Letter imagery deficits in a case of pure apraxic agraphia

Pure apraxic agraphia is a condition in which motor writing is impaired but limb praxis and nonmotor writing (typing, anagram letters) are preserved. This condition is believed to result from disruption of a parietal lobe graphemic area which generates grapheme representations used to program motor-writing patterns. We report a single case of pure apraxic agraphia in which defective letter imagery was evident. Results of writing and imagery evaluations suggest that the graphemic area may be responsible for generating letter images. In this respect, clinical evaluation of letter imagery abilities in cases of apraxic agraphia may be useful in identifying subtypes of the disorder resulting in a better understanding of the neuropsychological process involved in writing.     

Multiple component agraphia in a patient with atypical cerebral dominance: an error analysis

A 52-year-old man with atypical cerebral dominance (left-handed for writing but mixed handedness for other tasks) suffered an extensive right hemisphere stroke, resulting in a combination of deficits that has not been previously reported. There were profound visual constructive and visual perceptual disturbances and a spatial agraphia, which were consistent with a nondominant hemisphere lesion. There was also a severe apraxic agraphia, which is typically associated with a dominant hemisphere lesion, but no other signs of dominant hemisphere dysfunction such as linguistic disturbance or limb-motor apraxia were present. This case serves to highlight the functional and anatomical relationship between handwriting and other forms of praxis; the various sources of error in letter formation; the need to be specific in labeling and describing agraphias ; and the role of a detailed analysis of writing errors in delineating the neuropsychological processes involved in handwriting.     

Apraxia in corticobasal degeneration.

Corticobasal degeneration (CBD) is a degenerative disease that often presents with an asymmetric progressive ideomotor limb apraxia. Some apraxic subjects may fail to perform skilled purposive movements on command because they have lost the memories or representations that specify how these movements should be performed (representational deficit). In contrast, other apraxic subjects may have the movement representations but are unable to utilize the information contained in them to execute skilled purposive movements (production-execution deficit). To learn if the apraxic deficit in CBD is induced by a representational or a production-execution deficit, we tested three nondemented subjects with CBD on tasks requiring production of meaningful or meaningless gestures to command, gesture imitation, gesture discrimination, and novel gesture learning. A fourth subject with incomplete data also is presented. The results suggest that the apraxia associated with CBD is initially induced by a production-execution defect with relative sparing of the movement representations.     

Progressive agraphia can be a harbinger of degenerative dementia

By investigating three patients with progressive agraphia, we explored the possibility that this entity is an early sign of degenerative dementia. Initially, these patients complained primarily of difficulties writing Kanji (Japanese morphograms) while other language and cognitive impairments were relatively milder. Impairments in writing Kana (Japanese syllabograms), verbal language, executive function, visuo- and visuospatial cognition and memory were identified by neuropsychological testing. The agraphia was compatible with a peripheral type, based on deficits at the interface between the central letter selection and the graphemic motor execution (Patient 1) or at the stage of central letter selection as well (Patients 2 and 3). Agraphia was generally more prominent, although not exclusive, for Kanji probably because of later acquisition and larger total number of Kanji letters leading to lower frequency of use and familiarity per letter. Concurrent or subsequent emergence of non-fluent aphasia, ideomotor apraxia, executive dysfunction and asymmetric akinetic-rigid syndrome in two patients suggested degenerative processes involving the parietal-occipital-temporal regions, basal ganglia and striato-frontal projections. We propose that progressive agraphia may be one of the early symptoms of degenerative dementia such as corticobasal degeneration.     

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.     

Impaired finger dexterity in Parkinson's disease is associated with praxis function

A controversial concept suggests that impaired finger dexterity in Parkinson’s disease may be related to limb kinetic apraxia that is not explained by elemental motor deficits such as bradykinesia. To explore the nature of dexterous difficulties, the aim of the present study was to assess the relationship of finger dexterity with ideomotor praxis function and parkinsonian symptoms. Twenty-five patients with Parkinson’s disease participated in the study. Their left and right arms were tested independently. Testing was done in an OFF and ON state as defined by a modified version of the Movement Disorder Society-Unified Parkinson’s Disease Rating Scale (MDS-UPDRS). Finger dexterity was assessed by a coin rotation (CR) task and ideomotor praxis using a novel test of upper limb apraxia (TULIA), in which the patients were requested to imitate and pantomime 48 meaningless, as well as communicative and tool-related gestures. Coin rotation significantly correlated with TULIA irrespective of the motor state and arm involved, but not with the MDS-UPDRS. This association was significantly influenced by Hoehn and Yahr stage.The strong association of finger dexterity with praxis function but not the parkinsonian symptoms indicates that impaired finger dexterity in Parkinson’s disease may be indeed apraxic in nature, yet, predominantly in advanced stages of the disease when cortical pathology is expected to develop. The findings are discussed within a cognitive-motor model of praxis function.     

Limb apraxia and motor performance

This study was desinged to determine if motor deficits in limb apraxia are task specific. Non-brain-damaged patients and apraxic and nonapraxic patients with left hemisphere damage performed language and limb apraxia tests and six motor tasks with the left hand. Contrary to previous data, no significant group differences occurred on a finger tapping task. Although task complexity or sequencing requirements affected group differences, greatest apraxic impairment was noted on a task of precise steadiness, but only when its response inhibition requirements were increased. This pattern of deficits appears to be associated with greater involvement of the premotor area in the apraxic group.     

Cerebellar-induced apraxic agraphia: a review and three new cases

Apraxic agraphia is a writing disorder due to a loss or lack of access to motor engrams that program the movements necessary to produce letters. Clinical and functional neuroimaging studies have demonstrated that the neural network responsible for writing includes the superior parietal region and the dorsolateral and medial premotor cortex. Recent studies of two cases with atypical lesion localisations in the left thalamus and the right cerebellum support the hypothesis that the written language network is larger than previously assumed. The aim of this study is twofold: (1) to provide a survey of cases of apraxic agraphia published between 1973 and June 2010, and (2) to provide further evidence for a role of the cerebellum in writing via three additional cases who presented with apraxic agraphia after ischemic damage in the cerebellum. Functional neuroimaging studies by means of brain perfusion SPECT showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, syntax, naming, writing and executive functioning.     

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.     

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.     

Tool-use and the left hemisphere: What is lost in ideomotor apraxia?

Impaired tool related action in ideomotor apraxia is normally ascribed to loss of sensorimotor memories for habitual actions (engrams), but this account has not been tested against a hypothesis of a general deficit in representation of hand-object spatial relationships. Rapid reaching for familiar tools was compared with reaching for abstract objects in apraxic patients (N = 9) and in a control group with right hemisphere posterior stroke. The apraxic patients alone showed an impairment in rotating the wrist to correctly grasp an inverted tool but not when inverting the hand to avoid a barrier and grasp an abstract object, and the severity of the impairment in tool reaching correlated with pantomime of tool-use. A second experiment with two apraxic patients tested whether barrier avoidance was simply less spatially demanding than reaching for a tool. However, the patient with damage limited to the inferior parietal lobe still showed a selective problem for tools. These results demonstrate that some apraxic patients are selectively impaired in their interaction with familiar tools, and this cannot be explained by the demands of the task on postural or spatial representation. However, traditional engram theory cannot account for associated problems with imitation of novel actions nor the absence of any correlated deficit in recognition of the methods of grasp of common tools. A revised theory is presented which follows the dorsal and ventral streams model ( Milner & Goodale, 2008) and proposes preservation of motor control by the dorsal stream but impaired modulating input to it from the conceptual systems of the left temporal lobe.     

Crossed apraxia of speech: a case report

The present study reports on the first case of crossed apraxia of speech (CAS) in a 69-year-old right-handed female (SE). The possibility of occurrence of apraxia of speech (AOS) following right hemisphere lesion is discussed in the context of known occurrences of ideomotor apraxias and acquired neurogenic stuttering in several cases with right hemisphere lesion. A current hypothesis on AOS-the dual route speech encoding (DRSE) hypothesis-and predictions based on DRSE were utilized to explore the nature of CAS in SE. One prediction based on the DRSE hypothesis is that there should be no difference in the frequency of occurrence of apraxic errors on words and non-words. This prediction was tested using a repetition task. The experimental stimuli included a list of minimal pairs that signaled voice-voiceless contrasts in words and non-words. Minimal-pair stimuli were presented orally, one at a time. SE's responses were recorded using audio and videotapes. Results indicate that SE's responses were characterized by numerous voicing errors. Most importantly, production of real word minimal pairs was superior to that of non-word minimal pairs. Implications of these results for the DRSE hypothesis are discussed with regard to currently developing perspectives on AOS.     

Apraxia differs in corticobasal degeneration and left-parietal stroke: A case study.

Corticobasal degeneration (CBD) is a progressive disorder characterized by both cortical and basal ganglia dysfunction such as asymmetrical apraxia, and akinetic rigidity, involuntary movements, and cortical sensory loss. Although apraxia is a key finding for the differential diagnosis of CBD, it has not been determined whether the features of apraxia seen in subjects with CBD are similar to those features exhibited by subjects with left-hemisphere damage from stroke. Therefore, for both clinical purposes and in order to better understand the brain mechanisms that lead to apraxia in CBD, we studied praxis in a patient with CBD and compared him to patients who are apraxic from left-parietal strokes. We used three-dimensional movement analyses to compare the features of apraxic movement. This subject with CBD was a dentist whose initial complaint had been that he "forgot" how to use his tools in the mouths of his patients. Analyses were performed on the trajectories made when using a knife to actually slice bread, and when repetitively gesturing slicing made to verbal command. Movements of the left hand, wrist, elbow, and shoulder were digitized in 3-D space. Although the CBD subject was clearly apraxic, the features of his apraxia differed markedly from those of the subjects with lesions in the left parietal lobe. For movements to command, the CBD subject showed joint coordination deficits, but his wrist trajectories were produced in the appropriate spatial plane, were correctly restricted to a single plane, and, like control subjects, were linear in path shape. However, when he was actually manipulating the tool and object, all of these aspects of his trajectories became impaired. In contrast, the deficits of the apraxic subjects with left-parietal damage were most pronounced to verbal command with their movements improving slightly although remaining impaired during actual tool and object manipulation. Unlike patients with parietal strokes, patients with CBD have degeneration in several systems and perhaps deficits in these other areas may account for the differences in praxic behavior.     

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.     

Voice onset time characteristics in apraxia of speech

In order to examine the timing control of laryngeal and supralaryngeal articulatory adjustments in apraxia of speech, voice onset time (VOT) data for stop consonants in monosyllables /de/, /te/, /ge/ and /ke/ were obtained from four apraxic subjects and compared with those of fluent aphasic and normal (both young and aged) speakers (Experiment 1). The results indicated that the VOT distribution patterns of the apraxic patients differed markedly from those of the other speakers. A second experiment was conducted to determine whether the VOT distribution patterns of these four apraxic patients and of two normal controls would change with a lapse of time (Experiment 2). The results of the second experiment demonstrated marked changes in the VOT distribution patterns over time in half of the apraxic patients examined, but essentially no change in the normal controls.     

失用性失写的脑机制

失用性失写是指人们知道该如何书写, 但实际上却不会书写或错误书写的一种现象。失用性失写涉及的神经网络包括皮层与皮层连接、皮层与皮质下连接。其中前者包括额-顶叶相关脑区的连接、顶-枕叶相关脑区的连接以及额-顶-枕相关脑区的连接, 这些神经网络分别负责操作空间表征图像、回忆以及检索字母的形状等; 后者包括小脑与额-顶叶的连接、左丘脑-大脑皮质的连接以及纹状体-内囊-左额顶叶的连接等, 它们分别与图像输出程序、书写运动过程、操作指令的执行过程以及字素形成过程等有关。今后的研究应该集中在完善失用性失写的研究范式、深入探讨失用性失写的心理机制以及推动其康复工作的开展等方面。     

Task demands and limb apraxia in stroke

The present study was designed to examine the frequency and severity of apraxia in patients with left- or right-hemisphere stroke in both pantomime and imitation conditions and to compare the frequency of apraxia in each stroke group across the three patterns of apraxia described in Roy's model (Roy, 1996). Ninety-nine stroke patients and 15 age-matched healthy adults performed eight transitive gestures to pantomime and to imitation. Gestural performance was quantified as accuracy on five performance dimensions; a composite score, an arithmetic combination of the five performance dimensions, was used as an index of the overall accuracy. Analyses revealed a comparable proportion of patients in each stroke group were classified as apraxic in the imitation condition, but a higher proportion of left stroke patients were apraxic in the pantomime condition. The severity of apraxia in each stroke group and the performance dimensions affected were, however, comparable. Analyses of the patterns of apraxia (pantomime alone, imitation alone or apraxia in both conditions) revealed a higher frequency of apraxia in both stroke groups for the pattern reflecting apraxia in both conditions, indicating that a disruption at the movement execution stage of gesture performance was most common.     

Syllable frequency and syllable structure in apraxia of speech

Recent accounts of the pathomechanism underlying apraxia of speech (AOS) were based on the speech production model of Levelt, Roelofs, and Meyer, and Meyer (1999)1999. The apraxic impairment was localized to the phonetic encoding level where the model postulates a mental store of motor programs for high-frequency syllables. Varley and Whiteside (2001a) assumed that in patients with AOS syllabic motor programs are no longer accessible and that these patients are required to use a subsyllabic encoding route. In this study, we tested this hypothesis by exploring the influence of syllable frequency and syllable structure on word repetition in 10 patients with AOS. A significant effect of syllable frequency on error rates was found. Moreover, apraxic errors on consonant clusters were influenced by their position relative to syllable boundaries. These results demonstrate that apraxic patients have access to the syllabary, but that they fail to retrieve the syllabic motor patterns correctly. Our findings are incompatible with a subsyllabic route model of apraxia of speech.     

Pure agraphia and Gerstmann's syndrome as a visuospatial-language dissociation: an experimental case study

A right-handed man suffered a left parieto-occipital cerebral infarction, causing agraphia with Gerstmann's syndrome but without major aphasia, alexia, or apraxia. Oral spelling was superior to written spelling. Experiments were performed involving (1) analysis of errors in writing, (2) tasks of visual imagery, and (3) identifying letters drawn without leaving a visual trace. The results suggest that the agraphia and Gerstmann's syndrome are due to a dissociation of language skills and visuospatial skills caused by a dominant parieto-occipital lesion.     

Varieties of alexia from fusiform, posterior inferior temporal and posterior occipital gyrus lesions

Reading impairments of three alexia patients, two pure alexia and one alexia with agraphia, due to different lesions were examined quantitatively, using Kanji (Japanese morphogram) words, Kana (Japanese phonetic writing) words and Kana nonwords. Kana nonword reading was impaired in all three patients, suggesting that widespread areas in the affected occipital and occipitotemporal cortices were recruited in reading Kana characters (corresponding to European syllables). In addition, the findings in patient 1 (pure alexia for Kanji and Kana from a fusiform and lateral occipital gyri lesion) and patient 2 (pure alexia for Kana from a posterior occipital gyri lesion) suggested that pure alexia could be divided into two types, i.e. ventromedial type in which whole-word reading, together with letter identification, is primarily impaired because of a disconnection of word-form images from early visual analysis, and posterior type in which letter identification is cardinally impaired. Another type of alexia, alexia with agraphia for Kanji from a posterior inferior temporal cortex lesion (patient 3), results from deficient whole-word images of words per se, and thus should be designated "orthographic alexia with agraphia". To account for these impairments, a weighted dual-route hypothesis for reading is suggested.