Our objective was to evaluate diaphragm thicknesses during respiration by ultrasonography, and compare with conventional measurements of respiratory functions in patients with amyotrophic lateral sclerosis (ALS). Thirty-six consecutive ALS patients and 19 age-matched healthy volunteers participated. Ultrasonography of the diaphragm in the zone of apposition was performed. Maximal diaphragm thickness during the maximal inspiratory effort (DTmax) and minimum diaphragm thickness at the end expiratory position (DTmin) were measured using ultrasonography. The thickening ratio (TR), defined as the ratio of DTmin to DTmax, was calculated. All patients underwent conventional pulmonary function testing. Arterial blood gas analysis was also performed. The diaphragm was clearly identifiable by ultrasonography. DTmax, DTmin and the TR were all significantly decreased in ALS patients with %VC (vital capacity) < 80, compared with those in either ALS patients with %VC ≥ 80 or healthy controls. DTmax, DTmin and the TR were all significantly correlated with %VC. In addition, significant inverse correlations were found between all three parameters and pCO(2). The inter-observer reliability of measurements of diaphragm thickness was high. In conclusion, sonography of the diaphragm can provide additional or complementary information for assessing respiratory functions in patients with ALS.

impulsivity is a neuropsychiatric feature of parkinson's disease (pD). We investigated the pathophysiology of impulsivity in pD using resting-state functional magnetic resonance imaging (rs-fMRI). We investigated 45 patients with idiopathic PD and 21 healthy controls. Based on Barratt Impulsiveness Scale (BIS-11) score, PD patients were classified as higher (PD-HI) or lower impulsivity (pD-Li). functional connectivity (fc) between various large-scale brain networks were analysed using the conn toolbox. fc between the right frontoparietal network (fpn) and medial visual network (MVN) was significantly higher in PD-HI patients than PD-LI patients (false discovery rate [FDR]adjusted p = 0.0315). FC between the right FPN and MVN had a significant positive correlation with total BIS-11 score (FDR-adjusted p = 0.010) and the attentional impulsivity (FDR-adjusted p = 0.046) and non-planning impulsivity subscale scores (fDR-adjusted p = 0.018). On the other hand, motor impulsivity subscale score had a significant negative correlation with the FC between the defaultmode and salience networks (right supramarginal gyrus, fDR-adjusted p = 0.018; anterior cingulate cortex, fDR-adjusted p = 0.027); this trend was observed in healthy controls. The attentional and non-planning impulsivity, regarded as 'cognitive' impulsivity, may be associated with dysfunction in integration of perceptual information and flexible cognitive control in PD.

Introduction. The Questionnaire for Impulsive-Compulsive Disorders in Parkinson’s Disease (PD)-Rating Scale (QUIP-RS) was developed to assess the severity of impulsive and compulsive behaviors (ICBs) in PD. We aimed to validate the Japanese version of QUIP-RS and determine the characteristics of ICBs in Japan. Methods. We translated the QUIP-RS into Japanese, back-translated it to English, and obtained confirmation from the original author that the questionnaire remained appropriate. The participants for the validation study were 161 PD patients, identified by continuous sampling at two institutions, who were diagnosed with ICBs through a semistructured interview and completed the QUIP-RS-J. Sensitivity, specificity, and cutoff values were calculated using receiver operating characteristic (ROC) curves. Interinstitutional reliability and test-retest reliability were also assessed for a subset of participants. Results. Twenty-six (16.1%) participants were diagnosed with ICB. The optimal cutoff value of the QUIP-RS-J total score was 6, with area under the curve (AUC) = 0.889 and sensitivity/specificity of 0.92/0.71. Each subscale also showed high AUC (0.89–1.00), sensitivity (0.92–1.00), and specificity (0.71–1.00). Compared with the English version, the optimal cutoff point for binge eating was higher and hypersexuality lower. The total score tended to be higher when described by an informant. Conclusion. The present study validated the Japanese version of QUIP-RS. Use of QUIP-RS-J enables standardized assessment of ICBs and can be used in clinical research, including international multicenter studies.

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The aim of this study was to evaluate the accumulation of transition metals in the scalp hair of amyotrophic lateral sclerosis (ALS) patients in the Koza/Kozagawa/Kushimoto (K) area (K-ALS) in the Kii Peninsula, Japan. Metal contents were measured in the unpermed, undyed hair samples of 88 K-residents, 20 controls, 7 K-ALS patients, and 10 sporadic ALS patients using neutron activation analysis at the Research Reactor Institute, Kyoto University. A human hair standard and elemental standards were used as comparative standards. The contents of Zn, Mn, and V were higher, while that of S was lower in K-ALS patients than in the controls. The content of Mn in K-ALS patients negatively correlated with clinical durations. The content of Al was significantly higher in K-residents than in the controls, with 15.9 % of K-residents having high Mn contents over the 75th percentile of the controls. The contents of Zn, Mn, and V were high in the scalp hair of K-ALS patients and correlated with the content of Al. The accumulation of these transition metals may chronically increase metal-induced oxidative stress, which may, in turn, trigger the neuronal degeneration associated with K-ALS.