Outcome of visuospatial dysfunction assessment in patients with Parkinson's disease using mobile application software.

Background: Visuospatial dysfunction and cognitive impairment are common in Parkinson's disease (PD), which draw increasing attention in the current literature. But clinicians still lack rapid, effective and unified cognitive battery for visuospatial assessment. Objective: A new approach was studied to explore the feasibility of using mobile application software (APP) to evaluate visuospatial dysfunction in patients with PD and compared with traditional assessment tools. We aimed to verify the threshold score of the APP for early diagnosis. Materials and methods: A total of 41 patients with PD underwent assessments using several test modules including Digit Symbol Test (DST), Visual Organization Test (VOT), Facial Recognition Test (FRT), Vocabulary Memory Test (VMT) of this APP, as well as Clock Drawing Test (CDT), Cube Copying Test (CCT) and the Mini-Mental State Examination (MMSE) for comparison. Among the 41 PD patients, 30 individuals were found to have visuospatial dysfunction based on CDT score < 5 and CCT score of<18 while the remaining 11 patients served as control. Results: There were statistically significant differences in DST, VOT, and FRT scores (all p ≤ 0.001 for group comparisons). DST, VOT, and FRT-1 were significantly correlated with MMSE, CDT and CCT and the correlations were moderate or fairly strong. For visuospatial dysfunction diagnosis, all the areas under curves (AUC) of DST, VOT, and FRT-1 were statistically significant (p < 0.0001, p = 0.0002, and p = 0.0002, respectively). The estimates and 95% confidence intervals of AUC were 0.8303 (0.6868, 0.9739), 0.8045 (0.6423, 0.9668), and 0.7833 (0.6344, 0.9322), respectively. Their cut-off points for visuospatial dysfunction were 26, 17, and 19, respectively. After dichotomization by the cut-off points, DST had high sensitivity of 96.67% while VOT and FRT-1 had high specificity of 81.82 and 90.91%. Conclusion: This study demonstrated that visuospatial disorders was highly prevalent in PD patients, and the APP used in study could be a practical clinical screening tool for visuospatial ability assessment with high sensitivity and specificity.

A localized pallidal physiomarker in Meige syndrome.

Objectives: Oscillatory patterns in local field potentials (LFPs) have been recognized as disease-specific physiomarkers, particularly in the context of Parkinson's disease and cervical dystonia. This characteristic oscillatory feature is currently employed in adaptive deep brain stimulation (aDBS). However, for other types of dystonia, especially Meige syndrome, a distinct physiomarker of this nature is yet to be identified. Methods: Local field potentials were recorded during microelectrode-guided deep brain stimulation surgery from 28 patients with primary Meige syndrome. Before surgery, the severity of patients' motor syndrome were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale-Motor (BFMDRS-M). An instantaneous oscillation detection method was employed to identify true narrowband oscillations. Subsequently, a linear mixed effects model was utilized to examine the relationship between oscillatory activities (including power amplitude and burst duration) and symptom severity. Results: The focal peaks of "oscillatory activities" detected were predominantly concentrated in the narrow theta band (4-8 Hz), constituting 81.5% of the total detected oscillations in all recording sites near active DBS contacts in the globus pallidus internus (GPi). The linear mixed effects model revealed a positive correlation between the theta burst duration and the severity of preoperative motor impairment, but no correlation with postoperative motor scores. Additionally, there was no significant lateralization effect observed between the left and right GPi. Conclusion: Our findings suggest that the exaggerated narrowband theta activity (mainly the burst duration) in the GPi is predictive of dystonia symptom severity and may be used as a physiomarker for optimized DBS target during surgery and adaptive DBS for the treatment of Meige syndrome.

Staged Surgery for Intra-Extracranial Communicating Jugular Foramen Paraganglioma: A Case Report and Systematic Review.

Staged surgery strategy was preferred for patients with intra-extracranial communicating jugular foramen paraganglioma (IECJFP). A female patient who presented mild tinnitus, headache, and dizziness, together with preoperative related imaging, was diagnosed with a left intra-extracranial communicating jugular foramen lesion in November 2015 and accepted an initial operation for the intracranial tumor by retrosigmoid approach. The pathologic report was paraganglioma. In November 2021, a subtotal resection of the extracranial tumor was conducted for prominent lower cranial nerves (LCNs) deficit and middle ear involvement by infratemporal approach. In patients with IECJFP accompanied by LCNs deficit and middle ear involvement, an initial surgery for extracranial lesion and a second procedure for intracranial tumor were appropriate. However, the first operation for the intracranial lesion was preferred in IECJFP cases without LCNs deficit and middle ear involvement, as it could remove compression to the neurovascular structure and brain stem, clarify a pathological diagnosis, avoid a CSF leak, and prevent a severe neurological disorder from extracranial lesion excision. Subtotal resection of the extracranial tumor would be performed when lesion became larger combined with obvious LCNs disorder and tympanic cavity involvement. Consideration of specific staged surgical strategy for IECJFP in accordance with preoperative LCNs deficit and tympanic cavity involvement could prevent critical postoperative neurological deficit and improve quality of life in the long term.

Risk factors of herpes simplex virus reactivation after surgery for primary trigeminal neuralgia.

The objective of this study is to investigate the risk factors of oral or facial herpes simplex virus (HSV-1) infection after primary trigeminal neuralgia (PTN). The clinical data of 33 PTN patients admitted by the same surgeon in the neurosurgery were retrospectively analyzed. Among the 33 patients, 26 patients underwent microvascular decompression (MVD), 6 patients who have not been found the clear offending vessels during the operation underwent partial sensory rhizotomy (PSR), and only one underwent adhesive band separation. Thirteen patients with postoperative oral and facial HSV-1 infection were selected as the herpes group, and the remaining 20 patients without postoperative oral and facial HSV-1 infection were selected as the non-herpes group. The differences between the two groups were compared by statistical analysis of factors such as gender, age, operation mode, operation time, and serum HSV-1 antibody titer value before surgery. Compared with the non-herpes group, there were no statistically significant differences in sex ratio (P = 0.930), age composition (P = 0.261), or disease profile (P = 0.226). Twenty-six patients underwent MVD operation, eight of whom were infected, and the difference between the two groups was statistically significant (P = 0.029). The operation time of the herpes group was 10-30 min, which was significantly longer than that of the non-herpes group. The difference in operation time between the two groups was statistically significant (P = 0.023). Serum HSV-1-IgM was negative (< 0.9 COI) in all patients before surgery, but the positive rate of HSV-1-IgG (≥ 1.1 COI) was 97%, and the titer was greater than four times in 97% (32/33) of patients. The titer of IgG antibody in the herpes group was significantly lower than that in the non-herpes group, and the difference between the two groups was statistically significant (P = 0.017). The serum HSV-1-IgG in most of the PTN patients was positive. Latent HSV-1 in the trigeminal ganglion may be reactivate after PTN surgery to produce ipsilateral oral and facial herpes infection. The infection of HSV-1 reactivation after PTN surgery was positively correlated with the operation time but negatively correlated with the titer of HSV-1-IgG antibody before PTN surgery. The incidence of HSV-1 infection after PTN operation is related to different surgical procedures.

Comprehensive Analysis of REST/NRSF Gene in Glioma and Its ceRNA Network Identification.

We sought to clarify the clinical relationship between REST/NRSF expression and the prognosis of glioma and explore the REST-associated competitive endogenous RNA (ceRNA) network in glioma. We downloaded RNA-seq, miRNA-seq and correlated clinical data of 670 glioma patients from The Cancer Genome Atlas and analyzed the correlation between REST expression, clinical characteristics and prognosis. Differentially expressed genes (DEGs) were identified with DESeq2 and analyzed with Gene Ontology (GO) and the Kyoto Encyclopedia of Genes and Genomes (KEGG) using the Profiler package. Starbase was used to explore the regulatory interaction between REST and miRNAs or LncRNAs. The lncRNA-miRNA-REST ceRNA network was constructed with Cytoscape. RT-qPCR, WB, CCK8, wound-healing, and luciferase assays were performed to validate the ceRNA network. Results showed that REST expression was significantly higher in glioma patients than normal samples. Higher REST expression was significantly associated with worse overall survival, progression-free interval, and worse disease-specific survival in glioma patients. The DEGs of mRNA, miRNA, and lncRNA were identified, and GO and KEGG enrichment analyses were performed. Finally, REST-associated ceRNA networks, including NR2F2-AS1-miR129-REST and HOTAIRM1-miR137-REST, were experimentally validated. Thus, REST may be a prognostic biomarker and therapeutic target in glioma, and its regulatory network validated in this study may provide insights into glioma's molecular regulatory mechanisms.

Electrophysiological signatures predict clinical outcomes after deep brain stimulation of the globus pallidus internus in Meige syndrome.

OBJECTIVE: Deep brain stimulation (DBS) of the globus pallidus internus (GPi) has been shown to be a safe and effective alternative therapy for ameliorating medically refractory primary Meige syndrome. However, the associations between DBS target position and surrounding electrophysiological properties as well as patients' clinical outcomes remains largely unknown. In a large number of patients, we investigated electrophysiological features around stimulation targets and explored their roles in predicting clinical outcomes following bilateral GPi-DBS. METHODS: The locations of DBS active contacts along the long axis of the GPi in a standard space were calculated and compared among three groups with different clinical outcomes. The firing rates of individual neurons within the GPi were calculated for each patient and compared across the three groups. RESULTS: Compared with the bad group (poor clinical outcome), active contacts in the good group (good clinical outcome) and the best group (best clinical outcome) were located in the more posterior GPi. The average firing rates in the good and best groups were significantly higher than in the bad group, and this difference was pronounced within the ventral GPi. For the bad group, the average firing rates were significantly lower in the ventral than in the dorsal GPi. CONCLUSIONS: This study suggests that DBS of the posterior GPi may produce better clinical outcomes during primary Meige syndrome treatment and that higher GPi neuronal activity, particularly within the ventral part, can be used as a biomarker to guide DBS electrode implantation during surgery.

RNF213 gene polymorphism rs9916351 and rs8074015 significantly associated with moyamoya disease in Chinese population.

BACKGROUND: Gene polymorphism especially Ring Finger Protein 213 (RNF213) p.R4810K is one of the main cause of moyamoya disease (MMD) in Asian populations, especially among Japanese people. However, missense mutation may not explain the reduced frequency of MMD in Chinese patients. We performed a hospital based case-control study in a Chinese population to elucidate the possible underlying reasons. METHODS: Five gene polymorphism loci, namely, rs35692831, rs9916351, rs9913636, rs8074015 and rs112735431, were included. A total of 98 patients and 114 healthy controls were enrolled in the study. Genomic DNA was genotyped by Mass Array methods. RESULTS: A significant difference was observed between patients and healthy controls in rs9916351, rs9913636, and rs8074015 loci under three genotypes and allelic models (P<0.01). Logistic regression analysis revealed the significant differences under the dominant, recessive and additional model in rs9916351 [odds ratio (OR) =4.173, 95% confidence interval (CI): 2.290-7.606, P<0.001; OR =3.152, 95% CI: 1.585-6.267, P=0.001; OR =0.199, 95% CI: 1.727-3.764, P<0.001; respectively] and rs8074015 (OR =0.359, 95% CI: 0.206-0.627, P<0.001; OR =0.348, 95% CI: 0.148-0.81, P=0.015; OR =0.208, 95% CI: 0.311-0.703, P<0.001; respectively), even adjusting for age and gender. In addition, the haplotype rs9913636-rs8074015 under "GACG" showed significant association with MMD. CONCLUSIONS: Our results had revealed the polymorphism of RNF213 rs9916351 and rs8074015 were significantly associated with MMD especially in Chinese population.

Long-Term Efficacy of Deep Brain Stimulation of Bilateral Globus Pallidus Internus in Primary Meige Syndrome.

OBJECTIVE: Deep brain stimulation (DBS) of the internal segment of the globus pallidus (GPi) is an alternative therapy in ameliorating the clinical symptoms of primary Meige syndrome. Nevertheless, proof of its efficacy and safety is insufficient due to several case reports and small-sample clinical studies. This study aims to investigate postoperative long-term efficacy in patients undergoing DBS of the GPi for primary Meige syndrome. METHODS: We performed a retrospective study to assess the efficacy and safety of bilateral GPi stimulation in 40 patients with primary Meige syndrome who responded poorly to medical treatments or botulinum toxin injections. All participants were postoperatively followed up at the outpatient clinic, and their motor functions were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). The severity of patients' dystonia was evaluated before surgery and at follow-up neurostimu-lation. RESULTS: The implanted stimulator was turned on 1 month after surgery. All 40 patients received monopolar stimulation using the following parameters: voltage 2.5-3.5 V (average: 2.6 ± 0.8 V), frequency 60-160 Hz (average: 88.0 ± 21.3 Hz), and pulse width 60-185 µS (average: 90.0 ± 21.1 µS). In 28 of 40 patients, the symptoms had signifi-cantly improved within 1 week of stimulation. Most of the patients had been followed up for 6-24 months (average: 15.0 ± 7.8 months). The clinical symptoms of all patients had significantly improved. At 6, 12, and 24 months after surgery, the BFMDRS subscores of eyes, mouth, speech, and swallowing were significantly lower, and subscores of mouth movement showed progressively decreased with prolonged stimulation time. The overall improvement rate was 83%. Five adverse events occurred in the 40 patients; all of these events resolved without permanent sequelae. CONCLUSIONS: Bilateral GPi-DBS demonstrated satisfactory long-term efficacy in the treatment of primary Meige syndrome and could serve as an effective and safe option.

Long-Term Outcome of Repeat Microvascular Decompression for Hemifacial Spasm.

OBJECTIVE: Although repeat microvascular decompression (MVD) for hemifacial spasm (HFS) in patients with failed prior MVD is potentially curative, little is known about the long-term results of repeat MVD. We aimed to evaluate the long-term outcomes and complications after repeat MVD for HFS. METHODS: We performed repeat MVD on 78 consecutive patients who had undergone a prior MVD >1 year previously. Follow-up data were available for 58 patients, with a median follow-up period of 8.6 years (range, 6.9-10.2 years). The patients were assessed for intraoperative findings, relief results, and complications at discharge and at follow-up, as well as the associations between the preoperative characteristics and outcomes. RESULTS: At discharge, of 78 patients with repeat MVD, 72 (92.3%) achieved complete spasm resolution and 1 (2.6%) had significantly improved spasm resolution. Of all patients, 9% (7 of 78) presented short-term complications, including partial hearing loss, hemifacial paresis, and cerebrospinal fluid leak. At follow-up, 45 of 58 (77.6%) patients had complete relief and 10 of 58 (17.2%) had improved relief. Permanent complications occurred in 14 patients (24.1%), with partial hearing loss and mild hemifacial paresis being the most common. Despite the complications, 51 of 58 patients (91.4%) reported an excellent life quality. No significant correlation was found between preoperative characteristics, such as age, interval to prior MVD, or interval to recurrence, and outcomes including short-term or long-term relief results and complications. CONCLUSIONS: Repeat MVD provides lasting relief for most patients with persistent or recurrent HFS, albeit with a relatively high complication rate.