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BackgroundSpinocerebellar ataxia type 3 (SCA3) is the most common autosomal dominant hereditary ataxia, and, thus far, effective treatment remains low. Repetitive transcranial magnetic stimulation (rTMS) can improve the symptoms of spinal cerebellar ataxia, but the mechanism is unclear; in addition, whether any improvement in the symptoms is related to cerebellar metabolism has not yet been investigated. Therefore, the purpose of this study was to investigate the effects of low-frequency rTMS on local cerebellar metabolism in patients with SCA3 and the relationship between the improvement in the symptoms and cerebellar metabolism.MethodsA double-blind, prospective, randomized, sham-controlled trial was carried out among 18 SCA3 patients. The participants were randomly assigned to the real stimulation group (n = 9) or sham stimulation group (n = 9). Each participant in both the groups underwent 30 min of 1 Hz rTMS stimulation (a total of 900 pulses), differing only in terms of stimulator placement, for 15 consecutive days. To separately compare pre- and post-stimulation data (magnetic resonance spectroscopy (MRS) data and the International Cooperative Ataxia Rating Scale (ICARS) score) in the real and sham groups, paired-sample t-tests and Wilcoxon’s signed-rank tests were used in the analyses. The differences in the ICARS and MRS data between the two groups were analyzed with independent t-tests and covariance. To explore the association between the changes in the concentration of cerebellar metabolism and ICARS, we applied Pearson’s correlation analysis.ResultsAfter 15 days of treatment, the ICARS scores significantly decreased in both the groups, while the decrease was more significant in the real stimulation group compared to the sham stimulation group (p < 0.001). The analysis of covariance further confirmed that the total ICARS scores decreased more dramatically in the real stimulation group after treatment compared to the sham stimulation group (F = 31.239, p < 0.001). The values of NAA/Cr and Cho/Cr in the cerebellar vermis, bilateral dentate nucleus, and bilateral cerebellar hemisphere increased significantly in the real stimulation group (p < 0.05), but no significant differences were found in the sham stimulation group (p > 0.05). The analysis of covariance also confirmed the greater change in the real stimulation group. This study also demonstrated that there was a negative correlation between NAA/Cr in the right cerebellar hemisphere and ICARS in the real stimulation group (r = − 0.831, p = 0.02).ConclusionThe treatment with rTMS over the cerebellum was found to induce changes in the cerebellar local metabolism and microenvironment in the SCA3 patients. The alterations may contribute to the improvement of the symptoms of ataxia in SCA3 patients.

BackgroundNowadays, small peripheral pulmonary lesions (PPLs) are frequently detected and the prognosis of lung cancer depends on the early diagnosis. Because of the high fee and requiring specialized training, many advanced techniques are not available in many developing countries and rural districts.MethodsThree sets of opaque soft copper wires visible under the fluoroscopy (Flu) in the Flu-flexible bronchoscopy (FB) group (n = 24), which determined the three planes of the lesion, were respectively placed firmly on the surface of the chest wall with adhesive tape on the chest wall. The FB tip was advanced into the bronchus toward the crosspoint of the three perpendicular planes under Flu with careful rotation of a C-arm unit. Then the specimen were harvested focusing around the crosspoint for pathologic diagnosis. The rapid on-site evaluation (ROSE) procedure was also performed. The average Flu time during FB procedures were recorded and diagnostic accuracy rates in the Flu-FB group were compared with the other group guided by radial endobronchial ultrasound (R-EBUS) (n = 23).ResultsThe location of the core point of the lesion, whether it was visible or not under the fluoroscopy could be recognized by three-dimensional localization technique. The accuracy rates of diagnostic yields were 62.5% in the Flu-FB group, and was similar as 65.2% in the R-EBUS group (P > 0.05). However, in the Flu-FB group, there was a decreasing tendency on accurate diagnosis rates of lower lobe (LL) lesions when comparing with non-LL lesions (3/8 = 37.5% vs 12/16 = 75%, P = 0.091) while in the R-EBUS group it was similar (9/12 = 75% vs 6/11 = 54.6%, P = 0.278). In the Flu-FB group, fluoroscopy time was negatively correlated with the lesion length (r = −0.613, P = 0.001), however, there was no significant difference between the lesions invisible or not (5.83 ± 1.45 min vs 7.67 ± 2.02 min, P = 0.116) under the fluoroscopy, as well as no significant difference among SPN, mGGO and GGO (6.12 ± 2.05 min, 7.25 ± 1.33 min and 7.80 ± 2.02 min, P > 0.05).ConclusionsSmall PPL whether it is visible or not under fluoroscopy can be located accurately by our three-dimensional localization technique on chest wall surface and performed bronchoscopy procedures to increase diagnostic yields. It is more convenient, economical and reliable with the similar diagnostic yields than R-EBUS guided method.Trial registrationCurrent Controlled Trials ChiCTR-DDD-16009715. The date of registration: 3rd Nov, 2016. Retrospectively registered.

Objectives Spinocerebellar ataxia type 3 is a disorder within the brain network. However, the relationship between the brain network and disease severity is still unclear. This study aims to investigate changes in the white matter (WM) structural motor network, both in preclinical and ataxic stages, and its relationship with disease severity. Methods For this study, 20 ataxic, 20 preclinical SCA3 patients, and 20 healthy controls were recruited and received MRI scans. Disease severity was quantified using the SARA and ICARS scores. The WM motor structural network was created using probabilistic fiber tracking and was analyzed using graph theory and network‐based statistics at global, nodal, and edge levels. In addition, the correlations between network topological measures and disease duration or clinical scores were analyzed. Results Preclinical patients showed increasing assortativity of the motor network, altered subnetwork including 12 edges of 11 nodes, and 5 brain regions presenting reduced nodal strength. In ataxic patients assortativity of the motor network also increased, but global efficiency, global strength, and transitivity decreased. Ataxic patients showed a wider altered subnetwork and a higher number of reduced nodal strengths. A negative correlation between the transitivity of the motor network and SARA and ICARS scores was observed in ataxic patients. Interpretation Changes to the WM motor network in SCA3 start before ataxia onset, and WM motor network involvement increases with disease progression. Global network topological measures of the WM motor network appear to be a promising image biomarker for disease severity. This study provides new insights into the pathophysiology of disease in SCA3/MJD.