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Atypical dementia

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...
September 14, 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
Andreas Traschütz, Michael Thomas Heneka
Niemann-Pick type C disease (NPC) is a neurovisceral lysosomal storage disorder with a heterogeneous phenotype including ataxia, cognitive impairment, impairment of vertical saccades, and psychiatric symptoms, among many others. Based on clinical, genetic, and biomarker findings, recent guidelines put forward a screening for atypical and oligosymptomatic forms of NPC in clinical niches with an increased risk. Here, we report methods and results of a negative screening study in the niche of a memory clinic. We retrospectively and prospectively identified 83 patients with unclassified cognitive impairment (15 dementia, 46 mild cognitive impairment, and 22 progressive subjective cognitive decline) before 60 years of age (82 patients between 41 and 60 years)...
September 6, 2018: JIMD Reports
Sara Hall, Shorena Janelidze, Yulia Surova, Håkan Widner, Henrik Zetterberg, Oskar Hansson
Inflammation has been implicated in the pathogenesis of Parkinson's disease (PD). We here investigate levels of inflammatory biomarkers in cerebrospinal fluid (CSF) in PD and atypical parkinsonian disorders (APD) compared with neurologically healthy controls. We included 131 patients with PD and 27 PD with dementia (PDD), 24 with multiple system atrophy (MSA), 14 with progressive supranuclear palsy (PSP) and 50 controls, all part of the Swedish BioFINDER study. CSF was analyzed for CRP, SAA, IL-6, IL-8, YKL-40 and MCP-1 (CCL2) as well as α-synuclein (α-syn), tau, tau phosphorylated at Thr181 (P-tau), Aβ42 and NfL...
September 5, 2018: Scientific Reports
Inga Zerr, Peter Hermann
Rapidly progressive dementia is a syndrome caused by numerous disease entities. Accurate diagnosis is crucial as substantial proportion of these diseases is highly treatable. Others might implicate specific hygienic problems. Still, differential diagnosis remains challenging because of a huge overlap of clinical presentations. Areas covered: The paper reviews PubMed-listed research articles with a focus on diagnosis and treatment of diseases showing rapid cognitive decline such as inflammatory diseases, rapidly progressive neurodegenerative diseases, toxic-metabolic encephalopathies and prion diseases...
September 17, 2018: Expert Review of Neurotherapeutics
Ralf Kozian, Gregor Schmid, Sergiu Demian
Disturbances of thermoregulation are a well-known side effect of neuroleptic treatment, especially hyperthermia. Hypothermia can occur also during treatment with neuroleptics. Now hypothermia is more and more due to atypical neuroleptics in relation to 5-HT2A antagonism. We report the case of a patient (92 years) suffering from dementia with a hypothermia during treatment with aripiprazole.
August 27, 2018: Psychiatrische Praxis
Ignazio Cali, Fadi Mikhail, Kefeng Qin, Crystal Gregory, Ani Solanki, Manuel Camacho Martinez, Lili Zhao, Brian Appleby, Pierluigi Gambetti, Eric Norstrom, James A Mastrianni
Objective: To describe the clinicopathologic, molecular, and transmissible characteristics of genetic prion disease in a young man carrying the PRNP -G114V variant. Methods: We performed genetic, histologic, and molecular studies, combined with in vivo transmission studies and in vitro replication studies, to characterize this genetic prion disease. Results: A 24-year-old American man of Polish descent developed progressive dementia, aphasia, and ataxia, leading to his death 5 years later...
August 2018: Neurology. Genetics
Dae Lim Koo, Jee Young Lee, Hyunwoo Nam
OBJECTIVE: Rapid eye movement sleep behavior disorder (RBD) is a common sleep disturbance in patients with neurodegenerative disorders. We aimed to compare sleep parameters among the different types of RBD patients. METHODS: A total of 122 patients with dream enactment behavior were screened. Of these, 92 patients who were diagnosed with RBD by polysomnography were included in this study. Enrolled patients with RBD were classified into four groups based on the following diagnoses: idiopathic RBD (iRBD); RBD with Parkinson disease (PD-RBD); multiple system atrophy (MSA) with RBD (MSA-RBD); and dementia with Lewy bodies (DLB) with RBD (DLB-RBD)...
September 2018: Sleep Medicine
Daniela Monaco, Daniela Berg, Astrid Thomas, Vincenzo Di Stefano, Filomena Barbone, Michela Vitale, Camilla Ferrante, Laura Bonanni, Marta Di Nicola, Tonia Garzarella, Luciano Paolo Marchionno, Giovanni Malferrari, Rocco Di Mascio, Marco Onofrj, Raffaella Franciotti
Transcranial sonography (TCS) is a noninvasive, easily performed, and commonly available neuroimaging technique useful for the study of brain parenchyma in movement disorders. This tool has been increasingly used in the diagnosis of Parkinson's disease and atypical parkinsonism. The aim of the study was to evaluate the applicability of this technique as supportive tool in the early diagnosis of movement disorders. We performed TCS on 315 individuals which were diagnosed as healthy controls or affected by idiopathic Parkinson's disease, monogenetic subtypes of Parkinson's disease, atypical parkinsonism, and Dementia with Lewy bodies...
August 7, 2018: Neurological Sciences
Hirohisa Watanabe, Yuichi Riku, Kazuhiro Hara, Kazuya Kawabata, Tomohiko Nakamura, Mizuki Ito, Masaaki Hirayama, Mari Yoshida, Masahisa Katsuno, Gen Sobue
Multiple system atrophy (MSA) is an adult-onset, progressive neurodegenerative disorder. Patients with MSA show various phenotypes during the course of their illness, including parkinsonism, cerebellar ataxia, autonomic failure, and pyramidal signs. Patients with MSA sometimes present with isolated autonomic failure or motor symptoms/ signs. The median duration from onset to the concomitant appearance of motor and autonomic symptoms is approximately 2 years but can range up to 14 years. As the presence of both motor and autonomic symptoms is essential for the current diagnostic criteria, early diagnosis is difficult when patients present with isolated autonomic failure or motor symptoms/signs...
August 9, 2018: Journal of Movement Disorders
K Egger, M Rijntjes
The differential diagnosis of atypical dementia remains difficult. The use of positron emission tomography (PET) still represents the gold standard for imaging diagnostics. According to the current evidence, however, magnetic resonance imaging (MRI) is almost equal to fluorodeoxyglucose (FDG)-PET, but only when using new big data and machine learning methods. In cases of atypical dementia, especially in younger patients and for follow-up, MRI is preferable to computed tomography (CT). In the clinical routine, promising MRI procedures are e...
August 2018: Der Nervenarzt
R Laforce, J P Soucy, L Sellami, C Dallaire-Théroux, F Brunet, D Bergeron, B L Miller, R Ossenkoppele
Molecular imaging techniques using 18 F-fluorodeoxyglucose, amyloid tracers, and, more recently, tau ligands have taken dementia research by storm and undoubtedly improved our understanding of neurodegenerative diseases. The ability to image in vivo the pathological substrates of degenerative diseases and visualize their downstream impact has led to improved models of pathogenesis, better differential diagnosis of atypical conditions, as well as focused subject selection and monitoring of treatment in clinical trials aimed at delaying or preventing the symptomatic phase of Alzheimer's disease...
July 17, 2018: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
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