A Model-Based Approach to Understanding Apraxia in Corticobasal Syndrome

Limb apraxia is a neurological disorder of higher cognitive function characterized by an inability to perform purposeful skilled movements and not attributable to an elementary sensorimotor dysfunction or comprehension difficulty. Corticobasal Syndrome (CBS) is an akinetic rigid syndrome with asymmetric onset and progression with at least one basal ganglia feature (rigidity, limb dystonia or myoclonus) and one cortical feature (limb apraxia, alien hand syndrome or cortical sensory loss). Even though limb apraxia is highly prevalent in CBS (70–80%), very few studies have examined the performance of CBS patients on praxis measures in detail. This review aims to (1) briefly summarize the clinical, neuroanatomical and pathological findings in CBS, (2) briefly outline what limb apraxia is and how it is assessed, (3) to comprehensively review the literature on limb apraxia in CBS to date and (4) to briefly summarize the literature on other forms of apraxia, such as limb-kinetic apraxia and buccofacial apraxia. Overall, the goal of the review is to bring a model-based perspective to the findings available in the literature to date on limb apraxia in CBS.     

Error Analysis of Limb and Orofacial Praxis in Children with Developmental Motor Deficits

Two models have been suggested to depict the relationship between disorders of limb and orofacial praxis. The first views apraxia as a unitary disorder in which the underlying mechanisms for each type are similar, while the second model suggests that there are two separate praxis systems: one for planning and controlling limb gestures and a second one for planning and controlling orofacial movements. The purpose of this study was to investigate whether a common mechanism may underlie deficits in limb and orofacial praxis in children. This was done by analyzing the types of praxis errors demonstrated by children with developmental motor deficits and normal controls when performing limb and orofacial gestures. Results indicated that there was consistency across modalities (i.e., limb, orofacial) in the types of praxis errors made by children with motor deficits, providing support for the idea that a common mechanism may underlie disruptions to limb and orofacial praxis in children. This study also examined developmental trends in gestural representation and in types of praxis errors. The findings revealed a striking developmental maturation in gestural ability between the ages of 6 and 11 years for all children. However, over this age range, children with developmental motor deficits were impaired relative to normal controls.     

The role of the dynamic body schema in praxis: evidence from primary progressive apraxia

On an influential model of limb praxis, ideomotor apraxia results from damage to stored gesture representations or disconnection of representations from sensory input or motor output (Heilman & Gonzalez Rothi, 1993; Gonzalez Rothi et al., 1991). We report data from a patient with progressive ideomotor limb apraxia which cannot be readily accommodated by this model. The patient, BG, is profoundly impaired in gesturing to command, to sight of object, and to imitation, but gestures nearly normally with tool in hand and recognizes gestures relatively well. In addition, performance is profoundly impaired on imitation of meaningless gestures and on tasks requiring spatiomotor transformations of body-position information. We provide evidence that BG's apraxia is largely attributable to impairments external to the stored gesture system in procedures coding the dynamic positions of the body parts of self and others; that is, the body schema. We propose a model of a dynamic, interactive praxis system subserved by posterior parietal cortex in which stored representational elements, when present, provide "top-down" support to spatiomotor procedures computed on-line. In addition to accounting for BG's performance, this model accommodates a common pattern of ideomotor apraxia more readily than competing accounts.     

Spatial factors in visual attention: a reply to Crassini

Distances between stimuli and, derivatively, compactness of stimulus subsets are pervasive determiners of discrimination and classification performance. Contrary to Crassini (1986), these factors are sufficient to account for the major patterns in the chronometric data of Podgorny and Shepard (1978, 1983). Other factors, such as the dichotomous one distinguishing what Crassini terms unitary and nonunitary subsets, may exert some additional influence. But a convincing demonstration would require formulation of a quantitative model capable of being pitted against the distance-based model of Podgorny and Shepard within the context of their entire body of data.     

The relationship of limb apraxia severity to motor and language deficits

Ipsilateral motor and symbolic/linguistic skills were examined in normals and three left hemisphere damaged groups with no, mild, or moderate-severe limb apraxia. When the two apraxic groups were pooled they demonstrated poorer response inhibition and reading relative to the nonapraxic group. There were no significant group differences on motor or language tasks when the three brain damaged groups were separately compared. These results indicate there is a limited relationship between limb apraxia and some motor and linguistic skills, but the relationship was not greater for motor than linguistic skills.     

Cognition in action: testing a model of limb apraxia

Assessment of limb apraxia is still suffering from Liepmann's legacy and performance in gesture-processing tests is generally rendered by classifying patients' profile according to the classic clinical labels of ideomotor and ideational apraxia. At odds with other cognitive functions, interpretation of apraxia has suffered from a lack of a reliable model which does justice to its complexity. Recently such a model has been proposed (Rothi et al., 1991, 1997). In this article a modified version of this model is presented and predictions are made according to its functional architecture. Five different patterns of impairment of gesture processing are postulated. To validate the predicted performance profiles, 19 left-hemisphere-damaged patients were assessed by means of an ad hoc battery of four praxis tests. Four of the five predicted apraxia patterns were observed, the fifth being more equivocal. These results support the need to overcome the simplistic dichotomous view of apraxia and confirm the fruitfulness of a model of normal gesture processing in order to understand dissociations in apraxia.     

On the spatial distribution of visual attention

An implication of the data analysis and presentation of Podgorny and Shepard (1983) is that subjects are able to attend simultaneously to more than one square of a grid display when the squares are separated by unattended areas: Attention to such nonunitary areas produces similar benefits as attention to unitary areas. These benefits are reflected in reaction times (RTs), which were reported by Podgorny and Shepard (1983) as being related to a measure of spatial dispersion (compactness) of the attended areas, but this measure does not signify whether these areas are unitary or not. A reanalysis of part of Podgorny and Shepard's (1983) data shows that RTs to attended and unattended squares are almost identical when the attended areas are nonunitary. This reanalysis also shows that RTs are related to compactness for unitary attended areas but that this relation breaks down when attention is focused on nonunitary areas. In addition, Podgorny and Shepard's (1983) data are presented in a way that demonstrates the importance of the actual grid location of probes on RTs. The failure of compactness to reflect these aspects of the spatial nature of attention suggests that this metric is deficient when applied to the study of the spatial determinants of attention.     

Mutism associated with buccofacial apraxia and bihemispheric lesions

Mutism following brain trauma is quite common, is usually transient, and recovery of speech is essentially the rule. Lasting total absence of speech without aphasia is highly unusual. Three such patients, two of traumatic and one due to vascular origin showing buccofacial apraxia (BFA) and computerized tomography (CT) evidence of bilateral frontal lesions are reported. It is suggested that complete lasting mutism associated with BFA is a result of bihemispheric lesions affecting mainly the opercular part of the inferior frontal gyrus and immediate adjacent regions.     

The Assessment of Limb Apraxia: An Investigation of Task Effects and Their Cause

To test the effects of variations in the structure of tasks used to assess limb apraxia, eight tasks—differing in their modes of elicitation (tactile, verbal, visual, imitation) and/or types of movement elicited (transitive, intransitive, meaningless)—were administered to 25 carefully selected subjects with idiomotor limb apraxia. When standardized scores (based on the performance of 10 nonneurologically impaired control subjects) were used to measure the differences between tasks, no clinically significant task effects were found. Additional investigation was made of two causal hypotheses regarding the processes underlying limb apractic performance. The results of factor analyses and a search for double dissociations among individual subjects were consistent with the (severity) hypothesis that differences in task performances (raw scores) reflect degrees of task difficulty; and they failed to support the (disconnection) hypothesis that posits separate and independent neural/mental processes underlying task performances.     

Patterns of apraxia associated with the production of intransitive limb gestures following left and right hemisphere stroke

The model of apraxia proposed by Roy (1996) states that three patterns of apraxia should be observed across pantomime and imitation conditions. In the present analysis the frequency and severity of each pattern of apraxia were examined in a consecutive sample of left-(LHD) and right-hemisphere-damaged (RHD) patients during the production of intransitive limb gestures. The results indicated that a significant proportion of LHD and RHD patients were selectively impaired in formulating the ideational component of intransitive limb gestures.     

Neuropsychological differentiation of progressive aphasic disorders

The differentiation of subtypes of primary progressive aphasia (PPA) remains challenging. We aimed to identify optimum neuropsychological measures for characterizing PPA, to examine the relationship between behavioural change and subtypes of PPA and to determine whether characteristic profiles of language, working memory, and behavioural changes occur in PPA. Forty-seven patients with PPA and multi-domain Alzheimer's disease (AD) together with 19 age-matched controls underwent a large battery of working memory and language tests. We found that simple tasks of sentence ordering, narrative production, and buccofacial praxis were particularly useful in differentiating non-fluent/agrammatic variant PPA (nfvPPA) from other PPA subtypes, whereas a test of single word comprehension was useful in detecting semantic dementia (SD). No individual tests were discriminating for logopenic variant PPA (lvPPA) relative to nfvPPA. LvPPA and multidomain AD exhibited similar language profiles. A principal components analysis revealed that characteristic PPA profiles extended beyond the realms of language, in particular, the presence of apraxia in nfvPPA, behavioural changes in SD, and working memory deficits in lvPPA. These findings suggest that not all tests are equally discriminatory for PPA and highlight the importance of a test profile in differentiating PPA. These results also support the view that lvPPA is a focal form of AD and emphasize the difficulties classifying lvPPA.     

An update on the Conceptual-Production Systems model of apraxia: evidence from stroke

Limb apraxia is a neurological disorder characterized by an inability to pantomime and/or imitate gestures. It is more commonly observed after left hemisphere damage (LHD), but has also been reported after right hemisphere damage (RHD). The Conceptual-Production Systems model (Roy, 1996) suggests that three systems are involved in the control of purposeful movements: the conceptual, the production and the sensory/perceptual system. Depending on which system is damaged different patterns of apraxia are expressed. To determine the apraxia pattern, pantomime, delayed, and concurrent imitation tasks need to be administered, as well as conceptual tasks assessing one's knowledge of actions. Based on the model, eight patterns of apraxia should emerge. The purpose of this study is to determine whether these patterns are in fact observed in stroke patients and examine their frequency. If the performance of most stroke patients falls into one of the patterns, then we would have strong support for the conceptual-production model. Stroke (34 LHD and 39 RHD) patients and 27 age- and education-matched healthy controls participated in the study. Participants were assessed in four task modalities: pantomime, delayed imitation, concurrent imitation and conceptual knowledge (two tasks were used: tool naming by action and action identification). Patients were categorized as impaired on a task if they scored 2 SD below the mean performance of the controls for gesture production tasks, or below a cut-off score on the conceptual tasks. They were then classified into patterns depending on their performance on the four task modalities. Most patients (86%) fell into one of seven patterns originally predicted from the Conceptual-Production Systems model. The two most common patterns were deficits in pantomime and imitation with preserved gesture recognition and conduction apraxia (selective deficit in imitation). Four new patterns emerged, but mostly single cases of these were found. Overall, the study provides strong support for the Conceptual-Production Systems model.     

Dissociations of writing and praxis: Two cases in point

For normal writing it is essential that both motoric and linguistic competence be present; disruption of one or the other of these faculties may result in qualitatively different types of agraphia. Two right-handed patients became agraphic after left hemisphere lesions; pure apraxic agraphia in the absence of limb apraxia developed in one patient and pure linguistic agraphia in association with severe ideomotor limb apraxia in the other. The performance of these patients not only serves to illustrate the dissociation between the motoric and linguistic faculties that underlie writing but also confirms that ideomotor limb apraxia and apraxic agraphia are distinct and dissociable entities.     

An investigation of body part as object (BPO) responses in normal and brain-damaged adults

A test of simple pantomime was administered to three groups of adults and comparisons were made across groups of the incidence of subjects who exhibited body part as object (BPO) responses and of the mean frequency of occurrence of BPO in each group. The three groups were left-hemisphere-damaged aphasics (N = 28), right-hemisphere-damaged (N = 24), and normal controls (N = 28). The results indicated no significant differences among groups on the BPO measures. Also, to test the strength of association between the frequency of occurrence of BPO and measures of limb apraxia and severity of aphasia for the left-hemisphere-damaged aphasic group, correlation coefficients were obtained. The correlations were low and nonsignificant. The results of this investigation do not support the common clinical assumption that the occurrence of BPO during the performance of simple pantomimes is pathognomic for left-hemisphere pathology or associated with limb apraxia.     

Cohésion–non-cohésion des scores composites : valeurs seuils et interprétabilité. L’exemple du WISC-IV

When interpreting the composite scores of the Wechsler Intelligence scales, some authors recommended to determine whether the composite scores are unitary or not. It has been suggested that when variability among scores is too large, then the composite score does not provide a good estimate of the psychological attribute, and is not interpretable. The first objective of the paper is to demonstrate that the cut-off threshold values (23 points and 5 points) proposed by some authors are inadequate. Other cut-off threshold values should be used. Most importantly, the second goal of this paper is to demonstrate that the notion of unitary abilities that was guiding principle of interpretation is also inadequate. Indeed, nonunitary index still provides relevant information.     

A model‐based approach to limb apraxia in Alzheimer's disease

Limb apraxia is a neurological deficit characterized by an inability to pantomime and/or imitate gestures, which can result from neurodegenerative disorders such as Alzheimer's disease (AD). The major goal of the study was to describe comprehensively the apraxia deficits observed in AD patients and to relate those deficits to general cognitive status, measures of daily activity, and other neuropsychological measures. Limb apraxia was assessed on a variety of conceptual and gesture production tasks in 30 AD patients. As a group, AD patients were impaired across gesture production tasks: of note was the greater impairment in imitation, as opposed to pantomime, which was especially pronounced when patients were imitating with a delay. Imitation performance was best predicted by measures of visuospatial processing, while imitation with delay was best predicted by measures of working memory. In addition, pantomime in response to pictures of tools was less accurate than Pantomime to Verbal Command and holding the tool during performance did not decrease the participants' impairment, while introducing a verbal cue during imitation increased the severity of deficits. Furthermore, investigation into patterns of deficits clearly demonstrated that the nature of limb apraxia deficits observed in AD can be quite heterogeneous and that dissociations between the conceptual and the production system exist. Finally, we also report on significant correlations between general cognitive status and limb apraxia.     

Apraxia is not associated to a disproportionate naming impairment for manipulable objects

The relevance of gesture knowledge for the semantic representation of manipulable objects was investigated in a series of 15 patients with a focal left-hemisphere lesion. The patients were classified according to the presence/absence of ideomotor and ideational apraxia. We investigated picture naming and word-picture matching (pointing to a picture on verbal command) with stimuli including items from three categories: manipulable objects, non-manipulable objects, and animals. The analysis was performed at group level and at single-patient level. Nine patients were affected by ideational apraxia and nine by ideomotor apraxia: two cases presented ideomotor but not ideational apraxia, and two cases presented the opposite dissociation. Patients affected by ideational apraxia were more severely impaired in all tasks, but did not show a disproportionate impairment with the category of manipulable objects in contrast to the other categories. The presence of ideomotor apraxia did not cause a greater impairment in any task or category. Finally, we observed a patient with ideational apraxia who performed flawlessly in naming and word-picture matching for all the stimuli, including manipulable objects. In conclusion, we did not find a relationship between ideational apraxia and a disproportionate impairment of the semantic knowledge of manipulable objects.     

Response interference between functional and structural object‐related actions is increased in patients with ideomotor apraxia

We report data from two left hemisphere stroke patients with moderate‐to‐severe ideomotor apraxia who exhibited deficits in positioning their hands to use ‘conflict’ objects (objects grasped and used with different hand postures) relative to controls and patients with mild apraxia. These novel data support the claim that actions to common objects are subject to interference between multiple responses, and suggest that errors in apraxia may be attributed to deficient resolution of competition between appropriate and inappropriate actions.     

Interaction between verbal and gestural language in progressive aphasia: a longitudinal case study.

The objective of this longitudinal study is to investigate the on-line interaction between praxis and linguistic abilities in a progressive aphasia case. During 3 years of evolution, procedural discourse of a progressive aphasic patient was videotaped five times, allowing us to analyze the progression of both language and gestural production as well as the interaction between these two. We anticipated that, in the absence of apraxia, the patient would compensate for her speech deficit by producing progressively more and more meaningful gestures. Our compensatory hypothesis was confirmed but the compensation was not as efficient as one would expect given the absence of apraxia. With the progression of the speech deficit, the patient could not replace some verbs by pantomimes that were otherwise accompanying her discourse in the preceding testing sessions. We suggest that such a compensatory ability may constitute one important characteristic of the progressive aphasia syndrome.     

Contrasting speech patterns in apraxia of speech and phonemic paraphasia

This study was designed to determine if perceptual phonological analysis would reveal distinctions between patients with apraxia of speech and patients with phonemic paraphasic speech. Test findings from 10 Broca's aphasics with apraxia of speech were compared to findings from 10 paraphasic speakers (5 conduction and 5 Wernicke's aphasics). Several marked differences were revealed. Predominant locus of errors and relative difficulty of different classes of phonemic segments were significant discriminators. There was a nonsignificant trend for substituted phonemes to be further from target phonetically in the paraphasic patients. In addition, the two groups showed certain consistent differences in the types of errors they produced. Apraxic patients produced many errors of transitionalization, while sequencing errors were more typical of the patients with phonemic paraphasia. The findings are interpreted in relation to a neuropsychological model of speech. It is suggested that phonemic paraphasia represents a breakdown mainly in the retrieval of phonological word patterns, while apraxia of speech is characterized predominantly by a disturbance in encoding phonological patterns into appropriate speech movements.