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PubMed2006

Anatomical correlates of early mutism in progressive nonfluent aphasia.

Patients with progressive nonfluent aphasia (PNFA) can become mute early in the course of the disease. Voxel-based morphometry showed that PNFA is associated with left anterior insula and inferior frontal atrophy. In PNFA with early mutism, volume loss was more prominent in the pars opercularis and extended into the left basal ganglia. Damage to the network of brain regions involved in both coordination and execution of speech causes mutism in PNFA.

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PubMed2003

Speech and language in progressive nonfluent aphasia compared with early Alzheimer's disease.

BACKGROUND: Progressive nonfluent aphasia (PA) is a slow deterioration of language that remains relatively isolated from other cognitive or behavioral deficits for at least 2 years. The differentiation of PA from early Alzheimer's disease (AD) is important, given the presence of early language changes in AD. METHODS: A language assessment was administered to 15 patients who met established criteria for PA, 15 patients with clinically probable AD and mild dementia, and 15 normal control subjects. The language battery included verbal fluency, the Boston Naming Test with cuing and recognition, and an aphasia test battery with a motor speech exam. RESULTS: Pronounced literal paraphasic errors distinguished the PA patients from the AD patients. The PA group had anomia, decreased letter fluency, neologisms, difficulty on phrase repetition, decreased phrase length, and a decreased rate of verbal output. Interference from paraphasic anomia accounted for much of their decreased fluency. CONCLUSION: Many patients with PA have a primary defect in accessing sound-based representation of speech (phonemes), similar to conduction aphasia, possibly as a consequence of disturbed white matter tracts in the left superior temporal region.

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PubMed2003

Progressive anomia with preserved oral spelling and automatic speech.

We report a patient, Newton, with a progressive classical anomia resulting from focal degeneration of the left hemisphere. In naming tasks Newton spelt aloud picture names that he could not retrieve, indicating a dissociation between orthography and phonology. Unusually, his writing and letter-pointing performance were impaired and spelling was achieved only through alphabet recitation. A study of automatic speech tasks demonstrated strikingly preserved naming performance on automatic compared to nominative tasks. We argue that automatic tasks provide phonological cues that facilitate phonological activation. With progression of disease Newton has shown increasing difficulty reading and repeating words, which we interpret in terms of a progressive elevation in the threshold for activation of phonology. Phonological cueing of picture names has yielded superior naming than word reading and even repetition, a finding consistent with the notion that task characteristics influence likelihood of phonological activation and naming success, but contrary to the notion that there exist separate task-specific output systems. We conclude that Newton exhibits a unique pattern of deficits, which have theoretical relevance for the debate on the relationship between phonology and orthography, the role of automatic speech and the relationship between naming, reading and repetition.

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PubMed2007

Frontotemporal dementia.

Frontotemporal dementia (FTD) is an uncommon but important form of degenerative disease. It may make up 50% of dementia cases presenting before age 60. The symptoms are related to the anatomic areas affected. Neary divided the clinical syndromes into "frontotemporal dementia," "progressive nonfluent aphasia," and "semantic dementia." However, the pathology may extend beyond the frontal and temporal lobes and additional symptoms may be found. Although most cases are sporadic, some cases are genetic. The best-known genetic mutation causing FTD is frontotemporal dementia with parkinsonism, linked to the microtubule-associated protein tau on chromosome 17. There are other known genes and chromosome loci related to FTD. The most common pathology found is frontotemporal degeneration with ubiquitin inclusions. In contrast, FTD with Pick bodies is rare. Although there are strategies to help patients and their families, there is no known treatment for the disease.

Aphasia, Primary Progressive↗
PubMed2004

Initial complaints in frontotemporal lobar degeneration.

AIMS: Frontotemporal lobar degeneration (FTLD) is probably underrecognized. The goal of this study was to investigate initial complaints of both patients and their caregivers at first specialist referral. Also, we tried to assess whether misrecognition of symptoms contributed to diagnostic delay. METHODS: The case notes of all patients diagnosed with FTLD at the VU University Medical Center, Alzheimer Center of Amsterdam, The Netherlands, since 1998 were retrospectively reviewed. Only patients of whom detailed information of first specialist referral was available were included. The diagnosis of FTLD was based on the clinical diagnostic criteria of Neary and Snowden, supported by ancillary investigations. RESULTS: Forty-six patients with FTLD were included. Twenty-one patients had frontotemporal dementia (FTD), 17 semantic dementia (SD) and 8 progressive nonfluent aphasia (PA). The majority of the FTD patients presented without complaints or with somatic complaints and nearly a quarter of them expressed memory complaints. The presenting complaints of most of their caregivers differed from the patients' complaints and often consisted of cognitive complaints. In SD and PA, language problems but also forgetfulness were presented. Misrecognition of the initial symptoms in some cases seemed to have contributed to diagnostic delay. CONCLUSION: Presenting complaints in FTLD can be misleading. In our cohort, memory complaints occurred relatively often. A multidisciplinary approach, including a structured behavioral interview, is important to recognize symptoms of FTLD.

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PubMed2004

Longitudinal patterns of regional change on volumetric MRI in frontotemporal lobar degeneration.

The aim of this study was to assess the longitudinal patterns of regional change in the different syndromic variants of frontotemporal lobar degeneration (FTLD). Ten patients with semantic dementia, 7 with progressive non-fluent aphasia and 29 with frontotemporal dementia had two serial volumetric MR scans. Fluid registration was used to match serial scans from each individual. Voxel-level analysis of change across subject groups was performed using statistical parametric mapping. The analysis showed patterns of increased rates of volume loss (atrophy) in frontal, temporal and parietal regions in the whole FTLD group compared with controls. The different FTLD syndromes displayed different patterns of change. This technique gives an insight into disease evolution over time in these disorders and may be useful as a method of tracking change in clinical trials.

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PubMed2006

From progressive nonfluent aphasia to corticobasal syndrome: a case report of corticobasal degeneration.

In a previous report, we presented longitudinal clinical, cognitive and anatomical data of a right-handed woman, whose clinical picture evolved from progressive nonfluent aphasia with apraxia of speech to corticobasal syndrome (CBS) in the last stage of the disease. The patient died at age 57 and pathological examination revealed severe atrophy in the left frontal operculum and left premotor area. On histological examination, there was diffuse tau-positive pathology in gray and white cortical hemispheric gray and white matter, basal ganglia and substantia nigra, compatible with corticobasal degeneration (CBD). This case demonstrates the clinical overlap between frontotemporal lobar degeneration and CBD. In this case, early motor speech impairment predicted earlier and more accurately than CBS the presence of underlying tau-pathology and CBD.

Aphasia, Primary Progressive↗
PubMed2006

Non-fluent progressive aphasia: cerebral metabolic patterns and brain reserve.

Functional imaging studies suggest that brain reserve allows patients with Alzheimer's disease with more years of schooling to cope better with brain damage. No studies exist on patients with non-fluent progressive aphasia (NFPA). We aimed to explore metabolic patterns of patients with NFPA and to provide evidence for brain reserve in NFPA. 11 right-handed patients with NFPA and 16 age-matched controls underwent (18)F-FDG PET imaging. Scans of patients and controls were compared in SPM2. A linear regression analysis with glucose metabolism as dependent variable and years of schooling as the independent variable, adjusted for age, gender, and a total score of the CERAD neuropsychological battery was conducted. The NFPA group showed a hypometabolism of the left hemisphere including the middle frontal, and inferior temporal and angular gyri, and the bilateral caudate nuclei and thalami (p(corr)<0.05). The regression analysis revealed a significant inverse association between education and glucose metabolism in the left inferior temporal, parahippocampal, and supramarginal gyri (p(corr)<0.05). We conclude that brain reserve is also present in NFPA.

Age Distribution↗
PubMed2004

Confrontation naming and morphometric analyses of structural MRI in frontotemporal dementia.

We studied the neural basis for confrontation naming difficulty in 29 patients with frontotemporal dementia (FTD) by correlating naming with voxel-based morphometric analyses of gray matter volume in structural MRI. We found that naming is significantly impaired in FTD, including patients with semantic dementia (SD), progressive nonfluent aphasia (PNFA), and nonaphasic patients (NON-APH) with a disorder of social and executive functioning. Significant cortical atrophy was found in the left anterior temporal cortex in all three FTD subgroups relative to healthy seniors. We also found significant cortical atrophy in unique anatomic distributions in each FTD subgroup. This included: lateral, ventral, and parahippocampal regions of the left temporal lobe in SD; inferior, orbital, dorsolateral, and premotor regions of the left frontal lobe in PNFA, and bilateral frontal regions in NON-APH. Direct correlations between confrontation naming and gray matter volume revealed distinct patterns in each FTD subgroup. SD patients showed a significant correlation in the left lateral temporal cortex, PNFA patients in several left frontal regions, and NON-APH patients in the right dorsolateral prefrontal cortex. These findings suggest that confrontation naming is supported by a large-scale neural network, and that naming is compromised in FTD due to interruption of the network in several different ways.

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PubMed2003

Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula.

Progressive non-fluent aphasia (PNFA) is a syndrome in which patients lose the ability to communicate fluently in the context of relative preservation of single word comprehension and non-linguistic cognitive abilities. Neuroimaging in case studies with PNFA has failed to identify a consistent neural substrate for the language disorder. In this study of a group of patients (n=10) whose presenting complaint was progressive dysfluency, resting cerebral metabolism was measured using [18F]fluorodeoxyglucose-PET and analysed with the technique of statistical parametric mapping (SPM). Regional atrophy was assessed with voxel-based morphometry (VBM). Seven patients had a 'pure' PNFA syndrome, while the remaining three had additional features of a more pervasive dementia. Compared with controls, the patients showed hypometabolism in several regions that, most notably, included the left anterior insula/frontal opercular region. The VBM analysis revealed only one small area of atrophy in the left peri-Sylvian region. Analysis of the pure PNFA cases (n=7) relative to controls yielded qualitatively similar results to those of the whole group, suggesting that these cases were also at risk of a more generalized dementia, a finding borne out in subsequent follow-up of two cases to date. The PNFA group was then compared with a group with Alzheimer's disease (n=10) whose clinical profile did not include non-fluent aphasic features. In this analysis, the only persisting hypometabolic region was that centred over the left anterior insula. VBM did not identify any regional differences in atrophy between PNFA and Alzheimer's disease. In the light of current theories of fluent language production, the findings offer anatomical evidence that the breakdown in fluency is due to a motor articulatory planning deficit (speech apraxia) combined with a variable degree of agrammatism.

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PubMed2002

Frontotemporal dementia.

Frontotemporal dementia is a new name for clinical Pick's disease; the eponymic term PiD has been increasingly restricted to the pathologic variant with Pick bodies. This article describes the clinical picture, pathology, and genetic mechanisms of frontal lobe dementia. Frontotemporal dementia is still under-diagnosed and underestimated, partly because the individual components of the complex are considered separately or are considered "heterogeneous," a somewhat misleading adjective used in many descriptions. This article emphasizes the considerable overlap among the different varieties of frontotemporal dementia, as well as the distinctive features of each.

Aphasia, Primary Progressive↗
PubMed2000

The role of conceptual knowledge in object use evidence from semantic dementia.

It has been reported that patients with semantic dementia function well in everyday life and sometimes show striking preservation of the ability to use objects, even those specific objects for which the patient has degraded conceptual information. To explore this phenomenon in nine cases of semantic dementia, we designed a set of semantic tests regarding 20 everyday objects and compared performance on these with the patients' ability to demonstrate the correct use of the same items. We also administered a test of mechanical problem solving utilizing novel tools, on which the patients had completely normal ability. All but the mildest affected patient showed significant deficits of naming and on the visually based semantic matching tasks. Object use was markedly impaired and, most importantly, correlated strongly with naming and semantic knowledge. In a small number of instances, there was appropriate use of an object for which the patient's knowledge on the semantic matching tasks was no better than chance; but this typically applied to objects with a rather obvious relationship between appearance and use, or was achieved by trial and error. The results suggest that object use is heavily dependent upon object-specific conceptual knowledge, supplemented to some degree by a combination of visual affordances and mechanical problem solving.

Aphasia, Primary Progressive↗
PubMed2004

Patterns of referring of patients with frontotemporal lobar degeneration to psychiatric in- and out-patient services. Results from a prospective multicentre study.

Dementia with frontotemporal lobar degeneration (FTLD) is clinically characterized by the occurrence of various psychiatric symptoms. In a recent study, the hospital-based prevalence of FTLD and the circumstances of the patients' admission to German psychiatric state hospitals were estimated. On the basis of further continuous assessment, this original FTLD group (n = 33) has been enlarged to 58 patients. The authors here present demographic and clinical data, and reasons for admission to geriatric psychiatry hospitals in comparison with 17 patients, who primarily attended the Memory Disorders Clinic of the University of Regensburg. The results implicate that both institutions see patients with different clinical syndromes: (1) patients were primarily referred to the Memory Disorders Clinic presenting memory and/or speech difficulties as the leading symptoms; (2) major reasons for hospitalisation of patients with FTLD in geriatric psychiatry hospitals were behavioural disturbances; (3) late-onset FTLD (>65 years) was more common than previously assumed in both institutions, and (4) increasing age at admission increased the likelihood to obtain a limited diagnostic approach of brain imaging (only cranial computer tomography) to evaluate the cause of dementia.

Adult↗
PubMed2025

Comprehensive cross-sectional and longitudinal comparisons of plasma glial fibrillary acidic protein and neurofilament light across FTD spectrum disorders.

BACKGROUND: Therapeutic development for frontotemporal dementia (FTD) is hindered by the lack of biomarkers that inform susceptibility/risk, prognosis, and the underlying causative pathology. Blood glial fibrillary acidic protein (GFAP) has garnered attention as a FTD biomarker. However, investigations of GFAP in FTD have been hampered by symptomatic and histopathologic heterogeneity and small cohort sizes contributing to inconsistent findings. Therefore, we evaluated plasma GFAP as a FTD biomarker and compared its performance to that of neurofilament light (NfL) protein, a leading FTD biomarker. METHODS: We availed ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD) study resources to conduct a comprehensive cross-sectional and longitudinal examination of the susceptibility/risk, prognostic, and predictive performance of GFAP and NfL in the largest series of well-characterized presymptomatic FTD mutation carriers and participants with sporadic or familial FTD syndromes. Utilizing single molecule array technology, we measured GFAP and NfL in plasma from 161 controls, 127 presymptomatic mutation carriers, 702 participants with a FTD syndrome, and 67 participants with mild behavioral and/or cognitive changes. We used multivariable linear regression and Cox proportional hazard models adjusted for co-variates to examine the biomarker utility of baseline GFAP and NfL concentrations or their rates of change. RESULTS: Compared to controls, GFAP and NfL were elevated in each FTD syndrome but GFAP, unlike NfL, poorly discriminated controls from participants with mild symptoms. Similarly, both baseline GFAP and NfL were higher in presymptomatic mutation carriers who later phenoconverted, but NfL better distinguished non-converters from phenoconverters. We additionally observed that GFAP and NfL were associated with disease severity indicators and survival, but NfL far outperformed GFAP. Nevertheless, we validated findings that the GFAP/NfL ratio may discriminate frontotemporal lobar degeneration with tau versus TDP-43 pathology. CONCLUSIONS: Our head-to-head comparison of plasma GFAP and NfL as biomarkers for FTD indicate that NfL consistently outmatched GFAP as a prognostic and predictive biomarker for participants with a FTD syndrome, and as a susceptibility/risk biomarker for people at genetic risk of FTD. Our findings underscore the need to include leading biomarkers in investigations evaluating new biomarkers if the field is to fully ascertain their performance and clinical value.

Humans↗
PubMed1999

When traditional care falls short. Caring for people with atypical presentations of cortical dementia.

1 People with chronic dementias receive care and support with programs designed for people with Alzheimer's disease, meaning a significant minority of patients will not benefit as a result of atypical or unusual presentations of cortical decline. 2 When patients have atypical presentations of dementia, nurses must recognize the unusual symptoms, assess care needs, and develop appropriate management strategies. 3 For patients with chronic dementia, individualized care planning includes knowledge of: 1) the patient's culture; 2) their past habits, activities, and preferences; 3) their remaining abilities; 4) the regions of the brain affected, histopathology, and size of the affected area; 5) the usual trajectory for the disease type; and 6) external demands. 4 Conducting research on assessment and care of people with atypical presentations of dementia is very difficult because of their rare nature and lack of recognition by health practitioners who may diagnose the patients with Alzheimer's disease.

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PubMed2003

[Frontotemporal dementia--new understanding of Pick disease].

Frontotemporal dementia is a relatively new term, which encompasses several degenerative diseases involving the frontal and anterior temporal lobes. The article gives a short overview of new knowledge in this field. Clinical criteria are in focus, but neuropathological aspects are also mentioned.

Aphasia, Primary Progressive↗
PubMed2001

Distribution of cerebral cortical lesions in Pick's disease with Pick bodies: a clinicopathological study of six autopsy cases showing unusual clinical presentations.

We investigated six Japanese autopsy cases of Pick's disease with Pick bodies (PDPB) both clinically and pathologically, and examined the distribution of their cerebral cortical lesions using hemisphere and/or bisphere specimens. The lesions were classified into three categories (slight, moderate, and severe). Two patients with a clinical diagnosis of primary progressive apraxia and of slowly progressive aphasia had speech apraxia as their initial signs, and the other two patients were suspected as having Alzheimer's disease, with the clinical diagnosis of the remainder two patients being presenile dementia and depression, respectively. Extrapyramidal signs, believed to be rare in PDPB, were present in four patients. Severe lesions were multicentrically present in the cerebral cortices of all six cases. In two patients with speech apraxia, severe lesions were seen in the primary motor area, which generally has not been regarded as an "atrophic center" in Pick's disease. Furthermore, in a patient with depression, severe lesions were more widespread in the convexity than in the orbital region of the frontal lobe. The parietal lobes, including the postcentral gyrus usually believed to be spared in Pick's disease, were severely involved in three patients. We postulate that the clinical features of PDPB have a much wider spectrum than previously believed. In addition, we believe that the distribution of the cerebral cortical lesions in PDPB is more widespread than previously assumed, and that clinical manifestations of PDPB depend to some extent on the topographic distribution of the cerebral cortical lesions.

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