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Apathy and progressive supranuclear palsy

Ruwei Ou, Wei Song, Qianqian Wei, Ke Chen, Bei Cao, Yanbing Hou, Bi Zhao, Huifang Shang
Objectives. To explore the clinical correlates of nonmotor symptoms (NMS) in progressive supranuclear palsy (PSP) and their differences from healthy controls and patients with Parkinson's disease (PD). Methods. Twenty-seven PSP patients, 27 age- and gender-matched healthy controls (HC), and 27 age- and gender-matched PD patients were included for this case-control study. NMS were assessed using the Nonmotor Symptoms Scale (NMSS, including 9 domains). Results. All PSP patients reported NMS. The frequency and severity of "sleep/fatigue," "mood/apathy," "attention/memory," "gastrointestinal," "sexual dysfunction," and "miscellaneous" domains in PSP group were significantly higher than those in HC group (P < 0...
2016: Parkinson's Disease
Claire Boutoleau-Bretonnière, Christelle Evrard, Jean Benoît Hardouin, Laëtitia Rocher, Tiphaine Charriau, Frédérique Etcharry-Bouyx, Sophie Auriacombe, Aurélie Richard-Mornas, Florence Lebert, Florence Pasquier, Anne Sauvaget, Samuel Bulteau, Martine Vercelletto, Pascal Derkinderen, Cédric Bretonnière, Catherine Thomas-Antérion
BACKGROUND: The diagnosis of behavioral variant of frontotemporal dementia (bvFTD) relies primarily on clinical features and remains challenging. The specificity of the recently revised criteria can be disappointing, justifying development of new clinical tools. OBJECTIVE: We produced a behavioral inventory named DAPHNE. This scale (adapted from Rascovsky's criteria) explores six domains: disinhibition, apathy, perseverations, hyperorality, personal neglect and loss of empathy...
September 2015: Dementia and Geriatric Cognitive Disorders Extra
Biba R Stanton, Alan Carson
Apathy is an under-recognised and underestimated problem for people with chronic neurological disorders. Despite being common and disabling, it is seldom volunteered as a symptom by patients or even their caregivers. Yet apathy undoubtedly has an important impact on caregiver stress, functional disability and quality of life. A detailed clinical assessment can distinguish apathy from depression and allow clinicians to make practical suggestions to reduce the impact of symptoms on individual patients and their families...
February 2016: Practical Neurology
Christos Koros, Maria Stamelou
Progressive supranuclear palsy (PSP) an atypical parkinsonian with a common phenotype comprising early falls, the characteristic slowing of vertical saccades and a frontal syndrome with marked apathy (Richardson's syndrome). Currently, no effective symptomatic or neuroprotective treatment is available for PSP. Current medical have a limited role in PSP. Novel experimental treatments include davunetide or tideglusib, both inhibitors of glycogen synthase kinase-3 (GSK-3) that failed to improve the clinical outcome of PSP patients in two recent studies...
January 2016: Parkinsonism & related Disorders
Rik Ossenkoppele, Yolande A L Pijnenburg, David C Perry, Brendan I Cohn-Sheehy, Nienke M E Scheltens, Jacob W Vogel, Joel H Kramer, Annelies E van der Vlies, Renaud La Joie, Howard J Rosen, Wiesje M van der Flier, Lea T Grinberg, Annemieke J Rozemuller, Eric J Huang, Bart N M van Berckel, Bruce L Miller, Frederik Barkhof, William J Jagust, Philip Scheltens, William W Seeley, Gil D Rabinovici
A 'frontal variant of Alzheimer's disease' has been described in patients with predominant behavioural or dysexecutive deficits caused by Alzheimer's disease pathology. The description of this rare Alzheimer's disease phenotype has been limited to case reports and small series, and many clinical, neuroimaging and neuropathological characteristics are not well understood. In this retrospective study, we included 55 patients with Alzheimer's disease with a behavioural-predominant presentation (behavioural Alzheimer's disease) and a neuropathological diagnosis of high-likelihood Alzheimer's disease (n = 17) and/or biomarker evidence of Alzheimer's disease pathology (n = 44)...
September 2015: Brain: a Journal of Neurology
Tatjana Pekmezović, Milica Ječmenica-Lukić, Igor Petrović, Vladana Špica, Aleksandra Tomić, Vladimir S Kostić
The aim of this prospective cohort study that included 46 patients with progressive supranuclear palsy (PSP) was to estimate which demographic and clinical factors were the main contributors to the health-related quality of life (HRQoL) and how did the HRQoL change over a follow-up period of 1 year in these patients. The hierarchical regression analyses showed that the final models demonstrated that gender, included clinical variables and psychiatric/neuropsychological scales, accounted for 68% of the variance in the Physical Composite Score and 73% of the variance of the Mental Composite Score of the 36-item Short Form Health Survey (SF-36)...
September 2015: Journal of Neurology
Akihiro Shindo, Yukito Ueda, Shigeki Kuzuhara, Yasumasa Kokubo
BACKGROUND: The Kii peninsula of Japan is one of the foci of amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in the world. The purpose of this study is to clarify the neuropsychological features of the patients with ALS/PDC of the Kii peninsula (Kii ALS/PDC). METHODS: The medical interview was done on 13 patients with Kii ALS/PDC, 12 patients with Alzheimer's disease, 10 patients with progressive supranuclear palsy, 10 patients with frontotemporal lobar degeneration and 10 patients with dementia with Lewy bodies...
2014: BMC Neurology
James R Burrell, John R Hodges, James B Rowe
Corticobasal syndrome (CBS) and progressive supranuclear palsy (PSP) represent challenging neurodegenerative disorders for clinicians and nonclinical scientists alike. Although initially lumped together as "Parkinson's-Plus" syndromes, CBS and PSP are clinically and pathologically distinct from Parkinson's disease. It is now clear that behavioral and cognitive changes are common in both syndromes and affect impact quality of life and carer burden considerably. We briefly review the clinical, pathological, and neuroradiological features of each syndrome, followed by more detailed descriptions of the behavioral and cognitive deficits encountered in CBS and PSP...
April 15, 2014: Movement Disorders: Official Journal of the Movement Disorder Society
Federica Agosta, Sebastiano Galantucci, Marina Svetel, Milica Ječmenica Lukić, Massimiliano Copetti, Kristina Davidovic, Aleksandra Tomić, Edoardo G Spinelli, Vladimir S Kostić, Massimo Filippi
White matter (WM) tract alterations were assessed in patients with progressive supranuclear palsy (PSP) relative to healthy controls and patients with idiopathic Parkinson's disease (PD) to explore the relationship of WM tract damage with clinical disease severity, performance on cognitive tests, and apathy. 37 PSP patients, 41 PD patients, and 34 healthy controls underwent an MRI scan and clinical testing to evaluate physical disability, cognitive impairment, and apathy. In PSP, the contribution of WM tract damage to global disease severity and cognitive and behavioural disturbances was assessed using Random Forest analysis...
May 2014: Journal of Neurology
Eduardo Tolosa, Irene Litvan, Günter U Höglinger, David Burn, Andrew Lees, María V Andrés, Belén Gómez-Carrillo, Teresa León, Teodoro Del Ser
It is believed that glycogen synthase kinase-3 (GSK-3) hyperphosphorylates tau protein in progressive supranuclear palsy (PSP). The Tau Restoration on PSP (TAUROS) study was a double-blind, placebo-controlled, randomized trial to assess the efficacy, safety, and tolerability of tideglusib, a GSK-3 inhibitor, as potential treatment for PSP. The study enrolled 146 PSP patients with mild-to-moderate disease who were randomized to receive once-daily 600 mg tideglusib, 800 mg tideglusib, or placebo (ratio, 2:2:1) administered orally over 52 weeks...
April 2014: Movement Disorders: Official Journal of the Movement Disorder Society
Simona Spaccavento, Marina Del Prete, Angela Craca, Anna Loverre
BACKGROUND: Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progressive apraxia of speech (AOS) and aphasia. AIM: We aimed to investigate the clinical presentation of PSP, using a comprehensive multidimensional evaluation, and the disease response to various pharmacological treatments. METHODS: A 72-year-old right-handed male, with 17 years education, who first presented with aphasia, AOS, depression, apathy, and postural instability at 69 years; a complete neuropsychological evaluation, tapping the different cognitive domains, was performed...
2014: Clinical Interventions in Aging
Adam Gerstenecker, Kevin Duff, Benjamin Mast, Irene Litvan
Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder in which, classically, patients present with postural instability and falls, parkinsonism, and slowing of vertical saccades. PSP patients typically have deficits in cognitive functioning, difficulties with most daily activities, and present with notable behavioral disturbances-particularly apathy, impulsivity, and irritability. Using data from 154 patients meeting criteria for clinically probable PSP, domain and total scores of the Neuropsychiatric Inventory were examined and compared to demographics, disease severity, cognition, and motor features...
December 30, 2013: Psychiatry Research
Biba R Stanton, P Nigel Leigh, Robert J Howard, Gareth J Barker, Richard G Brown
Apathy is a neurocognitive syndrome of reduced goal-directed behaviour and is an important cause of disability in neurodegenerative disorders. Frontal-subcortical dysfunction is thought to be important in apathy, but the contribution of individual brain regions to different aspects of the apathy syndrome is poorly understood. We aimed to test the hypotheses that apathy in two distinct neurodegenerative disorders would be associated with frontal lobe atrophy and that reduced initiative and emotional blunting would be associated with distinct patterns of atrophy in functionally relevant brain areas...
October 2013: Journal of Neurology
Marla B Bruns, Keith A Josephs
Corticobasal syndrome (CBS) and progressive supranuclear palsy syndrome (PSPS) are two of the atypical Parkinsonism syndromes, in that patients exhibit rigidity, occasional tremor and postural instability, but do not symptomatically respond to dopamine replacement. CBS and PSPS can often present with complex cognitive difficulties and neuropsychiatric disturbances. Symptoms of depression, apathy, or agitation can be subtle and are often overlooked as reactions to learning a new diagnosis of Parkinsonism. These symptoms may be the earliest presenting evidence of CBS or PSPS, and these syndromes can be misdiagnosed with a primary psychiatric disorder rather than a neurodegenerative condition...
April 2013: International Review of Psychiatry
Kerstin Morcinek, Christoph Köhler, Jürgen Götz, Hannsjörg Schröder
The early occurrence of brainstem-related symptoms, e.g. gait and balance impairment, apathy and depression in Alzheimer's disease patients suggests brainstem involvement in the initial pathogenesis. To address the question whether tau filament forming mice expressing mutated human tau mirror histopathological changes observed in Alzheimer brainstem, the degree and distribution of neurofibrillary lesions as well as the pattern of cholinergic and monoaminergic neurons were investigated. The expression of the human tau transgene was observed in multiple brainstem nuclei, particularly in the magnocellular reticular formation, vestibular nuclei, cranial nerve motor nuclei, sensory trigeminal nerve nuclei, inferior and superior colliculi, periaqueductal and pontine gray matter, and the red nucleus...
February 25, 2013: Brain Research
Jeffrey C L Looi, Matthew D Macfarlane, Mark Walterfang, Martin Styner, Dennis Velakoulis, Jimmy Lätt, Danielle van Westen, Christer Nilsson
Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized by gait and postural disturbance, gaze palsy, apathy, decreased verbal fluency and dysexecutive symptoms, with some of these clinical features potentially having origins in degeneration of frontostriatal circuits and the mesencephalon. This hypothesis was investigated by manual segmentation of the caudate and putamen on MRI scans, using previously published protocols, in 15 subjects with PSP and 15 healthy age-matched controls...
November 30, 2011: Psychiatry Research
Junko Ishizaki, Masaru Mimura
Dysthymia is a depressive mood disorder characterized by chronic and persistent but mild depression. It is often difficult to be distinguished from major depression, specifically in its partially remitted state because "loss of interest" or "apathy" tends to prevail both in dysthymia, and remitted depression. Apathy may also occur in various psychiatric and neurological disorders, including schizophrenia, stroke, Parkinson's disease, progressive supranuclear palsy, Huntington's disease, and dementias such as Alzheimer's disease, vascular dementia, and frontotemporal dementia...
2011: Depression Research and Treatment
Keith A Josephs, Jennifer L Whitwell, Scott D Eggers, Matthew L Senjem, Clifford R Jack
BACKGROUND: Behavioral changes occur in progressive supranuclear palsy. This study aimed to identify the anatomic correlate of behavioral severity in progressive supranuclear palsy. METHODS: We performed standardized tests of behavioral severity (Frontal Behavioral Inventory), cognitive severity (Mini-Mental State Examination), motor severity (Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale Part III), and a 3.0-T volumetric head magnetic resonance imaging scan in 18 prospectively recruited subjects meeting National Institute of Neurological Diseases and Stroke-Society of Progressive Supranuclear Palsy criteria for probable progressive supranuclear palsy...
February 15, 2011: Movement Disorders: Official Journal of the Movement Disorder Society
Yusuke Yatabe, Mamoru Hashimoto, Keiichirou Kaneda, Kazuki Honda, Yusuke Ogawa, Seiji Yuuki, Shiho Matsuzaki, Atsuko Tuyuguchi, Hiroko Kashiwagi, Manabu Ikeda
BACKGROUND: Progressive supranuclear palsy (PSP) is a neurodegenerative disease characterized by supranuclear gaze palsy, postural instability, akinesia and other parkinsonism. Recently, the relationship between PSP and frontotemporal dementia (FTD) has been recognized, which includes clinical, pathological, biochemical and genetic features. However, there have been few studies that directly compared neuropsychiatric symptoms between PSP and FTD. The aim of the present study was to investigate comprehensive psychiatric and behavioural symptoms in PSP and compared them with those in FTD...
March 2011: Psychogeriatrics: the Official Journal of the Japanese Psychogeriatric Society
Pia Kvickström, Bengt Eriksson, Danielle van Westen, Jimmy Lätt, Christina Elfgren, Christer Nilsson
BACKGROUND: The clinical presentation in progressive supranuclear palsy (PSP), an atypical parkinsonian disorder, includes varying degrees of frontal dysexecutive symptoms. Using diffusion tensor imaging (DTI) and tractography (DTT), we investigated whether diffusion changes and atrophy of the inferior fronto-occipital fasciculus (IFO) occurs in PSP and if these changes correlate with disease stage and clinical phenotype. The corticospinal tract (CST), which is often involved in PSP, was investigated for comparison...
2011: BMC Neurology
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