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If brain lesions impair the allocation of attention to a representation of the body surface and the hand may serve as an attentional focus or "wand", one might expect that somatosensory deficits caused by cerebral lesions would be ameliorated by contact with the ipsilesional hand. To test this prediction, tactile detection tasks were administered to two subjects with right hemisphere lesions. Subject CB's left tactile extinction was investigated in conditions in which the degree of contact between the right and left hands and the spatial relationship between his hands was systematically varied. His left tactile extinction was significantly reduced by touch of the right hand. Similarly, extinction at the left knee was ameliorated by touch of the knee by the right hand; touch of the right foot had no effect. Subject NC's ability to detect a tactile stimulus delivered to the left side was systematically assessed in conditions in which the hands touched and the spatial relationship between the hands was varied. His ability to detect a touch on the left hand improved in conditions in which the left hand was touched by the right hand. This effect was not observed if direct contact between the two hands was prevented by inserting a thin cloth between the hands. For both subjects, placing the right hand in close proximity to the left hand or altering the spatial location of the hands relative to the body did not influence performance. These data demonstrate that the hand may serve as a conduit for attention and provide strong evidence for a distinct representation of the body surface that is at least in part independent of spatial representations.

The visual agnosias are an intriguing class of clinical phenomena that have important implications for current theories of high-level vision. Visual agnosia is defined as impaired object recognition that cannot be attributed to visual loss, language impairment, or a general mental decline. At least in some instances, agnostic patients generate an adequate internal representation of the stimulus but fail to recognize it. In this review, we begin by describing the classic works related to the visual agnosias, followed by a description of the major clinical variants and their occurrence in degenerative disorders. In keeping with the theme of this issue, we then discuss recent contributions to this domain. Finally, we present evidence from functional imaging studies to support the clinical distinction between the various types of visual agnosias.

Normal subjects were asked to produce the "first word that comes to mind" in response to pictures or words that differed with respect to manipulability and animacy. In separate analyses across subjects and items, normal subjects produced a significantly higher proportion of action words (that is, verbs) to pictures as compared to words, to manipulable as compared to non-manipulable stimuli and to inanimate as compared to animate stimuli. The largest proportion of action words was elicited by pictures of non-living, manipulable objects. Furthermore, associates to words matched standard word associates significantly more often than those elicited by pictures. These data suggest that pictures and words initially contact different forms of conceptual information and are consistent with an account of semantic organization that assumes that information is distributed across different domains reflecting the mode of acquisition of that knowledge.

Background: Patients with selective deficits in the naming and comprehension of animals, plants, and artifacts have been reported. These descriptions of specific semantic category deficits have contributed substantially to the understanding of the architecture of semantic representations., Objective: This study sought to further understanding of the organization of the semantic system by demonstrating that another semantic category, knowledge of the human body, may be selectively preserved., Methods: The performance of a patient with semantic dementia was compared with the performance of healthy controls on a variety of tasks assessing distinct types of body representations, including the body schema, body image, and body structural description., Results: Despite substantial deficits on tasks involving language and knowledge of the world generally, the patient performed normally on all tests of body knowledge except body part naming; even in this naming task, however, her performance with body parts was significantly better than on artifacts., Conclusions: The demonstration that body knowledge may be preserved despite substantial semantic deficits involving other types of semantic information argues that body knowledge is a distinct and dissociable semantic category. These data are interpreted as support for a model of semantics that proposes that knowledge is distributed across different cortical regions reflecting the manner in which the information was acquired.

It has been proposed that the underlying deficit for some simultanagnosics is the inability to bilaterally orient attention in space due to parietal damage. In five experiments, we examine the performance of a patient with simultanagnosia secondary to bilateral occipito-parietal lesions, IC, in naming pairs of line-drawings. With simultaneous presentation and disappearance of objects (Experiment 1), IC typically named a single object. IC's performance dramatically improved when the two drawings alternated every 500 ms (Experiment 2). This improvement was not due to the abrupt onset of the second drawing "capturing attention", as indicated by the results of Experiment 3. Experiments 4 and 5 demonstrated that the crucial factor in improving IC's performance with simultaneous presentation of visual objects was the offset of one of the two stimuli. We propose that IC's impairment in naming two objects is attributable to the inability to "unlock" attention from the first object detected to other objects in the array. Visual offset of the first object disengages attention from the first object, allowing it to be allocated to the second object.

Some accounts of body representations postulate a real-time representation of the body in space generated by proprioceptive, somatosensory, vestibular and other sensory inputs; this representation has often been termed the 'body schema'. To examine whether the body schema is influenced by peripheral factors such as pain, we asked patients with chronic unilateral arm pain to determine the laterality of pictured hands presented at different orientations. Previous chronometric findings suggest that performance on this task depends on the body schema, in that it appears to involve mentally rotating one's hand from its current position until it is aligned with the stimulus hand. We found that, as in previous investigations, participants' response times (RTs) reflected the degree of simulated movement as well as biomechanical constraints of the arm. Importantly, a significant interaction between the magnitude of mental rotation and limb was observed: RTs were longer for the painful arm than for the unaffected arm for large-amplitude imagined movements; controls exhibited symmetrical RTs. These findings suggest that the body schema is influenced by pain and that this task may provide an objective measure of pain.

We report a patient with autotopagnosia (JD) who was unable to code the position of body parts relative to each other and who failed to update the position of body parts after passive movements. JD's performance in Studies 1-4 suggest that her ability to code the dynamic location of body parts with respect to each other ("intrinsic egocentric" spatial coding) was impaired, and that she employed a compensatory strategy by means of which the location of body parts was computed with respect to objects in the environment ("extrinsic egocentric" spatial coding). Studies 5-8 suggest that JD's ability to update hand position information was impaired after passive relative to active movements of her arm. For example, she was impaired in reaching to a target after passive but not active movements of her hand. Taken together, these findings extend previously reported functions of extrinsic egocentric coding to the localisation of body parts and demonstrate a possible dissociation between body part localisation dependent upon proprioceptive and efference copy information.

Previous accounts of autotopagnosia (e.g., Ogden, 1985; Pick, 1908; Semenza, 1988) propose that the disorder is attributable to deficits in "mental images," visual body schema, or semantic representations. A recent account (Sirigu, Grafman, Bressler, & Sunderland, 1991b) posits deficits in visual structural descriptions of the human body and its parts, in the context of spared semantic and proprioceptivespatio-motor body representations, but provides no evidence bearing on the nature or format of the putatively damaged representation. We report data from a man with autotopagnosia consequent to lefthemisphere brain damage which bear directly on the nature of the representation impaired in the disorder. The subject, GL, is unable to localise body parts on himself or others, whether cued by verbal or visual input. In contrast, he uses body parts precisely in reaching and grasping tasks, correctly matches items of clothing to body parts, and localises the parts of animals and man-made objects without error. We also demonstrate that GL is unable to match pictured or real human body parts across shifts in orientation or changes in visual appearance, but can perform analogous matching tasks with animal body parts and man-made object parts. The data extend the account of Sirigu et al. (1991b) in suggesting that human body part localisation depends upon structural descriptions of human (but not animal) bodies that enable viewpoint-independent body part recognition and participate in the calculation of equivalence between the body parts of self and others across transformations in orientation.

Four experiments examined new associative learning in amnesia by contrasting the performance of 2 amnesic participants-1 (C.C.) with basal forebrain damage and the other (R.H.) with medial temporal lobe damage--and 3 controls. Both amnesic individuals were severely impaired on explicit memory measures but showed intact perceptual priming. On the new associations measures, only C.C., not R.H., exhibited learning by producing correct targets (HIJACKER) in the absence of perceptual cues for them (e.g., STAFF shot ???). When the perceptual cue (e.g., MEDICINE cured _I_C_P) was provided, both C.C. and R.H. showed learning. Transfer to information containing conceptually related targets (e.g., TERRORIST or BELCH) was reliably observed only in C.C. This finding was replicated with further reduction in perceptual overlap across original (LIGHTNING torched JUNGLE) and transfer (LIGHTNING burned WILDERNESS) sentences. Together, these findings delineate the role of experimental conditions, severity of amnesia, and different neuroanatomical structures in mediating new verbal learning in amnesia.

Disorders of reading are frequently encountered in patients with acquired cerebral lesions. Investigations in the past few decades have improved our understanding of these disorders. In this article we review the peripheral dyslexias, including neglect dyslexia, attentional dyslexia, and pure alexia (or alexia without agraphia), as well as the "central" dyslexias, including deep, surface, and phonological dyslexia. Current accounts of acquired dyslexia are also discussed. Finally, we briefly describe the reading tasks that serve to differentiate the different reading disorders.

Traumatic brain injury (TBI) is associated with impairments of attention, most typically measured through tests of information processing, or by subjective symptom endorsement by patients, families, and clinicians. We have previously shown increased rates of off-task behavior among patients with TBI versus controls as defined by videotaped records of independent work in distracting environments. In this research, we report on a more detailed method of coding such videotaped records which allows measurement of the precise number of off-task behaviors, their durations, and their relationship to distracting events. Using this method, we studied 20 patients with recent moderate-to-severe TBI and 20 demographically comparable controls as they performed independent work tasks while being subjected to controlled distracting events. This research confirms that patients are markedly less attentive than controls both in the presence of distractions and in their absence, that distractions have an influence on off-task behavior in both groups, and that the disruptive impact of distractors wanes relatively quickly for controls but not for patients. The duration of distraction produced by various classes of distracting events appeared similar for patients and controls, although the power to detect differences in behavioral duration between groups was limited. The pattern of inattentiveness among patients showed minimal relationship to measures of injury severity within this sample.

In subjects with parietal lobe lesions, performance on motor and language tasks differed as a function of the side of space to which subjects directed their attention or acted. Subjects with left parietal lesions performed better when attention was directed to stimuli in left hemispace (that is, the left side of their environment), and those with right parietal lesions showed a similar effect when attending to stimuli in right hemispace. Hemispace effects were not observed in subjects with lesions located elsewhere in the cerebral hemispheres, or in subjects with subcortical lesions. These data are consistent with the view that not only motor but also cognitive operations such as language, which do not appear to have any intrinsic spatial organization, are maintained in registration with spatial systems, and that this attention-requiring linkage confers a processing advantage.

A leading account of high-level visual recognition proposes that the recognition of faces, objects, and words is mediated by two processing capacities. Words are assumed to require the capacity to represent numerous parts, whereas faces are processed wholistically. and hence require the representation of complex units. Object recognition requires the capacity to represent both numerous and complex parts. As object recognition depends upon the same processing capacities underlying face and word recognition, this account predicts that patients with severe alexia and prosopagnosia should be deficient in tests of object recognition. We report a patient who is unable to recognize words or faces, yet performs relatively well on tests of object recognition. The two-capacity theory cannot accommodate this pattern of performance without additional assumptions.

An unselected group of right hemisphere, semi-acute stroke patients (n = 30) was run on a laboratory test of naturalistic action production and was found to commit errors of action at a higher rate than what was previously reported for recovering head injury patients [Schwartz et al., Naturalistic action impairment in closed head injury. Neuropsychology, 1997, 8, 59-72]. There were strong similarities in how these two patient groups responded to variations in task demands and in the pattern of errors they produced. Hemispatial biases were evident in the errors of right hemisphere patients with neglect but not those without neglect; and neglect patients also many errors that were unrelated to the spatial layout. We argue that a non-specific resource limitation--which might translate as reduced arousal or effort--is central to the breakdown of naturalistic action production after brain damage, and right hemisphere patients are especially vulnerable to this resource limitation and its behavioral consequences.

Subjects with left neglect often fail to use and, in some instances, recognize the left side of the body. We performed a series of investigations to determine if this deficit is, at least in part, attributable to an impairment in the "body schema," an internal three-dimensional, dynamic representation of the spatial and biomechanical properties of one's body. First, subjects were shown a series of pictures of a single hand and asked to determine if the stimulus was a right or left hand. Subjects with neglect but not other subjects with brain lesions identified pictures of left (contra-lesional) hands significantly less reliably than pictures of right hands. On the basis of evidence demonstrating that the identification of pictured hands involves the matching of the stimuli to an on-line mental representation of one's body, these data suggest that neglect may be associated with a disruption of, or failure to attend to, the body schema. Data from subsequent investigations contrasting patients with left neglect and Gerstmann's syndrome argue for a distinction between a body schema and a "body image," or conceptual representation of the body which articulates with language., (Copyright 1998 Academic Press.)

Optic ataxia, a disorder of visually guided reaching, has been attributed to a failure in the transmission of visuospatial information to motor systems. This account fails to explain why many patients w ith optic ataxia misreach only when targets are presented in non-foveal vision, but not w hen targets are view ed directly. We present data from two patients with unusually severe optic ataxia that suggest an alternative account of this relatively com mon pattern of misreaching. DP is utterly unable to reach to targets unless he gazes directly at them. We provide evidence that he is unable to program reaches in appropriate shoulder and hand-centred spatio-motor coordinates, but instead reaches using oculocentric coding. The gaze (and visual attention) of AM is frequently "captured" by his own hand, and his attentional allocation is influenced by the location at which he performs a hand movement. This suggests that he programs eye movements within hand-centred spatial coordinate frames. Taken together, these data suggest that residual reaching in optic ataxia may be enabled by linkage of the systems coding the spatio-m otor actions of the eye and hand.

Recent studies of pure alexia present a contradictory picture. Despite evidence of impaired letter identification in letter-by-letter readers, some patients are able to carry out lexical decision and other tasks under conditions of rapid presentation, although they are seldom able to identify these stim uli explicitly. We review evidence for both facets of pure alexic performance and offer an accountof this pattern in terms ofright-and left-hemisphere reading mechanisms. Specifically, we suggest that the right hemisphere supports performance in covert reading tasks, and that letter-by-letter reading is the product of the left hemisphere, operating on information transmitted from the right.

Clinical assessments of individuals with traumatic brain injury (TBI) typically report attentional difficulties, with distractibility prominent among these complaints. However, laboratory-based measures have often failed to find disproportionate distraction among patients with TBI, as compared to control participants. In this experiment, we tested 21 patients hospitalized for rehabilitation following recent TBI and 21 demographically comparable control subjects on a visual reaction time go-no-go task in which the target was preceded or followed by a brightly colored moving visual stimulus, appearing above the target location. Early distractors actually served as warning stimuli, improving accuracy and speed for both participant groups. Distractors occurring at or shortly after the time of target presentation had no significant impact on accuracy or response bias in either group, but did produce slowing of RT that was significantly greater for patients than for controls. The distractor that produced maximal slowing occurred 100 ms after the presentation of the target or foil. Repeated testing sessions led to reduction in the impact of the distractor and loss of the group difference in RT impact. The degree of RT slowing induced by distraction was modestly related to injury severity, as measured by the current score on the Disability Rating Scale, and the time until the patient first followed verbal commands. There was also a trend of greater RT slowing among individuals with focal orbitofrontal lesions, as assessed on neuroimaging studies. These results document a greater susceptibility to extraneous visual distraction among patients with TBI in comparison to controls. The fact that this difference appears only in the RT domain, and is greatest when the distractor follows the target, suggests that the primary impact of visual distractors is on response preparation and execution rather than target detection.

The authors sought to determine whether errors of action committed by patients with closed head injury (CHI) would conform to predictions derived from frontal lobe theories. In Study 1, 30 CHI patients and 18 normal controls performed routine activities, such as wrapping a present, under conditions of graded complexity. CHI patients committed more errors even on the simplest condition; but, except for a higher proportion of omitted actions, their error profile was very similar to that of controls. Study 2 involved a subset of patients whose performance in Study 1 was within normal limits. When these high functioning patients were asked to perform the routine tasks under still more taxing conditions, they, too, committed errors in excess of the control group. Accounts based on frontal mechanisms have a difficult time explaining the overall pattern of findings. An alternative based on limited-capacity resources is suggested.

We conducted three experiments to examine whether the anterior portion of the inferior temporal (IT) lobe is involved in the processing of visual objects in humans. In monkeys, damage to this region results in severe deficits in perception and in memory for visual objects. Our study was designed to examine both these processes in a patient (DM) with bilateral damage to the anterior portion of the inferior temporal lobe. Neuropsychological examination revealed a significant semantic impairment and a mild deficit in the discrimination of familiar objects from nonobjects. Despite these difficulties, the results of several studies indicated that DM was able to form and retain descriptions of the structure of objects. Specifically, DM showed normal perceptual priming for familiar and novel objects on implicit memory tests, even when the objects were transformed in size and left-right orientation. These results suggest that the anterior IT is not'involved in (1) the storage of pre-existing structural descriptions of known objects, (2) the ability to create new structural descriptions for novel objects, and (3) the ability to compute descriptions that are invariant with respect to changes in size and reflection. Instead, the anterior IT appears to provide the interface between structural descriptions of objects and their meanings.

We report two patients with right hemisphere lesions who demonstrate a double dissociation on visual imagery and visual perceptual tasks. One (M.N.) performed normally on a variety of standard tests for neglects as well as on measures of visual attention known to be sensitive to the presence of neglect, yet failed to report items from the left side of an imagined scene. In contrast, the other (C.I.) performed normally on tests of visual imagery but demonstrated substantial neglect on visual perceptual and visual attentional tasks. These data are not readily accommodated by accounts which attribute neglect to a single processing deficit, but suggest that the disorder is a heterogeneous syndrome attributable to disruptions of different aspects of spatial cognition.

Traumatic brain injury (TBI) is believed to have adverse effects on attention, but because of the multifaceted nature of the arousal and attentional network, the precise impact of TBI on various subcomponents of this network remains controversial. As part of a larger program of research on attention in TBI, we studied the effects of auditory warning signals on accuracy, reaction time, and response bias, as indices of phasic arousal in a visual go/no-go task. Warnings were presented randomly at various pre-stimulus intervals to a sample of recently injured patients and control subjects. For each subject and performance index, we examined the time interval required to reach maximal phasic arousal, the degree of performance change occurring at this maximal point, and the ability to maintain this state of increased arousal over longer warning intervals. After adjustment for baseline differences, there were no significant differences between patients and controls in any aspect of performance. Examination of effect sizes suggested that this was not due to limited statistical power, but that any differences between groups, if present, must have been small. In contrast, in our prior research on sustained attention and observable behaviors related to attention, large group differences have been found. These data suggest that mechanisms responsible for auditorily-induced phasic arousal responses are largely preserved in severe TBI.

Numerous accounts of pure alexia have suggested that prelexical impairment precludes rapid access to orthographic information in patients with the disorder. We report a patient with features of both pure and partially recovered deep dyslexia in whom we demonstrate prelexical deficits in maintaining a reliable abstract representation of the right side of letter arrays, as well as in modulating a "spotlight" of visual attention. These deficits, we suggest, encourage the patients's use of a letter-by-letter reading strategy; despite them, however, he demonstrates rapid, accurate reading of some, but not all classes of words. Furthermore, the patient's reading is influenced by both prelexical and lexical-semantic factors such that speed and accuracy are optimal for high imagiability nouns of few letters. Finally, the patient accurately names orally spelled words of all classes. Taken together, these data are consistent with the hypothesis that rapid reading may be enabled by lexical-semantic support from a right hemisphere-mediated processing system which recognizes words as whole, thereby mitigating the effect of the prelexical deficits.

Clinicians and families report that traumatic brain injury results in a variety of attention deficits. Numerous laboratory studies have documented slowing of information processing, alteration in event-related potentials, or difficulty attending to specific relevant task dimensions in the presence of redundant information. However, little is known about how these information processing abnormalities relate to observable behaviors in daily living or work environments, which presumably form the basis for clinicians' and families' reports. We developed a quantitative assessment of behavioral inattentiveness in both quiet and distracting environments, and demonstrated excellent interrater reliability. Using this assessment, we have studied 20 patients with recent traumatic brain injury and 20 demographically comparable control subjects. We have confirmed marked differences in behavioral attentiveness between patients and controls in both distracting and nondistracting environments.

Previous investigations have suggested that object-based neglect may reflect an impairment in attentional allocation that occurs relative to the intrinsic left and right of objects. We report a patient with apparent "object-based" neglect of 90 degrees rotated stimuli for whom the pattern of neglect was a function of task strategy. When the patient was instructed to visualize the rotated stimuli as if they were upright, i.e. mentally rotate them, he showed apparent "object-based" neglect to the left of the principal axes of the stimuli. In contrast, when instructed to refrain from mental rotation, neglect was apparent only with respect to his left, but not the left of the stimuli. Thus, the apparent "object-based" neglect of this patient may be attributed to a process of mental rotation of objects to upright, and subsequent neglect in viewer-centered or environment-centered coordinates. These data suggest a mechanism whereby object-based and viewer/environment-centered reference frames may be aligned, thereby causing viewer/environment-centered neglect to appear as if object-based.

Clinicians report that patients with traumatic brain injury (TBI) often have difficulty with tasks requiring sustained attention, and there are neuroanatomical and neurophysiological reasons to expect such deficits. Nevertheless, laboratory measures of sustained attention or vigilance in TBI have produced conflicting results. These inconsistencies may be due to patient heterogeneity as well as the fact that vigilance performance is dependent on highly specific features of the task design. We developed a visual vigilance task in which the influence of non-attentional factors was minimized and task difficulty for patients and controls made comparable. Performance was characterized with respect to vigilance level as well as vigilance decrement, using measures of perceptual discrimination, response bias, reaction time and reaction time variability. Twenty-six patients with recent TBI and 18 control subjects were tested on this task. A MANOVA of ranked scores revealed significantly different patient and control performance overall. Initial level of performance (vigilance level) was slower and more variable for patients than controls, and patients showed more conservative response biases. Deterioration over time (vigilance decrement) was steeper for patients than controls for reaction time, reaction time variability, and response bias. Deterioration in accuracy (D') did not differ significantly between patients and controls. Performance was not related to available measures of injury severity. Hypotheses relating arousal mechanisms to vigilance performance are discussed.

From a group of 22 patients with neglect, we identified five patients who neglected the left sides of "chimeric" figures composed of the conjoined right and left halves of drawings of two different objects; these subjects reported the left half-drawing significantly more accurately when a gap was introduced between the chimeric halves. The improvement in the "gap" condition cannot be attributed to the subjects' recognition that the right-sided stimuli were incomplete, as when whole figures were presented on the right along with left-sided half figures, the enhancement in reporting accuracy was maintained. For at least one patient, sufficient information was available about the neglected stimuli to support accurate recognition in a forced-choice task with foils visually similar to the targets. On the basis of these and other data, we propose that for these patients with neglect, visual processing systems subserving the registration of discrete object markers in a spatial map are at least relatively intact, but that there is an impairment in the allocation of attention to the left sides of these markers.

Although many patients with dominant hemisphere damage retain some reading ability, the anatomic substrate of the preserved reading remains controversial. We tested the hypothesis that the right hemisphere mediates the reading of some patients with acquired dyslexia by using Transcranial Magnetic Stimulation (TMS) to assess the effect of transient cortical stimulation on reading. A patient with (partially recovered) pure alexia was asked to read aloud briefly presented words, half of which were shown in association with TMS of the right or left hemisphere. Consistent with the right hemisphere reading hypothesis, stimulation of the right but not the left hemisphere disrupted oral reading.

The effect of the hemispace in which writing was performed was assessed in two left hemisphere stroke patients who demonstrated left-handed mirror writing. Both patients produced significantly more mirrored words when writing in right, as compared to left (body) hemispace. We suggest that writing in the right hemispace activates the left hemisphere. Further, we propose that mirror writing in the right hemispace is attributable to activation of the damaged left hemisphere spatial system, which fails to assist in the translation of right hand motor programs into those appropriate to the left hand. Writing in left hemispace, in contrast, activates the intact right hemisphere based spatial system which guides the execution or monitoring of motor productions in left hemispace. The result is writing which remains directionally correct when the left hand is used in left hemispace.

We report a patient who, after suffering infarcts involving the left temporo-parietal and left anterior cingulate regions, performed significantly worse on a variety of motor, language and sensory tasks when he acted in or attended to right hemispace. Performance on a number of tasks was better when acting in near as compared with far peri-personal space and with his head deviated to the left as compared with the right. Additionally, performance with the 'unaffected' left hand was often worse in right as compared with left hemispace. We propose that his deficits are attributable to a disruption of a left hemisphere-specific attentional system which normally serves to regulate the activation and articulation of left hemisphere processing modules.

A number of investigators have demonstrated that patients with pure alexia comprehend briefly presented words which they are unable to explicitly identify. We suggested previously that these patients may read by means of two distinct procedures: a laborious letter-by-letter method and a 'whole-word' procedure which, at least initially, does not support explicit word identification. We report a test of this proposal in a patient with pure alexia. We reasoned that if the patient had access to two distinct and incompatible procedures, he might be induced to switch from one to the other by changing task demands. We found that when instructed to name words, the patient employed a letter-by-letter strategy; in contrast, when instructed to make lexical decision or semantic judgements about rapidly presented words, he appeared to use a 'whole-word' strategy. These data support the hypothesis that two distinct procedures are available to this patient. We argue, further, that is necessary to suppress use of the letter-by-letter strategy to demonstrate whole word reading capability in pure alexics, and that failure to do so may account for negative findings in other cases reported in the literature.

Optic aphasia is a rare, visual modality-specific naming disorder. We describe a patient who developed this disorder subsequent to a left occipital lobe infarction. Like another case that we described previously, this patient performed normally on a variety of tasks assessing the recognition of objects he could not name. Additionally, although he never read aloud a single word, his performance on lexical decision and word comprehension tasks was far better than chance. We suggest that his performance was mediated by a right hemisphere semantic system.

Although aphasic patients with frontal lobe damage may demonstrate impaired retention of verbal material, significant anterograde memory disturbances have not, to our knowledge, been reported with a minor Broca's aphasia. We describe a patient with minor Broca's aphasia who exhibited an unusual and profound anterograde memory disturbance, especially for phonologically specified stimuli. We suggest that this disturbance is attributable to an impairment in the volitional, controlled search of stored phonological information.

Simultanagnosia is a disorder of visual perception characterized by the inability to interpret complex visual arrays despite preserved recognition of single objects. We report a series of investigations on a simultanagnosic patient which attempt to establish the nature of this visual processing disturbance. The patient performed normally on a feature detection task but was impaired on a test of attention-requiring visual search in which she was asked to distinguish between stimuli containing different numbers of targets. She was not impaired on a visual-spatial orienting task. She identified single briefly presented words and objects as rapidly and reliably as controls suggesting that access to stored structural descriptions was not impaired. With brief, simultaneous presentation of 2 words or drawings, she identified both stimuli significantly more frequently when the stimuli were semantically related than when they were unrelated. On the basis of these and other data, we suggest that the patient's simultanagnosia is attributable to an impairment in the process by which activated structural descriptions are linked to information coding the location of the object.

Most information processing accounts of reading incorporate either two or three mechanisms by which oral reading may be accomplished. The existence of a "third" mechanism by which words may be read lexically but without semantic mediation remains controversial. We report data from a patient (W.T.) with an infarction involving Wernicke's area who was unable to write, repeat, or comprehend words of low imageability yet read these and other words reliably; the patient could not, however, read nonwords. As the patient was unable to read on the basis of sublexical print-to-sound correspondences and, at least for words of low image-ability, was unable to read by means of a semantically mediated process, the data from this patient are most readily accommodated by models of reading incorporating three mechanisms.

Subjects with neglect of left hemispace may err to the right when bisecting lines. This error has been attributed either to a directional hypokinesia (failure to execute movements fully in or towards the contralateral hemispace) or to a failure to attend to or represent sensory information. Four subjects with neglect were tested on a line bisection task in which these two hypotheses were differentiated by preventing direct viewing of the line and instead using a video camera and monitor, each of which could be moved independently into right or left hemispace. The performance of 2 subjects was consistent with the predictions of the directional hypokinesia hypothesis; the performance of the other 2 subjects was consistent with the attention-representation deficit hypothesis. These data suggest that both a directional hypokinesia and an attention-representation deficit may each be a primary determinant of neglect.

Three distinct reading mechanisms have been proposed: the phonologic conversion system in which letters are converted to their sound equivalents (phonemes) before meaning is established, and two lexical or whole-word mechanisms in which a word (or root morpheme) is identified as a single unit rather than by phonologic conversion. The two lexical mechanisms differ in that one is inextricably linked to semantics but the other is not. The phonologic conversion system may depend more on the perisylvian phonologic system than do the lexical mechanisms, which may be mediated by left parietal or occipital areas or by the right hemisphere. Described here is a patient who had an infarction that partially isolated the perisylvian speech areas and disconnected the partially preserved lexical systems from the phonologic mechanism. He read visually presented orthographically irregular words but could not pronounce orally spelled irregular words. Although he could not read nonwords, he could pronounce orally spelled nonwords and orthographically regular words. These observations suggest that the lexical and phonologic conversion systems are functionally and anatomically distinct.

Two patients with mixed transcortical aphasia could repeat propositional speech but not affective prosody. These findings suggest that the intact perisylvian region responsible for propositional speech does not mediate effective prosody. We propose that affective prosody is incorporated into propositional speech by means of an interhemispheric mechanism and that the failure of these patients to repeat affective prosody was caused by a disconnection of the left perisylvian structures from the right hemisphere structures thought to mediate affective prosody.

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.

We tested the hypothesis that the right hemisphere is dominant for motor activation of "intention." If the right hemisphere is dominant for intention and if intention is a prerequisite for movement, motor impairment should be worse after an infarct in the right hemisphere than in the left. We asked nine subjects with right and nine with left hemisphere infarcts to elevate each shoulder independently. Subjects with right hemisphere lesions had more trouble moving the contralateral shoulder. The data are consistent with the hypothesis that the right hemisphere is dominant for motor activation or intention.

A 46-year-old female is described with prolonged, progressive dementia and a brain biopsy consistent with Creutzfeldt-Jakob disease (CJD). She had neither myoclonic jerks nor an electroencephalogram with periodic spikes and suppression. Five of her close relatives were also demented. The nosology of CJD was discussed in the light of this case in which histopathology was characteristic of spongiform encephalopathy but the clinical features were atypical. We concluded that it would be premature to expand the traditional diagnostic criteria to include such cases as having CJD but, at the same time, it would be prudent to handle tissue, linens and surgical instruments as if they were contaminated by the resistant agent of CJD.

One hundred sixteen patients, aged 8 to 82 years, with myasthenia gravis were treated with prednisone, 60 to 80 mg daily, until the onset of improvement, followed by lower-dose alternate-day therapy of several years' duration. Of all patients, 80.2% achieved either remission (27.6%) or marked improvement (52.6%). Moderate improvement occurred in 14.7%, and 5.2% showed no improvement. Increasing age correlated with a favorable outcome, but sex, duration of illness prior to treatment, severity and distribution of weakness at the time of onset of treatment, and presence of thymoma were not factors in the response to therapy.

Patients with hemispatial neglect perform activities poorly in the hemispace contralateral to the lesion. We postulate that hemispatial neglect induced by right hemisphere lesions may be associated with a directional hypokinesia: initiation of movements toward the hemispace contralateral to the lesion is affected more than movements toward the lesion. We tested 6 patients with hemispatial neglect caused by right hemisphere damage, 7 with left hemisphere damage and no neglect, and 12 controls. Patients with left hemispatial neglect initiated responses to left hemispace more slowly than toward right hemispace.

Destruction or disconnection of specific neuronal structures or failure to activate those structures may impair brain function. Because the right hemisphere seems dominant for mediating arousal, which is an important determinant of the capacity for cerebral activation, we predicted that subjects with right hemisphere damage would have a greater reduction in the capacity for cerebral activation than subjects with left hemisphere damage. A paradigm requiring that two simple tasks be performed singly and simultaneously was used to assess the capacity for activation. Subjects with right hemisphere damage had significantly greater impairment in the capacity for cerebral activation than subjects with left hemisphere damage. This impairment may partly explain the associations between right hemisphere damage and decreased ability to perform certain analytic and linguistic tasks.

We describe 4 patients who developed pure alexia after infarctions of the left cerebral hemisphere. All subjects employed a letter-by-letter strategy (with varying degrees of success) to explicitly identify visually presented words. Although all 4 subjects explicitly denied that they could identify briefly presented words, they all performed significantly better than chance on lexical decision and forced-choice semantic categorization tasks with briefly presented words which they could not explicitly identify. Three subjects regained the ability to explicitly identify briefly presented words; these subjects were more accurate with nouns than functors and words of high as compared with low imageability. Additionally, these subjects were impaired in the processing of suffixes. These data are not accommodated by the 'disconnection' account of pure alexia but are more consistent with the hypothesis that reading in these patients is mediated by the right hemisphere.

Impaired auditory comprehension and fluent but semantically empty speech in conjunction with preserved repetition characterize the syndrome of transcortical sensory aphasia (TSA). Repetition, however, may be mediated by at least two distinct processes--a lexical process that may involve the recognition and subsequent activation of discrete stored word representations and a nonlexical process that involves phonologic decoding and immediate phonologic encoding from immediate memory. We investigated the spontaneous speech, reading, and tendency to recognize and spontaneously correct syntactic errors in four patients with TSA: this analysis suggests there are two subtypes of TSA. We contend that in one subtype both the lexical and direct repetition (or speech production) mechanisms are preserved, but in the second subtype the lexical mechanism is disrupted and repetition is mediated by the nonlexical mechanism.

We studied patients with damage of either the right (RHD) or left hemisphere (LHD) and control subjects to determine whether the RHD patients had a global or limited prosodic defect. Compared with LHD patients and controls, RHD subjects had decreased comprehension of emotional prosody. Both LHD and RHD groups had more impaired comprehension of propositional prosody than controls, but the RHD and LHD groups did not differ. The right hemisphere, therefore, seems to be dominant for comprehending emotional prosody but not propositional prosody.

In this study, right-hemisphere-damaged (RHD) subjects performed significantly worse than LHD and NHD controls across a series of seven facial identity and facial affect tasks. Even when the patient groups were statistically equated on a measure of visuoperceptual ability, the RHD group remained impaired on three emotional tasks--naming, picking, and discriminating emotional faces. These findings suggest that the defects shown by RHD patients on facial affect tasks cannot be solely attributed to defects in visuoperceptual processing and that the right-hemisphere superiority for processing facial affect exists above and beyond its superiority for processing facial identity.

Optic aphasia is characterized by the ability to name from description and palpation but an inability to name visually-presented objects. Although originally attributed to a disconnection of visual information from object names, optic aphasia is often considered to be a mild form of visual agnosia. We describe a patient with optic aphasia who could access semantic information relevant to objects he could not name and comprehend written words he could not read. These data suggest that, at least in certain cases, this visual modality-specific naming impairment may not be attributable to impaired visual recognition. We suggest that this patient's preserved object recognition and reading comprehension was mediated by a semantic system supported by the right hemisphere.

Two studies were conducted in order to determine whether the poor performance of RHD patients on emotional prosody tasks could be attributed to a defect in perceiving/categorizing emotional prosody (processing defect) or to a problem in being distracted by the semantic content of affectively intoned sentences (distraction defect). In one study, patients with RHD, LHD or NHD listened to affectively intoned sentences in which the semantic content was congruent or incongruent with the emotional prosody. In a second study, the patients listened to affectively intoned sentences that had been speech filtered or unfiltered. Findings from these studies indicate that both processing and distraction defects are present in RHD patients.