Factors Contributing to the Severity and Laterality of Pisa Syndrome in Parkinson's Disease.

Objective: Pisa syndrome (PS) is a disabling postural deformity in Parkinson's disease (PD). We aimed to elucidate clinical factors determining the severity and laterality of PS in PD. Methods: In 54 PD patients with PS, we measured the clinical factors that are previously known to contribute to the occurrence of PS as follows: asymmetry of motor symptoms for the evaluation of asymmetric basal ganglia dysfunction, the degree and direction of subjective visual vertical (SVV) tilt for the misperception of body verticality, the canal paresis for unilateral peripheral vestibulopathy, and the tonic electromyographic (EMG) hyperactivity of paraspinal muscles for dystonia. Multivariable linear and logistic regression analyses were conducted to identify the clinical factors associated with the degree of truncal tilt, for the quantification of the severity of PS, and PS tilting to the less affected side, respectively. Results: The multivariable linear regression analyses revealed that the larger degree of SVV tilt (ß = 0.29, SE = 0.10, p = 0.005), right-sided SVV tilt (ß = 2.32, SE = 0.82, p = 0.007), and higher Hoehn and Yahr (HY) stage (ß = 4.01, SE = 1.29, p = 0.003) significantly increased the severity of PS. In the multivariable logistic regression analyses, greater asymmetry of motor symptoms [odds ratio (OR) = 2.01, 95% CI = 1.34-3.49] was significantly associated with PS tilting to the less affected side, while right-sided SVV tilt (OR = 0.02, 95% CI = 0.001-0.21), unilateral canal paresis (OR = 0.06, 95% CI = 0.003-0.79), and higher HY stage (OR = 0.04, 95% CI = 0.002-0.46) were associated with PS tilting to the more affected side. Conclusion: Misperception of verticality, asymmetric basal ganglia dysfunction, unilateral peripheral vestibulopathy, and motor disability are the clinical factors associated with the severity and laterality of PS in patients with PD.

Pisa Syndrome in Parkinson's Disease: Pathogenic Roles of Verticality Perception Deficits.

We elucidated whether verticality misperception is associated with the generation of Pisa syndrome (PS) in patients with Parkinson's disease (PD). To examine the heterogenous influence of verticality perception, we also identified the characteristics distinguishing between PD patients with PS who tilted toward the deviation of perceived verticality and those who did not. Subjective visual vertical (SVV) testing was performed in 54 PD patients with PS and 36 without PS to measure verticality perception. Other potential risk factors for PS were evaluated by assessing the asymmetry of motor symptoms, EMG activities of paraspinal muscles, bithermal caloric tests, back pain history, and Berg Balance Scale. Abnormal SVV (odds ratio (OR) 18.40, p = 0.006), postural imbalance (OR 0.71, p = 0.046), and unilateral EMG hyperactivity of paraspinal muscles (OR 39.62, p = 0.027) were independent contributors to PS. In subgroup analysis, EMG hyperactivity of paraspinal muscles contralateral to the leaning side and postural imbalance were associated with PD patients with PS who tilted toward the SVV deviation, whereas back pain was more frequent in those who did not. Verticality misperception is a potent risk factor for PS in PD and contributes differentially to PS depending on the congruence between its direction and PS direction, indicating distinct pathogenic roles.

The composite autonomic symptom scale 31 is a useful screening tool for patients with Parkinsonism.

Differentiation of multiple system atrophy with predominant parkinsonism (MSA-P) and Parkinson's disease (PD) is important, but an effective tool for differentiation has not been identified. We investigated the efficacy of the composite autonomic symptom scale 31 (COMPASS 31) questionnaire as a tool for evaluating autonomic function in parkinsonism patients. In this study, we enrolled drug-naïve patients with MSA-P and PD, and administered the COMPASS-31 and an objective autonomic dysfunction test (AFT). Demographic and clinical data, including parkinsonism and autonomic dysfunction, were compared between the two groups. Additionally, we determined the optimal COMPASS 31 cut-off score to differentiate MSA-P from PD for use as a screening tool. In this study, 27 MSA-P patients and 41 PD patients were recruited. The total COMPASS 31 score was well correlated with the objective AFT results. When we compared the COMPASS 31 score between the two groups, MSA-P patients showed higher total scores and sub-scores in the orthostatic intolerance, gastrointestinal, and bladder domains compared with PD patients. Similarly, MSA-P patients had more abnormalities in expiration to inspiration ratio, Valsalva ratio and pressure recovery time than PD patients in objective AFT. With 13.25 as the cut-off score for diagnosis of MSA-P, the total COMPASS-31 score demonstrated high sensitivity (92.6%) and moderate specificity (51.2%) with an area under the curve of 0.765. Based on our results, the COMPASS 31 is an effective tool for evaluation of autonomic function in patients with parkinsonism. The COMPASS-31 could be used as a sensitive and convenient screening tool, especially for the differentiation between MSA-P and PD.