Dementia, Amnestic Syndrome

INTRODUCTION: Visual naming ability reflects semantic memory retrieval and is a hallmark deficit of Alzheimer's disease (AD). Naming impairment is most prominently observed in the late-onset amnestic and logopenic variant Primary Progressive Aphasia (lvPPA) syndromes. However, little is known about how other patients across the atypical AD syndromic spectrum perform on tests of auditory naming, particularly those with primary visuospatial deficits (Posterior Cortical Atrophy; PCA) and early onset (EOAD) syndromes...

The hippocampus is a complex structure located in the mesial temporal lobe that plays a critical role in cognitive and memory-related processes. The hippocampal formation consists of the dentate gyrus, hippocampus proper, and subiculum, and its importance in the neural circuitry makes it a key anatomic structure to evaluate in neuroimaging studies. Advancements in imaging techniques now allow detailed assessment of hippocampus internal architecture and signal features that has improved identification and characterization of hippocampal abnormalities...

There is a longstanding ambiguity regarding the clinical diagnosis of dementia syndromes predominantly targeting executive functions versus behavior and personality. This is due to an incomplete understanding of the macro-scale anatomy underlying these symptomatologies, a partial overlap in clinical features, and the fact that both phenotypes can emerge from the same pathology and vice-versa. We collected data from a patient cohort of which 52 had dysexecutive Alzheimer's disease, 30 had behavioral variant frontotemporal dementia, seven met clinical criteria for behavioral variant frontotemporal dementia but had Alzheimer's disease pathology (behavioral Alzheimer's disease), and 28 had amnestic Alzheimer's disease...

OBJECTIVE: To evaluate the long-term effects of annual course therapy with Cerebrolysin on cognitive functioning and the risk of transition to dementia in relatives of patients with Alzheimer's disease (AD) with amnestic-type mild cognitive decline syndrome (aMCI) in comparison with the same group untreated relatives. MATERIAL AND METHODS: The cohort included 88 first-degree relatives of BA patients with aMCI syndrome aged 50 to 82 years (mean age 65.0±9.9 years) of which 46 people received course therapy with Cerebrolysin and 42 people were not treated...

INTRODUCTION: The Rey Auditory Verbal Learning Test (RAVLT) is a useful neuropsychological test for describing episodic memory impairment in dementia. However, there is limited research on its utility in early-onset Alzheimer's disease (EOAD). We assess the influence of amyloid and diagnostic syndrome on several memory scores in EOAD. METHODS: We transcribed RAVLT recordings from 303 subjects in the Longitudinal Early-Onset Alzheimer's Disease Study. Subjects were grouped by amyloid status and syndrome...

OBJECTIVE: Age-related dementia syndromes are often not related to a single pathophysiological process, leading to multiple neuropathologies found at autopsy. An amnestic dementia syndrome can be associated with Alzheimer's disease (AD) with comorbid transactive response DNA-binding protein 43 (TDP-43) pathology (AD/TDP). Here, we investigated neuronal integrity and pathological burden of TDP-43 and tau, along the well-charted trisynaptic hippocampal circuit (dentate gyrus [DG], CA3, and CA1) in participants with amnestic dementia due to AD/TDP, amnestic dementia due to AD alone, or non-amnestic dementia due to TDP-43 proteinopathy associated with frontotemporal lobar degeneration (FTLD-TDP)...

BACKGROUND: Over 55 million adults are living with dementia globally, which is projected to reach 157 million by 2050. Mild cognitive impairment (MCI), a syndrome of memory impairment with intact activities of daily living, may precede dementia by several years. Around 5-15% of individuals with MCI convert to dementia annually. Novel treatments which delay progression of MCI to dementia are urgently needed. Transcutaneous vagal nerve stimulation (tVNS) is a non-invasive neuromodulation technique that targets the vagus nerve...

Recent therapeutic advances provide enhanced motivation for accurate diagnosis of the underlying biologic etiologic causes of dementia. This review focuses on the importance of clinical recognition of Limbic-predominant Age-related TDP-43 Encephalopathy (LATE). LATE affects approximately one-quarter of older adults and produces an amnestic syndrome that is commonly mistaken for Alzheimer's disease (AD). While AD and LATE often co-occur in the same patients, these diseases differ in the protein aggregates driving neuropathology (amyloid/tau vs...

INTRODUCTION: In 2022 the DGN (Deutsche Gesellschaft für Neurologie) published an updated Transient Global Amnesia (TGA) guideline. TGA is characterized by a sudden onset of retrograde and anterograde amnesia for a period of one to a maximum of 24 h (with an average of 6 to 8 h). The incidence is estimated between 3 and 8 per 100,000 population/year. TGA is a disorder that occurs predominantly between 50 and 70 years. RECOMMENDATIONS: The diagnosis of TGA should be made clinically...

BACKGROUND: Posterior cortical atrophy (PCA) is a neurodegenerative syndrome characterised by progressive visuospatial and visuoperceptual impairment. Recent research shows that memory impairment can also occur as an early symptom of the condition and that the impairment can be ameliorated by providing support in the memory recall phase, for example, by presenting a related cue. In Alzheimer's disease (AD), which is defined by an amnestic syndrome, memory aids and strategies have been used to help support everyday memory, which in turn can have a positive impact on patient and carer outcomes...

BACKGROUND: Affective symptoms are dementia risk factors. Mild behavioral impairment (MBI) is a neurobehavioral syndrome that refines incorporation of psychiatric symptomatology into dementia prognostication by stipulating symptoms must emerge de novo in later life and persist for ≥6 months. Here, we investigated the longitudinal association of affective-MBI with incident dementia. METHODS: National Alzheimer Coordinating Centre participants with normal cognition (NC) or mild cognitive impairment (MCI) were included...

Abnormal reward processing is a hallmark of neurodegenerative diseases, most strikingly in frontotemporal dementia. However, the phenotypic repertoire and neuroanatomical substrates of abnormal reward behaviour in these diseases remain incompletely characterized and poorly understood. Here we addressed these issues in a large, intensively phenotyped patient cohort representing all major syndromes of sporadic frontotemporal dementia and Alzheimer's disease. We studied 27 patients with behavioural variant frontotemporal dementia, 58 with primary progressive aphasia (22 semantic variant, 24 non-fluent/agrammatic variant and 12 logopenic) and 34 with typical amnestic Alzheimer's disease, in relation to 42 healthy older individuals...

INTRODUCTION: Sleep-wake disturbances are a prominent feature of Alzheimer's disease (AD). Atypical (non-amnestic) AD syndromes have different patterns of cortical vulnerability to AD. We hypothesized that atypical AD also shows differential vulnerability in subcortical nuclei that will manifest as different patterns of sleep dysfunction. METHODS: Overnight electroencephalography monitoring was performed on 48 subjects, including 15 amnestic, 19 atypical AD, and 14 controls...

BACKGROUND: Transient epileptic amnesia (TEA) is a late-onset epilepsy syndrome encompassing transient iterative amnesias and interictal cognitive impairment, two features that overlap with incipient neurodegenerative dementias. We, therefore, examined the yield of CSF amyloid and tau biomarkers in TEA. METHODS: In this retrospective study, 127 TEA patients with unremarkable imaging findings were divided into 2 groups, namely, CSF (n = 71) and no-CSF (n = 56)...

In Germany, around 1.8 million people currently suffer from dementia and the numbers are increasing. The main cause of dementia is Alzheimer's disease. This is classically manifested in the form of an amnestic syndrome but also encompasses various atypical variants, especially in younger patients and in the clinical routine are not always easy to recognize. These are described in this narrative review with case studies. Posterior cortical atrophy (PCA) presents with visual disorders, in the logopenic variants of primary progressive aphasia (lvPPA) impaired word retrieval is the main symptom, in the frontal variant of Alzheimer's disease behavioral disorders are prominent and in corticobasal syndrome (CBS) an akinetic rigid Parkinson's syndrome with alien limb phenomenon...

The dentate gyrus (DG), a key hippocampal subregion in memory processing, generally resists phosphorylated tau accumulation in the amnestic dementia of the Alzheimer's type due to Alzheimer's disease (DAT-AD), but less is known about the susceptibility of the DG to other tauopathies. Here, we report stereologic densities of total DG neurons and tau inclusions in thirty-two brains of human participants with autopsy-confirmed tauopathies with distinct isoform profiles-3R Pick's disease (PiD, N = 8), 4R corticobasal degeneration (CBD, N = 8), 4R progressive supranuclear palsy (PSP, N = 8), and 3/4R AD (N = 8)...

Besides the typical amnestic presentation, neuropathological studies indicate that Alzheimer's disease (AD) may present with atypical clinical pictures. The relative frequencies of typical and atypical or mixed presentations within the entire spectrum of AD remain unclear, while some mixed or atypical presentations may have not received adequate attention for them to be included in diagnostic criteria. We investigated the spectrum of clinical presentations in patients with the AD CSF biomarker profile (high tau and phospho-tau, low Aβ42 levels), hospitalized in a tertiary academic center...

OBJECTIVE: Little effort has been made in the past to validate depressive pseudodementia based on hypothesis-driven approaches. We extended this concept to individuals with amnestic Mild Cognitive Impairment and Major Depression, that is, pseudodepressive amnestic disorder. We tested two hypotheses consistent with the presentations and mechanisms associated with this potential syndrome: improvements in cognition would be significantly correlated with improvements in depression after treatment (Hypothesis 1), and if not confirmed, the presence of such an association could be identified once moderator variables were taken into account (Hypothesis 2)...

BACKGROUND: Dementia syndrome is one of the most devastating conditions in older adults. As treatments to stop neurodegeneration become available, accurate and timely diagnosis will increase in importance. One issue is that cognitive performance sometimes does not match the corresponding level of neuropathology, affecting diagnostic accuracy. Cognitive reserve (CR), which can preserve cognitive function despite underlying neuropathology, explains at least some variability in cognitive performance...

BACKGROUND: Confusion and hallucinations in geriatric patients are frequent symptoms and typically associated with delirium, late-life psychosis or dementia syndromes. A far rarer but well-established differential in patients with rapid cognitive deterioration, acute psychosis, abnormal movements and seizures is autoimmune encephalitis. Exemplified by our case we highlight clinical and economic problems arising in management of geriatric patients with cognitive decline and psychotic symptoms...