF ctDNA derived from IDH-mutant diffuse astrocytomas, while CSF ctDNA mutations of RB1 and EGFR were found in IDH-wild-type GBM. IDH mutation was detected in LrGG, whereas Rb1 mutation was more commonly detected in GBM. CONCLUSIONS: CSF ctDNA detection can be an alternative method as liquid biopsy in gliomas.

The role of noninvasive brain stimulation for behavioral and psychological symptoms of dementia: a systematic review and meta-analysis.

OBJECTIVE: This meta-analysis aimed at evaluating and comparing the efficacy of noninvasive brain stimulation (NIBS) techniques on the behavioral and psychological symptoms of dementia (BPSD). METHODS: An exhaustive literature retrieval was performed on PubMed, Embase, Cochrane Library, and Web of Science until October 2019. The primary outcome was the relative changes in BPSD severity scores immediately after NIBS and at the last follow-up visit. Subgroup analyses were conducted to compare the efficacy of repetitive transcranial magnetic stimulation (rTMS) and transcranial direct current stimulation (tDCS). Changes in the severity scores after NIBS were also analyzed with restriction to patients with Alzheimer's disease (AD). RESULTS: Ten studies with 324 patients were included, out of which 7 studies involved patients with AD. The analysis results indicated that NIBS significantly improved the BPSD outcome immediately after stimulation (SMD, 0.31; 95% CI, 0.10-0.52; P = 0.005), but not at the last follow-up visit (0.15; - 0.11-0.41; 0.25). Our subgroup analyses suggested that the favorable effects of rTMS remained significant at the last follow-up visit (0.57; 0.18-0.96; 0.004). This discrepancy maybe caused by the continuously insignificant outcomes of tDCS on the whole data. The results for AD patients immediately after stimulation (0.37; 0.12-0.61; 0.003) and at the last follow-up visit (0.29; - 0.19-0.76; 0.24) were both largely similar to those in the whole patient group with dementia. CONCLUSIONS: rTMS, rather than tDCS, was capable of persistently improving the BPSD at an early stage after treatment. More trials are warranted to confirm our results before the establishment of final conclusions.

Morphological and Biochemical Properties of Human Astrocytes, Microglia, Glioma, and Glioblastoma Cells Using Fourier Transform Infrared Spectroscopy.

BACKGROUND With infiltration, high-grade glioma easily causes the boundary between tumor tissue and adjacent tissue to become unclear and results in tumor recurrence at or near the resection margin according to the incomplete surgical resection. Fourier transform infrared spectroscopy (FTIR) technique has been demonstrated to be a useful tool that yields a molecular fingerprint and provides rapid, nondestructive, high-throughput and clinically relevant diagnostic information. MATERIAL AND METHODS FTIR was used to investigate the morphological and biochemical properties of human astrocytes (HA), microglia (HM1900), glioma cells (U87), and glioblastoma cells (BT325) cultured in vitro to simulate the infiltration area, with the use of multi-peak fitting and principal component analysis (PCA) of amide I of FTIR spectra and the use of hierarchical cluster analysis (HCA). RESULTS We found that the secondary structures of the 4 types of cells were significantly different. The contents of a-helix structure in glial cells was significantly higher than in the glioma cells, but the levels of ß-sheet, ß-turn, and random coil structures were lower. The 4 types of cells could be clearly separated with 85% for PC1 and 12.2% for PC2. CONCLUSIONS FTIR can be used to distinguish between human astrocytes, microglia, glioma, and glioblastoma cells in vitro. The protein secondary structure can be used as an indicator to distinguish tumor cells from glial cells. Further tissue-based and in vivo studies are needed to determine whether FTIR can identify cerebral glioma.

Associations of histological and molecular alterations with invasion of the corpus callosum in gliomas.

BACKGROUND: Glioma invading the corpus callosum (CC) accounts for approximately 14% of gliomas and is thought to be more aggressive. However, there is still a lack of studies on the pathogenesis and molecular features of this condition. Here, we examined the occurrence association of CC invasion with respect to patients' clinical, pathological, and genetic characteristics. METHODS: First, a cohort of 331 patients was included, with 86 cases (26%) that were diagnosed with invasion glioma. They were all analyzed for basic clinical and pathological characteristics and four routinely tested glioma molecular markers. Second, 29 pairs of patients who underwent deep sequencing of 68 glioma molecular alterations were selected from both groups for in-depth analysis. RESULTS: The results of the first part showed that there was no difference between the two groups in terms of the basic factors in univariate analysis, while in multivariate logistic analysis, WHO grade was the risk factor for CC invasion (p = 0.001). The results of the second part showed that the paired groups had different genetic expression profiles, which highlighted glioma invading the CC as a distinct biological entity. PDGFRA mutation (PDGFRAmut) was present in 9 patients with invasive gliomas (31%), but only in one case (3.4%) in the control group (OR 17.331; 95% CI 1.987-151.156). CONCLUSION: Our data revealed the clinical, pathological, and genetic characteristics of glioma invading the CC and showed that it may be associated with glioma WHO grade and PDGFRAmut, but not other factors. Thus, the risk signaling pathway may offer potential therapeutic targets for this disease.

Clinical features and long-term surgical outcomes of pure spinal epidural cavernous hemangioma-report of 23 cases.

BACKGROUND: Pure spinal epidural cavernous malformation (CM) is a rare hypervascular disease that is easily misinterpreted as other imaging-similar epidural lesions. The demographic characteristics, therapeutic strategies, and surgical outcomes associated with this vascular entity remains unclear. METHODS: A retrospective review of patients with pathologically proven, pure epidural CM from 2001 to 2018 was conducted. All data that included clinical manifestations, radiographic features, and treatment modalities were analyzed. RESULTS: Twenty-three consecutive patients with an average age of 51.5 ± 8.4 years old (range 38-70 years old) were included; of these, 52.2% were female patients. Clinical manifestation included chronic progressive nerve root disturbance syndrome in 7 patients (30.4%) and myelopathy in 16 patients (69.6%). The CM level was predominately thoracic (n = 14, 60.9%) or lumbar (n = 6, 26.1%), with the dorsal epidural space (n = 14, 60.9%) the most common site. The initial clinical diagnoses were schwannoma (n = 11, 52.2%), meningioma (n = 5, 21.7%), angioma (n = 3, 13.1%), recurrent CMs (n = 2, 8.7%), and metastatic tumor (n = 1, 4.3%). Fifteen lesions (65.2%) were isointense on T1-weighted images, and all lesions were hyperintense on T2-weighted images, with homogenously strong enhancement observed in 17 lesions (73.9%). Total resection was achieved in 18 patients (78.3%) and usually resulted in excellent clinical outcomes (n = 21, 91.3%). No patients experienced recurrence of symptoms, and lesion relapse during follow-up. CONCLUSION: Total surgical removal of epidural CM can usually achieve satisfactory outcomes in patients with a chronic clinical course and should be recommended. Subtotal removal of tumors can also benefit patients, and guaranteed a long recurrent free time after surgery. A good preoperative neurological condition usually leads to good outcomes.

A morphometric study of the atlanto-occipital joint in adult patients with Chiari malformation type I.

BACKGROUND: There are many morphometric studies on Chiari malformation type I (CMI) patients, most of which focus on the posterior cranial fossa (PCF). Less attention has been paid to the atlanto-occipital joint. In this study, we aim to evaluate the morphological characteristics of the atlanto-occipital joint in CMI patients. MATERIALS AND METHODS: The cervical CT imaging data of adult patients diagnosed with CMI but without any bony malformation in craniovertebral junction (CVJ) who were treated by the authors between January 2014 and December 2019 were retrospectively analyzed. The equal number of sex and age-matched healthy individuals were included as the control group. The morphometric analysis was performed by measuring the length and depth of the atlanto-occipital joint, and the depth/length ratio was calculated to evaluate the curvature of the joint. RESULTS: A total of 47 patients (15 males and 32 females) were included. The mean age of patients was 47.49 ± 9.01 years (range 19-62 years). The mean depth/length ratio of the atlanto-occipital joint in CMI patients was 0.141 ± 0.065 (range 0.027 - 0.274), which was smaller than that of control individuals (0.228 ± 0.057, range 0.069 - 0.379). And the difference was statistically significant (p < .01). CONCLUSIONS: The atlanto-occipital joints in CMI patients are significantly flatter compared with those in healthy controls. This morphological variation could lead to differences of the atlanto-occipital stability between CMI patients and normal population, which may be related to the pathogenesis of CMI.

Image-based analysis and long-term clinical outcomes of deep brain stimulation for Tourette syndrome: a multisite study.

BACKGROUND: Deep brain stimulation (DBS) can be an effective therapy for tics and comorbidities in select cases of severe, treatment-refractory Tourette syndrome (TS). Clinical responses remain variable across patients, which may be attributed to differences in the location of the neuroanatomical regions being stimulated. We evaluated active contact locations and regions of stimulation across a large cohort of patients with TS in an effort to guide future targeting. METHODS: We collected retrospective clinical data and imaging from 13 international sites on 123 patients. We assessed the effects of DBS over time in 110 patients who were implanted in the centromedial (CM) thalamus (n=51), globus pallidus internus (GPi) (n=47), nucleus accumbens/anterior limb of the internal capsule (n=4) or a combination of targets (n=8). Contact locations (n=70 patients) and volumes of tissue activated (n=63 patients) were coregistered to create probabilistic stimulation atlases. RESULTS: Tics and obsessive-compulsive behaviour (OCB) significantly improved over time (p<0.01), and there were no significant differences across brain targets (p>0.05). The median time was 13 months to reach a 40% improvement in tics, and there were no significant differences across targets (p=0.84), presence of OCB (p=0.09) or age at implantation (p=0.08). Active contacts were generally clustered near the target nuclei, with some variability that may reflect differences in targeting protocols, lead models and contact configurations. There were regions within and surrounding GPi and CM thalamus that improved tics for some patients but were ineffective for others. Regions within, superior or medial to GPi were associated with a greater improvement in OCB than regions inferior to GPi. CONCLUSION: The results collectively indicate that DBS may improve tics and OCB, the effects may develop over several months, and stimulation locations relative to structural anatomy alone may not predict response. This study was the first to visualise and evaluate the regions of stimulation across a large cohort of patients with TS to generate new hypotheses about potential targets for improving tics and comorbidities.

Dysregulation of resting-state functional connectivity in patients with Cushing's disease.

PURPOSE: To explore the anatomical distance-dependent functional connectivity patterns in patients with active phase of Cushing's disease (CD) and to evaluate the associations between hypercortisol exposure and regional normalized functional connectivity strengths (nFCSs). METHODS: Based on the fMRI data in 32 CD patients and 32 healthy controls (HCs), we computed the nFCSs for each voxel in the brain and further divided them into long-range and short-range nFCSs. General linear models was used to investigate between-group differences in these nFCS metrics and the correlations between the nFCSs and clinical variables. RESULTS: Compared with HC, CD patients showed dysregulation of the nFCSs mainly in the default mode network. They showed an overall higher nFCS in bilateral parahippocampal cortex mainly owing to the disruption of long-range nFCS and a relatively lower nFCS in bilateral posterior cingulate cortex (PCC), bilateral lateral parietal cortex (LP), and right prefrontal cortex (PFC). In addition, their long-range nFCS was lower in the bilateral anterior cingulate cortex, PCC, and LP; short-range nFCS was lower in the bilateral PFC. Notably, the positive correlation between the nFCSs in their right parahippocampal cortex and serum cortisol levels at 08:00 remained significant after taking the anatomical distance into consideration. CONCLUSION: The discrepant functional connectivity patterns found in our study indicated a hypercortisol-associated, distance-dependent disruption of resting-state functional connectivity in patients with active CD. We provide novel insights into the impacts of hypercortisol exposure and the pathophysiologic mechanisms of CD, which may facilitate advances in CD intervention ultimately.