Dementia Syndrome Research Articles

Utility of empathy informant report in FTD differential diagnosis.

Loss of empathy is a hallmark feature of behavioral variant frontotemporal dementia (bvFTD). Change in socioemotional functioning identified by others is often the primary initial presenting concern in this disorder, in contrast to more subtle early cognitive changes and limited patient insight. The present study examined the predictive utility of an empathy informant-report measure for discriminating clinician-diagnosed bvFTD from other dementia syndromes. Data from the National Alzheimer's Coordinating Center (NACC) database were used to study individuals with bvFTD (n = 406) and other dementia syndromes (n = 385). Participants were administered neuropsychological measures and collateral informants completed an informant-report of empathy. Informants reported that patients with bvFTD demonstrated significantly lower levels of empathic concern [F(1, 789) = 120.91, p < .001, η2 = 0.13] and perspective taking [F(1, 789) = 153.08, p < .001, η2 = 0.16] than patients with other dementia syndromes. These differences were not attributable to the level of global cognitive impairment. Empathy scores were not significantly associated with any neurocognitive measure when controlling for age. ROC curve analyses showed fair to good clinical utility of the informant-report empathy measure for distinguishing bvFTD from non-bvFTD, whereas a traditional measure of executive functioning failed to differentiate the groups. These findings indicate that informant ratings of empathy offer a unique source of clinical information that may be useful in detecting neurobehavioral changes specific to bvFTD before a clear neurocognitive pattern emerges on testing.

Olfactory Bulb Integrity in Frontotemporal Dementia and Alzheimer’s Disease

Olfactory dysfunction is highly prevalent in dementia syndromes, including Alzheimer's disease (AD) and frontotemporal dementia (FTD). The structural integrity of the olfactory bulb (OB) is thought to play a critical role in odor detection and identification, but no MRI study has measured OB volume in FTD, or measured OB volume longitudinally in AD. To measure OB volume in FTD and AD patients longitudinally using MRI. This study measured OB volumes using MRI in patients diagnosed with behavioral-variant FTD (n = 55), semantic dementia (n = 34), progressive non-fluent aphasia (n = 30), AD (n = 50), and healthy age-matched controls (n = 55) at their first visit to a dementia research clinic ('baseline'). Imaging data in patients 12-months later were analyzed where available (n = 84) for longitudinal assessment. Volumes of subcortical and cortical olfactory regions ('olfactory network') were obtained via surface-based morphometry. Results revealed that in AD and FTD at baseline, OB volumes were similar to controls, whereas volumes of olfactory network regions were significantly reduced in all patient groups except in progressive non-fluent aphasia. Longitudinal data revealed that OB volume became significantly reduced (10-25% volume reduction) in all dementia groups with disease progression. Olfactory dysfunction is common in patients diagnosed with AD or FTD, but our results indicate that there is no detectable volume loss to the OBs upon first presentation to the clinic. Our findings indicate that the OBs become detectably atrophied later in the disease process. OB atrophy indicates the potential usefulness for OBs to be targeted in interventions to improve olfactory function.

Derivation and validation of a 90-day unplanned hospital readmission score in older patients discharged form a geriatric ward.

To derive and validate a 90-day unplanned hospital readmission (UHR) score based on information available to non-hospital based care providers. Retrospective longitudinal study with cross-validation method. Participants were older adults (≥ 65years) admitted to a geriatric short-stay department in a general hospital in France. Patients were split into a derivation cohort and a validation cohort. We recorded demographic information, medical history, and concurrent clinical characteristics. The main outcome was 90-day UHR. Data obtained from hospital discharge letters were used in a logistic regression model to construct a predictive score, and to identify risk groups for 90-day UHR. In total, 750 and 250 aged adults were included in both the derivation and the validation cohorts. Mean age was 87.2 ± 5.2years, most were women (68.1%). Independent risk factors for 90-day UHR were: use of mobility aids (p = .02), presence of dementia syndrome (p = .02), history of recent hospitalisation (p = .03), and discharge to domiciliary home (p = .005). From these four risk factors, three groups were determined: low-risk group (score < 4), medium-risk group (score between 4 and 6), and high-risk group (score ≥ 6). In the derivation cohort the 90-day UHR rates increased significantly across risk groups (14%, 22%, and 30%, respectively). The 90-day UHR score had the same discriminant power in the derivation cohort (c-statistic = 0.63) as in the validation cohort (c-statistic = 0.63). This score makes it possible to identify aged adults at risk of 90-day UHR and to target multidisciplinary interventions to limit UHR for patients discharged from a Geriatric Short-Stay Unit.

Dementia in People with Severe/Profound Intellectual (and Multiple) Disabilities: Applicability of Items in Dementia Screening Instruments for People with Intellectual Disabilities

ABSTRACT Introduction Diagnosing dementia in people with severe/profound intellectual (and multiple) disabilities (SPI(M)D) is complex. Whereas existing dementia screening instruments as a whole are unsuitable for this population, a number of individual items may apply. Therefore, this study aimed to identify applicable items in existing dementia screening instruments. Methods Informant interviews about 40 people with SPI(M)D were conducted to identify applicable items in the Dementia Scale for Down Syndrome, Behavioral and Psychological Symptoms of Dementia in Down Syndrome II scale, Dementia Questionnaire for persons with Mental Retardation and Social competence Rating scale for people with Intellectual Disabilities. Results Among 193 items, 101 items were found applicable, categorized in 5 domains: behavioral and psychological functioning (60 items), cognitive functioning (25), motor functioning (6), activities of daily living (5) and medical comorbidities (5). Conclusion Identifying applicable items for people with SPI(M)D is an essential step in developing a dedicated dementia screening instrument for this population.

Pisa syndrome in dementia with Lewy bodies: A Chinese multicenter study

BackgroundPisa syndrome (PS) is rarely reported in Dementia with Lewy bodies (DLB). The aim of this article is to investigate the prevalence rate of PS and the correlation with clinical features evaluated in patients with DLB. MethodsA total of 209 DLB patients were consecutively recruited and underwent standardized clinical evaluation in our multicenter study. The associations between PS and clinical factors were evaluated. ResultsThe prevalence rate of PS in patients with DLB was 15.3%, which was higher in the moderate and severe stages than mild cognitive impairment and mild stages (81.2% vs. 18.8%). Patients with PS had a longer duration of disease (P = 0.020) and parkinsonism (P = 0.003), higher scores of NPI (P = 0.028), ADL (P = 0.002) and UPDRS part III (P < 0.001), lower scores of clock drawing test (P = 0.009), visuospatial/executive abilities (P = 0.018), attention (P = 0.020), language and praxis (P = 0.020), registration (P = 0.012), greater H&Y stage (P < 0.001), and higher proportion of cholinesterase inhibitors used (P = 0.044) than those without PS. Longer disease duration (OR = 1.166, P = 0.023), presence of parkinsonism (OR = 7.971, P = 0.007), moderate and severe dementia (OR = 3.215, P = 0.021) were associated with the presence of PS. Patients had a longer duration of PS (P = 0.014) and lower mean age of onset (P = 0.040) in the group with severe lateral trunk flexion. ConclusionThe development of PS may be associated with longer disease duration, the presence of parkinsonism and severe stages of dementia in DLB. Cholinesterase inhibitors may have a correlation with PS. The severity of lateral flexion is related to the duration of PS and mean age of onset.

The Carriers: What the Fragile X Gene Reveals About Family, Heredity, and Scientific Discovery.

Back to table of contents Previous article Book ReviewsFull AccessThe Carriers: What the Fragile X Gene Reveals About Family, Heredity, and Scientific DiscoveryJames A. Bourgeois, O.D., M.D.James A. BourgeoisSearch for more papers by this author, O.D., M.D.Published Online:22 Aug 2022https://doi.org/10.1176/appi.neuropsych.22010008AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail by Anne Skomorowsky, M.D. New York, Columbia University Press, 2022, 280 pages.In The Carriers, Dr. Anne Skomorowsky—a consultation-liaison psychiatrist, clinical instructor in psychiatry at the New York University (NYU) Grossman School of Medicine and attending psychiatrist at NYU Langone Hospital—offers us a thorough, sensitive, and provocative account of the lives of patients with the premutation, or carrier state, of fragile X disorders—many clustered in families of multiple fragile X–affected individuals. In this relatively slim volume, the author offers a concise yet thoroughly informative account of the genetic and clinical history of the ascertainment of fragile X syndrome, a critical distinction between fragile X syndrome and its carrier state, detailed and poignant narratives of many affected patients, and the evolution of the diagnosis, classification, and clinical research paradigm into this important condition.Fragile X–related conditions are of great interest to both psychiatrists and neurologists in that there may be manifestations in both the psychiatric and neurologic symptom and syndrome domains. Indeed, so many patients suffer in both domains that we really ought to consider these conditions as prototypical “neuropsychiatric” disorders, similar in conceptual inclusion to multiple sclerosis and Parkinson’s disease.Whereas full-spectrum fragile X syndrome is generally better known and usually manifests in childhood with various degrees of cognitive and behavioral impairment (though the spectrum of illness ranges from the subtle to the severely impairing), Dr. Skomorowsky focuses her book on the carrier or premutation states. The book’s thesis is that for most patients the carrier-state illness is not just a milder version of fragile X syndrome, but is a distinct, separate illness with different genetic deficits, pathophysiology, and multisystemic clinical manifestations, which have been ascertained only in the past 20 years. Additional chapters address the history of ascertainment of genetically caused intellectual disability, the ultimately successful search for the fragile X gene, and the evolution of fragile X disorders clinical research.One fascinating aspect of assessing clinical illness in carriers, particularly psychiatric comorbid illness, is that many carriers are actively involved in raising one or more full-spectrum fragile X children, such that the axiomatic (yet, critically, incomplete) assumption of clinicians is often that the stresses of raising affected children are the genesis of the carriers’ often significant psychiatric comorbidity. Dr. Skomorowsky persuasively shows, by citations of literature, interviews with many fragile X researchers, and her own interviews with affected patients, that the psychiatric comorbidity in carriers is primarily part of the phenotype of the carrier premutation state itself. Carriers need empathic clinical care on this basis alone.The book emphasizes the multigenerational nature of the carrier/premutation conditions. Although young adult females who carry the premutation are prone to ovarian dysfunction, other systemic manifestations, anxiety and depressive disorders, and problems with social behavior, middle-aged and older male carriers (the grandfathers of full-mutation fragile X children and fathers of the adult female carriers) are at risk for an often debilitating fragile X–associated tremor-ataxia syndrome (FXTAS, acronym pronounced “fax-tass”), which is a mixed movement disorder and dementia syndrome that can lead to significant debility. One of the many instructive ironies in fragile X research is that FXTAS was initially described by the noted fragile X clinical researcher Randi Hagerman, M.D., making FXTAS perhaps the only adult degenerative dementia syndrome discovered by a pediatrician. In many cases, young adult carriers are “pulled two ways” between attending to their own impaired children and assisting their progressively ill fathers, all while managing their own, often clinically impairing, symptoms.Throughout the text, Dr. Skomorowsky highlights the need for an empathic approach, the need for physician-patient alliance, and an appreciation of the coping strategies exhibited by fragile X carrier patients while they manage their own variable symptoms in the broader context of affected family members. As clinical and basic sciences advance apace, it is both inevitable and desirable that additional neuropsychiatric illnesses will be discovered to have a genetic and molecular basis. The recent experience with fragile X premutation carrier states illustrates this magnificently. Psychiatrists curious about genetically determined psychiatric illness—which should be all of us—will find this book a valuable introduction to the world of fragile X illnesses and of patients coping with them.Central Texas Division, Baylor Scott & White Health, Dallas; Department of Medical Education, College of Medicine, Texas A&M University Health Science Center, Temple.Send correspondence to Dr. Bourgeois ([email protected]).Dr. Bourgeois reports no financial relationships with commercial interests. FiguresReferencesCited byDetailsCited byNone Volume 35Issue 1 Winter 2023Pages 102-102 Metrics KeywordsDementiaGeneticsPDF download History Received 10 January 2022 Accepted 15 April 2022 Published online 22 August 2022 Published in print 1 January 2023

A-117 Posterior Cortical Atrophy Case Study: Neuropsychological Assessment Remains Gold Standard Diagnostic Tool of Choice

Abstract Objective: Posterior cortical atrophy (PCA) is a rare, early onset, neurodegenerative syndrome that is characterized by impaired visuospatial/visual-constructional skills. Patients describe vague visual symptoms; however, ocular assessments are usually normal. Alzheimer's disease is the most common underlying cause of PCA; however, brain MRI is often unrevealing early in the disease. As a result, accurate diagnosis is often delayed. Method: The patient was initially referred in 2016 for a concussion evaluation following a work-related head injury when she was 54. Initial MRI of the brain was unremarkable. Neuropsychological testing was repeated in 2019, which was the first diagnostic test to establish a diagnosis of PCA. Subsequently, MRI of the brain with volumetric analysis showed total hippocampal volume to be at the 99th percentile and no pattern of cerebral volume loss to suggest a specific diagnosis. However, CSF biomarker assays were consistent with a diagnosis of Alzheimer’s disease. Results: The patient’s initial cognitive profile in 2016 reflected mild cognitive impairment, multidomain type with severely impaired visual-constructional skills, which were unrelated to her concussion. Neuropsychological testing was repeated in 2019, which showed interval progressive cognitive and functional decline. Profound visual-constructional impairments were again noted (see image), and she met criteria for PCA. Neuropsychological testing was repeated in 2021 and showed progression to moderate dementia with features of Gerstmann's syndrome. Conclusions: In spite of sophisticated neuroimaging techniques and increasing access to CSF biomarker analysis, neuropsychological assessment remains the gold standard diagnostic tool of choice for neurologists faced with rare dementia syndromes, such as PCA. See patient video at: https://youtu.be/9HdAKSL5alg

Clinical utility of the Saint Louis University Mental Status Examination (SLUMS) in a mixed neurological sample: Proposed revised cutoff scores for normal cognition, mild cognitive impairment, and dementia

Early detection of cognitive impairment is of paramount importance in clinical settings, with several brief screening tools having been developed for that purpose. The present study sought to evaluate the clinical utility of the Saint Louis University Mental Status examination (SLUMS) at identifying examinees with normal cognition, mild cognitive impairment, or dementia syndrome using the criterion of a comprehensive neuropsychological assessment. Two hundred sixty-three examinees (M age = 67.84 ± 12.72; 59.3% female; 81.4% white) were referred for comprehensive neuropsychological evaluation at a private, Mid-Atlantic medical center. Using original cutoff scores, the SLUMS correctly classified just over half (55.1%) of examinees. Classification statistics suggested modified cutoff scores for mild cognitive impairment (≤24) and dementia (≤17) with strong discriminability between cognitive status groups (AUCs ranged from .834 to .986). These proposed revised cutoff scores improved overall concordance between SLUMS and diagnostic conclusions from comprehensive clinical neuropsychological testing, correctly classifying nearly two-thirds of examinees (65.4%). The SLUMS and its revised cutoff scores appear to have clinical utility for cognitive screening in primary care and neurological settings to inform treatment plans and appropriate referrals for comprehensive neuropsychological assessment.

Systematic review of pharmacological management in Creutzfeldt-Jakob disease: no options so far?

The Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that manifests as a rapidly progressive dementia syndrome. Currently, CJD has no cure, and many patients die within the first year, but some drugs are being studied as options for managing this condition. To evaluate the effectiveness of pharmacological treatments offered to patients with CJD as a means to increase survival and reduce cognitive deterioration. A systematic review of the literature was performed using 4 independent reviewers and 1 extra reviewer to resolve possible divergences in the search and analysis of papers indexed in MedLINE (PubMed), SciELO and Lilacs databases. The Medical Subject Heading (MeSH) terms used were: prion diseases, Creutzfeldt-Jakob disease, pharmacologic therapy, therapeutics, quinacrine, doxycycline, flupirtine, and pentosan polysulfate, with the Boolean operators AND and OR. This search included controlled clinical trials, uncontrolled clinical trials, and case series published from the year 2000 onwards, in the English language. A total of 85 papers were found using the descriptors used. At the end of the selection analyses, 9 articles remained, which were analyzed fully and individually. None of the drugs evaluated proved significantly effective in increasing survival in patients with CJD. Flupirtine appears to have a beneficial effect in reducing cognitive deterioration in patients with CJD. However, additional studies are needed to establish better evidence and therapeutic options for the management of patients with CJD.

The Relationship Between Prior Cancer Diagnosis and All-Cause Dementia Progression Among US Adults.

Cancer-related cognitive impairment (CRCI), a frequent effect of cancer and its treatments, shares common cognitive symptoms with dementia syndromes. Cross-sectional studies demonstrate an inverse relationship between cancer and dementia. However, the longitudinal relationship between dementia decline and cancer has not been investigated. To evaluate the association between cancer and longitudinal progression of dementia. We extracted electronic health record data from July 2003 to February 2020 from a single academic medical center. We identified dementia and cancer history prior to dementia using ICD-9/10 codes. We measured cognitive decline with the Alabama Brief Cognitive Screener (ABCs). We used adjusted linear mixed models to estimate baseline cognition and rate of progression by cancer history, including differences by race. The study included 3,809 participants with dementia, of which 672 (17.6%) had cancer history. Those with cancer history had higher baseline cognition (β: 1.07, 95% CI: 0.45, 1.69), but similar rate of decline. Non-Hispanic Blacks had lower cognitive scores at baseline and throughout follow-up regardless of cancer status compared to non-Hispanic Whites and other races/ethnicities with and without cancer history. In this longitudinal retrospective study, participants with cancer history demonstrate better cognition at dementia diagnosis and no difference in cognitive decline than those without cancer history. Smoking and comorbidities attenuate this association and results indicate non-Hispanic Blacks have worse cognitive outcomes in dementia regardless of cancer history than other race/ethnicity groups. Further exploration of the role of smoking, comorbidities, and race/ethnicity on cancer and dementia-related cognitive decline is needed.

Increased Serum NSE and S100B Indicate Neuronal and Glial Alterations in Subjects Under 71 Years With Mild Neurocognitive Disorder/Mild Cognitive Impairment.

BackgroundMild cognitive impairment (MCI) is considered a pre-stage of different dementia syndromes. Despite diagnostic criteria refined by DSM-5 and a new term for MCI – “mild neurocognitive disorder” (mild NCD) – this diagnosis is still based on clinical criteria.MethodsTo link mild NCD to the underlying pathophysiology we assessed the degree of white matter hyperintensities (WMH) in the brain and peripheral biomarkers for neuronal integrity (neuron-specific enolase, NSE), plasticity (brain-derived neurotrophic factor, BDNF), and glial function (S100B) in 158 community-dwelling subjects with mild NCD and 82 healthy controls. All participants (63–79 years old) were selected from the Leipzig-population-based study of adults (LIFE).ResultsSerum S100B levels were increased in mild NCD in comparison to controls (p = 0.007). Serum NSE levels were also increased but remained non-significant after Bonferroni-Holm correction (p = 0.04). Furthermore, age by group interaction was significant for S100B. In an age-stratified sub-analysis, NSE and S100B were higher in younger subjects with mild NCD below 71 years of age. Some effects were inconsistent after controlling for potentially confounding factors. The discriminatory power of the two biomarkers NSE and S100B was insufficient to establish a pathologic threshold for mild NCD. In subjects with mild NCD, WMH load correlated with serum NSE levels (r = 0.20, p = 0.01), independently of age.ConclusionOur findings might indicate the presence of neuronal (NSE) and glial (S100B) injury in mild NCD. Future studies need to investigate whether younger subjects with mild NCD with increased biomarker levels are at risk of developing major NCD.

The Association of Physical and Mental Illness and Self-Harm Resulting in Hospitalization: A Population-Based Study of Older Adults in South Korea.

Self-harm injury among older adults is a pressing problem that demands social attention in South Korea. This study sought to identify the association between physical and mental illness and hospitalization following self-harm injuries, compared to non-self-harm injuries, among older adults living in Korea. We analyzed individuals aged 65 and older who were admitted to hospitals either for self-harm or non-self-harm from a population-based survey of the Korea National Hospital Discharge In-depth Injury Survey (KNHDIS). A logistic regression analysis was performed. Compared with non-self-harm-related hospitalization, self-harm hospitalization was associated with higher odds of depression, other disorders of the nervous system, malignancies, alcohol misuse and dependence, and drug-related dependence. Dementia, anxiety disorder, diabetes, arthritis, cerebral palsy, and other paralytic syndromes had a lower likelihood of leading to self-harm than non-self-harm hospitalization. The findings of this study can inform medical professionals to identify older adults with a heightened risk of self-harming behavior leading to hospitalization.

CURVED MULTIFORMAT IMAGING OF STRUCTURAL MRI AS A BIOMARKER FOR PROFILING OF VARIOUS DEMENTIA - AN ALGORITHMIC APPROACH WITH MR/PET.

Dementia is one of the most common clinical entities for which neuroimaging is done for diagnosis and characterization of the sub-types. Advanced neuroimaging modalities like MR-PET have added to the armamentarium of the neuroradiologists for the diagnosis of specific types of dementia syndromes. However, advanced molecular imaging is expensive and is not widely available particularly in underdeveloped countries. Structural imaging with MRI in addition to the clinical work up remains the most useful approach to the diagnosis of dementia in our setup. We describe a simple technique of curved multiplanar reconstruction of brain images with the use of a simple reconstruction software which can help in recognition of pattern of atrophy in dementias and thus improve the diagnostic accuracy of structural MRI. Using this approach of pattern recognition, we suggest a simplified algorithm for diagnosis of sub-types of dementia. In our study, we applied this technique for demonstration of pattern of atrophy which correlated with metabolic changes on PET in a variety of dementia cases using simultaneous MR-PET imaging and may act as a low cost and reliable biomarker to profile dementia subtypes. To the best of our knowledge this is the first study to show the utility of curved multiplanar reconstruction in dementias.

Hypertension and cognitive function: a review of life-course factors and disparities.

Dementia is a life-course condition with modifiable risk factors many from cardiovascular (CV) origin, and disproportionally affects some race/ethnic groups and underserved communities in the USA. Hypertension (HTN) is the most common preventable and treatable condition that increases the risk for dementia and exacerbates dementia pathology. Epidemiological studies beginning in midlife provide strong evidence for this association. This study provides an overview of the differences in the associations across the lifespan, and the role of social determinants of health (SDoH). Clinical trials support HTN management in midlife as an avenue to lower the risk for late-life cognitive decline. However, the association between HTN and cognition differs over the life course. SDoH including higher education modify the association between HTN and cognition which may differ by race and ethnicity. The role of blood pressure (BP) variability, interactions among CV risk factors, and cognitive assessment modalities may provide information to better understand the relationship between HTN and cognition. Adopting a life-course approach that considers SDoH, may help develop tailored interventions to manage HTN and prevent dementia syndromes. Where clinical trials to assess BP management from childhood to late-life are not feasible, observational studies remain the best available evidence.

Brain functional topology differs by sex in cognitively normal older adults.

IntroductionLate onset Alzheimer’s disease (AD) is the most common form of dementia, in which almost 70% of patients are women.HypothesisWe hypothesized that women show worse global FC metrics compared to men, and further hypothesized a sex-specific positive correlation between FC metrics and cognitive scores in women.MethodsWe studied cognitively healthy individuals from the Alzheimer’s Disease Neuroimaging Initiative cohort, with resting-state functional Magnetic Resonance Imaging. Metrics derived from graph theoretical analysis and functional connectomics were used to assess the global/regional sex differences in terms of functional integration and segregation, considering the amyloid status and the contributions of APOE E4. Linear mixed effect models with covariates (education, handedness, presence of apolipoprotein [APOE] E4 and intra-subject effect) were utilized to evaluate sex differences. The associations of verbal learning and memory abilities with topological network properties were assessed.ResultWomen had a significantly lower magnitude of the global and regional functional network metrics compared to men. Exploratory association analysis showed that higher global clustering coefficient was associated with lower percent forgetting in women and worse cognitive scores in men.ConclusionWomen overall show lower magnitude on measures of resting state functional network topology and connectivity. This factor can play a role in their different vulnerability to AD.Significance statementTwo thirds of AD patients are women but the reasons for these sex difference are not well understood. When this late onset form dementia arises is too late to understand the potential causes of this sex disparities. Studies on cognitively healthy elderly population are a fundamental approach to explore in depth this different vulnerability to the most common form of dementia, currently affecting 6.2 million Americans aged 65 and older are, which means that >1 in 9 people (11.3%) 65 and older are affected by AD. Approaches such as resting-state functional network topology and connectivity may play a key role in understanding and elucidate sex-dependent differences relevant to late-onset dementia syndromes.

Communication Bridge™-2 (CB2): an NIH Stage 2 randomized control trial of a speech-language intervention for communication impairments in individuals with mild to moderate primary progressive aphasia

BackgroundPrimary progressive aphasia (PPA) is a clinical dementia syndrome. Impairments in language (speaking, reading, writing, and understanding) are the primary and persistent symptoms. These impairments progress insidiously and devastate communication confidence, participation, and quality of life for persons living with PPA. Currently, there are no effective disease modifying treatments for PPA. Speech-language interventions hold promise for mitigating communication challenges and language symptoms. However, evidence regarding their efficacy in PPA is of low quality and there are currently no rigorous randomized trials.MethodCommunication Bridge™-2 (CB2) is a Stage 2, superiority, single-blind, randomized, parallel group, active-control, behavioral clinical trial delivered virtually within a telehealth service delivery model to individuals with PPA. Ninety carefully characterized participants with clinically confirmed PPA will be randomized to one of two speech-language intervention arms: (1) Communication Bridge™ a dyadic intervention based in communication participation therapy models that incorporates salient training stimuli or (2) the control intervention a non-dyadic intervention based in impairment therapy models addressing word retrieval and language production that incorporates fixed stimuli. The superiority of Communication Bridge™ over the Control arm will be evaluated using primary outcomes of communication confidence and participation. Other outcomes include accuracy for trained words and scripts. Participants complete two therapy blocks over a 12-month period. Outcomes will be measured at baseline, at each therapy block, and at 12 months post enrollment.DiscussionThe CB2 trial will supply Level 2 evidence regarding the efficacy of the Communication Bridge™ intervention delivered in a telehealth service delivery model for individuals with mild to moderate PPA. An important by-product of the CB2 trial is that these data can be used to evaluate the efficacy of speech-language interventions delivered in both trial arms for persons with PPA. The impact of these data should not be overlooked as they will yield important insights examining why interventions work and for whom, which will advance effectiveness trials for speech-language interventions in PPA.Trial RegistrationClinicalTrials.govNCT03371706. Registered prospectively on December 13, 2017.

Wandering syndrome in dementia — nosospecific characteristics and clinical-psychopathоtological features

The work was performed at the Department of Psychiatry, Psychology and Sexology of Danylo Halytsky’s Lviv National Medical University and the clinical departments of the Lviv Regional Clinical Psychiatric Hospital. We studied the clinical and psychopathological features of wandering syndrome in patients with neuropsychiatric symptoms of dementia due to vascular, neurodegenerative pathologies and mixed genesis among patients with moderate and severe dementia who underwent inpatient and outpatient treatment due to these symptoms. The aim was to study and compare the parameters of wandering syndrome among patients with different types of dementia. A reliable association was found between the following characteristics of wandering syndrome and types of dementia: wandering that occurred only in the middle of the night was significantly more common in vascular dementia than in neurodegenerative dementia; cases of wandering, accompanied by disorientation, regardless of the time of day, mostly occurred with vascular dementia; attempts to escape and escape from the apartment — more often in patients with vascular than neurodegenerative dementia; loss of orientation when leaving the usual place of residence was observed mainly among patients with mixed dementia; the intensity of wandering in the number and frequency of wandering acts correlates with cases of mixed dementia. The study of the features of wandering allows us to examine this phenomenon in more detail to predict the course of both individual neuropsychiatric syndrome and the disease as a whole, as well as to provide comprehensive and effective care for this group of patients.

Prognostic and diagnostic value of cerebrospinal fluid analysis in neurodegenerative dementia diseases

Cerebrospinal fluid (CSF) analysis is an important diagnostic tool in the assessment of dementia. For the differentiation of Alzheimer's disease from other etiologies of dementia syndromes, established biological markers could be helpful to confirm a distinctive neuropathology. Whereas negative CSF findings can rule out the majority of primarily neurodegenerative disorders, overlapping biomarker profiles remain adiagnostic challenge. Therefore, it is important to interpret CSF results within aspecific clinical context. Furthermore, atypical CSF data can be challenging and require profound knowledge of preanalytics, biomarker profiles and the broad spectrum of diseases associated with cognitive decline. Beyond the Alzheimer's disease clinical spectrum, current studies aim at investigating CSF biomarkers to better differentiate tauopathies, TDP43(Transactive response DNA binding protein 43 kDa)-proteinopathies and synucleinopathies.

Artificial ulcer with perforating bone defect on the scalp due to repeated - scratching attacks in dementia syndrome

We report on a 72-year-old demented patient with a palm-sized skin defect on the scalp, who has been manipulating her head several times a day with her fingers but also with sharp objects for many months. A 4 × 6 cm circular, cleanly granulated ulcer was visible on the high parietal surface. On general examination, the patient was little affected: no fever, no signs of inflammation around the ulcer, no evidence of meningitis. There was no histological evidence of the presence of a malignant or benign tumor. A CT of the skull showed a bony defect measuring 2.6 × 3.3 cm without involvement of the dura. The whole body CT without pathological findings. First, a protective helmet was made for the patient. Subsequently, a titanium plate was surgically inserted as a bone substitute and the defect was closed by means of transpositionoplasty. In the two-year follow-up, no tumor disease was detected. Due to the lack of evidence of tumorigenesis in a follow-up period of two years, we evaluate the lesion as an artificial ulcer with perforating bone defect caused by repeated scratching attacks in the context of dementia syndrome.

121 The Queen Square tests of auditory cognition: defining hearing deficits and dis- ability in dementia

BackgroundHearing impairment has emerged as a potent association of cognitive decline in dementia and a promising treatment target. To realise this promise, we need to resolve fundamental questions concerning the roles of peripheral versus central auditory deficits in different dementias.MethodsUsing a novel psychoacoustic battery – the Queen Square Tests of Auditory Cognition (QSTAC)– we combined pre-existing and novel measures of peripheral and central hearing functions, to charac- terise discrete ‘auditory phenotypes’ across dementia syndromes. Customised auditory symptom ques- tionnaires (completed by patients’ caregivers) were used to capture daily-life hearing-related disability and care burden. Patient groups: Alzheimer’s disease, frontotemporal dementia, primary progressive aphasia (PPA) and control. Neuroanatomical associations of specific hearing deficits were assessed using voxel-based morphometry.ResultsSyndromic signatures of peripheral and central auditory dysfunction were identified. Alzheimer’s disease was associated with prominent impairments of auditory scene analysis and dichotic listening. PPA syndromes and behavioural variant FTD (BvFTD) were principally associated with deficits of sound pattern analysis, sound identity, emotion recognition and degraded speech perception, which stratified according to sub-syndrome (logopoenic, semantic, nonfluent).ConclusionsTaken together, our findings suggest that major dementias have characteristic and differenti- ated auditory phenotypes, reflecting a complex interplay of peripheral hearing and auditory cognitive dysfunction.jeremy.johnson@doctors.org.uk