Progressive Supranuclear Palsy: Neuropsychopathological, Therapeutical and Bioethical Aspects

2018 ◽  
Vol 15 (10) ◽  
pp. 959-963 ◽  
Author(s):  
Ignazio Vecchio ◽  
Cristina Tornali ◽  
Giulia Malaguarnera ◽  
Nicola Luigi Bragazzi ◽  
Michele Malaguarnera

The progressive supranuclear palsy is a progressive neurodegenerative disease characterized by Parkinsonism, oculomotor abnormalities, early postural instability and cognitive impairment. Neurodegeneration in PSP is associated with tau protein, but the mechanisms by which tau abnormalities lead to cell dysfunction and death are not well understood. Neuro-behavioural problems related to the fear and loss of autonomy can determinate many bioethical implications. Careful planning involving patients’ families, academic and industry researchers were necessary to ensure improvement in quality of life.

2006 ◽  
Author(s):  
A. Schrag ◽  
C. Selai ◽  
N. Quinn ◽  
A. Lees ◽  
I. Litvan ◽  
...  

2021 ◽  
pp. 030157422110234
Author(s):  
Mohamed Iqbal J

Huntington’s disease is a progressive neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms. Dystonia of muscles is a characteristic feature of this condition. A case of Huntington’s disease, with orofacial dystonia, leading to severe uncontrolled biting of the lips, was referred by the Department of Neurology. Deep traumatic ulcerations were found in both upper and lower lips. A simple Essix retainer was fabricated and inserted, which acted as a barrier for the teeth from injuring the lips. The ulcers showed complete resolution in 3 to 4 weeks. The vacuum-formed retainers resulted in a good fit and resisted removal by the uncontrolled contortions of the orofacial muscles. The Essix retainer can be effectively used in improving the quality of life of patients, with Huntington’s disease, having such dystonia-related injuries to lips.


2021 ◽  
pp. 1-12
Author(s):  
Sergiu Albu ◽  
Nicolas Rivas Zozaya ◽  
Narda Murillo ◽  
Alberto Garcia-Molina ◽  
Cristian Andres Figueroa Chacón ◽  
...  

BACKGROUND: Coronavirus disease 2019 (COVID-19) patients present long-lasting physical and neuropsychological impairment, which may require rehabilitation. OBJECTIVES: The current cross-sectional study characterizes post COVID-19 sequelae and persistent symptoms in patients in an outpatient rehabilitation program. METHODS: Thirty patients [16 post-ICU and 14 non-ICU; median age = 54(43.8–62) years; 19 men] presenting sequelae and/or persistent symptoms (>3 months after acute COVID-19) were selected of 41 patients referred for neurorehabilitation. Patients underwent physical, neuropsychological and respiratory evaluation and assessment of impact of fatigue and quality of life. RESULTS: The main reasons for referral to rehabilitation were: fatigue (86.6%), dyspnea (66.7%), subjective cognitive impairment (46.7%) and neurological sequelae (33.3%). Post-ICU patient presented sequelae of critical illness myopathy and polyneuropathy, stroke and encephalopathy and lower forced vital capacity compared to non-ICU patients. Cognitive impairment was found in 63.3% of patients, with a similar profile in both sub-groups. Increased physical fatigue, anxiety and depression and low quality of life were prevalent irrespective of acute COVID-19 severity. CONCLUSIONS: The variability of post COVID-19 physical and neuropsychological impairment requires a complex screening process both in ICU and non-ICU patients. The high impact of persistent symptoms on daily life activities and quality of life, regardless of acute infection severity, indicate need for rehabilitation.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Pei Ern Mary Ng ◽  
Sean Olivia Nicholas ◽  
Shiou Liang Wee ◽  
Teng Yan Yau ◽  
Alvin Chan ◽  
...  

AbstractTo address the paucity of research investigating the implementation of multi-domain dementia prevention interventions, we implemented and evaluated a 24-week, bi-weekly multi-domain program for older adults at risk of cognitive impairment at neighborhood senior centres (SCs). It comprised dual-task exercises, cognitive training, and mobile application-based nutritional guidance. An RCT design informed by the Reach, Effectiveness, Adoption, Implementation, Maintenance framework was adopted. Outcome measures include cognition, quality of life, blood parameters, and physical performance. Implementation was evaluated through questionnaires administered to participants, implementers, SC managers, attendance lists, and observations. The program reached almost 50% of eligible participants, had an attrition rate of 22%, and was adopted by 8.7% of the SCs approached. It was implemented as intended; only the nutritional component was re-designed due to participants’ unfamiliarity with the mobile application. While there were no between-group differences in cognition, quality of life, and blood parameters, quality of life reduced in the control group and physical function improved in the intervention group after 24 weeks. The program was well-received by participants and SCs. Our findings show that a multi-domain program for at-risk older adults has benefits and can be implemented through neighborhood SCs. Areas of improvement are discussed.Trial registration: ClinicalTrials.gov NCT04440969 retrospectively registered on 22 June 2020.


2021 ◽  
pp. 1-8
Author(s):  
Alexander Pantelyat ◽  
Lenora Higginbotham ◽  
Liana Rosenthal ◽  
Diane Lanham ◽  
Vanessa Nesspor ◽  
...  

<b><i>Introduction:</i></b> There is growing interest in using patient-reported outcomes as end points in clinical trials, such as the progressive supranuclear palsy quality of life (PSP-QoL) scale. However, this tool has not been widely validated and its correlation with validated motor scales has not been explored. To evaluate the potential utility of using PSP-QoL as an outcome, it is important to examine its relationship with a standard scale used to evaluate neurologic parameters, such as the PSP Rating Scale. <b><i>Methods:</i></b> PSP-QoL and PSP Rating Scale scores were gathered from 60 clinically diagnosed PSP patients, including patients with Richardson syndrome PSP (PSP-RS, <i>n</i> = 43) and those with non-RS PSP variants (<i>n</i> = 17). Linear regression analysis adjusted for age, sex, and disease duration was used to evaluate the cross-sectional relationship between the total and subscale scores of the 2 instruments. <b><i>Results:</i></b> Among 60 PSP patients, there was a significant correlation between total PSP-QoL and PSP Rating Scale scores. The physical and mentation subscales of each instrument also demonstrated significant correlations. Comparisons among PSP subtypes indicated that worsening PSP-QoL Total and Physical subscale scores correlated with worsening PSP Rating Scale gait subscale scores more strongly for the non-RS PSP variants than for PSP-RS. <b><i>Discussion:</i></b> There is a significant association between the total scores and many of the subscale scores of the PSP-QoL and the PSP Rating Scale. Additionally, the relationship between these measures may differ for PSP-RS and non-RS variants. These findings suggest that the PSP-QoL may be useful in clinical trials as a patient-reported outcome measure. Large prospective multicenter studies utilizing the PSP-QoL are necessary to examine its relationship to disease evolution and changes in the PSP Rating Scale.


2018 ◽  
Vol 12 (3) ◽  
pp. 220-232 ◽  
Author(s):  
Elizabeth Karol ◽  
Dianne Smith

Aim:The objective of this article is to identify and analyze what is known about characteristics in and around the home that support well-being for those with cognitive impairment. This could provide direction for designers of homes in general, but specifically for designers trying to meet the needs of people with cognitive impairment.Background:It has been established that there is a relationship between psychological well-being and a person’s environment. Research also shows that particular design aspects can reduce the impact of cognitive impairment. However, there is limited design expertise in the Australian housing market to create supportive spaces which will help to reduce the impact of the disability for those with cognitive impairment.Method:A literature review was carried out to determine the extent and details of what is known about the relationship of home design and its impact on emotional, psychological, or social well-being for people with cognitive impairment.Conclusions:The study indicates that researchers in various disciplines understand that pragmatic design inputs such as thermal comfort and adequate lighting are important for people with cognitive impairment. In addition, some researchers have shown or surmise that there are other “intangible” designer-controlled elements that have beneficial impacts on people with cognitive impairment. Details of these intangible elements are sparse, and how much they might improve the quality of life for a person with cognitive impairment is not well understood. Further research is required to meet a growing need.


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