scholarly journals The Clinical Spectrum of Young Onset Dementia Points to Its Stochastic Origins

2021 ◽  
pp. 1-17
Author(s):  
Peter K. Panegyres
Keyword(s):  
Small Vessel Disease ◽  
Age Of Onset ◽  
Gene Mutations ◽  
Lewy Body Disease ◽  
Corticobasal Degeneration ◽  
Young Onset ◽  
Clinical Presentations ◽  
Eeg Data ◽  
Common Causes ◽  
Young Onset Dementia

Background: Dementia is a major global health problem and the search for improved therapies is ongoing. The study of young onset dementia (YOD)—with onset prior to 65 years—represents a challenge owing to the variety of clinical presentations, pathology, and gene mutations. The advantage of the investigation of YOD is the lack of comorbidities that complicate the clinical picture in older adults. Here we explore the origins of YOD. Objective: To define the clinical diversity of YOD in terms of its demography, range of presentations, neurological examination findings, comorbidities, medical history, cognitive findings, imaging abnormalities both structural and functional, electroencephagraphic (EEG) data, neuropathology, and genetics. Methods: A prospective 20-year study of 240 community-based patients referred to specialty neurology clinics established to elucidate the nature of YOD. Results: Alzheimer’s disease (AD; n = 139) and behavioral variant frontotemporal (bvFTD; n = 58) were the most common causes with a mean age of onset of 56.5 years for AD (±1 SD 5.45) and 57.1 years for bvFTD (±1 SD 5.66). Neuropathology showed a variety of diagnoses from multiple sclerosis, Lewy body disease, FTD-MND, TDP-43 proteinopathy, adult-onset leukoencephalopathy with axonal steroids and pigmented glia, corticobasal degeneration, unexplained small vessel disease, and autoimmune T-cell encephalitis. Non-amnestic forms of AD and alternative forms of FTD were discovered. Mutations were only found in 11 subjects (11/240 = 4.6%). APOE genotyping was not divergent between the two populations. Conclusion: There are multiple kinds of YOD, and most are sporadic. These observations point to their stochastic origins.

Young-onset dementia and MRI changes in a patient with subclinical liver cirrhosis due to chronic hepatitis C

2011 ◽  
Vol 2 (4) ◽  
Author(s):  
Marina Boban ◽  
Branko Malojčić
Keyword(s):  
Chronic Hepatitis ◽  
Hepatic Encephalopathy ◽  
Hepatitis C ◽  
Chronic Hepatitis C ◽  
Wide Spectrum ◽  
Decompensated Liver Disease ◽  
Young Onset ◽  
Clinical Presentations ◽  
Young Onset Dementia ◽  
Mri Changes

AbstractYoung-onset dementia (before age of 65) is relatively infrequent and presents a challenge in everyday neurological practice due to wide spectrum of clinical presentations and diversity of underlying etiology. When cognitive deficits are accompanied with liver dysfunction different etiologies should be considered. We present a case report of a young patient with subclinical decompensated liver disease due to underlying chronic hepatitis C, presented with the mildest form of hepatic encephalopathy spectrum, called minimal (subclinical) hepatic encephalopathy and characteristic MRI changes.

Small Vessel Disease and Associations with Cerebrospinal Fluid Amyloid, Tau, and Neurodegeneration (ATN) Biomarkers and Cognition in Young Onset Dementia

2020 ◽  
Vol 77 (3) ◽  
pp. 1305-1314
Author(s):  
Chathuri Yatawara ◽  
Kok Pin Ng ◽  
Anne Cristine Guevarra ◽  
Benjamin Wong ◽  
TingTing Yong ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Cognitive Impairment ◽  
Grey Matter ◽  
Small Vessel Disease ◽  
Small Vessel ◽  
Memory Clinic ◽  
Young Onset ◽  
Vessel Disease ◽  
Young Onset Dementia ◽  
Global Cognition

Background: Small vessel disease (SVD) and Alzheimer’s disease (AD) frequently coexist; however, it remains unclear how they collectively affect cognition. Objective: We investigated associations between SVD and AD biomarkers, namely amyloid, tau, and neurodegeneration (ATN) in young onset dementia (YOD) and explored how SVD and ATN interact to affect cognition. Methods: 80 YOD individuals were recruited from a memory clinic. SVD burden (SVD+) was operationalized as a score >1 on the Staals scale and ATN was measured using cerebrospinal fluid (CSF). Results: SVD+ was associated with lower CSF Aβ1–42 (B = –0.20, 95% CI: –0.32 to –0.08) and greater neurodegeneration, indexed as hippocampal atrophy (B = –0.24, 95% CI: –0.40 to –0.04). SVD+ was not associated with tau. Cognitive impairment was associated with CSF Aβ1–42 (B = –0.35, 95% CI: –0.55 to –0.18) but not SVD. Rather, SVD was indirectly associated with cognition via reduced CSF Aβ1–42, specifically with global cognition (B = –0.03, 95% CI: –0.09 to –0.01) and memory (B = 0.08, 95% CI: –.01 to .21). SVD was indirectly associated with cognition via increased neurodegeneration in grey matter (Global cognition: B = –0.06, 95% CI: –0.17 to –0.03; Memory: B = 0.05, 95% CI: 0.01 to 0.18) and the hippocampus (Global cognition: B = –0.05, 95% CI: –0.11 to –0.01; Memory: B = 0.06, 95% CI: 0.01 to 0.17). Conclusion: In YOD, SVD burden was associated with AD pathology, namely CSF Aβ1–42. SVD indirectly contributed to cognitive impairment via reducing CSF Aβ1–42 and increasing neurodegeneration.

scholarly journals Autoantibody profiles and clinical association in Thai patients with autoimmune retinopathy

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Aulia Rahmi Pawestri ◽  
Niracha Arjkongharn ◽  
Ragkit Suvannaboon ◽  
Aekkachai Tuekprakhon ◽  
Vichien Srimuninnimit ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Age Of Onset ◽  
Male Gender ◽  
Blurred Vision ◽  
Autoimmune Retinopathy ◽  
Immune Mediated ◽  
Underlying Malignancy ◽  
Clinical Presentations ◽  
Treatment Plans ◽  
Autoantibody Profiles

AbstractAutoimmune retinopathy (AIR) is a rare immune-mediated inflammation of the retina. The autoantibodies against retinal proteins and glycolytic enzymes were reported to be involved in the pathogenesis. This retrospective cohort study assessed the antiretinal autoantibody profiles and their association with clinical outcomes of AIR patients in Thailand. We included 44 patients, 75% were females, with the overall median age of onset of 48 (17–74, IQR 40–55.5) years. Common clinical presentations were nyctalopia (65.9%), blurred vision (52.3%), constricted visual field (43.2%), and nonrecordable electroretinography (65.9%). Underlying malignancy and autoimmune diseases were found in 2 and 12 female patients, respectively. We found 41 autoantibodies, with anti-α-enolase (65.9%) showing the highest prevalence, followed by anti-CAII (43.2%), anti-aldolase (40.9%), and anti-GAPDH (36.4%). Anti-aldolase was associated with male gender (P = 0.012, OR 7.11, 95% CI 1.54–32.91). Anti-CAII showed significant association with age of onset (P = 0.025, 95% CI − 17.28 to − 1.24), while anti-α-enolase (P = 0.002, OR 4.37, 95% CI 1.83–10.37) and anti-GAPDH (P = 0.001, OR 1.87, 95% CI 1.32–2.64) were significantly associated with nonrecordable electroretinography. Association between the antibody profiles and clinical outcomes may be used to direct and adjust the treatment plans and provide insights in the pathogenesis of AIR.

scholarly journals Driving Cessation in Patients Attending a Young-Onset Dementia Clinic: A Retrospective Cohort Study

2018 ◽  
Vol 8 (1) ◽  
pp. 190-198 ◽  
Author(s):  
Latha Velayudhan ◽  
Sarah Baillon ◽  
Gabriela Urbaskova ◽  
Laura  McCulloch ◽  
Samuel  Tromans ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Female Gender ◽  
University Teaching ◽  
Mediation Effect ◽  
Health Concern ◽  
Driving Cessation ◽  
Factors Affecting ◽  
Young Onset ◽  
People With Dementia ◽  
Young Onset Dementia

Background: Although driving by persons with dementia is an important public health concern, little is known about driving cessation in younger people with dementia. We aimed to determine the prevalence and factors affecting driving cessation in individuals with and without dementia aged under 65 years attending a memory clinic in a European setting. Methods: Subjects were consecutive patients assessed at a specialist memory service at a university teaching hospital between 2000 and 2010. The data collected included demographic, clinical, standardized cognitive assessments as well as information on driving. Dementia diagnosis was made using ICD-10 criteria. Results: Of the 225 people who were or had been drivers, 32/79 (41%) with young-onset dementia (YOD) stopped driving compared to 25/146 (17%) patients who had cognitive impairment due to other causes. Women were more likely to cease driving and voluntarily than men (p < 0.001). Diagnosis of YOD was associated with driving cessation (1.193, 95% CI 0.570–1.815, p ≤ 0.001), and was mediated by impairment in praxis with the highest indirect mediation effect (0.754, 95% CI 0.183–1.401, p = 0.009). Conclusions: YOD diagnosis, female gender, and impairment in praxis have a higher probability for driving cessation in those under 65 years of age with cognitive impairment.

scholarly journals ‘This is Killing me Inside’: The Impact of Having a Parent with Young-Onset Dementia

Dementia ◽  
2017 ◽  
Vol 18 (3) ◽  
pp. 1089-1107 ◽  
Author(s):  
Helen J Aslett ◽  
Jaci C Huws ◽  
Robert T Woods ◽  
Joanne Kelly-Rhind
Keyword(s):  
Interpretative Phenomenological Analysis ◽  
Service Providers ◽  
Phenomenological Analysis ◽  
Parent Support ◽  
Young Onset ◽  
Roles And Responsibilities ◽  
Affected Parent ◽  
Young Onset Dementia ◽  
The Impact ◽  
Depth Interviews

This study explored the experience of young adults having a parent with young-onset dementia. In-depth interviews were undertaken with five participants aged between 23 and 36 years of age and these were analysed using interpretative phenomenological analysis. Participants were found to experience a number of stresses in relation to their parent’s illness, many of which were linked to loss and guilt. Five main themes were identified related to relationship changes, shifts in roles and responsibilities, support for the non-affected parent, support for self and the impact of living with their own potential risk of dementia. These findings are discussed in relation to the existing literature and suggest that individuals with a parent with young-onset dementia have needs which service providers should consider in the wider context of young-onset dementia care.

P2-207: Prevalence of Family History of Dementia in a Specialist Young-Onset Dementia Clinic Cohort

2016 ◽  
Vol 12 ◽  
pp. P700-P700
Author(s):  
Yang-Lin Ting ◽  
Levinia Lim ◽  
Nagaendran Kandiah ◽  
Adeline Su Lyn Ng
Keyword(s):  
Family History ◽  
Young Onset ◽  
History Of ◽  
Young Onset Dementia

The experiences and needs of children living with a parent with young onset dementia: results from the NeedYD study

2013 ◽  
Vol 26 (12) ◽  
pp. 2001-2010 ◽  
Author(s):  
Joany K. Millenaar ◽  
Deliane van Vliet ◽  
Christian Bakker ◽  
Myrra J. F. J. Vernooij-Dassen ◽  
Raymond T. C. M. Koopmans ◽  
...  
Keyword(s):  
Coping Styles ◽  
Specific Information ◽  
Structured Interviews ◽  
Specific Knowledge ◽  
Young Onset ◽  
Care And Support ◽  
Young Parent ◽  
Young Onset Dementia ◽  
The Impact ◽  
Family Centered

ABSTRACTBackground:Children of patients with young onset dementia (YOD) who are confronted with a parent who has a progressive disease, often assist in caregiving tasks, which may have a great impact on their lives. The objective of the present study is to explore the experiences of children living with a young parent with dementia with a specific focus on the children's needs.Methods:Semi-structured interviews with 14 adolescent children between the ages of 15 and 27 years of patients with YOD were analyzed using inductive content analysis. Themes were identified based on the established codes.Results:The emerging categories were divided into three themes that demonstrated the impact of dementia on daily life, different ways of coping with the disease, and children's need for care and support. The children had difficulties managing all of the responsibilities and showed concerns about their future. To deal with these problems, they demonstrated various coping styles, such as avoidant or adaptive coping. Although most children were initially reluctant to seek professional care, several of them expressed the need for practical guidance to address the changing behavior of their parent. The children felt more comfortable talking to someone who was familiar with their situation and who had specific knowledge of YOD and the available services.Conclusion:In addition to practical information, more accessible and specific information about the diagnosis and the course of YOD is needed to provide a better understanding of the disease for the children. These findings underline the need for a personal, family-centered approach.

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