Unraveling the enigma of transient global amnesia.
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Amnesia↗
Biomedical subjects
J R Hodges
Publications and source records attributed to J R Hodges.
Word reading in Alzheimer's disease: cross-sectional and longitudinal analyses of response time and accuracy data.
Using a list of high- and low-frequency regular and exception words, we measured the reading performance of three groups of subjects: patients with mild dementia of the Alzheimer's type (DAT) at presentation (Stage 1) and 2-3 years later (Stage 2); moderately severe DAT patients; and control subjects. In the longitudinally studied patients, there was a dramatic increase in response times (RTs) from Stage 1 to Stage 2, but little change in either error rate or pattern. By contrast, a comparison of the Stage 2 with the Moderate patients revealed similar RTs but a significant increase in error rate for the Moderate group, particularly on low-frequency exception words. These two results for word naming were almost exactly mirrored by effects in picture naming. We conclude that correct word reading, especially for less common words with atypical spelling-sound correspondences, is compromised in DAT only when the disease produces a significant deterioration of semantic memory. These results are relevant to current theories about normal and impaired reading processes.
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Face processing impairments after encephalitis: amygdala damage and recognition of fear.
Face processing and facial emotion recognition were investigated in five post-encephalitic people of average or above-average intelligence. Four of these people (JC, YW, RB and SE) had extensive damage in the region of the amygdala. A fifth post-encephalitic person with predominantly hippocampal damage and relative sparing of the amygdala (RS) participated, allowing us to contrast the effects of temporal lobe damage including and excluding the amygdala region. The findings showed impaired recognition of fear following bilateral temporal lobe damage when this included the amygdala. For JC, this was part of a constellation of deficits on face processing tasks, with impaired recognition of several emotions. SE, YW and RB, however, showed relatively circumscribed deficits. Although they all had some problems in recognizing or naming famous faces, and had poor memory for faces on the Warrington Recognition Memory Test, none showed a significant impairment on the Benton Test of Facial Recognition, indicating relatively good perception of the face's physical structure. In a test of recognition of basic emotions (happiness, surprise, fear, sadness, disgust and anger), SE, YW and RB achieved normal levels of performance in comparison to our control group for all emotions except fear. Their results contrast with those of RS, with relative sparing of the amygdala region and unimpaired recognition of emotion, pointing clearly toward the importance of the amygdala in the recognition of fear.
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A reversal of the temporal gradient for famous person knowledge in semantic dementia: implications for the neural organisation of long-term memory.
On tests of autobiographical memory, patients with semantic dementia demonstrate significantly better retrieval of episodic events from the recent past compared with the distant past. This reversal of the Ribot effect has been attributed to the relative sparing of the hippocampal complex in the disorder. Current computational models of long-term memory predict a similar time-dependent pattern of impairment on tests of remote semantic memory. Five patients with semantic dementia were tested on recognition (familiarity) and identification (knowledge) of famous names selected from four different time-periods: 1950's, 1980's, 1990-1993 (early 1990's) and 19941996 (current). As expected, it was found that one patient DM (who had focal left temporal lobe atrophy) showed no significant impairment on recognition of famous names, but was significantly better at producing information about people who were currently famous compared to people famous in the other three time-periods. The other four patients (who had bilateral temporal lobe damage) showed better recognition of famous names from the current time-period (and to a lesser extent the 1950's), yet were profoundly impaired on the identification component, producing very little information across all four time-periods. The results are discussed with respect to current views of the neural organisation of person-specific and general semantic memory.
Aphasia, Wernicke↗
Naming in semantic dementia--what matters?
One of the major symptoms of semantic dementia (or progressive fluent aphasia) is profound word-finding difficulties. We present here a cross-sectional study of the factors affecting picture naming in semantic dementia based on data obtained from eight patients, together with a longitudinal analysis of naming in another patient. Various properties and attributes of the objects were entered into a series of regression analyses in order to predict which items the patients could or could not name. The analyses showed that object familiarity, word frequency and age-of-acquisition predicted naming success for the group and, in most cases, for each individual patient, irrespective of lesion site or overall naming success. We propose that the pattern of naming in semantic dementia is best described in terms of reduced semantic activation within a cascading/interactive speech production system. We suggest that object familiarity, and possibly word frequency, reflect the inherent robustness of individual semantic representations to the decay process in terms of both quantity and quality of experience. Age-of-acquisition and word frequency (at a phonological-lexical level) predicts naming success, because frequent, early-acquired words are relatively easy to activate even with reduced semantic "input".
Adult↗
Knowing about people and naming them: can Alzheimer's disease patients do one without the other?
It has recently been suggested that patients with semantic breakdown may show the phenomenon of so-called "naming without semantics". If substantiated, this finding would clearly have a major impact on theories of face and object processing, all of which assume that access to semantic knowledge is a prerequisite for successful naming. In order to investigate this issue, we studied recognition, identification (the ability to provide accurate information), and naming of 50 famous faces by 24 patients with mild to moderate dementia of Alzheimer type (DAT) and 30 age-matched controls. The DAT group was impaired in all three conditions. An analysis of the concordance between identification and naming by each patient, for each stimulus item, established that naming a famous face was possible only with semantic knowledge sufficient to identify the person. Our data support the hypothesis that naming is not possible unless semantic information associated with the target is available. Naming without semantics, therefore, did not occur in patients with DAT. By contrast, there were 206 instances (17% of the total responses) in which the patients were able to provide detailed, accurate identifying information yet were unable to name the person represented. The implication of these findings for models of face identification and naming are discussed.
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Category specific semantic loss in dementia of Alzheimer's type. Functional-anatomical correlations from cross-sectional analyses.
In the context of focal brain injury, selective loss of semantic knowledge in the domain of either natural kinds or artefacts is usually considered to reflect the differential importance of temporal and frontoparietal regions to the representations of perceptual and functional attributes, respectively. It is harder to account far as a feature of a more diffuse process, and previous cross-sectional analyses of patients with dementia of Alzheimer's type (DAT) have differed over whether category effects occur. In our series of 58 patients with probable DAT, we demonstrated a significant group advantage for artefacts, and explored possible reasons for the inconsistency of this finding in other studies. A multiple single-case strategy revealed not only individuals with consistent advantages for artefacts but also individuals with consistent advantages for natural kinds. By ranking the individuals according to measures of naming performance and global intellectual ability, we showed that the strength of the group advantage for artefacts was dependent on the former but not the latter variable. The findings are discussed in the context of two competing theories of semantic breakdown in DAT. One differentiates between domains of knowledge in terms of the structure of semantic representations within a single distributed network; the other emphasizes the importance of different brain regions in the category distinction. We conclude that our findings are in keeping with the predictions of the latter hypothesis.
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Dissociating executive mechanisms of task control following frontal lobe damage and Parkinson's disease.
Twelve patients with focal damage of the frontal cortex and 12 patients with mild, medicated, early stage Parkinson's disease switched between letter- and digit=naming tasks on every second trial of a task-switching paradigm. Compared with age- and IQ-matched control performance, patients with left-sided, but not right-sided, frontal damage exhibited markedly increased time costs associated with these predictable switches only when there was a general incidence of interference or 'crosstalk' between the tasks, and particularly so when the available task cues were relatively weak and arbitrary. The same patients also showed evidence of an increased sensitivity to the facilitatory and inhibitory effects of previous processing, when required to switch between tasks. Both groups of patients (with left- or right-sided frontal damage) exhibited slow, disorganized performance early in practice. In contrast to these frontal effects, the Parkinson's disease patients showed little indication of larger time costs of task switches but they did show progressive increases in the error costs, while age- and IQ-matched control subjects showed reductions. We propose that while both left and right frontal cortical areas are involved in the organization of cognitive and motor processes in situations involving novel task demands, only the left frontal cortex is involved in the dynamic reconfiguring between already-established task-sets, and specifically, that it is the site of an executive mechanism responsible for the modulation of exogenous task-set activity. Finally, dopaminergic transmission, along the nigrostriatal pathway, may be implicated in sustaining various cognitive and motor processes over prolonged periods, including the operation of those executive control mechanisms that accomplish reconfiguring between task-sets.
Adult↗
Acquisition of post-morbid vocabulary and semantic facts in the absence of episodic memory.
Disagreement exists in the literature over whether it is possible for new semantic (factual) information to be learned in the absence of episodic (event) memory. We report the case of R.S., a 49-year-old amnesic man, who we found to have acquired information about famous people, public events and new vocabulary during the 13-year period since he became amnesic, despite having no measurable anterograde episodic memory function and a profound loss of autobiographical memory. These data suggest that at least some severe amnesics are able to acquire new semantic knowledge, provided that critical temporal neocortical regions are spared. We discuss these findings in the context of recent connectionist models of memory.
Amnesia↗
Evidence for specific cognitive deficits in preclinical Huntington's disease.
The performance of 54 subjects genetically at risk for Huntington's disease was examined in double-blind fashion on a series of computerized tests from the Cambridge Neuropsychological Test Automated Battery. None of the subjects exhibited clinical movement disorder characteristic of Huntington's disease. Of the 54 subjects, 22 were Huntington's disease mutation carriers and 32 were non-carriers. On a comprehensive battery of neuropsychological tests previously shown to be sensitive to the early stages of clinical Huntington's disease, Huntington's disease mutation carriers exhibited highly specific cognitive deficits. In particular, Huntington's disease mutation carriers performed significantly less well than non-carriers, matched for age and IQ, on tests of attentional set shifting and semantic verbal fluency. Furthermore, performance on these two tests was significantly correlated, even after partialling out the effects of age and IQ. It is suggested that these cognitive impairments relate to a common deficit in inhibitory control mechanisms, under the control of striatofrontal mechanisms, and that such a deficit is present in Huntington's disease mutation carriers prior to the onset of definite motor symptomatology. The implications for the nature of the cognitive decline seen in Huntington's disease, and possible future treatment strategies, are discussed.
Adult↗
Transient epileptic amnesia: a description of the clinical and neuropsychological features in 10 cases and a review of the literature.
OBJECTIVES: To clarify the clinical and neuropsychological aspects of transient epileptic amnesia (TEA) based on 10 personally studied cases as well as review of 21 previously published cases; and to propose tentative diagnostic criteria for the diagnosis of TEA. METHODS: All 10 patients and informants underwent a standardised clinical interview. The radiological and neurophysiological (EEG) data were also reviewed in all cases. The diagnosis of transient epileptic amnesia was made on the basis of the following criteria: (1) there was a history of recurrent witnessed episodes of transient amnesia; (2) cognitive functions other than memory were judged to be intact during typical episodes by a reliable witness; (3) there was evidence for a diagnosis of epilepsy. This evidence was provided by either (a) wake or sleep EEG, or (b) the co-occurrence of other seizure types (if their roughly concurrent onset or close association with episodes of transient amnesia suggested a connection), or (c) a clear cut response to anticonvulsant therapy, or by a combination of these three factors. In addition all patients were administered a comprehensive neuropsychological test battery designed to assess verbal and non-verbal anterograde memory and retrograde memory for famous personalities and personal events. Their results were compared with those of 25 age and IQ matched normal controls. RESULTS: TEA usually begins in later life, with a mean age of 65 years in this series. Episodes are typically brief, lasting less than one hour, and recurrent, with a mean frequency of three a year. Attacks on waking are characteristic. Repetitive questioning occurs commonly during attacks. The anterograde amnesia during episodes is, however, often incomplete so that patients may later be able to "remember not being able to remember". The extent of the retrograde amnesia during attacks varies from days to years. Most patients experience other seizure types compatible with an origin in the temporal lobes, but transient amnesia is the only manifestation of epilepsy in about one third of patients. Epileptiform abnormalities arising from the temporal lobes are most often detected on interictal sleep EEG. Despite normal performance on tests of anterograde memory, many patients complain of persistent interictal disturbance of autobiographical memory, involving a significant but variable loss of recall for salient personal episodes. The epochs affected may predate the onset of epilepsy by many years. CONCLUSIONS: TEA is an identifiable syndrome and comprises episodic transient amnesia with an epileptic basis, without impairment of other aspects of cognitive function. Future studies should consider the question of whether TEA reflects ictal activity or a postictal state, and the mechanism of the persistent autobiographical amnesia. It is hypothesised that the latter may result in part from impairment of very long term memory consolidation as a result of epileptic activity in mesial temporal structures.
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Dementia and aphasia in motor neuron disease: an underrecognised association?
OBJECTIVES: To determine the prevalence and nature of global cognitive dysfunction and language deficits in an unselected population based cohort of patients with motor neuron disease (MND). METHODS: A battery of neuropsychological and language tests was administered to patients presenting consecutively over a 3 year period to a regional neurology service with a new diagnosis of sporadic motor neuron disease. RESULTS: The 18 patients could be divided on the basis of their performance into three groups: Three patients were demented and had impaired language function (group 1); two non-demented patients had an aphasic syndrome characterised by word finding difficulties and anomia (group 2). Major cognitive deficits were therefore found in five of the 18 patients (28%). The remaining 13 performed normally on the test battery apart from decreased verbal fluency (group 3). CONCLUSIONS: The prevalence of cognitive impairment in MND in this population based study of an unselected cohort was higher than has been previously reported. Language deficits, especially anomia, may be relatively frequent in the MND population. Aphasia in MND may be masked by dysarthria and missed if not specifically examined.
Adult↗
Functional neuroanatomy of the semantic system: divisible by what?
Studies of patients with brain damage suggest that specific brain regions may be differentially involved in representing/processing certain categories of conceptual knowledge. With regard to the dissociation that has received the most attention--between the domains of living things and artifacts--a debate continues as to whether these category-specific effects reflect neural implementation of categories directly or some more basic properties of brain organization. The present positron emission tomography (PET) study addressed this issue by probing explicitly for differential activation associated with written names of objects from the domains of living things or artifacts during similarity judgments about different attributes of these objects. Subjects viewed triads of written object names and selected one of two response words as more similar to a target word according to a specified perceptual attribute (typical color of the objects) or an associative attribute (typical location of the objects). The control task required a similarity judgment about the number of syllables in the target and response words. All tasks were performed under two different stimulus conditions: names of living things and names of artifacts. Judgments for both domains and both attribute types activated an extensive, distributed, left-hemisphere semantic system, but showed some differential activation-particularly as a function of attribute type. The left temporo-occipito-parietal junction showed enhanced activity for judgments about object location, whereas the left anteromedial temporal cortex and caudate nucleus were differentially activated by color judgments. Smaller differences were seen for living and nonliving domains, the positive findings being largely consistent with previous studies using objects; in particular, words denoting artifacts produced enhanced activation in the left posterior middle temporal gyrus. These results suggest that, within a distributed conceptual system activated by words, the more prominent neural distinction relates to type of attribute.
Adult↗
Patients with features similar to Huntington's disease, without CAG expansion in huntingtin.
OBJECTIVE: To describe characteristics of gene-negative patients with clinical features of Huntington's disease (HD), exploring likely etiologies. BACKGROUND: When a direct gene test became definitive for diagnosis of HD, we discovered a number of patients in our clinics in Baltimore, MD, and Cambridge, UK, believed or suspected to have HD who did not have the triplet repeat expansion. METHODS: Patients were examined using standardized instruments, and given full neurologic and psychiatric evaluations. Those negative for HD were tested for dentatorubro-pallidoluysian atrophy, SCA-1, SCA-3, SCA-2, SCA-6, and other conditions as indicated. RESULTS: Of 15 patients, 7 received specific diagnoses or appear to be sporadic cases, 4 have a possible but uncertain relation to HD, and 4 have unknown familial progressive movement disorders. CONCLUSIONS: This last group of patients might be properly described as phenocopies of HD, some of which may be caused by unidentified triplet repeat expansions.
Adult↗
Can frontotemporal dementia and Alzheimer's disease be differentiated using a brief battery of tests?
OBJECTIVE: To compare the performance of patients with frontotemporal dementia (FTD) and Alzheimer's disease (AD) on a range of simple neuropsychological tests. DESIGN: A battery of neuropsychological tests easily applied at the bedside, consisting of traditional tests of memory, attention and executive function, were given together with tests of motor sequencing and examination of frontal release signs. In addition, we devised a theoretically motivated test of dual attention-a story with distraction which also contained a 'social dilemma'. SETTING: Specialist memory and cognitive disorders clinic. PATIENTS: 12 patients with FTD and 12 patients with AD, matched for overall level of dementia on the Mini-Mental State Examination, were selected. RESULTS: In general, the difference in results between FTD and AD patients was small. However, a composite score derived from the presence of a grasp and pout reflex, the number of perseverations during category fluency for animals and response to the social dilemma within the two stories produced a sensitivity of 83.3% and specificity of 91.6%. There was also a highly significant difference between patients with FTD and AD in scores achieved on the Clinical Dementia Rating Scale reflecting the marked change in behaviour that patients with FTD suffer, even at a stage when memory functions are well preserved. CONCLUSION: Traditional neuropsychological tests were poor at differentiating cases of FTD and AD; however, a composite (SIFTD) score appears potentially useful but requires prospective validation. Better methods of assessing the changes in comportment that characterize the early stages of FTD are required.
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A comparison of semantic memory in vascular dementia and dementia of Alzheimer's type.
OBJECTIVE: To determine whether semantic memory is impaired in vascular dementia and to assess the utility of semantic memory measures in differentiating vascular dementia from dementia of Alzheimer's type (DAT). DESIGN: Case-control study. PATIENTS: Ten patients with Cambridge Mental Disorders in the Elderly (CAMDEX) diagnosis of 'definite' mild or moderate vascular dementia (mean age 77) were individually matched with 10 patients with a CAMDEX diagnosis of 'definite' DAT on the basis of age, education, sex, premorbid IQ (as measured by the National Adult Reading Test) and performance on the Cambridge Cognitive Examination (CAMCOG). In addition, 10 age, sex and education matched volunteer or relative controls were assessed. OUTCOME MEASURES: A detailed semantic memory test battery consisting of five subtests: category fluency, picture naming, picture sorting, word-picture matching and generation of verbal definitions. RESULTS: Compared to normal controls, both patient groups were impaired on all subtests of the semantic battery with the exception of the word-picture matching test. No differences were found between the vascular dementia and DAT groups on any of the measures. CONCLUSIONS: Impairment of semantic memory is a feature of both vascular dementia and DAT. Tests of semantic memory appear, therefore, of little value in differentiating between these two major causes of dementia. Further work is required to determine whether the nature of the processing deficit is the same in these conditions.
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Knowing where and knowing what: a double dissociation.
We report a double dissociation between visuo-spatial abilities and semantic knowledge (knowledge of the names and attributes of objects and people), in two brain-injured people with longstanding stable impairments, using a wide range of tests to explore the extent of the dissociation, MU, who has bilateral lesions of occipito-parietal cortex, shows severe spatial disorientation with relatively well-preserved semantic knowledge. He is contrasted with JBR, who has bilateral temporal lobe damage and shows severe semantic problems and no impairment on visuo-spatial tasks. Our findings thus demonstrate a double dissociation between the performance of semantic and spatial tasks by MU and JBR. This pattern is consistent with Ungerleider and Mishkin's (1982) neurophysiological hypothesis of separable cortical visual pathways; one which is specialised for spatial perception and follows a dorsal route from occipital to parietal lobes, and the other following a more ventral route from occipital to temporal lobes, whose target is semantic information needed in specifying what an object is.
Adult↗
Preservation of famous person knowledge in a patient with severe post anoxic amnesia.
A 58 year old patient (ES) suffered severe anterograde and retrograde amnesia following prolonged cardiac arrest with presumed hypoxic brain damage. Personally relevant autobiographical memory was severely impaired as was knowledge of public events. In contrast, knowledge of famous people was very well preserved. This findings has implications for the organisation of remote memory. Knowledge of people is clearly represented independently from autobiographical memory. We argue that this pattern of profound autobiographical amnesia may result from either multifocal neocortical damage (as in this case) or a failure of the "thematic framework" system involved in the active reconstruction of autobiographical memory, as hypothesised in another patient with thalamic infarction who showed a similar pattern of results (Hodges and McCarthy, 1993).
Amnesia↗