Neurodegenerative Diseases最新文献

Objective: This retrospective study analyzed the clinical characteristics and prognosis of the elderly amyotrophic lateral sclerosis (ALS) population in a large sample.

Methods: The study included 1,005 patients with sporadic ALS admitted to Chinese PLA General Hospital between March 2011 and March 2021. We stratified the ALS patients into young and old groups using 2 cutoffs for the age at disease onset (≥65 or ≥70 years old) and compared their demographic, clinical, and survival data.

Results: The mean onset age of all patients was 52.79 ± 10.55 years, with 123 (12.24%) having a disease onset ≥65 years and 44 (4.38%) having an onset ≥70 years. There were 624 (62.1%) male patients. More bulbar-onset cases were in the late-onset group (p = 0.001). The sex distribution, time from onset to diagnosis, and the time of symptom spread from spinal or bulbar localization to a generalized localization did not differ between groups. Late-onset patients progressed more rapidly and had a significantly shorter survival.

Conclusions: Chinese ALS patients have an earlier age at onset and a relatively smaller proportion of old onset than European and Japanese patients. Elderly patients are more likely to have bulbar onset, which is related to rapid progression and a shorter survival.

Background: Many factors affect sexuality in the elderly such as dementia which is a common cause of inappropriate sexual behaviors. These behavioral disturbances are distressing, disruptive, and impair the care of the patient.

Summary: The onset of dementia does not erase sexuality. Sexual expression can be an important aspect of well-being for older adults with dementia. This study gives a general overview about the relationship between sexuality and cognitive impairment. It starts with a general discussion of sexual aspects in the elderly. This is followed by research studies in this field including effects of dementia on sexual life, sexuality issues related to cognitive decline, inappropriate sexual behaviors in dementia patients, and sexuality in healthcare institutions. We discuss also ethical aspects in relation with sexuality and dementia. Finally, we show different approaches to treat inappropriate sexual behaviors.

Key messages: The discussion of sexuality in dementia raises many medical and ethical concerns. Inappropriate sexual behaviors are estimated to occur in about 7%-25% of demented patients. The question is how to address such a delicate subject and discuss it in an easy way without making the patient feel humiliated or mistreated. This narrative review reveals sexual problems and difficult questions encountered in daily practice with patients suffering from cognitive impairment.

Objectives: In the present study, inflammatory factors, including interleukin (IL) and tumor necrosis factor-α (TNF-α) in the peripheral blood of patients with sporadic amyotrophic lateral sclerosis (sALS), were evaluated, and the issue of whether these variables were associated with the progression and severity of the disease examined.

Methods: Data on inflammatory factors, including IL-1, IL-2, IL-6, IL-8, IL-10, and TNF-α, were retrospectively collected from 248 sALS patients admitted to the Chinese PLA General Hospital between March 2018 and March 2021. The relationships between the variables and clinical features, including gender, age at onset, site of onset, time from onset to hospital admission, ALS functional rating scale score, and diagnostic category were analyzed.

Results: IL-1, IL-2, IL-6, IL-8, IL-10, and TNF-α levels were elevated in 43.75%, 7.04%, 16.42%, 25.35%, 1.41%, and 50.72% of ALS patients, respectively, compared with the normal value range. IL-2 and IL-6 levels were inversely associated with the ALS functional rating scale score (r = -0.280, p = 0.004 and r = -0.198, p = 0.048).

Conclusion: Elevated levels of inflammatory cytokines support the hypothesis of an inflammatory response in ALS, and IL-2 and IL-6 may be used as an inflammation-related biomarker for disease severity.

Objective: We aimed to investigate costly punishment in patients with Huntington's disease (HD).

Background: HD is an autosomal dominant neurodegenerative disease with motor, cognitive, and psychiatric symptoms. As neuropsychiatric abnormalities often precede motor symptoms, we wanted to assess whether costly punishment is part of the neuropsychological profile of patients with HD.

Methods: A total of 40 non-demented subjects were prospectively enrolled in this study with a between-subject design comparing manifest HD patients (n = 18) to healthy controls (HC; n = 22). All participants performed 8 rounds of a costly punishment task, in which money was shared unevenly in 5 rounds or in a fair manner in the remaining 3 rounds. Participants then had to decide whether they wanted to punish the trustee. Furthermore, all participants underwent neuropsychological background tasks.

Results: HD patients performed worse in the neuropsychological background tests compared to HC (all p values <0.05). Moreover, HD patients punished more often in fair (Wald χ2 = 5.03, p = 0.025) but not in unfair rounds (Wald χ2 = 1.63, p = 0.202).

Conclusions: Our results demonstrate increased costly punishment during fair conditions in HD patients. Whether this behaviour is due to a lack of recognition of social norms, an impairment in top-down inhibition, or an effect of antidopaminergic medication remains unclear.

Background: Coronavirus disease 2019 (COVID-19), the far-reaching pandemic, has infected approximately 185 million of the world's population to date. After infection, certain groups, including older adults, men, and people of color, are more likely to have adverse medical outcomes. COVID-19 can affect multiple organ systems, even among asymptomatic/mild severity individuals, with progressively worse damage for those with higher severity infections.

Summary: The COVID-19 virus, severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), primarily attaches to cells through the angiotensin-converting enzyme 2 (ACE2) receptor, a universal receptor present in most major organ systems. As SARS-CoV-2 binds to the ACE2 receptor, its bioavailability becomes limited, thus disrupting homeostatic organ function and inducing an injury cascade. Organ damage can then arise from multiple sources including direct cellular infection, overactive detrimental systemic immune response, and ischemia/hypoxia through thromboembolisms or disruption of perfusion. In the brain, SARS-CoV-2 has neuroinvasive and neurotropic characteristics with acute and chronic neurovirulent potential. In the cardiovascular system, COVID-19 can induce myocardial and systemic vascular damage along with thrombosis. Other organ systems such as the lungs, kidney, and liver are all at risk for infection damage. Key Messages: Our hypothesis is that each injury consequence has the independent potential to contribute to long-term cognitive deficits with the possibility of progressing to or worsening pre-existing dementia. Already, reports from recovered COVID-19 patients indicate that cognitive alterations and long-term symptoms are prevalent. This critical review highlights the injury pathways possible through SARS-CoV-2 infection that have the potential to increase and contribute to cognitive impairment and dementia.

Background: Excessive daytime sleepiness (EDS) in Parkinson's disease (PD) may occur because of dysfunction on the brain areas in controlling wakefulness; however, the pathophysiology of EDS in PD has not been completely clarified. The Pb component of a middle-latency auditory evoked response (MLR) is generated from the cholinergic ascending reticular activating system (ARAS) projecting to the auditory cortex via the thalamus. We examined the association between EDS and the Pb component in patients with PD.

Methods: Participants were 38 patients with nondemented PD and 18 age-matched controls. EDS was evaluated using the Japanese version of the Epworth Sleepiness Scale (JESS). PD patients were classified into the high sleepiness (HS) group and the low sleepiness (LS) group by the score of JESS. MLRs were recorded from the scalp with each earlobe as a reference under presentation of 1-Hz and 65- to 90-dB click sounds.

Results: There was no difference in age, duration, and motor function between the HS PD and the LS PD groups. Peak latencies of Pb were not different between PD group and controls; however, Pb amplitudes were significantly increased in the HS PD group compared with the LS PD group and controls.

Conclusion: One of the mechanisms of EDS in PD was suggested to be dysregulation of cholinergic neurons from the ARAS projecting to cortical cholinergic neurons.

Background: In Parkinson's disease (PD), verb-naming tasks (VNTs) have been proposed as superior to noun-naming ones in detecting language deficits, although such a hypothesis is not supported at a statistical level.

Objectives: The main aim of this study was to provide diagnostic accuracy evidence for a VNT and noun-naming task (NNT) in detecting cognitive impairment (CI) in PD patients.

Method: Thirty-three consecutive PD patients were subdivided into participants with (PD-CI; N = 12) or without CI (cognitively unimpaired, PD-CU; N = 21), based on a raw score ≤25 or >25 on the Mini-Mental State Examination, respectively. The NNT and VNT by Neuropsychologia [2006 Jan;44(1):73-89] were administered. Diagnostic accuracy of the NNT and VNT was assessed through receiver-operating characteristics analyses by comparing PD-CU to PD-CI patients. At the optimal cut-off, sensitivity, specificity, positive and negative predictive values (PPV, NPV), and likelihood ratios (LR+, LR-) were separately tested for the NNT and VNT against PD-CU versus PD-CI classification.

Results: Diagnostic accuracy was higher for the NNT (AUC = 0.85; p = 0.001) versus the VNT (AUC = 0.68; p = 0.092). Consistently, the NNT yielded higher sensitivity, specificity, and post-test features than the VNT (NNT: sensitivity = 0.75, specificity = 0.81, PPV = 0.69, NPV = 0.85, LR+ = 3.94, LR- = 0.31; VNT: sensitivity = 0.67, specificity = 0.67, PPV = 0.53, NPV = 0.78, LR+ = 2, LR- = 0.5).

Conclusions: In accordance with the Movement Disorders Society guidelines, NNTs are diagnostically sound psychometric instruments to discriminate PD patients with versus without CI. However, these findings need replication by (1) employing a gold standard different from the Mini-Mental State Examination, which does not capture the full range of CI in this population and (2) subdividing PD patients into those with mild CI and dementia.