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Biomedical subjects

J R Hodges

Publications and source records attributed to J R Hodges.

At least 91 records · Page 5Linked to original sources
PubMed1999

"What" and "how": evidence for the dissociation of object knowledge and mechanical problem-solving skills in the human brain.

Patients with profound semantic deterioration resulting from temporal lobe atrophy have been reported to use many real objects appropriately. Does this preserved ability reflect (i) a separate component of the conceptual knowledge system ("action semantics") or (ii) the operation of a system that is independent of conceptual knowledge of specific objects, and rather is responsible for general mechanical problem-solving skills, triggered by object affordances? We contrast the performance of three patients-two with semantic dementia and focal temporal lobe atrophy and the third with corticobasal degeneration and biparietal atrophy-on tests of real object identification and usage, picture-based tests of functional semantic knowledge, and a task requiring selection and use of novel tools. The patient with corticobasal degeneration showed poor novel tool selection and impaired use of real objects, despite near normal semantic knowledge of the same objects' functions. The patients with semantic dementia had the expected deficit in object identification and functional semantics, but achieved flawless and effortless performance on the novel tool task. Their attempts to use this same mechanical problem-solving ability to deduce (sometimes successfully but often incorrectly) the use of the real objects provide no support for the hypothesis of a separate action-semantic system. Although the temporal lobe system clearly is necessary to identify "what" an object is, we suggest that sensory inputs to a parietal "how" system can trigger the use of objects without reference to object-specific conceptual knowledge.

Aged↗
PubMed1999

Is a picture worth a thousand words? Evidence from concept definitions by patients with semantic dementia.

Nine patients with semantic dementia (the temporal lobe variant of frontotemporal dementia) were asked to define concrete concepts either from presentation of a picture of the object or from its spoken name. As expected, the patients with the most severe semantic impairment produced the least detailed definitions, and the quality of the definitions overall was significantly related to concept familiarity. Further analyses of the definitions were designed to assess two key theoretical aspects of semantic organization. (i) Do objects and their corresponding names activate conceptual information in two neuroanatomically separable (modality-specific) semantic systems? If so, then-apart from any expected commonality in performance attributable to factors such as concept familiarity-one would not predict striking item-specific similarities in a patient's picture- and word-elicited definitions. (ii) Do sensory/perceptual features and more associative/functional attributes of conceptual knowledge form two neuroanatomically separable subsystems? If so, then one would predict significant dissociations in the prominence of these two types of information in the patients' definitions. The results lead us to favor a model of the semantic system that is divided by attribute type but not by modality.

Aged↗
PubMed1999

Myotonic dystrophy and progressive cognitive decline: a common condition or two separate problems?

We report the case of NG, a 43-year old woman with myotonic dystrophy (MYD) who has shown a slow decline in both motor and cognitive abilities since her referral to us at age 32. MYD is an autosomal dominant disorder characterised by weakening and wasting of the muscles together with impaired muscle relaxation. Cognitive abilities are usually little affected in the adult onset form, although there is a high risk of cognitive impairment in those with childhood onset. Cognitive decline is also typically associated with maternal inheritance. NG, who was diagnosed with MYD at the age of 18, inherited it from her father. We report the decline in NG's cognitive abilities over 11 years of longitudinal assessment, and consider whether she is an atypical MYD patient or whether the MYD and cognitive decline are attributable to two separate pathological processes.

Adolescent↗
PubMed1999

Episodic memory: new insights from the study of semantic dementia.

The study of patients with semantic dementia, the temporal variant of frontotemporal dementia, has provided new insights into the interaction between episodic and semantic memory, and the different roles played by various structures in the temporal lobe. Recent findings indicate that the syndrome of semantic dementia can inform us about the organisation of long-term memory and the relationship between semantic memory and other cognitive systems.

Dementia↗
PubMed1999

Evidence for impaired sentence comprehension in early Alzheimer's disease.

We investigated sentence comprehension in 46 patients with probable minimal (very mild), mild, or moderate dementia of the Alzheimer type (DAT), comparing their performance on the Test for the Reception of Grammar (TROG), with that of 20 age- and education-matched controls. Performance on the TROG was generally related to dementia severity, independent of lexicosemantic and working memory (digit span) impairments, but related to at least 1 measure of attention. Some patients in the minimal group showed sentence comprehension deficits while others in the moderate group did not, indicating that DAT may impair sentence comprehension at the very earliest stages of disease, but that its effects are heterogeneous. Patients were most impaired on sentences with 2 propositions and noncanonical word order, suggesting difficulties with both interpretative and postinterpretative stages of sentence processing. Further investigation is needed into the relationship between attentional processes, interpretative and postinterpretative stages of syntactic processing in DAT.

Age Factors↗
PubMed1999

The differentiation of semantic dementia and frontal lobe dementia (temporal and frontal variants of frontotemporal dementia) from early Alzheimer's disease: a comparative neuropsychological study.

The authors compared age-matched groups of patients with the frontal and temporal lobe variants of frontotemporal dementia (FTD; dementia of frontal type [DFT] and semantic dementia), early Alzheimer's disease (AD), and normal controls (n = 9 per group) on a comprehensive neuropsychological battery. A distinct profile emerged for each group: Those with AD showed a severe deficit in episodic memory with more subtle, but significant, impairments in semantic memory and visuospatial skills; patients with semantic dementia showed the previously documented picture of isolated, but profound, semantic memory breakdown with anomia and surface dyslexia but were indistinguishable from the AD group on a test of story recall; and the DFT group were the least impaired and showed mild deficits in episodic memory and verbal fluency but normal semantic memory. The frontal and temporal presentations of FTD are clearly separable from each other and from early AD.

Age Factors↗
PubMed1999

Relearning and subsequent forgetting of semantic category exemplars in a case of semantic dementia.

Over 15 months of longitudinal assessment, a patient with semantic dementia, D.M., improved on tests of naming and category fluency for a specific set of items (Experiment 1). The authors attribute this to his home drill with the names of these concepts plus pictures and descriptions of them. In Experiment 2, D.M. produced significantly more exemplars on category fluency for semantic categories that he had been practicing at home than for nonpracticed categories, an effect that cannot be attributed to an inherent difference between the 2 sets because the fluency performance of control participants revealed no significant difference between the 2 sets. In Experiment 3, D.M. rehearsed some of his previously nonpracticed categories daily for a period of 2 weeks: His fluency scores on the experimental categories improved substantially, but they declined once he ceased the daily drill. The results are discussed with respect to current views of long-term memory, particularly new word learning and forgetting, and to current techniques for facilitating word finding in aphasia.

Aphasia↗
PubMed1999

Disrupted temporal lobe connections in semantic dementia.

Semantic dementia refers to the variant of frontotemporal dementia in which there is progressive semantic deterioration and anomia in the face of relative preservation of other language and cognitive functions. Structural imaging and SPECT studies of such patients have suggested that the site of damage, and by inference the region critical to semantic processing, is the anterolateral temporal lobe, especially on the left. Recent functional imaging studies of normal participants have revealed a network of areas involved in semantic tasks. The present study used PET to examine the consequences of focal damage to the anterolateral temporal cortex for the operation of this semantic network. We measured PET activation associated with a semantic decision task relative to a visual decision task in four patients with semantic dementia compared with six age-matched normal controls. Normals activated a network of regions consistent with previous studies. The patients activated some areas consistently with the normals, including some regions of significant atrophy, but showed substantially reduced activity particularly in the left posterior inferior temporal gyrus (iTG) (Brodmann area 37/19). Voxel-based morphometry, used to identify the regions of structural deficit, revealed significant anterolateral temporal atrophy (especially on the left), but no significant structural damage to the posterior inferior temporal lobe. Other evidence suggests that the left posterior iTG is critically involved in lexical-phonological retrieval: the lack of activation here is consistent with the observation that these patients are all anomic. We conclude that changes in activity in regions distant from the patients' structural damage support the argument that their prominent anomia is due to disrupted temporal lobe connections.

Anomia↗
PubMed1999

Huntington's disease progression. PET and clinical observations.

Using serial [(11)C]SCH 23390- and [11C]raclopride-PET, we have measured the rate of loss of striatal dopamine D1 and D2 receptor binding over a mean of 40 months in nine asymptomatic adult Huntington's disease mutation carriers, four patients with symptomatic disease, seven mutation-negative controls and three subjects at risk for the disease. Eight of the nine asymptomatic Huntington's disease mutation carriers had serial [11C]raclopride-PET and showed a mean annual loss of striatal D2 binding of 4.0%. Only five of these eight, however, showed active progression, and they had a mean annual loss of D2 binding of 6.5%. All nine asymptomatic mutation carriers had serial [11C]SCH 23390-PET and showed a mean annual loss of striatal D1 binding of 2. 0%. Four of these subjects demonstrated active progression and they had a mean annual loss of 4.5%. Our four symptomatic Huntington's disease patients showed a mean annual loss of D2 binding of 3.0% and of D1 binding of 5.0%. Loss of striatal D1 and D2 binding was significantly greater in the known mutation carriers than in the combined at-risk and gene-negative groups (P < 0.05). At follow-up PET all subjects were clinically assessed using the Unified Huntington's Disease Rating Scale. Scores for motor function and total functional capacity correlated with PET measures of striatal dopamine receptor binding both in the asymptomatic mutation carriers (D1, P < 0.01) and across the combined asymptomatic and clinically affected Huntington's disease mutation carrier group (D1 and D2, P < 0.001). We conclude that PET measures of striatal D1 and D2 dopamine binding can be used to identify asymptomatic Huntington's disease mutation carriers who are actively progressing and who would thus be suitable for putative neuroprotective therapies. Measures of disease progression rates in Huntington's disease patients and asymptomatic mutation carriers will be of critical importance in future trials of experimental restorative treatments.

Adult↗
PubMed1999

Attention and executive deficits in Alzheimer's disease. A critical review.

In this review we summarize the progress that has been made in the research on attentional and executive deficits in Alzheimer's disease. Like memory, attention is now recognized as consisting of subtypes that differ in their function and anatomical basis. We base our review upon a classification of three subtypes of attention: selective, sustained and divided. This model derives from lesion studies, animal electrophysiological recordings and functional imaging. We examine how these subcomponents of attention can be reconciled with neuropsychological models of attentional control, particularly the Supervisory Attentional System and the Central Executive System of Shallice and Baddeley, respectively. We also discuss the relationship of attention to the concept of executive function. Current evidence suggests that after an initial amnesic stage in Alzheimer's disease, attention is the first non-memory domain to be affected, before deficits in language and visuospatial functions. This is consistent with the possibility that difficulties with activities of daily living, which occur in even mildly demented patients, may be related to attentional deficits. It appears that divided attention and aspects of selective attention, such as set-shifting and response selection, are particularly vulnerable while sustained attention is relatively preserved in the early stages. The phenomenon of cognitive slowing in Alzheimer's disease and normal ageing emphasizes the need to discriminate quantitative changes in attention dysfunction from qualitative changes which may be specifically related to the disease process. The neuropathological basis of these attentional deficits remains unsettled, with two competing hypotheses: spread of pathology from the medial temporal to basal forebrain structures versus corticocortical tract disconnection. Finally we discuss the difficulties of comparing evidence across studies and look at the implications for the design of future studies and future directions that may be fruitful in the research on attention in Alzheimer's disease.

Animals↗
PubMed1999

Specific cognitive deficits in mild frontal variant frontotemporal dementia.

Eight patients with relatively mild frontal variant frontotemporal dementia (fvFTD) were compared with age- and IQ-matched control volunteers on tests of executive and mnemonic function. Tests of pattern and spatial recognition memory, spatial span, spatial working memory, planning, visual discrimination learning/attentional set-shifting and decision-making were employed. Patients with fvFTD were found to have deficits in the visual discrimination learning paradigm specific to the reversal stages. Furthermore, in the decision-making paradigm, patients were found to show genuine risk-taking behaviour with increased deliberation times rather than merely impulsive behaviour. It was especially notable that these patients demonstrated virtually no deficits in other tests that have also been shown to be sensitive to frontal lobe dysfunction, such as the spatial working memory and planning tasks. These results are discussed in relation to the possible underlying neuropathology, the anatomical connectivity and the hypothesized heterogeneous functions of areas of the prefrontal cortex. In particular, given the nature of the cognitive deficits demonstrated by these patients, we postulate that, relatively early in the course of the disease, the ventromedial (or orbitofrontal) cortex is a major locus of dysfunction and that this may relate to the behavioural presentation of these patients clinically described in the individual case histories.

Adult↗
PubMed1999

Dyspraxia in a patient with corticobasal degeneration: the role of visual and tactile inputs to action.

OBJECTIVES: To investigate the roles of visual and tactile information in a dyspraxic patient with corticobasal degeneration (CBD) who showed dramatic facilitation in miming the use of a tool or object when he was given a tool to manipulate; and to study the nature of the praxic and neuropsychological deficits in CBD. METHODS: The subject had clinically diagnosed CBD, and exhibited alien limb behaviour and striking ideomotor dyspraxia. General neuropsychological evaluation focused on constructional and visuospatial abilities, calculation, verbal fluency, episodic and semantic memory, plus spelling and writing because impairments in this domain were presenting complaints. Four experiments assessed the roles of visual and tactile information in the facilitation of motor performance by tools. Experiment 1 evaluated the patient's performance of six limb transitive actions under six conditions: (1) after he described the relevant tool from memory, (2) after he was shown a line drawing of the tool, (3) after he was shown a real exemplar of the tool, (4) after he watched the experimenter perform the action, (5) while he was holding the tool, and (6) immediately after he had performed the action with the tool but with the tool removed from his grasp. Experiment 2 evaluated the use of the same six tools when the patient had tactile but no visual information (while he was blindfolded). Experiments 3 and 4 assessed performance of actions appropriate to the same six tools when the patient had either neutral or inappropriate tactile feedback-that is, while he was holding a non-tool object or a different tool. RESULTS: Miming of tool use was not facilitated by visual input; moreover, lack of visual information in the blindfolded condition did not reduce performance. The principal positive finding was a dramatic facilitation of the patient's ability to demonstrate object use when he was holding either the appropriate tool or a neutral object. Tools inappropriate to the requested action produced involuntary performance of the stimulus relevant action. CONCLUSIONS: Tactile stimulation was paramount in the facilitation of motor performance in tool use by this patient with CBD. This outcome suggests that tactile information should be included in models which hypothesise modality specific inputs to the action production system. Significant impairments in spelling and letter production that have not previously been reported in CBD have also been documented.

Apraxias↗
PubMed1999

Cognition, language and behaviour in motor neurone disease: evidence of frontotemporal dysfunction.

Cognitive symptoms accompanying motor neurone disease (MND) have been recognized and described since the late 19th century. Numerous reports from Europe, North America and Japan suggest existence of a syndrome that can be described as MND/dementia. Typically, psychiatric and cognitive changes, strongly reminiscent of frontotemporal dementia, precede the occurrence of the classical signs and symptoms of MND by several months. In a small number of patients a similar picture can be heralded by a progressive aphasia leading ultimately to mutism. While the syndromes of MND/dementia and MND/aphasia constitute a comparatively small group, subtle but consistent cognitive alterations have also been observed in the majority of nondemented MND patients. Although generally much less pronounced, their pattern, affecting mostly frontal-executive functions, resembles that of MND/dementia. Postmortem examination results, describing pathological changes in the frontal lobes, and functional neuroimaging studies, showing abnormal pattern of frontal activation, add more weight to the hypothesis linking MND to the frontotemporal dementia.

Behavior↗
PubMed1999

Early diagnosis of the frontal variant of frontotemporal dementia: how sensitive are standard neuroimaging and neuropsychologic tests?

OBJECTIVE: To examine the role of structural (magnetic resonance imaging [MRI]) and functional (single photon emission computed tomography [SPECT]) imaging and neuropsychologic evaluation in the early diagnosis of frontal variant frontotemporal dementia (fvFTD). BACKGROUND: Current criteria for FTD stress the need for neuropsychologic and functional neuroimaging abnormalities, yet caregivers report lengthy histories of behavioral change. It is not known when, in the course of the disease, these investigations become abnormal, because few longitudinal studies have been reported. METHOD: Longitudinal study of two patients with serial neuropsychologic evaluation and MRI and HMPAO-SPECT scanning. RESULTS: Both patients, men aged 49 and 50, had major changes in personality, behavior, and social conduct that progressed over 5 to 6 years in a way that conformed to the clinical picture of fvFTD. There was remarkably little abnormality on neuropsychologic testing, and MRI and HMPAO-SPECT findings initially were normal. Over time, however, abnormalities on SPECT, frontal atrophy on MRI, or a neuropsychologic profile more typical of fvFTD developed in both patients. CONCLUSIONS: Standard neuropsychologic tests and conventional brain imaging techniques (MRI and SPECT) may not be sensitive to the early changes in fvFTD that occur in the ventromedial frontal cortex, and better methods of accurate early detection are required. These findings are relevant to the diagnostic criteria for FTD.

Atrophy↗
PubMed1998

Single word production in nonfluent progressive aphasia.

We present an experiment investigation of the spoken single word production of two patients with nonfluent progressive aphasia. In Experiment 1, a task effect (reading > repetition > naming) suggested that phonological information available from task stimuli facilitated the patients' speech production; a length effect reflected the increased difficulty of phonological processing required for long words compared with that required for shorter words. Experiment 2 compared repetition, reading, copying, and writing to dictation tasks and demonstrated that a correspondence between input and output modality also facilitated performance. Experiment 3 showed that the patients' access to appropriate phonology in reading was positively related to the degree of correlation between orthographic and phonological forms. These results are discussed with reference to an account of pathologically weakened connections between nodes in an interactive spreading activation model of speech production of the type described by Dell (1986).

Aged↗