Clinical Parkinsonism Related Disorders最新文献

Introduction

Methods

Results

Conclusions

Introduction

The present study characterized the degeneration of nigrostriatal dopaminergic neurons in the early stages of parkinsonian disorders using integrative neuroimaging analysis with neuromelanin-sensitive MRI and ¹²³I-FP-CIT dopamine transporter (DAT) SPECT.

Methods

Thirty-one, 30, and 29 patients with progressive supranuclear palsy (PSP), corticobasal syndrome (CBS) with abnormal specific binding ratio (SBR) in either hemisphere (mean ± 2SD), and parkinsonism-predominant multiple system atrophy (MSA-P), respectively, were enrolled. Neuromelanin-related contrast (NRC) in the substantia nigra (NRC_SN) and locus coeruleus (NRC_LC) and the SBR of DAT SPECT were measured. All the patients underwent both examinations simultaneously within five years after symptom onset. After adjusting for interhemispheric asymmetry on neuromelanin-related MRI contrast using the Z-score, linear regression analysis of the NRC_SN and SBR was performed for the most- and least-affected hemispheres, as defined by the interhemispheric differences per variable (SBR, NRC_SN, standardized [SBR + NRC_SN]) in each patient.

Results

Although the variables did not differ significantly between PSP and CBS, a significant correlation was found for CBS in the most-affected hemisphere for all the definitions, including the clinically defined, most-affected hemisphere. No significant correlation was found between the NRC_SN and SBR for any of the definitions in either PSP or MSA-P.

Conclusion

Together with the findings of our previous study of dementia with Lewy bodies (DLB) and Parkinson’s disease (PD), the present findings indicated that neural degeneration in the disorders examined may be categorized by the significance of the NRC_SN-SBR correlation in PD and CBS and its non-significance in DLB, PSP, and MSA-P.

Background

Lower extremity dystonia (LED) is a frequent complication of Parkinson disease (PD). Treatment with botulinum neurotoxinA (BoNTA) over 8 years was retrospectively reviewed.

Cases

14 patients with LED received an average of 3.86 injections (1–8). Mean interval was 40 weeks (median of 25). Average dose was 182 units. Injections were well-tolerated. Using a 6 point scale, there was an average of 3.37 point improvement in disability after each session, with average duration of 28.56 weeks (median 11 weeks). After mean follow-up of 101 weeks, disabling dystonia was not present in 11 of 14 patients.

Conclusions

Botulinum toxin is safe and effective in PD related LED. Good response to the first two injection sessions was significantly associated with greater likelihood of long-term response. Assertive BoNTA dosing may lead to sustained remission of symptoms. As natural history of LED in PD has not been reported, prospective placebo-controlled studies are needed.

Background

Bradyphrenia, best thought of as the mental equivalent of bradykinesia, has been described in several disorders of the brain including Parkinson’s disease and schizophrenia; however, little is known about this phenomenon in Huntington’s Disease (HD).

Objective

The aim of this study was to investigate the presence of bradyphrenia in HD using the Computerized Test of Information Processing (CTiP), an easy to administer and objective task that assesses cognitive processing speed with increasing task complexity.

Methods

This study included 211 participants: Huntington’s Disease Integrated Staging System (HD-ISS) Stage 0 [n = 28], Stage 1 [n = 30], Stage 2 [n = 48] and Stage 3 [n = 48], and healthy controls (HC) [n = 57]. The CTiP incorporates three subtests: Simple Reaction Time (SRT), which assesses baseline motor function; Choice Reaction Time (CRT), with an added decisional component; and Semantic Search Reaction Time (SSRT), with an added conceptual component. SRT scores were subtracted from CRT and SSRT scores to establish a motor-corrected measure of central conduction time, which was used to operationalize bradyphrenia.

Results

HD-ISS and HC within-group reaction times differed significantly when comparing motor-corrected CRT vs SSRT (all ps < 0.0001). Furthermore, the magnitude of these differences increased with HD disease stage (p < 0.0001). An ROC analysis determined that motor-corrected within-subject differences significantly distinguished Stage 2 + 3 from Stage 0 + 1 (AUC = 0.72, p < 0.0001).

Conclusions

We report evidence of bradyphrenia in HD that increases with disease progression. This processing deficit, which can be quantified using the CTiP, has the potential to greatly impact HD daily life and warrants additional research.

Background

Research Question

Methods

Results

Conclusion

Introduction

Progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), and multiple system atrophy (MSA) are rare neurodegenerative diseases associated with rapid decline and require complex symptom management. Caregiving responsibilities significantly increase with progression of these atypical Parkinsonian syndromes, yet care burden in these syndromes has not been researched extensively to date.

Methods

The Zarit Burden Interview (ZBI) was used to assess burden in care partners of patients clinically diagnosed with PSP, CBS, or MSA seen in specialty interdisciplinary clinics at two academic movement disorders centers. Univariable and multivariable regression analyses were performed to evaluate cross-sectional demographic and clinical determinants of care partner burden.

Results

A total of 139 care partners completed the ZBI (59.0% PSP, 28.1% MSA, 12.9% CBS). Cohorts at both medical centers were similar across all variables. Female gender of both patients and care partners was independently associated with higher ZBI scores. Additionally, MSA-Parkinsonian type was significantly associated with lower total care partner burden compared to PSP and CBS.

Conclusion

Several determinants of higher care partner burden in atypical Parkinsonian syndromes were identified, particularly female gender and diagnosis. Healthcare professionals can consider this information when assessing individualized needs of patients and care partners and referring to disease-specific resources. Additionally, this study’s methods and results highlight the potential to further explore interdisciplinary care as a means of comprehensive evaluation and support for those with atypical Parkinsonism.