Objective: To estimate the prevalence of frontotemporal dementia (FTD) and other degenerative early-onset dementias in a geographically defined population. Background: Early-onset dementia (at age Methods: Case ascertainment was by review of case records of three specialist clinic databases and inpatient admissions at a university hospital in Cambridge, United Kingdom, for patients with dementia who were Results: A total of 108 patients (66 men and 42 women) with dementia with onset before they were 65 years of age were identified, of whom 60 were Conclusions: Frontotemporal dementia is a more common cause of early-onset dementia than previously recognized and appears to be more common in men.

The prevalence of frontotemporal dementia

For more than a decade, researchers have refined criteria for the diagnosis of dementia with Lewy bodies (DLB) and at the same time have recognized that cognitive impairment and dementia occur commonly in patients with Parkinson disease (PD). This article addresses the relationship between DLB, PD, and PD with dementia (PDD). The authors agreed to endorse "Lewy body disorders" as the umbrella term for PD, PDD, and DLB, to promote the continued practical use of these three clinical terms, and to encourage efforts at drug discovery that target the mechanisms of neurodegeneration shared by these disorders of alpha-synuclein metabolism. We concluded that the differing temporal sequence of symptoms and clinical features of PDD and DLB justify distinguishing these disorders. However, a single Lewy body disorder model was deemed more useful for studying disease pathogenesis because abnormal neuronal alpha-synuclein inclusions are the defining pathologic process common to both PDD and DLB. There was consensus that improved understanding of the pathobiology of alpha-synuclein should be a major focus of efforts to develop new disease-modifying therapies for these disorders. The group agreed on four important priorities: 1) continued communication between experts who specialize in PDD or DLB; 2) initiation of prospective validation studies with autopsy confirmation of DLB and PDD; 3) development of practical biomarkers for alpha-synuclein pathologies; 4) accelerated efforts to find more effective treatments for these diseases.

DLB and PDD boundary issues: Diagnosis, treatment, molecular pathology, and biomarkers

Frontotemporal lobar degeneration (FTLD) is characterized clinically by progressive changes in social, behavioral, and/or language function.1-3 In the frontal or social/dysexecutive variant of FTLD, there is an early change in social comportment and personality often associated with disinhibition, apathy, and lack of insight. In contrast, the aphasic variant of FTLD is further classified as either progressive nonfluent aphasia, characterized by effortful speech and phonemic errors,4 or semantic dementia, manifested by severe problems with naming and understanding word and object meaning.5 In both the social/dysexecutive and aphasic variants, there is relative preservation of memory, especially in early stages. In addition, some patients with FTLD manifest a motor disorder such as parkinsonism or motor neuron disease (MND).6,7

Multiple neuropathological abnormalities are associated with FTLD,3,8 and this pathological heterogeneity is reflected in a recent classification scheme from an frontotemporal dementia (FTD) work group that was the first to incorporate both immunohistochemical and biochemical data in the diagnostic algorithm (Fig 1).3 Two broad pathological subdivisions of FTD are recognized: brains with tau-positive pathology (tauopathies), and brains free of tau pathology (see Fig 1). The tauopathies are subdivided into several distinct entities, including Pick’s disease (PiD), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), argyrophilic grain disease, tangle-predominant senile dementia, and patients with MAPT gene mutations collectively referred to as frontotemporal dementia with parkinsonism linked to chromosome 17, or FTDP-17. In contrast, brains from the remaining patients lack tau-positive inclusions and are further categorized by the presence or absence of ubiquitin-positive inclusions, designated frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U)9-11 and dementia lacking distinctive histopathology (DLDH), respectively.12-14 A new pathological entity recently was described in FTLD-U patients, designated neuronal intermediate filament inclusion disease, that is characterized by neuronal intermediate filament-positive aggregates in neurons of affected brain regions.15-18 It also should be emphasized that both Alzheimer’s disease (AD) pathology and cortical Lewy bodies formed by filamentous α-synuclein aggregates (dementia with Lewy bodies) may present with clinical features indistinguishable from the FTLD syndromes described earlier (see Fig 1).12,19,20



Fig 1

Algorithm for the neuropathological diagnosis of patients with frontotemporal lobar degeneration (FTLD). This algorithm represents an oversimplification of the scheme used to diagnose FTLD patients and does not reflect the complexity of the cases or the ...



FTLD clinical syndromes have been associated with specific neuropathological disorders by some researchers,7 although this association is controversial.3 With the development of therapies that target the underlying pathology and cell biology of specific diseases, classification of patients with various pathological entities will become increasingly important. The clinical and pathological features have been described, but few studies have performed detailed clinicopathological correlations of large series of FTLD patients. In this study, we sought to determine the clinical and/or neuropsychological features that correlate with the underlying pathology in a large series of subjects with a neuropathological diagnosis of FTD who were evaluated over a 10-year period.

/pdf/frontotemporal-dementia-clinicopathological-correlations-48ie904g89.pdf

Frontotemporal dementia: clinicopathological correlations.

Aims: The Korsakoff syndrome is a preventable memory disorder that usually emerges (although not always) in the aftermath of an episode of Wernicke's encephalopathy. The present paper reviews the clinical and scientific literature on this disorder. Methods: A systematic review of the clinical and scientific literature on Wernicke's encephalopathy and the alcoholic Korsakoff syndrome. Results: The Korsakoff syndrome is most commonly associated with chronic alcohol misuse, and some heavy drinkers may have a genetic predisposition to developing the syndrome. The characteristic neuropathology includes neuronal loss, micro-haemorrhages and gliosis in the paraventricular and peri-aqueductal grey matter. Lesions in the mammillary bodies, the mammillo-thalamic tract and the anterior thalamus may be more important to memory dysfunction than lesions in the medial dorsal nucleus of the thalamus. Episodic memory is severely affected in the Korsakoff syndrome, and the learning of new semantic memories is variably affected. ‘Implicit’ aspects of memory are preserved. These patients are often first encountered in general hospital settings where they can occupy acute medical beds for lengthy periods. Abstinence is the cornerstone of any rehabilitation programme. Korsakoff patients are capable of new learning, particularly if they live in a calm and well-structured environment and if new information is cued. There are few long-term follow-up studies, but these patients are reported to have a normal life expectancy if they remain abstinent from alcohol. Conclusions: Although we now have substantial knowledge about the nature of this disorder, scientific questions (e.g. regarding the underlying genetics) remain. More particularly, there is a dearth of appropriate long-term care facilities for these patients, given that empirical research has shown that good practice has beneficial effects.

/pdf/the-korsakoff-syndrome-clinical-aspects-psychology-and-57qgwf30dc.pdf

The Korsakoff syndrome: clinical aspects, psychology and treatment.

Presenile Dementia or Early Onset Dementia (EOD) is a public health problem, it differs from Senile Dementia, and encloses a significant number of cases; nevertheless, it is still poorly understood and underdiagnosed. This study aims to review the prevalence and etiology of EOD, comparing EOD with Senile Dementia, as well as to show the main causes of EOD and their prevalence in population and non-population based studies. The computer-supported search used the following databases: Pubmed/Medline, ISI Web of Knowledge and Scielo. The search terms were alcohol-associated dementia, Alzheimer’s disease, dementia, Creutzfeldt-jakob disease, dementia with lewy bodies, early onset dementia, frontotemporal lobar degeneration, Huntington’s disease, mixed dementia, neurodegenerative disorders, Parkinson’s disease dementia, presenile dementia, traumatic brain injury, vascular dementia. Only papers published in English and conducted from 1985 up to 2012 were preferentially reviewed. Neurodegenerative diseases are the most common etiologies seen in EOD. Among the general population, the prevalence of EOD was found to range between 0 to 700 per 100.000 habitants in groups of 25-64 years old, with an increasing incidence with age. The progression of EOD was found to range between 8.3 to 22.8 new cases per 100.000 in those aged under 65 years. Alzheimer's disease (AD) is the major etiology, followed by Vascular Dementia (VaD) and Frontotemporal Lobar Degeneration (FTLD). A larger number of epidemiological studies to elucidate how environmental issues contribute to EOD are necessary, thus, we can collaborate in the planning and prevention of services toward dementia patients.

https://repositorio.bc.ufg.br/bitstream/ri/16819/5/Artigo%20-%20Renata%20Teles%20Vieira%20-%202013.pdf

Epidemiology of early-onset dementia: a review of the literature

The clinical characteristics and the level of service use were studied in a cohort of 200 patients referred for the investigation of presenile dementia, having had the onset of symptoms before the age of 65. The most common diagnoses given were Alzheimer's disease, vascular dementia and depressive pseudodementia. The level of psychiatric morbidity was high, depression insomnia, anxiety and aggression being the most common symptoms. As regards rate of service use, 22% of the Liverpool patients were in residential care after 1 year of follow-up, in contrast to 8% initially. We conclude that there is a need for specialized multidisciplinary services for this group of patients, both diagnostic and supportive.

The early onset dementias: a study of clinical characteristics and service use

Both Parkinson’s disease (PD) and dementia with Lewy bodies (DLB) share a common neuropathological marker, the presence of Lewy bodies in brain stem and basal forebrain nuclei. DLB, in addition, is associated with Lewy bodies in the neocortex, and, in it’s more common form, with Alzheimer-type pathological markers, particularly amyloid plaques. Published neuropsychological studies have focused on the differential profiles of DLB and Alzheimer’s disease (AD). However, it is presently unclear whether DLB should be classified as a variant of AD or PD. In the present study we compare a healthy age-matched control group with three groups of patients, one with DLB, and two with PD. One of the PD groups was early in the course (PD-E) and the second, more advanced group (PD-A), was matched on severity of cognitive impairment with the DLB group. The results show that DLB was associated with a different pattern of neuropsychological impairment than the PD-A group, particularly in tests believed to be mediated by prefrontal cortical regions.

/pdf/intellectual-mnemonic-and-frontal-functions-in-dementia-with-2wb2zbq5vh.pdf

Intellectual, mnemonic, and frontal functions in dementia with Lewy bodies: A comparison with early and advanced Parkinson's disease.

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The early onset dementias: a study of clinical characteristics and service use

Intellectual, mnemonic, and frontal functions in dementia with Lewy bodies: A comparison with early and advanced Parkinson's disease.