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Clinical and pathological characterization of progressive aphasia.

OBJECTIVE: The clinical and neuropathological categorization of patients presenting with progressive aphasia is an area of controversy. This study aimed to characterize a large group of progressive aphasic patients from a single center (n = 38), first clinically by case note review, and then pathologically. METHODS: Hierarchical cluster analysis of the cases according to their clinical language deficits was used to establish an unbiased, data-driven classification. RESULTS: This analysis revealed two groups of cases corresponding to the syndromes of progressive nonfluent aphasia (n = 23) and semantic dementia (n = 15). Postmortem analysis showed a majority in both groups of pathologies from the spectrum of frontotemporal lobar degeneration: the most frequent were non-Alzheimer's disease (AD) tauopathy in the nonfluent cases (10 of 23) and frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions in the fluent cases (8 of 15). Despite rigorous exclusion of cases with clinically significant memory deficits or other cognitive impairments, the pathology of AD was present in approximately one third of each group (overall 12 of 38), although often with an atypical neuroanatomical distribution. INTERPRETATION: Progressive aphasia is best seen as a composite of two conditions, on both clinical and pathological levels: progressive nonfluent aphasia and semantic dementia.

Alzheimer Disease↗

Language deviations in aphasia: a frequency analysis.

Thirty right-handed left hemisphere-damaged patients were taken and divided into five groups (transcortical motor, Broca, conduction, Wernicke, and anomic aphasia). Language deviations were scored and analyzed for the Picture Description (Plate No. 1, The Cookie Theft), Repetition (Words, High and Low Probability Sentences), and Naming (Responsive Naming, Confrontation, and Body-part naming) subtests of the Boston Diagnostic Aphasia Examination--Spanish version (Goodglass & Kaplan, 1979). A classification of paraphasias is proposed. Language deviations were scores for the following groups: Literal paraphasias (phoneme omissions, additions, displacements, and substitutions), verbal paraphasias (formal, morphologic, semantic, and unrelated), syntagmatic paraphasias, circumlocutions (object description and instrumental function), indefinite anaphors, and neologisms. Frequency of different types of language deviations is presented in the five aphasia groups. It was found that some paraphasic errors appeared in several aphasia groups; others were characteristics of specific aphasic syndromes.

Adolescent↗

Crossed aphasia in a dextral: a test of the Alexander-Annett theory of anomalous organization of brain function.

A case of crossed aphasia is presented in a strongly right-handed 77-year-old white female without history of familial sinistrality or prior neurological illness. She developed a right middle cerebral artery infarction documented by CT and accompanied by obvious clinical signs of a conduction aphasia with some resolution but continuing obvious language defect after 9 weeks in rehabilitation. Comprehensive neuropsychological and aphasia testing suggested anomalous lateralization of phonologic-output aspects of language, emotional prosody, motor planning and body schema modules with usual lateralization of lexical-semantic aspects of language and visuo-spatial functions. Experimental validation of the uncrossed lexical-semantic aspects of language using tachistoscope methods found support for the Alexander-Annett theory that different aspects of language can be dissociated in their lateralization. The subject had difficulty identifying a semantic associate of a picture presented to the left visual field (7 errors out of 10) relative to right visual field presentation (2 errors out of 10). Bilateral free naming errors (6 and 5 errors in the left and right visual fields, respectively) occurred consistent with the aphasic presentation, suggesting phonologic-output dysfunction from the right cerebral vascular accident. Implications of the results for aphasia classification are discussed.

Aged↗

Acquired crossed aphasia in dextral children revisited.

In contrast to the estimated low incidence of crossed aphasia in dextral adults (among 1%), crossed aphasia in children has been considered a common finding for almost a century. However, reviewing the literature on crossed aphasia in dextrals (CAD) and its related topics from 1975 onward, we encountered only 5 children in a corpus of 180 cases (2.7%). Critical analysis rendered three of the reported cases ambiguous and hence not suitable to draw potentially relevant conclusions. In this review, the neurobehavioral manifestations of the two representative childhood CAD cases are analyzed and compared with adult CAD and acquired childhood aphasia (ACA). In the light of our findings, which support the position of innate cerebral specialization for language, the long-standing controversy as to whether lateralized hemispheric specialization for language is innate or develops progressively during maturation is briefly discussed.

Aphasia↗

Aphasia in bilinguals.

The neurophysiological and the neurolinguistic basis of multilingualism is not yet fully elucidated. A study of the occurrence of aphasia in multilingual patients and the pattern of recovery may help to clarify some unsolved problems. For e.g. is the ability to use a second language stored in a different area of the left hemisphere and what is the extent of involvement of the right hemisphere in language skills? When recovery from aphasia occurs, what factors determine the rate of recovery of different languages and the priorities of recovery? To understand some of the problems, a study of aphasia in multilinguals was conducted in Madras, South India, where multilingualism is common. 88 patients were studied with 40 healthy controls, using standard protocols. It was found that the pattern of recovery was dispersed widely in time, rate, level, degree and between the languages known. No support was obtained for the notion that the patients' mother tongue recovers first, nor was support found for the importance of language proficiency viewed globally. Evidence was found that the languages in which routine thinking, mental calculations and praying were carried out were the ones most resistant to damage in brain insults. It is interesting that these functions are all highly overlearned, acquired early in life and used frequently over the course of many years. Another observation made was that the incidence of crossed aphasia was fairly high, in both uni- and multi-linguals, with the latter showing a slightly higher incidence.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Corticobasal degeneration with primary progressive aphasia and accentuated cortical lesion in superior temporal gyrus: case report and review.

A 57-year-old woman showed progressive sensory aphasia as an initial symptom, and then developed total aphasia within 6 years and, finally, severe dementia. Neuropathologically, the cerebral cortex was most severely affected in the superior and transverse temporal gyri, and subsequently in the inferior frontal gyrus, especially in the pars opercularis. The degeneration in the subcortical grey matter was most severe in the substantia nigra, and it was moderate to mild in the ventral part of thalamus, globus pallidus and striatum. Cytopathologically, in addition to achromatic ballooned neurons, massive taupositive types of cytosekeletal abnormalities were observed both in neurons and glia, mainly in the degenerating region. This cytoskeletal pathology coincided with that reported in corticobasal degeneration (CBD). On Bodian staining, only a few neurofibrillary tangles were found in the entorhinal pre-alpha layer and substantia nigra. Pick's bodies and senile plaques could not be found. This case is thought to represent a type of CBD, but with its cortical lesion focus located in the speech area instead of the frontoparietal region. A survey of 28 pathologically evaluated cases of CBD revealed two similar cases, both of which began with progressive aphasia and presented cortical degeneration in the superior temporal gyrus. An overview of CBD cases clarified the features in another group of cases, in which the cerebral accentuated focus was shifted forward from the central region, clinically resembling Pick's disease. The clinical manifestations in CBD seem to be the expression of these diverse cortical lesions. Primary progressive aphasia may include cases of CBD with involvement of the language center.

Aphasia, Primary Progressive↗

Localization of cerebral lesions in aphasia--a computer aided comparison between men and women.

It is still a matter of debate whether there are differences between men and women concerning the localization of higher cerebral functions. To further elucidate this problem we conjointly evaluated the aphasia protocols and corresponding computer-assisted tomography (CT) scans of 49 men and 35 women who presented with unilateral ischemic cerebral lesions. Both, the aphasia tests and CT scans, were fed into computer programs warranting a high degree of objectivity. Comparing the four main aphasic syndromes we found differences for each of them displaying a more posterior extension of lesions with global and amnestic aphasia in men and larger lesions with Broca and Wernicke aphasia in women. When all templates were compared, men displayed a wider extension within the left hemisphere than women whose lesions were more focused in the perisylvian area. This study confirms earlier findings claiming that aphasic men and women differ regarding the anterior-posterior extension and the frequency of lesions within the classical language zones. However, while the predominance of men's lesions in the posterior areas was in accordance with previous findings, the greater overall scatter within the left hemisphere contradicts published studies.

Adolescent↗

Functional connectivity between cortical hand motor and language areas during recovery from aphasia.

Previous data indicate that in healthy subjects, there is a connectivity between cortical areas for hand movement and language on the left hemisphere. This link is possibly mediated by the so-called mirror neuron system. The present study investigated the functional relationship between linguistic and hand movement processing in patients who were recovering from post-stroke aphasia. The excitability of the right- and left-hand motor cortex during language production in patients who were recovering from post-stroke aphasia and age-matched controls was investigated. As control, phonation was investigated. Hand motor cortex excitability was assessed with Motor Evoked Potentials which were elicited by Transcranial Magnetic Stimulation (TMS). In patients, reading aloud enhanced the excitability of the right hemispheric hand motor cortex, whereas phonation had no effect on hand motor cortex excitability. In the control group, an increased excitability of the left hemispheric hand motor system was found during reading aloud in accordance with previous data. The present data suggest a functional connectivity between regions mediating hand movements and reading. This may indicate that the right hemisphere participates in language processing as far as involved in single word reading in patients recovering from aphasia. The coactivation between cerebral representations of hand movements and language may be used therapeutically for aphasia rehabilitation.

Adult↗

Functional activation studies of word processing in the recovery from aphasia.

Some reviews on theories of recovery in aphasia put an emphasis on neural network models based on empirical data from evoked-potentials in aphasia as an approach to mapping recovery of cognitive function to neural structure. We will focus here on what we call an "anatomical" approach to look at recovery in aphasia. "Anatomical" theories of recovery stated by classical aphasiologists have contributed to the understanding of language representations in the human brain. But many aspects of these theories can only be investigated by using modern techniques of lesion analysis, psychometric assessment and functional imaging. Whereas structure-function relations have been primarily established by looking for the association of deficit symptoms with certain lesions, functional activation methods offer a means to study more directly the functional anatomy of recovered or retained functions in neuropsychological patients. To falsify or build up anatomical theories of recovery we will propose a stepwise approach of inference. The methodological pitfalls of this approach will be discussed by focussing on anatomical hypotheses of semantic word comprehension and its impairment and recovery in aphasia.

Aphasia↗

Is math lateralised on the same side as language? Right hemisphere aphasia and mathematical abilities.

The main purpose of the present study was to learn how mathematical abilities are located and develop in the brain with respect to language. Mathematical abilities were assessed in six right-handed patients affected by aphasia following a lesion to their non-dominant hemisphere (crossed aphasia) and in two left-handed aphasics with a right-sided lesion. Acalculia, although in different degrees, was found in all cases. The type of acalculia depended on the type of aphasia, following patterns that have been previously observed in the most common aphasias resulting from left hemisphere lesions. No sign of right hemisphere or spatial acalculia (acalculia in left lateralised right-handed subjects) was detected. These results suggest that, as a rule, language and calculation share the same hemisphere. A primitive computational mechanism capable of recursion may be the precursor of both functions.

Aphasia↗

Overt propositional speech in chronic nonfluent aphasia studied with the dynamic susceptibility contrast fMRI method.

This study examined activation levels in the left (L) supplementary motor area (SMA) and the right (R) SMA (separately), and activation in nine R perisylvian language homologues during overt, propositional speech in chronic nonfluent aphasia patients. Previous functional imaging studies with a variety of chronic aphasia patients have reported activation in these regions during different language tasks, however, overt propositional speech has not been examined. In the present research, four nonfluent aphasia patients were studied during overt elicited propositional speech at 4-9 years post-single L hemisphere stroke, which spared the SMA. The dynamic susceptibility contrast (DSC) method of functional MRI was used to calculate relative cerebral blood volume (relCBV) for cortical regions of interest (ROIs) during the first-pass bolus of gadolinium during two conditions: (1) pattern (silent viewing of checkerboard patterns) and (2) story (overt, elicited propositional speech describing sequential pictures, which formed a story). During the story condition, controls had significantly higher relCBV in L SMA than in R SMA; aphasics, however, had significantly higher relCBV in R SMA than in L SMA. During the pattern condition, no significant differences were observed between the L SMA and the R SMA for either controls or aphasics. In addition, aphasics had significantly higher relCBV in the R sensorimotor mouth during story than pattern. This R sensorimotor mouth relCBV was also significantly higher in aphasics than controls during story, and the two groups did not differ during pattern. The overall mean relCBV for the nine R perisylvian ROIs was significantly higher for aphasics than controls during both story and pattern. These results suggest that poor modulation, including possible over-activation of R sensorimotor mouth and other R perisylvian language homologues may underlie in part, the hesitant, poorly articulated, agrammatic speech associated with nonfluent aphasia.

Adult↗

Global aphasia due to thalamic hemorrhage: a case report and review of the literature.

A 55-year-old patient was admitted with weakness in the right extremities and with significant language deficits. Both computed tomography (CT) and positron-emission tomography (PET) using 2-fluoro-2-deoxy-D-glucose were done within 1 month and repeated at 10 months after onset. Language evaluation was done using parts of the Western Aphasia Battery (WAB) and the Boston Diagnostic Aphasia Examination (BDAE) at 1 month after admission, at 3 months, and at 10 months. The initial CT scan revealed hemorrhage in the left thalamus with edema surrounding the hemorrhage. A follow-up CT scan after 10 months showed an old hemorrhage in the left thalamus with no new lesions. The initial PET scan revealed hypometabolism in the left thalamus, and a repeat PET scan at 10 months showed reduced uptake in the left frontal, left parietal, and left temporal cortex. An initial language evaluation showed impaired auditory and reading comprehension, poor verbal expression, impaired repetition, and difficulty with naming and with sentence completion. The patient was diagnosed with global aphasia. Follow-up language evaluation at 3 months and 10 months showed only minimal improvement in his communication. Global aphasia due to lesions in the thalamic region is rare and the prognosis is poor.

Aphasia↗

Piracetam as an adjuvant to language therapy for aphasia: a randomized double-blind placebo-controlled pilot study.

OBJECTIVE: To determine whether piracetam 4.8 g/day together with intensive language therapy improved language function more than language therapy alone. DESIGN: Double-blind, placebo-controlled parallel group study. SETTING: Referral speech and language clinic of a university department of neurology. PATIENTS: Sixty-six inpatients with aphasia present between 4 weeks and 36 months. INTERVENTIONS: Intensive language therapy for 6 weeks in all patients. Thirty-two patients received piracetam 4.8 g daily and 34 patients received placebo. MAIN OUTCOME MEASURE: The Aachen Aphasia Test (AAT), a standardized procedure for evaluating the severity of aphasia, was performed at baseline and after 6 weeks' treatment. RESULTS: In 50 patients evaluated for efficacy, a trend toward improvement in the active group was observed in all subtests of the AAT. This trend was statistically significant for absolute differences in recovery of "written language" and "profile level." CONCLUSION: Piracetam appears to have a positive adjuvant effect on the recovery of aphasia in patients receiving intensive language therapy.

Adult↗

Crossed aphasia: one or more syndromes?

Seven strongly right-handed patients developed aphasia following a right hemisphere vascular lesion documented by computerized tomography. One patient had a severe unilateral neglect, indication of its presence were evident in three and absent in three patients. The Token Test scores were significantly higher than in matched controls. Two patients had Broca aphasia, four had Wernicke aphasia and one had agraphia. The correlation between type of aphasia and locus of lesion was not much different from that normally found in standard left hemisphere brain damaged aphasics.

Aphasia↗

Evolution of aphasia in the first year post-onset.

Evolution of aphasia was studied in the first year of recovery in 43 patients sustaining left hemisphere strokes with language impairment. Observations were made daily during acute hospitalization and subsequently at approximately one, two, three, four-to-six and seven-to-twelve month intervals post-stroke. Aphasias were classified according to standard criteria. A significantly larger percentage of the sample (59%) exhibited evolution of aphasia than in previous studies. This was attributed to earlier and more intensive patient observations in this investigation. Most changes occurred within the first two weeks of recovery. Two patterns of evolution were clearly present in the sample, one being early rapid change and the other a more gradual evolution. An expansion and integration of Gloning and Quatember's (1964) model of evolution is proposed on the basis of combined results from several studies. Also, the influence of aging, evolution of aphasia and the emergence of dementia post-stroke are discussed.

Adult↗

A comparison of relaxation training and syntax stimulation for chronic nonfluent aphasia.

UNLABELLED: This study examined the effects of relaxation training and syntax stimulation on the spoken language abilities of a 59-year-old male with chronic, nonfluent aphasia of moderate severity. Relaxation training consisted of progressive muscle relaxation (PMR) and guided imagery (GI), whereas the syntax stimulation was a modified version of the Helm Elicited Program for Syntax Stimulation (HELPSS) [(1981). Helm Elicited Language Program for Syntax Stimulation. Austin, TX: Pro-Ed.]. These treatments were applied in the context of a single-subject alternating treatments plus baseline design. Results indicated that although both treatments produced improvements in spoken language, syntax stimulation was associated with larger improvements, particularly in terms of the proportions of grammatical utterances, correct information units (CIUs), and successful utterances produced by the participant. Analysis of treatment order, however, indicated that the participant's best performances of the syntax treatment and of the probe tasks occurred when relaxation training preceded syntax stimulation. These findings suggest that the simplicity and psychological benefits of relaxation training make it a complementary component to traditional linguistic programs for aphasia. EDUCATIONAL OBJECTIVES: (1) The reader will understand how psychological responses to stress may affect the language processing abilities of adults with aphasia. (2) The reader will be able to describe how relaxation training complements a traditional language treatment approach for remediating spoken language abilities of adults with chronic nonfluent aphasia.

Aphasia, Broca↗

Motivating for infrastructure change: toward a communicatively accessible, participation-based stroke care system for all those affected by aphasia.

UNLABELLED: This paper provides a rationale for changing the base upon which healthcare services for individuals with stroke and aphasia can be provided. It is a nuts-and-bolts summary of the interactions between the Aphasia Institute and the West Greater Toronto Stroke Network who worked together to effect meaningful change. Further, the article provides a practical set of guidelines for others to use, should they wish to effect such change. LEARNING OUTCOMES: As a result of reading this article, the participant will be able to (1) develop a strong rationale changing the infrastructure related to healthcare services for individuals and families who have incurred stroke and aphasia; (2) describe the activities undertaken at the Aphasia Institute for accomplishing these changes; (3) refer to a practical set of guidelines for effecting infrastructural change.

Aphasia↗

Primary progressive aphasia and Pick complex.

Ten autopsied patients from a prospectively followed, clinically defined, neuropsychologically and radiologically documented cohort with primary progressive aphasia were histologically characterized. All were variants of frontotemporal degeneration (Pick complex): Pick body dementia, n=3, corticobasals degeneration (CBD), n=4, and tau and synuclein negative ubiquitinated inclusions of the motor neuron disease type, n=3. All shared superficial cortical spongiosis, neuronal loss, and gliosis. Although most patients had fluent anomic aphasia at onset, all progressed to a nonfluent or mute state. Comprehension, episodic memory, and activities of daily living were initially preserved. Three cases with Pick body dementia had verbal apraxia and stuttering at onset. Two of the patients with CBD pathology were older than the average primary progressive aphasia (PPA). All patients developed secondary syndromes either of frontotemporal dementia (FTD) and/or extrapyramidal-apraxic manifestations (CBD). By the time autopsy was obtained, the pathology appeared outside the language areas. Progressive aphasias secondary to Alzheimer's disease (AD) were excluded on the basis of early loss of memory and comprehension.Rather than the previously emphasized histological heterogeneity, clinically probable PPA has a predictive value of a group of related pathologies, collectively named frontotemporal degeneration, or Pick complex. This series of autopsied cases provides evidence for the clinical and pathological overlap of PPA with FTD and CBD, and contributes to the diagnostic and neuropsychological definition of PPA.

Aphasia↗