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Atypical dementia

Tessa A Hulshof, Sytse U Zuidema, Peter J K van Meer, Christine C Gispen-de Wied, Hendrika J Luijendijk
OBJECTIVES: To assess baseline imbalances in placebo-controlled trials of atypical antipsychotics in dementia, and their association with neuropsychiatric symptoms (NPS), extrapyramidal symptoms (EPS), and mortality. METHOD: We searched for trials in multiple sources. Two reviewers extracted baseline characteristics and outcomes per treatment group. We calculated direction, range, pooled mean, and heterogeneity in the baseline differences, and used meta-regression for the relationship with the outcomes...
December 4, 2018: International Journal of Methods in Psychiatric Research
Paresh A Malhotra
Alzheimer's Disease (AD) is characteristically perceived as primarily being a disorder of episodic memory, with prominent attentional impairments more typically being associated with other neurodegenerative conditions, such as Dementia with Lewy Bodies. However, attention is also affected early on in Alzheimer's, particularly in individuals with young onset and atypical syndromes. In addition, some initial symptoms that are apparently due to episodic memory loss may be secondary to failures of attentional processes...
November 8, 2018: Current Opinion in Psychology
James R Burrell, John R Hodges
Frontotemporal dementia (FTD) and related diseases are important causes of younger-onset dementia. Falls may be a source of morbidity and mortality in FTD, but remain underreported, and very few high-quality studies have been performed. In this chapter, we briefly review the clinical features of FTD and related syndromes such as motor neuron disease (MND) and atypical parkinsonian syndromes, such as progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). Falls are frequently encountered in patients who present with FTD syndromes...
2018: Handbook of Clinical Neurology
Leila Sellami, Synthia Meilleur-Durand, Anne-Marie Chouinard, David Bergeron, Louis Verret, Stéphane Poulin, Léonie Jean, Marie-Pierre Fortin, Yannick Nadeau, Pierre Molin, Stéphanie Caron, Joël Macoir, Carol Hudon, Rémi W Bouchard, Robert Laforce
INTRODUCTION: Early recognition of atypical dementia remains challenging partly because of lack of cognitive screening instruments precisely tailored for this purpose. METHODS: We assessed the validity and reliability of the Dépistage Cognitif de Québec (DCQ;, a newly developed cognitive screening test, to detect atypical dementia using a multicenter cohort of 628 participants. Sensitivity and specificity were compared to the Montreal Cognitive Assessment (MoCA)...
November 27, 2018: Dementia and Geriatric Cognitive Disorders
Jennifer A Watt, Tara Gomes, Susan E Bronskill, Anjie Huang, Peter C Austin, Joanne M Ho, Sharon E Straus
BACKGROUND: Trazodone is increasingly prescribed for behavioural and psychological symptoms of dementia, but little is known about its risk of harm. Our objective was to describe the comparative risk of falls and fractures among older adults with dementia dispensed trazodone or atypical antipsychotics. METHODS: The study cohort included adults with dementia (excluding patients with chronic psychotic illnesses) living in long-term care and aged 66 years and older...
November 26, 2018: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
John R Younce, Albert A Davis, Kevin J Black
 The atypical antipsychotic ziprasidone has been considered inappropriate for use in patients with Parkinson's disease (PD), as most atypical antipsychotics worsen parkinsonism. However, the current evidence for safety and efficacy of ziprasidone in PDP has not been evaluated in a systematic fashion. We review published experience with ziprasidone for treating psychosis in PD via systematic search of MEDLINE, Embase, Cochrane CENTRAL, and with terms related to "ziprasidone" and "Parkinson's disease", inclusive of case reports and prospective studies...
November 21, 2018: Journal of Parkinson's Disease
Roongroj Bhidayasiri, Jirada Sringean, Stephen G Reich, Carlo Colosimo
To establish a clinical diagnosis of a parkinsonian disorder, physicians rely on their ability to identify relevant red flags, in addition to cardinal features, to support or refute their working diagnosis in an individual patient. The term 'red flag', was originally coined in 1989 to define the presence of non-cardinal features that may raise a suspicion of multiple system atrophy (MSA), or at least suggest alternative diagnosis to Parkinson's disease (PD). Since then, the term 'red flag', has been consistently used in the literature to denote the clinical history or signs that may signal to physicians the possibility of an atypical parkinsonian disorder (APD)...
October 6, 2018: Parkinsonism & related Disorders
Peter Swann, John T O'Brien
ABSTRACTObjectives:Visual hallucinations are a common symptom in dementia and Parkinson's disease and have been associated with greater cognitive and functional decline, but optimal management strategies are unclear. We review the frequency and pathogenesis of visual hallucinations in dementia and Parkinson's disease and examine the evidence base for their management. DESIGN: We undertook a systematic review of the visual hallucinations in dementia, searching studies published between January 1980 and July 2017 using PubMed with the search terms visual hallucinations AND review AND (dementia OR parkinson*)...
November 6, 2018: International Psychogeriatrics
Akio Sakamoto, Ryuzo Arai, Takeshi Okamoto, Yosuke Yamada, Hodaka Yamakado, Shuichi Matsuda
BACKGROUND Ischemic fasciitis is a rare condition that occurs in debilitated and immobilized individuals, usually overlying bony protuberances. Because the histology shows a pseudosarcomatous proliferation of atypical fibroblasts, and because the lesion can increase in size, ischemic fasciitis can mimic sarcoma. Beta-propeller protein-associated neurodegeneration (BPAN) arises in infancy and is due to mutations in the WDR45 gene on the X chromosome. BPAN results in progressive symptoms of dystonia, Parkinsonism, and dementia once the individual reaches adolescence or early adulthood, and is usually fatal before old age...
October 20, 2018: American Journal of Case Reports
Colin P Doherty, Eoin O'Keeffe, James Keaney, Brian Lawlor, Robert F Coen, Michael Farrell, Matthew Campbell
A history of brain trauma has long been acknowledged as increasing an individual's risk of developing dementia in later life. The underlying mechanisms that belie this pre-disposition are, however, very poorly understood. Here, we report a clinical-neuropathological correlation of a man who presented at the age of 66 with a progressive complex atypical dementia with early and prominent neurobehavioral symptoms. His neurological condition continued to decline up to his death at the age of 74. During the compilation of his clinical history, it was established that the subject had experienced a single severe traumatic brain injury (TBI) aged 12 years in 1954 resulting in loss of consciousness, hospitalization, and coma for a number of days after which he was deemed to have recovered...
October 19, 2018: Clinical Neuropathology
Iñigo Gabilondo, Verónica Llorens, Trinidad Rodriguez, Manuel Fernández, Tomas Pérez Concha, Marian Acera, Beatriz Tijero, Ane Murueta-Goyena, Rocío Del Pino, Jesús Cortés, Juan Carlos Gómez-Esteban
PURPOSE: To identify myocardial sympathetic denervation patterns suggestive of Lewy body (LB) pathology in patients with genetic and idiopathic parkinsonisms by 123 I-metaiodobenzylguanidine (MIBG) scintigraphy. METHODS: We retrospectively analysed myocardial MIBG images acquired with a dual-head gamma camera and low-energy high-resolution collimator (LEHR) in 194 patients with suspected synucleinopathy or atypical parkinsonism, including 34 with genetic Parkinson's disease (PD; 4 PARK1, 8 PARK2 and 22 PARK8), 85 with idiopathic PD (iPD), 6 with idiopathic REM sleep behaviour disorder (iRBD), 17 with dementia with LB (DLB), 40 with multiple system atrophy (MSA) and 12 with progressive supranuclear palsy (PSP), and in 45 healthy controls...
October 15, 2018: European Journal of Nuclear Medicine and Molecular Imaging
J Cummings, C Ballard, P Tariot, R Owen, E Foff, J Youakim, J Norton, S Stankovic
Psychosis is common across dementia types with a prevalence of 20% to 70%. Currently, no pharmacologic treatment is approved for dementia-related psychosis. Atypical antipsychotics are frequently used to treat these disorders, despite significant safety concerns. Pimavanserin, a selective 5-HT2A inverse agonist/antagonist, was approved in the U.S. for treating hallucinations and delusions associated with Parkinson's disease psychosis (PDP). Patients in the pimavanserin group experienced a significant (p=0.001) improvement in Scale for the Assessment of Positive Symptoms - Parkinson's disease (SAPS-PD) scores vs...
2018: Journal of Prevention of Alzheimer's Disease
Davide Vito Moretti
AIMS: Success in treating patients with atypical parkinsonian syndromes, namely progressive supranuclear palsy (PSP), cortico-basal degeneration (CBD), multiple system atrophy (MSA), Parkinson's disease with dementia (PDD), and Lewy body dementia with (LBD), remains exceedingly low. The present work overviews the most influential research literature collected on MEDLINE, ISI Web of Science, Cochrane Library, and Scopus for available treatment in atypical parkinsonisms without time restriction...
October 7, 2018: CNS Neuroscience & Therapeutics
Jochen Hammes, Alexander Drzezga, Thilo van Eimeren
PURPOSE OF REVIEW: Differential diagnosis of atypical Parkinson syndromes (APS) is difficult as clinical presentations may vary and as there is a strong overlap between disease entities. Aggregations of misfolded and hyperphosphorylated tau proteins are the common denominator of many of these diseases. RECENT FINDINGS: Several tau targeting positron emission tomography (PET) tracers have been evaluated as possible biomarkers in APS in the recent years. For Parkinson's disease, dementia with Lewy bodies, progressive supranuclear palsy, and corticobasal degeneration, promising results have been reported with regard to the ability to detect the presence of disease and to discriminate patients from controls...
October 6, 2018: Current Neurology and Neuroscience Reports
Toshihide Ogawa, Shinya Fujii, Keita Kuya, Shin-Ichiro Kitao, Yuki Shinohara, Mana Ishibashi, Yoshio Tanabe
An accurate diagnosis of Parkinson's disease (PD) is a prerequisite for therapeutic management. In spite of recent advances in the diagnosis of parkinsonian disorders, PD is misdiagnosed in between 6 and 25% of patients, even in specialized movement disorder centers. Although the gold standard for the diagnosis of PD is a neuropathological assessment, neuroimaging has been playing an important role in the differential diagnosis of PD and is used for clinical diagnostic criteria. In clinical practice, differential diagnoses of PD include atypical parkinsonian syndromes such as dementia with Lewy bodies, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, caused by a striatal dopamine deficiency following nigrostrial degeneration...
September 2018: Yonago Acta Medica
William J Bosl
Mental, neurological, and neurodevelopmental (MNN) disorders impose an enormous burden of disease globally. Many MNN disorders follow a developmental trajectory. Thus, defining symptoms of MNN disorders may be conceived as the end product of a long developmental process. Many pharmaceutical therapies are aimed at the end symptoms, essentially attempting to reverse pathological brain function that has developed over a long time. A new paradigm is needed to leverage the developmental trajectory of MNN disorders, based on measuring brain function through the life span...
2018: Neurodiagnostic Journal
A Lunati, S Lesage, A Brice
The cause of Parkinson's disease (PD) remains unknown in most patients. Since 1997, with the first genetic mutation known to cause PD described in SNCA gene, many other genes with Mendelian inheritance have been identified. We summarize genetic, clinical and neuropathological findings related to the 27 genes reported in the literature since 1997, associated either with autosomal dominant (AD): LRRK2, SNCA, VPS35, GCH1, ATXN2, DNAJC13, TMEM230, GIGYF2, HTRA2, RIC3, EIF4G1, UCHL1, CHCHD2, and GBA; or autosomal recessive (AR) inheritance: PRKN, PINK1, DJ1, ATP13A2, PLA2G6, FBXO7, DNAJC6, SYNJ1, SPG11, VPS13C, PODXL, and PTRHD1; or an X-linked transmission: RAB39B...
September 20, 2018: Revue Neurologique
Abdul Qayyum Rana, Abdul Rehman Qureshi, Omer Siddiqui, Zainab Sarfraz, Ruqqiyah Rana, Alexander Shtilbans
Pain is common in atypical parkinsonism such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and dementia with Lewy bodies (LBD). In this study, a systematic review and meta-analysis was conducted and peer-reviewed literature was searched to determine the prevalence and types of pain in four atypical parkinsonism syndromes: MSA, PSP, CBD and LBD. The results show that pain was prevalent mainly in MSA patients in comparison to PSP and CBD patients. Pain was reported at an early stage and was found in females, with limb pain being the most common, followed by neck and back pain...
September 20, 2018: Journal of Neurology
Gregory S Day, Erik S Musiek, John C Morris
BACKGROUND: Published approaches to the evaluation and management of patients with rapidly progressive dementia (RPD) have been largely informed by experience at academic hospitals and national centers specializing in the diagnosis of Creutzfeldt-Jakob disease. Whether these approaches can be applied to patients assessed within lower-acuity outpatient settings is unknown. METHODS: A total of 96 patients with suspected RPD were assessed within the Washington University School of Medicine (Saint Louis, MO) outpatient memory clinic from February 2006 to February 2016...
October 2018: Alzheimer Disease and Associated Disorders
Amanda M Liesinger, Neill R Graff-Radford, Ranjan Duara, Rickey E Carter, Fadi S Hanna Al-Shaikh, Shunsuke Koga, Kelly M Hinkle, Sarah K DiLello, McKenna F Johnson, Adel Aziz, Nilufer Ertekin-Taner, Owen A Ross, Dennis W Dickson, Melissa E Murray
Women reportedly make up two-thirds of Alzheimer's disease (AD) dementia sufferers. Many estimates regarding AD, however, are based on clinical series lacking autopsy confirmation. The Florida Autopsied Multi-Ethnic (FLAME) cohort was queried for AD cases with a total of 1625 identified ranging in age from 53 to 102 years at death. Standard neuropathologic procedures were employed and clinical information was retrospectively collected. Clinicopathologic and genetic data (MAPT and APOE) were stratified by sex...
September 15, 2018: Acta Neuropathologica
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