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Introduction: The diagnosis of diseases known as synucleinopathies, Parkinson's disease (PD), multiple system atrophy (MSA) and Lewy body dementia (DLB), is predominantly based on clinical criteria. However, diagnostic uncertainty may persist until late in the disease process leading to delays in diagnosis and medical mismanagement. Skin biopsy detection of phosphorylated alpha-synuclein (P-SYN) is a sensitive and specific technique that increases diagnostic sensitivity of synucleinopathies, although the clinical utility of this test has not been fully explored. Methods: To determine the role of skin biopsy in the diagnosis of synucleinopathies we performed a retrospective chart review of patients who underwent skin biopsy for detection of P-SYN in the evaluation of neurodegenerative disease at a tertiary care academic institution to investigate the change in diagnosis and medical management based on the results of skin biopsy detection of P-SYN. Results: We included 97 patients suspected to have a synucleinopathy: 54 with PD, 19 with DLB and 24 with MSA. After skin biopsy testing for P-SYN, 78% of patients had a change in their clinical care with 66% having a change in their diagnosis and 55% having a change in their treatment. Changes in diagnosis were most common in patients with parkinsonism with prominent action tremor (93%), lower-extremity predominant parkinsonism (postural instability and gait dysfunction) (90%), and parkinsonism with predominant cognitive dysfunction (76%). Discussion: In patients with suspected synucleinopathies, skin biopsy detection of P-SYN had a high level of clinical utility leading to changes in clinical diagnosis and treatment. [ABSTRACT FROM AUTHOR]

IntroductionThe diagnosis of diseases known as synucleinopathies, Parkinson’s disease (PD), multiple system atrophy (MSA) and Lewy body dementia (DLB), is predominantly based on clinical criteria. However, diagnostic uncertainty may persist until late in the disease process leading to delays in diagnosis and medical mismanagement. Skin biopsy detection of phosphorylated alpha-synuclein (P-SYN) is a sensitive and specific technique that increases diagnostic sensitivity of synucleinopathies, although the clinical utility of this test has not been fully explored.MethodsTo determine the role of skin biopsy in the diagnosis of synucleinopathies we performed a retrospective chart review of patients who underwent skin biopsy for detection of P-SYN in the evaluation of neurodegenerative disease at a tertiary care academic institution to investigate the change in diagnosis and medical management based on the results of skin biopsy detection of P-SYN.ResultsWe included 97 patients suspected to have a synucleinopathy: 54 with PD, 19 with DLB and 24 with MSA. After skin biopsy testing for P-SYN, 78% of patients had a change in their clinical care with 66% having a change in their diagnosis and 55% having a change in their treatment. Changes in diagnosis were most common in patients with parkinsonism with prominent action tremor (93%), lower-extremity predominant parkinsonism (postural instability and gait dysfunction) (90%), and parkinsonism with predominant cognitive dysfunction (76%).DiscussionIn patients with suspected synucleinopathies, skin biopsy detection of P-SYN had a high level of clinical utility leading to changes in clinical diagnosis and treatment.

Background: Metacognition is the ability to monitor and self-assess cognitive performance. It can be impaired in neurodegenerative diseases, with implications for daily function, and the ability of patients to reliably report their symptoms to health professionals. However, metacognition has not been systematically assessed in early-mid stage Parkinson’s disease (PD) and REM sleep behavioral disorder (RBD), a prodrome of PD. Objectives: This study aimed to evaluate metacognitive accuracy and selfconfidence in PD and RBD patients across various cognitive tasks. Methods: We conducted detailed computerized cognitive assessments with 19 cognitive tasks within an established PD and RBD cohort. Participants selfrated their performance post-task. Metacognitive accuracy was calculated by comparing these ratings against objective performance and further analyzed against clinical and mental health factors. Results: PD and RBD patients’ metacognitive accuracy aligned with control subjects. However, they exhibited lower confidence across cognitive domains, reflecting their reduced cognitive performance. A notable inverse correlation was observed between their confidence and MDS-UPDRS I and II scales and HADS anxiety and depression scores. Conclusion: Our findings indicate that patients with early to mid-stage PD and RBD are generally aware of their cognitive status, differing from other neurological disorders. The inverse relationship between patient confidence and symptoms of depression, anxiety, and daily life challenges underscores the impact of emotional and functional difficulties on their self-perception of cognitive abilities. This insight could be significant for understanding how these conditions affect mental health, aiding clinicians in developing more effective patient care strategies. [ABSTRACT FROM AUTHOR]

Isolated REM sleep behavior disorder (iRBD) is an early stage of synucleinopathy with most patients progressing to Parkinson's disease (PD) or related conditions. Quantitative susceptibility mapping (QSM) in PD has identified pathological iron accumulation in the substantia nigra (SN) and variably also in basal ganglia and cortex. Analyzing whole‐brain QSM across iRBD, PD, and healthy controls (HC) may help to ascertain the extent of neurodegeneration in prodromal synucleinopathy. 70 de novo PD patients, 70 iRBD patients, and 60 HCs underwent 3 T MRI. T1 and susceptibility‐weighted images were acquired and processed to space standardized QSM. Voxel‐based analyses of grey matter magnetic susceptibility differences comparing all groups were performed on the whole brain and upper brainstem levels with the statistical threshold set at family‐wise error‐corrected p‐values <.05. Whole‐brain analysis showed increased susceptibility in the bilateral fronto‐parietal cortex of iRBD patients compared to both PD and HC. This was not associated with cortical thinning according to the cortical thickness analysis. Compared to iRBD, PD patients had increased susceptibility in the left amygdala and hippocampal region. Upper brainstem analysis revealed increased susceptibility within the bilateral SN for both PD and iRBD compared to HC; changes were located predominantly in nigrosome 1 in the former and nigrosome 2 in the latter group. In the iRBD group, abnormal dopamine transporter SPECT was associated with increased susceptibility in nigrosome 1. iRBD patients display greater fronto‐parietal cortex involvement than incidental early‐stage PD cohort indicating more widespread subclinical neuropathology. Dopaminergic degeneration in the substantia nigra is paralleled by susceptibility increase, mainly in nigrosome 1. [ABSTRACT FROM AUTHOR]

BackgroundMetacognition is the ability to monitor and self-assess cognitive performance. It can be impaired in neurodegenerative diseases, with implications for daily function, and the ability of patients to reliably report their symptoms to health professionals. However, metacognition has not been systematically assessed in early-mid stage Parkinson’s disease (PD) and REM sleep behavioral disorder (RBD), a prodrome of PD.ObjectivesThis study aimed to evaluate metacognitive accuracy and self-confidence in PD and RBD patients across various cognitive tasks.MethodsWe conducted detailed computerized cognitive assessments with 19 cognitive tasks within an established PD and RBD cohort. Participants self-rated their performance post-task. Metacognitive accuracy was calculated by comparing these ratings against objective performance and further analyzed against clinical and mental health factors.ResultsPD and RBD patients’ metacognitive accuracy aligned with control subjects. However, they exhibited lower confidence across cognitive domains, reflecting their reduced cognitive performance. A notable inverse correlation was observed between their confidence and MDS-UPDRS I and II scales and HADS anxiety and depression scores.ConclusionOur findings indicate that patients with early to mid-stage PD and RBD are generally aware of their cognitive status, differing from other neurological disorders. The inverse relationship between patient confidence and symptoms of depression, anxiety, and daily life challenges underscores the impact of emotional and functional difficulties on their self-perception of cognitive abilities. This insight could be significant for understanding how these conditions affect mental health, aiding clinicians in developing more effective patient care strategies.

Purpose: The purpose of this study was to evaluate the association between clinically possible rapid eye movement (REM) sleep behavioral disorder (pRBD) and orthostatic hypotension (OH) in PD patients, as well as to explore the mechanisms underlying the association. Methods: PD patients (n = 116) were assigned to a group with OH (PD-OH) or without OH (PD-NOH). General demographic and clinical data were collected. A series of scales were used to assess the clinical symptoms in the two groups. Results: A total of 27 patients (23.3%) had OH. The PD-OH group showed significantly higher H-Y staging score and significantly higher frequencies of pRBD, anxiety, depression, and cognitive impairment than the PD-NOH group. Binary logistic regression analysis identified the following factors as independently associated with PD-OH: H-Y staging [odds ratio (OR) 2.565, 95% confidence interval (CI) 1.160–5.673; P = 0.020], RBD (OR 7.680, 95% CI 1.944–30.346; P = 0.004), UPDRS II (OR 1.021, 95% CI 0.980–1.063; P = 0.020), depression (OR 7.601, 95% CI 1.492–38.718; P = 0.015), and cognitive impairment (OR 0.824, 95% CI 0.696–0.976; P = 0.025). Conclusions: Our results suggest that pRBD is an independent risk factor for OH in patients with PD. We speculate that there may be a close relationship between RBD and OH, which requires attention. Early diagnosis of RBD may help predict the appearance of OH in PD patients. [ABSTRACT FROM AUTHOR]

The diagnosis of Parkinson disease (PD) is based on the presence of bradykinesia and either resting tremor or rigidity and there should be no features from the history or examination to suggest an alternative cause of parkinsonism. In addition to the motor manifestations of PD, there is a long list of nonmotor symptoms, several of which occur before motor signs and are considered "prodromal" PD. These are classified as neuropsychiatric, autonomic, sleep, and sensory. There are many medical options for the treatment of PD but levodopa remains the mainstay. Deep brain stimulation and other advanced therapies are also available., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Abstract: Suvorexant is a new insomnia drug, and it is generally safe and well tolerated. Here, we report a rare but potentially important adverse effect of suvorexant in a patient with Parkinson disease., (© 2017 American Academy of Sleep Medicine)