The Experts below are selected from a list of 693 Experts worldwide ranked by ideXlab platform
Murray Grossman - One of the best experts on this subject based on the ideXlab platform.
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Semantic categorisation of novel objects in frontotemporal dementia
Cognitive Neuropsychology, 2020Co-Authors: Phyllis Koenig, Edward E. Smith, Murray GrossmanAbstract:Impaired semantic memory is ubiquitous in frontotemporal dementia (FTD), including patients with semantic dementia (SD), Progressive Nonfluent Aphasia (PNFA) and nonaphasic FTD patients with a deficit in executive and social functioning (EXEC/SOC). One hypothesis attributes this to the degradation of specific categories of knowledge in semantic memory. This study explores the alternate hypothesis that impaired semantic memory in FTD can also reflect limitations in the categorisation processes that determine object meaning. Patients were taught a novel semantic category under two conditions: rule-based categorisation, where executive resources support the evaluation of specific features to determine category membership; and similarity-based categorisation, where category membership is determined by the overall resemblance of an item to a prototype or recalled exemplars. In the first experiment, patients learned a novel category composed of highly salient features. For SD patients, we found category members...
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Social cognition, executive functioning, and neuroimaging correlates of empathic deficits in frontotemporal dementia.
Journal of Neuropsychiatry and Clinical Neurosciences, 2011Co-Authors: Paul J. Eslinger, Peachie Moore, Chivon Anderson, Murray GrossmanAbstract:The authors investigated aspects of interpersonal sensitivity and perspective-taking in relation to empathy, social cognitions, and executive functioning in 26 frontotemporal dementia (FTD) patients. Behavioral-variant FTD (bvFTD) patients were significantly impaired on caregiver assessments of empathy, although self-ratings were normal. Progressive Nonfluent Aphasia and semantic-dementia samples were rarely abnormal. In bvFTD, empathy ratings were found to be correlated with social cognition and executive functioning measures, but not depression. Voxel-based morphometry revealed that reduced empathic perspective-taking was related to bifrontal and left anterior temporal atrophy, whereas empathic emotions were related to right medial frontal atrophy. Findings suggest that bvFTD causes multiple types of breakdown in empathy, social cognition, and executive resources, mediated by frontal and temporal disease.
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why are patients with Progressive Nonfluent Aphasia Nonfluent
Neurology, 2010Co-Authors: D Gunawardena, Corey T Mcmillan, Brian B Avants, Murray GrossmanAbstract:Progressive Nonfluent Aphasia (PNFA) is one clinical presentation of frontotemporal lobar degeneration (FTLD).1,2 PNFA may be a clinical marker of tauopathies such as dementia with Pick bodies, corticobasal degeneration (CBD), and Progressive supranuclear palsy (PSP),3,4 and thus it is critical to understand the characteristics of this condition. The hallmark feature of PNFA is reduced speech fluency.5,6 In this study, we investigated 3 potential sources of Nonfluent speech attributed to PNFA, including grammatic simplifications and errors,1,5 a motor-related disorder associated with speech-sound errors known as apraxia of speech (AOS),3,7 and executive difficulty that limits mental search for words.8,9 An executive deficit also is seen in patients with FTLD with a disorder of personality and executive functioning known as behavioral-variant frontotemporal dementia (bvFTD).2,10 Since these patients also have reduced speech fluency,11 it is important to distinguish between reduced fluency in bvFTD and PNFA. In PNFA, left inferior frontal cortex (IFC) atrophy is associated with grammatic processing,12 and an fMRI study shows reduced left IFC recruitment during grammatic processing.13 Patients with PNFA also have atrophy in left insula, an area associated with motor-speech errors,7 and in left dorsolateral prefrontal cortex, an area associated with mental search.14,15 In this study, we related fluency, grammar, executive functioning, and speech-sound production to quantitative measures of cortical thickness. We predicted that Nonfluent speech would be related to limited grammatic processing in PNFA and reduced executive functioning in bvFTD. Moreover, in PNFA, we expected reduced WPM to be associated with IFC thinning, and that this would overlap with regions implicated in grammatic difficulty.
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sentence comprehension and voxel based morphometry in Progressive Nonfluent Aphasia semantic dementia and nonaphasic frontotemporal dementia
Journal of Neurolinguistics, 2008Co-Authors: Jonathan E Peelle, Peachie Moore, Luisa Vesely, Corey T Mcmillan, Vanessa Troiani, Murray GrossmanAbstract:Abstract To investigate the basis for impaired sentence comprehension in patients with frontotemporal dementia (FTD) we assessed grammatical comprehension and verbal working memory in 88 patients with three distinct presentations: Progressive Nonfluent Aphasia (PNFA), semantic dementia (SD), and nonaphasic patients with a disorder of social comportment and executive processing (SOC/EXEC). We related sentence comprehension and working memory performance to regional cortical volume in a subgroup of 29 patients with structural MRI scans using voxel-based morphometry. PNFA patients exhibited the greatest difficulty with sentence comprehension and were especially impaired with grammatically complex sentences, which correlated with atrophy in left inferior frontal cortex. Working memory performance in these same patients correlated with a proximal but distinct left inferior frontal region. SD patients’ sentence comprehension scores correlated with left inferolateral temporal lobe damage, which we hypothesize reflects impairments in lexical processing. We did not observe any consistent relationship between cortical atrophy and sentence comprehension impairment in SOC/EXEC patients, suggesting the deficits in this subgroup may be due to more variable declines in executive resources.
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syntactic and thematic components of sentence processing in Progressive Nonfluent Aphasia and nonaphasic frontotemporal dementia
Journal of Neurolinguistics, 2007Co-Authors: Jonathan E Peelle, Ayanna N Cooke, Peachie Moore, Luisa Vesely, Murray GrossmanAbstract:We used an online word-monitoring paradigm to examine sentence processing in healthy seniors and frontotemporal dementia patients with Progressive Nonfluent Aphasia (PNFA) or a nonaphasic disorder of social and executive functioning (SOC/EXEC). Healthy seniors were sensitive to morphosyntactic, major grammatical subcategory, and selection restriction violations in a sentence. PNFA patients were insensitive to grammatical errors, but showed reasonable sensitivity to thematic matrix violations, consistent with a differential grammatical processing impairment. By contrast, SOC/EXEC patients showed partial sensitivity to grammatical errors but were insensitive to thematic violations. These findings support a dissociation between grammatical and thematic components of sentence processing. Specifically, they are consistent with a grammatical processing deficit in PNFA patients, and impairment in the formation of a coherent thematic matrix in SOC/EXEC patients.
Anna Richardson - One of the best experts on this subject based on the ideXlab platform.
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sporadic creutzfeldt jakob disease presenting as Progressive Nonfluent Aphasia with speech apraxia
Alzheimer Disease & Associated Disorders, 2013Co-Authors: Christopher Kobylecki, Jennifer C Thompson, Matthew Jones, Samantha J Mills, Sandip Shaunak, James W Ironside, Julie S Snowden, Anna RichardsonAbstract:Progressive non-fluent Aphasia (PNFA) is typically associated with pathological changes consistent with frontotemporal lobar degeneration. A 65-year-old male presented with effortful speech, markedly impaired naming and features of speech apraxia, consistent with PNFA. Perceptuospatial function, calculation and executive function were intact. Brain SPECT showed left perisylvian hypoperfusion. He deteriorated profoundly over the subsequent eight months, with appearances on diffusion-weighted magnetic resonance imaging typical of sporadic Creutzfeldt-Jakob disease, which was confirmed pathologically at postmortem examination. While the presence of PNFA with speech apraxia is thought to predict underlying tauopathy, sporadic Creutzfeldt-Jakob disease may mimic this presentation and present in a highly circumscribed form not previously described.
Bruce L. Miller - One of the best experts on this subject based on the ideXlab platform.
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eLS - Frontal Lobe Disorders: Frontotemporal Dementia (Pick Disease)
Encyclopedia of Life Sciences, 2009Co-Authors: Maria Carmela Tartaglia, Bruce L. MillerAbstract:Frontotemporal dementia (FTD) is a neurodegenerative disorder that clinically relates to three distinct phenotypes, behavioural variant FTD, semantic dementia (SD) and Progressive Nonfluent Aphasia (PNFA), each is associated with focal atrophy. The clinical syndromes observed in these illnesses, encompassing diverse symptoms including loss of social skills, apathy, disinhibition, repetitive and compulsive behaviours, Progressive inability to represent the self and others, loss of word meaning, and inability to express oneself, are determined by injury to specific anatomical structures. Frontotemporal lobar degeneration (FTLD) is the pathological term that encompasses all three syndromes. Ubiquitin and tau are two different pathological substrates that have been implicated in these syndromes and their anatomical predilection determines the phenotype. The genetics associated with FTD have also proven to be more diverse than anticipated. These illnesses provide a unique opportunity to investigate clinicoanatomical relationships for specific symptoms. Key concepts Frontotemporal dementia (FTD) is a clinical term that encompasses a heterogeneous group of patients that share focal degeneration within the anterior frontal, temporal and insular regions. This term includes: behavioural variant of FTD (bvFTD), Progressive Nonfluent Aphasia (PNFA) and semantic dementia (SD). Frontotemporal lobar degeneration (FTLD) is the pathological term that encompasses all three syndromes. Behavioural variant frontotemporal dementia (bvFTD) leads to a change in personality and behaviour. PNFA is a disorder of Nonfluent speech and language. SD is a disorder of semantic knowledge for words. FTD is the second most common dementia in those less than 65 years of age as it accounts for 5–6% of all dementias and is responsible for up to 17% of early onset (
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Left frontal lobe contributions to concept formation: a quantitative MRI study of performance on the Delis-Kaplan Executive Function System Sorting Test.
Journal of Clinical and Experimental Neuropsychology, 2009Co-Authors: Eric M. Fine, Bruce L. Miller, Dean C. Delis, David Dean, Victoria Beckman, Howard J. Rosen, Joel H. KramerAbstract:This study examined relationships between lobar volumes and performance on the Delis–Kaplan Executive Function System (D-KEFS) Sorting Test, a standardized measure of concept formation. There were 89 participants: 19 patients with probable Alzheimer's disease, 25 patients with frontotemporal dementia, 13 patients with semantic dementia, 12 patients with Progressive Nonfluent Aphasia, 9 patients with probable Progressive supranuclear palsy, 2 patients with possible Progressive supranuclear palsy, and 9 healthy participants. We used BRAINS2 software to generate volumes of the right and left frontal, temporal, and parietal lobes. Multiple regression analysis indicated that, after controlling for Mini-Mental State Examination scores, intracranial volume, and demographic variables, only the left frontal lobe significantly predicted performance on the D-KEFS Sorting Test.
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The logopenic/phonological variant of primary Progressive Aphasia
Neurology, 2008Co-Authors: Maria Luisa Gorno-tempini, Jennifer M Ogar, Simona Maria Brambati, Nina F Dronkers, Alessandra Marcone, Valeria Ginex, Daniela Perani, Valentina Garibotto, Stefano F. Cappa, Bruce L. MillerAbstract:Objective: Primary Progressive Aphasia (PPA) is characterized by isolated decline in language functions. Semantic dementia and Progressive Nonfluent Aphasia are accepted PPA variants. A “logopenic” variant (LPA) has also been proposed, but its cognitive and anatomic profile is less defined. The aim of this study was to establish the cognitive and anatomic features of LPA. Methods: Six previously unreported LPA cases underwent extensive neuropsychological evaluation and an experimental study of phonological loop functions, including auditory and visual span tasks with digits, letters, and words. For each patient, a voxel-wise, automated analysis of MRI or SPECT data were conducted using SPM2. Results: In LPA, speech rate was slow, with long word-finding pauses. Grammar and articulation were preserved, although phonological parAphasias could be present. Repetition and comprehension were impaired for sentences but preserved for single words, and naming was moderately affected. Investigation of phonological loop functions showed that patients were severely impaired in digit, letter, and word span tasks. Performance did not improve with pointing, was influenced by word length, and did not show the normal phonological similarity effect. Atrophy or decreased blood flow was consistently found in the posterior portion of the left superior and middle temporal gyri and inferior parietal lobule. Conclusions: Logopenic Progressive Aphasia (LPA) is a distinctive variant of primary Progressive Aphasia. Cognitive and neuroimaging data indicate that a deficit in phonological loop functions may be the core mechanism underlying the LPA clinical syndrome. Recent studies suggest that Alzheimer disease may be the most common pathology underlying the LPA clinical syndrome. GLOSSARY: AD = Alzheimer disease; BA = Brodmann area; CDR = Clinical Dementia Rating; CVLT-MS = California Verbal Learning Test–Mental Status Edition; ECD = ethyl cysteinate dimer; FWHM = full-width at half-maximum; GM = gray matter; LPA = logopenic Progressive Aphasia; MMSE = Mini-Mental State Examination; PNFA = Progressive Nonfluent Aphasia; PPA = primary Progressive Aphasia; Rey-O = Rey–Osterrieth; SemD = semantic dementia; VBM = voxel-based morphometry; WAB = Western Aphasia Battery; WAIS-III = Wechsler Adult Intelligence Scale, Third Edition.
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Progressive Nonfluent Aphasia and its characteristic motor speech deficits
Alzheimer Disease & Associated Disorders, 2007Co-Authors: Jennifer M Ogar, Bruce L. Miller, Simona Maria Brambati, Nina F Dronkers, Maria Luisa GornotempiniAbstract:Progressive Nonfluent Aphasia (PNFA) is a clinical le characterized by motor speech impairment and syndrome characterized by motor speech impairment and agrammatism, with relative sparing of single word comprehension and semantic memory. PNFA has been associated with the characteristic pattern of left anterior insular and posterior frontal atrophy, including the motor and premotor regions and Broca's area. Postmortem histopathologic evidence has shown that PNFA is usually associated with tau pathology, although focal Alzheimer disease pathology and tau-negative, ubiquitin-TDP-43 inclusions also have been reported in association with this clinical syndrome. We performed a detailed analysis of motor speech errors in 18 patients with PNFA and investigated their neural correlates using voxel-based morphometry on magnetic resonance imaging scans. Seven patients demonstrated only apraxia of speech (AOS) errors, whereas 11 showed AOS along with dysarthria. Slow rate of speech, effortful articulation with groping, and consonant distortions were the most common AOS errors. Hypernasality was the most represented dysarthric feature and dysarthria was most often classified as spastic, hypokinetic, or mixed spastic-hypokinetic. Neuroimaging results demonstrated that patients with AOS-only and AOS plus dysarthria showed atrophy in the left posterior frontal, anterior insular, and basal ganglia regions when compared with controls. Patients with AOS plus dysarthria showed greater damage than patients with AOS-only in the left face portion of primary motor cortex and left caudate. PNFA is a distinct frontotemporal lobar degeneration clinical syndrome associated with characteristic clinical, neuroimaging, and pathologic features. The clinical features are driven by the severity of left frontal and caudate damage.
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Frontotemporal dementia and semantic dementia: anatomic variations on the same disease or distinctive entities?
Alzheimer Disease & Associated Disorders, 2007Co-Authors: Bruce L. MillerAbstract:: Research into the non-Alzheimer dementias has exploded over the last 2 decades, and frontotemporal lobar degeneration has emerged as the most common cause of dementia in patients below the age of 65. In 1998, an international consortium of investigators focusing on this disease entity met and agreed upon the classification of 3 subtypes of frontotemporal lobar degeneration, using regional atrophy to distinguish them. These are: a frontally predominant form called frontotemporal dementia, a temporally predominant form called semantic dementia, and a left-frontally predominant syndrome, called Progressive Nonfluent Aphasia. With the rise of genetic and neuropathologic findings, however, the usefulness of this subtype classification has been called into question. This paper discusses the major pertinent findings, and the implications of classifying the disease on the basis of atrophy versus genetic or molecular mechanisms.
Jason D. Warren - One of the best experts on this subject based on the ideXlab platform.
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Apraxia in Progressive Nonfluent Aphasia
Journal of Neurology, 2010Co-Authors: Jonathan Daniel Rohrer, Martin N. Rossor, Jason D. WarrenAbstract:The clinical and neuroanatomical correlates of specific apraxias in neurodegenerative disease are not well understood. Here we addressed this issue in Progressive Nonfluent Aphasia (PNFA), a canonical subtype of frontotemporal lobar degeneration that has been consistently associated with apraxia of speech (AOS) and in some cases orofacial apraxia, limb apraxia and/or parkinsonism. Sixteen patients with PNFA according to current consensus criteria were studied. Three patients had a corticobasal syndrome (CBS) and two a Progressive supranuclear palsy (PSP) syndrome. Speech, orofacial and limb praxis functions were assessed using the Apraxia Battery for Adults-2 and a voxel-based morphometry (VBM) analysis was conducted on brain MRI scans from the patient cohort in order to identify neuroanatomical correlates. All patients had AOS based on reduced diadochokinetic rate, 69% of cases had an abnormal orofacial apraxia score and 44% of cases (including the three CBS cases and one case with PSP) had an abnormal limb apraxia score. Severity of orofacial apraxia (but not AOS or limb apraxia) correlated with estimated clinical disease duration. The VBM analysis identified distinct neuroanatomical bases for each form of apraxia: the severity of AOS correlated with left posterior inferior frontal lobe atrophy; orofacial apraxia with left middle frontal, premotor and supplementary motor cortical atrophy; and limb apraxia with left inferior parietal lobe atrophy. Our findings show that apraxia of various kinds can be a clinical issue in PNFA and demonstrate that specific apraxias are clinically and anatomically dissociable within this population of patients.
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Progressive supranuclear palsy syndrome presenting as Progressive Nonfluent Aphasia a neuropsychological and neuroimaging analysis
Movement Disorders, 2010Co-Authors: Jonathan Daniel Rohrer, Dominic C Paviour, Adolfo M Bronstein, Sean S Osullivan, Andrew J Lees, Jason D. WarrenAbstract:There is currently considerable interest in the clinical spectrum of Progressive Nonfluent Aphasia (PNFA) and Progressive supranuclear palsy (PSP) and the intersection of these two entities. Here, we undertook a detailed prospective clinical, neuropsychological, and neuroimaging analysis of 14 consecutive patients presenting with PNFA to identify cases meeting clinical criteria for PSP. These patients had further detailed assessment of extrapyramidal and oculomotor functions. All patients had high-resolution MR brain volumetry and a cortical thickness analysis was undertaken on the brain images. Four patients presenting with PNFA subsequently developed features of a PSP syndrome, including a typical oculomotor palsy. The neuropsychological profile in these cases was similar to other patients with PNFA, however, with more marked reduction in propositional speech, fewer speech errors, less marked impairment of literacy skills but more severe associated deficits of episodic memory and praxis. These PSP-PNFA cases had less prominent midbrain atrophy but more marked prefrontal atrophy than a comparison group of five patients with pathologically confirmed PSP without PNFA and more prominent midbrain atrophy but less marked perisylvian atrophy than other PNFA cases. In summary, although the PSP-PNFA syndrome overlaps with PNFA without PSP, certain neuropsychological and neuroanatomical differences may help predict the development of a PSP syndrome. (C) 2010 Movement Disorder Society
John R. Hodges - One of the best experts on this subject based on the ideXlab platform.
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Clinical and pathological characterization of Progressive Aphasia.
Annals of Neurology, 2020Co-Authors: Jonathan A Knibb, Karalyn Patterson, John H Xuereb, John R. HodgesAbstract:Objective The clinical and neuropathological categorization of patients presenting with Progressive Aphasia is an area of controversy. This study aimed to characterize a large group of Progressive aphasic patients from a single center (n = 38), first clinically by case note review, and then pathologically. Methods Hierarchical cluster analysis of the cases according to their clinical language deficits was used to establish an unbiased, data-driven classification. Results This analysis revealed two groups of cases corresponding to the syndromes of Progressive Nonfluent Aphasia (n = 23) and semantic dementia (n = 15). Postmortem analysis showed a majority in both groups of pathologies from the spectrum of frontotemporal lobar degeneration: the most frequent were non–Alzheimer's disease (AD) tauopathy in the Nonfluent cases (10 of 23) and frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions in the fluent cases (8 of 15). Despite rigorous exclusion of cases with clinically significant memory deficits or other cognitive impairments, the pathology of AD was present in approximately one third of each group (overall 12 of 38), although often with an atypical neuroanatomical distribution. Interpretation Progressive Aphasia is best seen as a composite of two conditions, on both clinical and pathological levels: Progressive Nonfluent Aphasia and semantic dementia. Ann Neurol 2006;59:156–165
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ecological assessment of emotional enhancement of memory in Progressive Nonfluent Aphasia and alzheimer s disease
Journal of Alzheimer's Disease, 2014Co-Authors: John R. Hodges, Fiona Kumfor, Olivier PiguetAbstract:BACKGROUND: Events which are imbued with emotion are typically remembered vividly and with more confidence than similar non-emotional events. The extent that emotional enhancement of memory is compromised in neurodegenerative disorders is unclear, despite differential effects of dementia on emotion processing ability. OBJECTIVE: To examine emotional enhancement of memory using an ecologically valid task in Progressive Nonfluent Aphasia (PNFA), an expressive language subtype of frontotemporal dementia, in comparison to Alzheimer's disease (AD) and matched-controls. METHODS: Twenty-five dementia patients (13 PNFA, 12 AD) and 10 controls viewed either an emotionally arousing or a closely matched non-emotional story. Multiple-choice recognition memory was tested after a 1-hour delay. The alternate story was presented two weeks later. RESULTS: PNFA showed a similar level of memory for the emotional and neutral story, whereas both controls and AD remembered significantly more details from the emotional than the neutral story. Correlation analyses indicated that in PNFA, emotional story memory correlated with reduced emotion recognition, whereas in AD, neutral story memory correlated with visual recall memory performance only. Furthermore, in PNFA, reduced emotional memory enhancement was associated with increased carer stress and depression. CONCLUSION: Emotional memory enhancement is absent in PNFA, whereas emotion facilitates memory for real-life events in AD. Disrupted emotional memory enhancement in PNFA is associated with reduced emotion recognition ability, suggesting that widespread emotion processing dysfunction is present in this disease. Crucially, loss of emotional enhancement influences carer wellbeing, which represents an important avenue for future studies to examine.
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Grey and white matter brain network changes in frontotemporal dementia subtypes
Translational Neuroscience, 2013Co-Authors: Tim Nguyen, John R. Hodges, Maxime Bertoux, Claire O’callaghan, Samrah Ahmed, Michael HornbergerAbstract:Background Frontotemporal dementia (FTD) comprises of three clinical syndromes, behavioural-variant frontotemporal dementia (bvFTD), semantic dementia (SV-PPA), and Progressive Nonfluent Aphasia (NFV-PPA) with unique underlying neuroanatomical deficits. To date, however, grey matter structural differences and their connecting white matter tracts in this network have been mostly characterised in comparison to controls, whereas within FTD subtype comparisons in the same patients have not been explored.
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Frontotemporal dementias: Recent advances and current controversies
Annals of Indian Academy of Neurology, 2010Co-Authors: Cristian E. Leyton, John R. HodgesAbstract:Frontotemporal dementia (FTD) syndromes comprise a heterogeneous group of neurodegenerative conditions characterized by atrophy in the frontal and temporal lobes. Three main clinical variants are recognized: Behavioral variant (bv-FTD), Semantic dementia (SD), and Progressive Nonfluent Aphasia (PNFA). However, logopenic/phonological (LPA) variant has been recently described, showing a distinctive pattern of brain atrophy and often associated to Alzheimer’s disease pathology. The diagnosis of FTD is challenging, since there is clinical, pathological, and genetic overlap between the variants and other neurodegenerative diseases, such as motoneuron disease (MND) and corticobasal degeneration (CBD). In addition, patients with gene mutations (tau and progranulin) display an inconsistent clinical phenotype and the correspondence between the clinical variant and its pathology is unpredictable. New cognitive tests based on social cognition and emotional recognition together with advances in molecular pathology and genetics have contributed to an improved understanding. There is now a real possibility of accurate biomarkers for early diagnosis. The present review concentrates on new insights and debates in FTD.
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Application of Addenbrooke's cognitive examination to diagnosis and monitoring of Progressive primary Aphasia.
Dementia and Geriatric Cognitive Disorders, 2010Co-Authors: Cristian E. Leyton, Michael Hornberger, Eneida Mioshi, John R. HodgesAbstract:Background/Aims: Primary Progressive Aphasia (PPA) comprises 2 main variants: semantic dementia (SD) and Progressive Nonfluent Aphasia (PNFA). Addenbrooke’s Cognitive Examination (ACE) has become widely used for the diagnosis of dementias. Less information, however, is available about its ability to detect and monitor changes in cognition in PPA. We aimed to analyse the sensitivity and longitudinal changes of ACE scores in 2 subforms of PPA. Methods: We included 63 SD and 45 PNFA cases, all of whom had at least 2 assessments. Sensitivity levels, annualised rates of change and difference in scores over time on repeated ACE measurements were calculated. Results: A cut-off of 88 points detected 95% of the PNFA and SD cases. Longitudinal analysis showed an average annual decline of 10 points per year, with no significant difference between groups. Conclusion: The ACE is a useful tool for detecting and tracking the evolution of PPA.
