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Atypical dementia

Zoltan Lukacs, Miriam Nickel, Simona Murko, Paulina Nieves Cobos, Angela Schulz, René Santer, Alfried Kohlschütter
PURPOSE: CLN2 disease is a genetic disorder caused by dysfunction of the lysosomal enzyme tripeptidyl peptidase 1 (TPP1) that belongs to the neuronal ceroid lipofuscinoses (NCL) and leads to epilepsy, dementia, and death in young persons. CLN2 disease has recently become treatable by enzyme replacement, which can only be effective when the disease is diagnosed early. We have investigated the reliability of a test for TPP1 deficiency in dried blood specimens (DBS) to detect CLN2 disease...
February 13, 2019: Clinica Chimica Acta; International Journal of Clinical Chemistry
Raya Elfadel Kheirbek, Ali Fokar, John T Little, Marshall Balish, Nawar M Shara, Malaz A Boustani, Maria Llorente
Background: Antipsychotics are prescribed to treat various symptoms in older adults, however, their safely in this context has not been fully evaluated. The objective was to evaluate mortality risks associated with off- label use of antipsychotics among older adults with no pre-existing mental illness or dementia relative to those with diagnosis of dementia. Methods: Data (2007-2015) were derived from Department of Veterans Affairs registries for 730,226 patients (≥65 years) with no baseline serious mental illness, dementia)...
February 11, 2019: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
Mariana Nassif Kerbauy, Denise da Cunha Pasqualin, Jerusa Smid, Rogerio Iquizli, Lucila Nassif Kerbauy, Ricardo Nitrini, Guilherme Carvalhal Ribas, Luciano Neder, Nelson Hamerschlak
RATIONALE: Lymphomatosis cerebri is a rare form of PCNSL, characterized by diffuse infiltration of lymphoma cells in cerebral parenchyma, without mass-formation and mild or no contrast enhancement on magnetic resonance (MR) imaging. There are less than 50 cases described in the literature under the term Lymphomatosis cerebri. PATIENT CONCERNS: A 74-year-old man presented to our service with progressive dementia for 12 months and accelerated cognitive decline within the last two months...
February 2019: Medicine (Baltimore)
Toshinari Odawara, Yuta Manabe, Osamu Konishi
BACKGROUND: Dementia with Lewy bodies (DLB) is a progressive form of dementia, accompanied by a range of behavioural and psychological symptoms. The aim of this study was to identify current clinical practice for the treatment of DLB in Japan. METHODS: We conducted a survey of medical doctors engaged in the management of dementia in Japan. Participants were divided into two groups: psychiatrists (Group P) and neurologists or neurosurgeons (Group NS). Doctors completed a questionnaire and we analysed their responses to compare the two groups with regard to diagnosis and treatment of DLB, and in particular the treatment of behavioural and psychological symptoms of dementia (BPSD)...
February 5, 2019: Psychogeriatrics: the Official Journal of the Japanese Psychogeriatric Society
Tomoya Kon, Fumiaki Mori, Akira Arai, Yasuo Miki, Kunikazu Tanji, Hidekachi Kurotaki, Masahiko Tomiyama, Koichi Wakabayashi
Globular glial tauopathy (GGT) is a group of 4-repeat tauopathies characterized by widespread globular glial inclusions (GGIs). GGT is now classified into three subtypes based on the distribution and morphology of the GGIs. We report an autopsy case of GGT in an 85-year-old woman who presented with semantic dementia, a rare phenotype in GGT. Postmortem examination revealed marked atrophy of the frontotemporal and motor cortices and corticospinal tract degeneration with widespread occurrence of globular neurofibrillary tangles and GGIs...
February 5, 2019: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Linda A Hershey, Rhonda Coleman-Jackson
Dementia with Lewy bodies (DLB) is a complex disease that involves a variety of cognitive, behavioral and neurological symptoms, including progressive memory loss, visual hallucinations, parkinsonism, cognitive fluctuations and rapid eye movement sleep behavior disorder (RBD). These symptoms may appear in varying combinations and levels of severity in each patient who is seen in the clinic, making diagnosis and treatment a challenge. DLB is the third most common of all the neurodegenerative diseases behind both Alzheimer's disease and Parkinson's disease (PD)...
January 25, 2019: Drugs & Aging
Abdalla Z Mohamed, Paul Cumming, Jürgen Götz, Fatima Nasrallah
PURPOSE: Traumatic brain injury (TBI) and posttraumatic stress disorder (PTSD) have emerged as independent risk factors for an earlier onset of Alzheimer's disease (AD), although the pathophysiology underlying this risk is unclear. Postmortem studies have revealed extensive cerebral accumulation of tau following multiple and single TBI incidents. We hypothesized that a history of TBI and/or PTSD may induce an AD-like pattern of tau accumulation in the brain of nondemented war veterans...
January 7, 2019: European Journal of Nuclear Medicine and Molecular Imaging
Giuseppe Di Fede, Marcella Catania, Cristiana Atzori, Fabio Moda, Claudio Pasquali, Antonio Indaco, Marina Grisoli, Marta Zuffi, Maria Cristina Guaita, Roberto Testi, Stefano Taraglio, Maria Sessa, Graziano Gusmaroli, Mariacarmela Spinelli, Giulia Salzano, Giuseppe Legname, Roberto Tarletti, Laura Godi, Maurizio Pocchiari, Fabrizio Tagliavini, Daniele Imperiale, Giorgio Giaccone
Prion diseases are neurodegenerative disorders which are caused by an accumulation of the abnormal, misfolded prion protein known as scrapie prion protein (PrPSc ). These disorders are unique as they occur as sporadic, genetic and acquired forms. Sporadic Creutzfeldt-Jakob Disease (CJD) is the most common human prion disease, accounting for approximately 85-90% of cases, whereas autosomal dominant genetic forms, due to mutations in the prion protein gene (PRNP), account for 10-15% of cases. Genetic forms show a striking variability in their clinical and neuropathological picture and can sometimes mimic other neurodegenerative diseases...
January 3, 2019: Acta Neuropathologica Communications
Rhian Convery, Simon Mead, Jonathan D Rohrer
Frontotemporal dementia (FTD) is a heterogeneous group of disorders causing neurodegeneration within a network of areas centred on the frontal and temporal lobes. Clinically, patients present with behavioural symptoms (behavioural variant FTD) or language disturbance (primary progressive aphasia), although there is an overlap with motor neurone disease and atypical parkinsonian disorders. Whilst neuroimaging commonly reveals abnormalities in the frontal and temporal lobes, a closer review identifies a more complex picture with a variable asymmetry of neuronal loss, widespread subcortical involvement, and in many cases more posterior cortical atrophy...
December 24, 2018: Neuropathology and Applied Neurobiology
Bushra Elhusein, Ahmed Radwan, Abdalla Khairi, Mohamed Ahmed
We present the case of a 34-year-old Jordanian woman who was referred to mainstream mental health services because of irritability, agitation, loss of appetite, withdrawal from family activities and sleeping difficulties. She was initially diagnosed with major depressive disorder but subsequently showed very poor response to antidepressant therapy. Her presentation gradually and dramatically progressed into full blown dementia within couple of years. Brain MRI showed atrophic cortical changes and subcortical white matter alterations consistent with Alzheimer's dementia...
December 3, 2018: BMJ Case Reports
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)...
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...
January 2019: Clinical Neuropathology
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