Three variant forms of subcortical aphasia in Chinese stroke patients

Five right-handed patients with subcortical aphasia that involved the left hemisphere subcortical lesion sites were subjected to CT scans. Given their etiology, two cases were infarctions and the other three were hemorrhages. Two of the patients presented an involvement of the anterior limb of the internal capsule and of the basal ganglia and an anterior superior white-matter lesion extension. In both cases slow scanty dysarthric speech was noted; one had markedly impaired auditory comprehension, and the others were only partially impaired. The third patient presented an involvement of the posterior limb of the internal capsule and of the thalamus and a posterior paraventricular white-matter lesion extension. He had poor auditory comprehension, echolalia, and fluent speech. The last two patients presented an involvement of the internal capsule, the basal ganglia, and the thalamus and an anterior posterior paraventricular white-matter lesion extension. The latter two showed poor auditory comprehension with nonfluent and scanty spontaneous speech. The speech sounds were nonsensical monosyllabic words with a pattern similar to that of global aphasia. All patients had lasting right hemiplegia.     

Basal ganglia participation in language pathology

Language disturbances were studied in 40 patients with well-demarcated vascular lesions of the speech-dominant hemisphere. Computerized cranial tomography was used for localization of the lesion. Special emphasis was given to the analysis of automatized speech and repetitive verbal phenomena.Subcortical infarctions with basal ganglia involvement led to transient aphasia although long-lasting abnormalities of language could be detected in these patients. Aphasia was more severe if a cortical lesion was combined with a basal ganglia lesion. Automatisms and recurring utterances occurred only with combined cortical and basal ganglia lesions. A lesion of Wernicke's area alone, without involvement of prerolandic structures or subcortical nuclei, was sufficient to produce long-lasting aphasia, whereas lesions of Broca's area alone produced only transient language disturbances. The results are compatible with a recent theory of multiple cerebral representation of function.     

Semantic dementia and persisting Wernicke's aphasia: linguistic and anatomical profiles

Few studies have directly compared the clinical and anatomical characteristics of patients with progressive aphasia to those of patients with aphasia caused by stroke. In the current study we examined fluent forms of aphasia in these two groups, specifically semantic dementia (SD) and persisting Wernicke’s aphasia (WA) due to stroke. We compared 10 patients with SD to 10 age- and education-matched patients with WA in three language domains: language comprehension (single words and sentences), spontaneous speech and visual semantics. Neuroanatomical involvement was analyzed using disease-specific image analysis techniques: voxel-based morphometry (VBM) for patients with SD and overlays of lesion digitized lesion reconstructions in patients with WA. Patients with SD and WA were both impaired on tasks that involved visual semantics, but patients with SD were less impaired in spontaneous speech and sentence comprehension. The anatomical findings showed that different regions were most affected in the two disorders: the left anterior temporal lobe in SD and the left posterior middle temporal gyrus in chronic WA. This study highlights that the two syndromes classically associated with language comprehension deficits in aphasia due to stroke and neurodegenerative disease are clinically distinct, most likely due to distinct distributions of damage in the temporal lobe.     

Linguistic and neuropsychological deficits in crossed conduction aphasia. Report of three cases

This study describes the linguistic and neuropsychological findings in three right-handed patients with crossed conduction aphasia. Despite the location of the lesion in the right hemisphere, all patients displayed a combination of linguistic deficits typically found in conduction aphasia following analogous damage to the left hemisphere. Associated cognitive deficits varied across the three patients. In addition, all cases showed deficits classically attributed to non-dominant hemisphere damage (visuoperceptual deficits and reduced figural memory). As a result, lesion-behaviour relationships in our study sample indicate both dominant and non-dominant qualities of the right hemisphere.     

An open-label trial of bromocriptine in nonfluent aphasia: a qualitative analysis of word storage and retrieval

Anomia is a commonly found in aphasia and has been attributed to a loss of representations (storage deficit) or to a loss of access to these representations (retrieval deficit). Bromocriptine, a dopamine agonist, was tested on four patients, two men and two women, with nonfluent aphasia. The patients were tested in an open-label ABBA design using a stochastic model that measured the degree of storage and retrieval deficits. All patients showed significant improvements in word retrieval. Bromocriptine may be a useful adjunct in the treatment of selected patients with a nonfluent aphasia in which retrieval deficits play a major role.     

Comparison of metabolic rates, language, and memory in subcortical aphasias

Four patients with subcortical lesions and either aphasia or amnesia were compared to four patients with cortical lesions and aphasia. Each patient had [18F] fluorodeoxyglucose positron-emission computed tomography, language, and memory evaluations. Marked metabolic depression was found in the thalamus and caudate in all patients. The subcortical patients had only mild left-cortical changes, while the cortical patients showed marked cortical metabolic changes in the left hemisphere. Language changes were mild in the subcortical patients, while moderate to severe in the cortical patients. All patients showed severe verbal memory dysfunction. The only common abnormalities in the two groups were metabolic changes in the thalamus and severity of verbal memory dysfunction. These findings suggest a relationship between verbal memory and thalamic function.     

Self-monitoring behavior in a case of severe auditory agnosia with aphasia

This case report describes an unusual combination of speech and language deficits secondary to bilateral infarctions in a 62-year-old woman. The patient was administered an extensive series of speech, language, and audiologic tests and was found to exhibit a fluent aphasia in which reading and writing were extremely well preserved in comparison to auditory comprehension and oral expression, and a severe auditory agnosia. In spite of her auditory processing deficits, the patient exhibited unexpected self-monitoring ability and the capacity to form acoustic images on visual tasks. The manner in which she corrected and attempted to correct her phonemic errors, while ignoring semantic errors, suggests that different mechanisms may underlie the monitoring of these errors.     

Subcortical Aphasia

We critically review the literature on subcortical aphasia, suggest that a number of traditional concepts regarding mechanisms of aphasia are inconsistent with now abundant data, and propose several new hypotheses. The absence of aphasia in 17 reported cases of dominant hemisphere striatocapsular infarction and the finding of nearly every conceivable pattern of language impairment in 33 different reported cases of striatocapsular infarction provide strong evidence against a major direct role of the basal ganglia in language and against disconnection or diaschisis as mechanisms of nonthalamic subcortical aphasia. However, detailed consideration of the vascular events leading to striatocapsular infarction strongly suggests that associated linguistic deficits are predominantly related to sustained cortical hypoperfusion and infarction not visible on structural imaging studies. Thalamic disconnection, as may occur with striatocapsular infarcts with extension to the temporal stem and putamenal hemorrhages, may also contribute to the language deficits in some patients. Review of the literature on thalamic infarction, in conjunction with previously unreported anatomic details of four cases, suggests that what infarcts in the tuberothalamic artery territory and the occasional infarcts in the paramedian artery territory associated with aphasia have in common is damage to the frontal lobe–inferior thalamic peduncle–nucleus reticularis–center median system that may be involved in regulating the thalamic gate in attentional processes. Disruption of attentional gating in the pulvinar and lateral posterior nuclei resulting from such lesions may impair selection of specific neuronal networks in the projection field of these nuclei that serve as the substrate for lexical–semantic function, which is in effect a disruption of a type of working memory, as defined by Goldman–Rakic. We define this as a defect of selective engagement.     

The neuropsychology of paramedian thalamic infarction

A longitudinal study of three patients with CT-scan documented paramedian thalamic infarctions (bilateral, primarily right, unilateral left) is reported and the neuropsychology of human paramedian thalamic infarction is reviewed. The neuropsychological deficits following these selected lesions, the nature of the clinical memory disorder, and the neuroanatomy of memory are discussed. The significance of cortical/subcortical relationship in explaining observed behavioral changes is emphasized. Brain damage with maximum involvement in the dorsomedial nuclei and mamillothalamic tracts appears to cause primarily a memory disorder and frontal-limbic behavioral changes, the severity and profile of deficits depending on lesion extent and location. Both anterograde and remote memory loss may be present. Asymmetry in memory at the level of the thalamus was observed, following the left-verbal, right-nonverbal dichotomy.     

Maternal behavior of mice with cingulate cortical, amygdala, or septal lesions.

Nest building, pup retrieving, and pup survival and growth were evaluated in primiparous control mice and those with cingulate cortical, neocortical, amygdala, or septal lesions. Mice with neocortical or amygdala lesions showed little or no deficits in maternal behavior. Mice with septal lesions were severely impaired in all aspects of maternal care. These mice did not build nests, showed a variety of aberrant behaviors during pup retrieving, and their pups died or gained significantly less weight than those of controls. Mice with cingulate cortical lesions retrieved pups more slowly than controls, and retrieving deficits were correlated with the extent of retrograde degeneration found in the anterior thalamic nuclei.     

The neural basis of syntactic deficits in primary progressive aphasia

Patients with primary progressive aphasia (PPA) vary considerably in terms of which brain regions are impacted, as well as in the extent to which syntactic processing is impaired. Here we review the literature on the neural basis of syntactic deficits in PPA. Structural and functional imaging studies have most consistently associated syntactic deficits with damage to left inferior frontal cortex. Posterior perisylvian regions have been implicated in some studies. Damage to the superior longitudinal fasciculus, including its arcuate component, has been linked with syntactic deficits, even after gray matter atrophy is taken into account. These findings suggest that syntactic processing depends on left frontal and posterior perisylvian regions, as well as intact connectivity between them. In contrast, anterior temporal regions, and the ventral tracts that link frontal and temporal language regions, appear to be less important for syntax, since they are damaged in many PPA patients with spared syntactic processing.     

Semantic aphasia: A neglected entity

Three cases of semantic aphasia are reported. Computerized brain tomography showed bilateral temporo-parieto-occipital junction hemorrhages in one patient, and left parieto-occipital junction infarctions in the other two patients. The auditory comprehension defect of the three patients was characterized by preserved understanding of single words and impaired understanding of grammatically complex constructions. It is suggested that this comprehension defect reflects an inability to fully grasp the meaning of words and grammatical constructions imbued with spatial or quasi-spatial significance. Each of the three patients showed a complex spatial disorder that included constructional apraxia, spatial agnosia, and elements of Gerstmann's syndrome. The aphasic as well as the spatial disorder of semantic aphasics may be manifestations of a common defect in the perception of spatial relationships produced by left temporo-parieto-occipital region damage.     

Jargonagraphia in a case of frontotemporal dementia.

Jargonagraphia is known to occur after discrete brain lesions but not in primary degenerative dementia. We report a patient with frontotemporal dementia who developed jargonagraphia and nonfluent aphasia. Written output was graphically preserved but consisted of short words intermingled with abstruse neologisms. MRI showed predominant right frontotemporal cortical atrophy accompanied by white matter hyperintensities in the right anterior subcallosal periventricular region. Diagnosis and MRI were corroborated by extensive neuropathological findings obtained 8 months later. The agraphia in this case is discussed with reference to both specific macroscopic and microscopic pathoanatomical lesions. We suggest that jargonagraphia can appear in frontotemporal dementia depending on the localization of lesions.     

Linguistic and nonlinguistic priming in aphasia

Studies of real-time processing in aphasia suggest that linguistic symptoms may be due to deficits in activation dynamics rather than loss of linguistic knowledge. To investigate the domain specificity of such processing deficits, we compared performance by Italian-speaking fluent aphasics, nonfluent aphasics, and normal controls in a linguistic priming task (grammatical gender) with their performance in a color-priming task that requires no verbal mediation. Normal or larger than normal color-priming effects were demonstrated in both aphasic groups. Gender priming did not reach significance in either group, even though the patients displayed above-chance sensitivity to gender class and gender agreement in their accuracy scores. The demonstration of spared gender knowledge despite impaired gender priming underscores the utility of on-line techniques in the study of aphasia. The demonstration of spared color priming suggests that priming deficits in aphasia are either (1) specific to speech and language or (2) specific only to those sensorimotor and attentional processes that language shares with other nonlinguistic systems.     

Adynamic Aphasia: A Transcortical Motor Aphasia with Defective Semantic Strategy Formation

Adynamic aphasia is a form of transcortical motor aphasia characterized by sparse but otherwise normal spontaneous speech that may improve when concepts are introduced by external stimuli. Akinesia, impaired concept formation, inertia of concept generation, a defective semantic network, damage or impaired access to the verbal output lexicon, and defective semantic strategy formation have been proposed to account for this disorder. We studied a patient with adynamic aphasia and frontal lobe systems dysfunction due to bilateral striatocapsular infarctions. The patient was not akinetic but did demonstrate inertia of concept generation that could be overcome with prompting. However, prompting did not improve the number of concepts generated. He demonstrated a generally intact verbal lexicon and semantic network and normal lexical priming. However, his ability to sort closely related items into different classes without prior cuing regarding the nature of the classes was defective. Although his verbal memory was normal, he appeared to use a serial rather than a semantic strategy to recall items. Finally, despite normal lexical priming, he was impaired on a letter fluency task. These results most clearly demonstrate a defect in semantic strategy formation but indicate an additional and possibly related deficit in concept formation and a partial deficit in lexical strategy formation. All of these deficits appear to reflect impairment in the hierarchical organization of knowledge specific to the task at hand. This appears to be a key component of executive functions supported by frontal lobe systems.     

Variable solutions to the same problem: aberrant practice effects in object naming by three aphasic patients

Although deficits in confrontation naming are a common consequence of damage to the language areas of the left cerebral hemisphere, some patients with aphasia show relatively good naming ability. We measured effects of repeated practice on naming latencies for a set of pictured objects by three aphasic patients with near-normal naming ability and by neurologically intact young and older adults. While the non-injured participants showed a systematic reduction in overall mean latencies and reduced trial-to-trial latency variability, the aphasic patients did not. Examination of the latency distributions suggests that successful naming by aphasic patients may come about by different underlying operations.     

Real-time examinations of lexical processing in aphasics

We argue that the lesion localizing value of disruptions to modular information processing systems emerges most clearly from on-line analyses of processing. In this respect we seek to show that left anterior (but not left posterior) damage causes slowed information access and we discuss the manner in which this slowing might yield some of the specific syntactic limitations charted in Broca's aphasia. The general possibility we raise is that the cortical area implicated in Broca's aphasia is not necessarily the locus of syntactic representations, but rather sustains particular time-based operating characteristics that in turn sustain normal real-time parsing.This research was supported in part by NIH grants NS11408 and NS06209 to Boston University School of Medicine and by the Research Service of the Veterans Administration.     

Apathy in frontotemporal dementia: behavioral and neuroimaging correlates

We investigated the occurrence of goal-directed motivational change in the form of apathy in patients with frontotemporal dementia (FTD), particularly those with behavioral variant social and executive deficits (bvFTD). Standardized behavioral inventory was employed to survey and compare apathy ratings from patients and caregivers. In cases of bvFTD, apathy ratings were further related to measures of social cognition, executive function, and atrophy on brain MRI. Results indicated that caregivers rated bvFTD patients as having significantly elevated apathy scores though patient self-ratings were normal. Caregiver and self-ratings of FTD samples with progressive nonfluent aphasia and semantic dementia did not differ from healthy controls and their informants. In the bvFTD sample, caregiver apathy scores were not correlated with general cognitive screening or depression scores, but were significantly correlated with social cognition and executive function measures. Voxel-based morphometry revealed that apathy ratings in bvFTD were related to prominent atrophy in the right caudate (including the ventral striatum), the right temporo-parietal junction, right posterior inferior and middle temporal gyri, and left frontal operculum- anterior insula region. Findings suggest that bvFTD is associated with a significant breakdown in goal-directed motivated behavior involving disruption of cortical-basal ganglia circuits that is also related to social and executive function deficits.     

Development of speech and language following bilateral frontal lesions

Language and speech were studied in a young child with perinatally acquired bifrontal lesions. Bilateral frontal pathology seriously interfered with the development of intelligible speech and resulted in a persistent expressive aphasia. Analysis of the neuropsychological profile indicated impairments in intelligence and language comprehension. These deficits, however, were considered secondary to the profound speech programming disorder. The findings indicate that, despite the plasticity of the immature central nervous system, bilateral frontal injury sustained at an early age precludes the development of intelligible speech. Furthermore, structurally intact cortical regions outside the territories of the speech zones fail to mediate normal speech and language development.