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J R Hodges

Publications and source records attributed to J R Hodges.

At least 73 records · Page 4Linked to original sources
PubMed2000

The rise and fall of frequency and imageability: noun and verb production in semantic dementia.

This study examines the impact of progressive degeneration of conceptual knowledge on the content words used in connected speech elicited using the Cookie Theft picture description (Goodglass & Kaplan. 1983). We began with an analysis of control subjects' descriptions with regard to word types and their frequency and imageability. Because the impairment of conceptual knowledge in semantic dementia is graded by concept familiarity, we created a model of a standardized normal Cookie Theft description that was then progressively degraded by the systematic removal of lower bands of word frequency. We drew two main predictions from this model: reduced availability of the lower bands of word frequency should result in (a) an apparent deficit for noun retrieval in relation to verb retrieval and (b) an apparent reverse imageability effect. Results from a longitudinal study. in which three patients with semantic dementia each described the Cookie Theft picture on three occasions during the progression of their disease, confirmed these predictions. An additional cross-sectional analysis, adding narratives from a larger number of cases, demonstrated that the decline in ability to produce suitable words for the picture description is closely related to the extent of semantic impairment as measured in tests of word comprehension and production. Both verbs and nouns are affected by the degradation of semantic memory; the fact that the impairment to noun production is manifested earlier and more catastrophically may be attributed to the relatively lower frequency of these terms.

Aged↗
PubMed2000

Phonological and articulatory impairment in Alzheimer's disease: a case series.

We demonstrate that phonological and articulatory impairments may occur at presentation or early in the course of Alzheimer's disease, contrary to claims that these aspects of language production are relatively preserved until the final stages of this disease. Six patients with pathologically confirmed Alzheimer's disease (AD) and four patients with clinically diagnosed dementia of the Alzheimer's type (DAT) presented with one of five different clinical profiles: nonfluent progressive aphasia, mixed progressive aphasia, progressive aphasia diagnosed as DAT from neuropsychological assessment, initial amnestic syndrome with prominent phonological errors, and biparietal syndrome. Analysis of their conversational speech, single-word production, and performance of highly familiar series speech tasks such as counting revealed false start errors, phonological paraphasias, and/or articulatory difficulty. Neuropathological changes were located in left perisylvian regions consistent with speech and language impairment but atypical for Alzheimer's disease.

Alzheimer Disease↗
PubMed2000

Semantic dementia: clinical, radiological and pathological perspectives.

Semantic dementia (SD) is a recently described clinical syndrome characterised by an acquired progressive inability to name or comprehend common concepts, with little or no distortion of the phonological and syntactic aspects of language, and relative sparing of other aspects of cognition, such as episodic memory, nonverbal problem-solving, and perceptual and visuo-spatial skills. The cognitive locus of this syndrome appears to lie in the permanent store of long-term memory representing general world knowledge-semantic memory. The anatomical distribution of atrophy is less well-defined, and the contribution of various imaging modalities is discussed in the context of a body of 45 published and unpublished cases. We conclude that involvement of the left infero-lateral temporal cortex is the critical area in the genesis of SD. SD probably always represents a non-Alzheimer neurodegenerative process; a variety of pathological lesions may be present, and possible causes, together with debates about their correct classification, are discussed.

Dementia↗
PubMed2000

Non-verbal semantic impairment in semantic dementia.

The clinical presentation of patients with semantic dementia is dominated by anomia and poor verbal comprehension. Although a number of researchers have argued that these patients have impaired comprehension of non-verbal as well as verbal stimuli, the evidence for semantic deterioration is mainly derived from tasks that include some form of verbal input or output. Few studies have investigated semantic impairment using entirely non-verbal assessments and the few exceptions have been based on results from single cases ([3]: Breedin SD, Saffran EM, Coslett HB. Reversal of the concreteness effect in a patient with semantic dementia. Cognitive Neuropsychology 1994;11:617-660, [12]: Graham KS, Becker JT, Patterson K, Hodges JR. Lost for words: a case of primary progressive aphasia? In: Parkin A, editor. Case studies in the neuropsychology of memory, East Sussex: Lawrence Erlbaum, 1997. pp. 83-110, [21]: Lambon Ralph MA, Howard D. Gogi aphasia or semantic dementia? Simulating and assessing poor verbal comprehension in a case of progressive fluent aphasia. Cognitive Neuropsychology, (in-press). This study employed sound recognition and semantic association tasks to investigate the nature of the verbal and non-verbal comprehension deficit in 10 patients with semantic dementia. The patients were impaired on both verbal and non-verbal conditions of the assessments, and their accuracy on these tasks was directly related to their scores on a range of other tests requiring access to semantic memory. Further analyses revealed that performance was graded by concept and sound familiarity and, in addition, identified significant item consistency across the different conditions of the tasks. These results support the notion that the patients' deficits across all modalities were due to degradation within a single, central network of conceptual knowledge. There were also reliable differences between conditions. The sound-picture matching task proved to be more sensitive to semantic impairment than the word-picture matching equivalent, and the patients performed significantly better on the picture than word version of a semantic association test. We propose that these differences arise directly from the nature of the mapping between input modality and semantic memory. Words and sounds have an arbitrary relationship with meaning while pictures benefit from a degree of systematicity with conceptual knowledge about the object.

Aphasia↗
PubMed2000

The impact of semantic memory impairment on spelling: evidence from semantic dementia.

We assessed spelling and reading abilities in 14 patients with semantic dementia (with varying degrees of semantic impairment) and 24 matched controls, using spelling-to-dictation and single-word reading tests which manipulated regularity of the correspondences between spelling and sound, and word frequency. All of the patients exhibited spelling and reading deficits, except at the very earliest stages of disease. Longitudinal study of seven of the patients revealed further deterioration in spelling, reading, and semantic memory. The performance of both subject groups on both spelling and reading was affected by regularity and word frequency, but these effects were substantially larger for the patients. Spelling of words with exceptional (or more precisely, unpredictable) sound-to-spelling correspondences was most impaired, and the majority of errors were phonologically plausible renderings of the target words. Reading of low frequency words with exceptional spelling-to-sound correspondences was also significantly impaired. The spelling and reading deficits were correlated with, and in our interpretation are attributed to, the semantic impairment.

Alzheimer Disease↗
PubMed2000

The role of speech production in auditory-verbal short-term memory: evidence from progressive fluent aphasia.

We report investigations of auditory-verbal short-term memory (AVSTM) in a patient with progressive fluent anomic aphasia. Despite having apparently normal AVSTM as measured by digital span, FM was significantly impaired in immediate serial recall of short sequences of familiar words, and even in reproducing a single word after a filled delay of just a few seconds. In both tasks, unlike normal subjects, she produced numerous phonological errors, often consisting of phonological segments from the intended target word concatenated with segments from other words in the stimulus sequence. Her success in these tasks was modulated (i) consistently by word frequency (high > low), (ii) inconsistently by word imageability (high > low), and (iii) most dramatically by 'nameability': that is, FM was much more likely to reproduce a word correctly in AVSTM if it was a word that she could also produce successfully in picture-naming tasks. On the basis of these and additional experiments designed to exclude other interpretations, we conclude that AVSTM may be crucially supported by activation of the lexical phonological representations responsible for production of content words in speech.

Anomia↗
PubMed2000

Insights from semantic dementia on the relationship between episodic and semantic memory.

An influential theory of long-term memory, in which new episodic learning is dependent upon the integrity of semantic memory, predicts that a double dissociation between episodic and semantic memory is not possible in new learning. Contrary to this view, we found, in two separate experiments, that patients with impaired semantic memory showed relatively preserved performance on tests of recognition memory if the stimuli were perceptually identical between learning and test. A significant effect of semantic memory was only seen when a perceptual manipulation was introduced in the episodic task. To account for these findings, we propose a revision to current models of long-term memory, in which sensory/perceptual information and semantic memory work in concert to support new learning.

Aged↗
PubMed2000

The nature and staging of attention dysfunction in early (minimal and mild) Alzheimer's disease: relationship to episodic and semantic memory impairment.

The development of cholinergic therapies for Alzheimer's disease (AD) has highlighted the importance of understanding the role of attentional deficits and the relationship between attention and memory in the earliest stages of the disease. Variability in the tasks used to examine aspects of attention, and in the disease severity, between studies makes it difficult to determine which aspects of attention are affected earliest in AD, and how attentional impairment is related to other cognitive modules. We tested 27 patients in the early stages of the disease on the basis of the MMSE (minimal 24-30 corresponding to minimal cognitive impairment, very mild or possible AD in other classifications; and mild 18-23) on a battery of attentional tests aimed to assess sustained, divided, and selective attention, plus tests of episodic memory, semantic memory, visuoperceptual and visuospatial function, and verbal short-term memory. Although the mildly demented group were impaired on all attentional tests, the minimally impaired group showed a preserved ability to sustain attention, and to divide attention based on a dual-task paradigm. The minimally demented group had particular problems with response inhibition and speed of attentional switching. Examination of the relationship between attention and other cognitive domains showed impaired episodic memory in all patients. Deficits in attention were more prevalent than deficits in semantic memory suggesting that they occur at an earlier stage and the two were partially independent. Impairment in visuoperceptual and visuospatial functions and verbal short-term memory were the least common. Although attention is impaired early in AD, 40% of our patients showed deficits in episodic memory alone, confirming that amnesia may be the only cognitive deficit in the earliest stages of sporadic AD.

Aged↗
PubMed2000

Intervening with everyday memory problems in dementia of Alzheimer type: an errorless learning approach.

Dementia of Alzheimer Type (DAT) is increasingly detected at an earlier stage of the disorder, when interventions to assist with everyday memory difficulties might be most valuable. Some learning is possible in DAT and a number of factors have been identified which may facilitate performance, although applications to everyday memory problems have been limited. The concept of errorless learning has not previously been directly examined in relation to DAT, but might provide a useful additional strategy. In the present study, 6 participants with early stage DAT (MMSE scores 21 - 26) received individually tailored interventions, based on errorless learning principles and targeted at a specific everyday memory problem. Five of the participants showed significant improvement on the target measures, and maintained this improvement up to 6 months later. The results suggest that it is feasible to intervene with everyday memory problems in the early stages

Activities of Daily Living↗
PubMed2000

Atypical and typical presentations of Alzheimer's disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases.

There has been increasing awareness that some slowly progressive focal cortical syndromes can be the presenting features of Alzheimer's disease, but pathological evidence has been sparse. This clinico-pathological series presents our experience with pathologically proven atypical as well as typical Alzheimer's disease presentations. We report and compare four patterns of presentation: a typical pattern with initial amnesic syndrome (n = 4 cases), progressive visual dysfunction (n = 1), progressive biparietal syndrome (n = 2) and progressive aphasia (n = 6). The aphasic presentations include both fluent and non-fluent aphasic syndromes. The neuropsychological profiles and neuroimaging clearly reflect the presenting clinical features, and show a close relationship to the distribution of pathology in these cases. Of note was the sparing of medial temporal structures (hippocampus and/or entorhinal cortex) in several aphasic cases and the severe occipito-parietal involvement in those with prominent visuospatial disorders at presentation. Our data demonstrate the wide spectrum of Alzheimer's disease presentations. The recognition of atypical presentations of Alzheimer's disease is important when attempting to make an early accurate pre-morbid diagnosis of neurodegenerative disease.

Aged↗
PubMed2000

Visual object and visuospatial cognition in Huntington's disease: implications for information processing in corticostriatal circuits.

The primate visual system contains two major streams of visual information processing. The ventral stream is directed into the inferior temporal cortex and is concerned with visual object cognition, whereas the dorsal stream is directed into the posterior parietal cortex and is concerned with visuospatial cognition. Both of these processing streams send projections to the basal ganglia, and the ventral stream may also receive reciprocal connections from the basal ganglia. Although a role for the basal ganglia in visual object and visuospatial cognition has been suggested, little work has been carried out in this area in humans. The primary site of neuropathology in Huntington's disease is the basal ganglia, and hence Huntington's disease provides an important model for the role of the human basal ganglia in visual object and visuospatial cognition, and its breakdown in disease. We examined performance on a wide battery of tests of both visual object and visuospatial recognition memory, working memory, attention, associative learning and perception, enabling us to specify more fully the role of the basal ganglia in visual object and visuospatial cognition, and the disruption of these processes in Huntington's disease. Huntington's disease patients exhibited deficits on tests of pattern and spatial recognition memory; showed impaired simultaneous matching and delay-independent delayed matching-to-sample deficits; showed spared accuracy but impaired reaction times in visual search; were impaired in spatial but not visual object working memory; and showed impaired pattern-location associative learning. The results of our investigations suggest a particular role for the striatum in context-dependent action selection, in line with current computational theories of basal ganglia function.

Adult↗
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↗
PubMed2000

Tau gene mutation K257T causes a tauopathy similar to Pick's disease.

Exonic and intronic mutations in Tau cause neurodegenerative syndromes characterized by frontotemporal dementia and filamentous tau protein deposits. Here we describe a K257T missense mutation in exon 9 of Tau. The proband, a 47-yr-old male, presented with severe personality changes followed by semantic memory loss. A diagnosis of Pick's disease was made. The symptoms progressed until death at age 51. The proband's brain showed a marked frontotemporal atrophy that was most pronounced in the temporal lobes. Numerous tau-immunoreactive Pick bodies were present in the neocortex and the hippocampal formation, as well as in some subcortical brain regions. Their appearance and staining characteristics were indistinguishable from those of sporadic Pick's disease. Diffuse staining for hyperphosphorylated tau was also observed in some nerve cell bodies. Immunoblot analysis of sarkosyl-insoluble tau showed 2 major bands of 60 and 64 kDa and 2 very minor bands of 68 and 72 kDa. Upon dephosphorylation, these bands resolved into 6 bands consisting of 3-repeat and 4-repeat tau isoforms, with an overall preponderance of 3-repeat tau. Isolated tau filaments were narrow, irregularly twisted ribbons. Biochemically, recombinant tau proteins with the K257T mutation showed a reduced ability to promote microtubule assembly, suggesting that this may be the primary effect of the mutation. In addition, the K257T mutation was found to stimulate heparin-induced assembly of 3-repeat tau into filaments. Taken together, the present findings indicate that the K257T mutation in Tau can cause a dementing condition similar to Pick's disease.

Frontal Lobe↗
PubMed2000

Corticobasal ganglionic degeneration and/or frontotemporal dementia? A report of two overlap cases and review of literature.

OBJECTIVE: According to the existing viewpoint, Corticobasal degeneration (CBD) is thought of as a predominantly extrapyramidal motor disorder that is distinct and unrelated to frontotemporal dementia (FTD), the most common form of non-Alzheimer dementias. A lack of understanding of the aetiopathogenesis, and poor correlation between the pathology and the clinical syndromes, has resulted in a disparity in the classification of cases of non-Alzheimer dementias. This report intends to highlight the overlap between FTD and CBD in the light of the evolution of these terms, and to discuss the implications of these findings on the nosology of CBD and the classification of non-Alzheimer dementias. METHODS AND RESULTS: Two cases who presented with cognitive dysfunction, which, on comprehensive neuropsychological testing warranted an antemortem diagnosis of FTD are reported. A detailed necropsy study of their brains, however, favoured a pathological diagnosis of CBD. The literature on the overlap between CBD and FTD is also reviewed. CONCLUSIONS: Firstly, evidence is emerging to suggest that the clear distinction drawn between FTD and CBD by the existing viewpoint, needs revision. Secondly, until such time that a comprehensive classification of non-Alzheimer dementias is evolved, it may be better to distinguish between the clinical and pathological levels of description and to classify cases, in vivo, on the basis of the clinical phenotype.

Aged↗
PubMed2000

Which neuropsychiatric and behavioural features distinguish frontal and temporal variants of frontotemporal dementia from Alzheimer's disease?

OBJECTIVES: To investigate the prevalence of changes in mood, personality, and behaviour in frontotemporal dementia (FTD) and Alzheimer's disease (AD) and hence, which features reliably distinguish between them. To establish whether the frontal and temporal variants of FTD are characterised by different behavioural changes. METHODS: A questionnaire was designed to assess a wide range of neuropsychiatric changes; it incorporated features reported in previous studies of FTD and components of the neuropsychiatric inventory.(1) This was completed by 37 carers of patients with Alzheimer's disease (AD) and 33 patients with frontotemporal dementia (FTD), comprising 20 with temporal variant FTD (tv FTD) or semantic dementia and 13 with frontal variant FTD (fv FTD). An exploratory principal components factor analysis and discriminant function analysis was applied. RESULTS: Factor analysis showed four robust and meaningful symptom clusters: factor 1-stereotypic and eating behaviour; factor 2-executive dysfunction and self care; factor 3-mood changes; factor 4-loss of social awareness. Only stereotypic and altered eating behaviour and loss of social awareness reliably differentiated AD from FTD with no effect of disease severity. By contrast, executive dysfunction, poor self care, and restlessness showed a significant effect of disease severity only, with the more impaired patients scoring more highly. Changes in mood were found to be equally prevalent in the three patient groups. Analysis of individual symptoms showed increased rates of mental rigidity and depression in the patients with semantic dementia compared with those with fv FTD. Conversely, the latter group showed greater disinhibition. Discriminant function analysis correctly classified 71.4% overall and 86.5% of the patients with AD. CONCLUSIONS: This questionnaire disclosed striking differences between patients with FTD and AD, but only stereotypic behaviour, changes in eating preference, disinhibition, and features of poor social awareness reliably separated the groups. The patients with fv FTD and semantic dementia were behaviourally very similar, reflecting the involvement of a common network, the ventral frontal lobe, temporal pole, and amygdala. Dysexecutive symptoms and poor self care were found to be affected by the severity of the disease, reflecting perhaps spread to dorsolateral prefrontal areas relatively late in the course of both FTD and AD. This questionnaire may be of value in the diagnosis and the monitoring of therapies.

Aged↗
PubMed2000

A voxel-based morphometry study of semantic dementia: relationship between temporal lobe atrophy and semantic memory.

The cortical anatomy of 6 patients with semantic dementia (the temporal lobe variant of frontotemporal dementia) was contrasted with that of a group of age-matched normal subjects by using voxel-based morphometry, a technique that identifies changes in gray matter volume on a voxel-by-voxel basis. Among the circumscribed regions of neuronal loss, the left temporal pole (Brodmann area 38) was the most significantly and consistently affected region. Cortical atrophy in the left hemisphere also involved the inferolateral temporal lobe (Brodmann area 20/21) and fusiform gyrus. In addition, the right temporal pole (Brodmann area 38), the ventromedial frontal cortex (Brodmann area 11/32) bilaterally, and the amygdaloid complex were affected, but no significant atrophy was measured in the hippocampus, entorhinal, or caudal perirhinal cortex. The degree of semantic memory impairment across the 6 cases correlated significantly with the extent of atrophy of the left anterior temporal lobe but not with atrophy in the adjacent ventromedial frontal cortex. These results confirm that the anterior temporal lobe is critically involved in semantic processing, and dissociate its function from that of the adjacent frontal region.

Aged↗