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Prion protein codon 129 genotype prevalence is altered in primary progressive aphasia.

The prion protein (PrP) is central to the prion diseases, although a role in other neurodegenerative diseases has been postulated. A common polymorphism (Met or Val) at codon 129 of the PrP gene (PRNP) features prominently in the risk and phenotype, of prion disease, and an abnormality in its distribution frequency may signal a role for PrP in other diseases. We conducted a case-control study to compare the PRNP codon 129 genotype distribution in Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS), and primary progressive aphasia (PPA), including 281 AD, 256 ALS, 39 PPA, and 415 healthy control subjects. Statistical analysis was applied to determine the presence or absence of disease-specific genotype associations. The distribution of codon 129 genotypes was similar among healthy control, AD, and ALS subjects, although the heterozygous state was significantly overrepresented (age-adjusted odds ratio, 8.47) in PPA, a rare condition of unknown cause. Although these findings do not entirely exclude a role for PrP in AD or ALS, they do not support the codon 129 genotype as a risk factor for either disease. However, the strong association between heterozygosity and PPA raises new questions about its cause and the role of PrP in other neurodegenerative diseases.

Aged↗

Measurement of early spontaneous recovery from aphasia with stroke.

The amount of spontaneous change in aphasia was quantified in 44 right-handed patients who were aphasic following a first and unihemispheric stroke. All were initially tested less than 14 days after onset and were reevaluated at a mean of 30 days. The Porch Index of Communicative Ability was administered for objective and precise measurement. Forty-one of the patients demonstrated significant improvement, a finding which must be taken into account in evaluating any claims for effectiveness of speech therapy.

Adult↗

Mechanisms of recovery from aphasia: evidence from serial xenon 133 cerebral blood flow studies.

In 21 patients who suffered aphasia resulting from left hemisphere ischemic infarction, the xenon 133 inhalation cerebral blood flow technique was used to measure cerebral blood flow within 3 months and 5 to 12 months after stroke. In addition to baseline measurements, cerebral blood flow measurements were also carried out while the patients were performing purposeful listening. In patients with incomplete recovery of comprehension and left posterior temporal-inferior parietal lesions, greater cerebral blood flow occurred with listening in the right inferior frontal region in the late studies than in the early studies. In patients with nearly complete recovery of comprehension and without left posterior temporal-inferior parietal lesions, early listening studies showed diffuse right hemisphere increases in cerebral blood flow. Later listening studies in this latter patient group showed greater cerebral blood flow in the left posterior temporal-inferior parietal region. The study provides evidence for participation of the right hemisphere in language comprehension in recovering aphasics, and for later return of function in left hemisphere regions that may have been functionally impaired early during recovery.

Adult↗

Crossed aphasia with visceral situs inversus.

A patient presenting the association of anomalous cerebral dominance for language, as revealed by crossed aphasia, and partial visceral situs inversus is described. Although it cannot be excluded that the two abnormalities coincided by chance, it is suggested that they both resulted from a single aberration of development.

Aged↗

Working memory and aphasia.

This study investigated the relationship between working memory capacity and reading comprehension in aphasia. A measurement of working memory capacity was obtained using a modified version of Daneman and Carpenter's (1980) Reading Span Task. Sets of sentences ranging in length from one to six words were presented to 22 aphasic subjects who were required to retain the terminal words following each sentence for subsequent recognition. The maximum number of words retrieved was used as an index of working memory capacity. Two versions of the task (listening and reading) were presented depending on the subjects' ability to read. Strong positive correlations were found between working memory capacity, reading comprehension, and language function. These results support the notion that the ability of aphasic individuals to comprehend language is predictable from their working memory capacities.

Adult↗

Factors predicting success in picture naming in Alzheimer's disease and primary progressive aphasia.

The aim of the present study was to determine the predictive value of all important variables in the picture naming performance of 8 patients with probable Alzheimer's disease (AD) and 8 patients with primary progressive aphasia (PPA). The experimental investigation controlled for (i) visual complexity, (ii) name agreement on dominant response, (iii) age of acquisition, (iv) frequency, (v) word length, (vi) concept familiarity, and (vii) category membership. The results of the multiple regression analyses showed that age of acquisition and name agreement were significant for 10/16 subjects. Visual complexity, frequency, familiarity, and category were also significant for four patients respectively. Word length had no effect. These results are at variance with those of series of patients with AD (Gaillard et al., 1998) and with PPA (Lambon Ralph et al., 1998) where concept familiarity was found to be one of the most predictive factors of naming success.

Aged↗

Proper names and noun-to-determiner movement in aphasia: a case study.

Proper names (PN) have properties that are different from those of common names (CN) and as such underlie different syntactic rules. According to one of these rule, in many languages, PN, unlike CN, do not take the determiner. It has been suggested that this happens because PN move themselves from noun position to determiner position. The present study addressed the question of whether noun to determiner movement should be just considered a formal description or rather reflects actual processing. A single aphasia case study and a group study on Alzheimer's patients provide experimental support to the view that movement from nominal to determiner position reflects some psychological computation.

Aged↗

Locative prepositional phrases in severe aphasia.

Six severely aphasic patients were trained on C-VIC, a computerized alternative communication system. Patients with both global and Broca's aphasia were able to produce and comprehend locative prepositions using C-VIC. The Broca's aphasic patients were able to produce and interpret symbol order correctly in C-VIC locative prepositional phrases, despite their difficulties in performing the same tasks using English. One patient's verbal production of locative prepositional phrases improved markedly after C-VIC training. These results suggest that the "mapping" impairment suffered by some Broca's aphasics may be ameliorated by use of alternative communication system, such as C-VIC.

Aphasia↗

Memory impairment and executive control in individuals with stroke-induced aphasia.

The purpose of this study was to examine memory abilities of aphasic individuals in relation to site of neurological lesion. Fourteen individuals with stroke-induced aphasia (7 with anterior lesions; 7 with posterior lesions) and 14 demographically matched control subjects were given selected tests of short-term memory (STM) and long-term memory (LTM). Stroke patients were impaired relative to control subjects on tests of verbal memory, with greater impairment of LTM associated with anterior lesions and greater impairment of STM associated with posterior lesions. Verbal memory performance did not correlate highly with language ability, and did not appear to be simply a consequence of language impairment. Executive control deficits were postulated as explanatory of the LTM impairment associated with anterior lesions.

Aged↗

The effects of task variation on the production of grammatical morphology in Broca's aphasia: a multiple case study.

Generally task variation is not taken into account in discussions about agrammatism in Broca's aphasia. Both free speech and speech under constrained conditions are classified as "agrammatic speech." In this study, we investigated the effects of task variation on the character of agrammatic speech. We employed three types of task situations, an interview and two picture description tasks, to elicit speech from 16 Dutch and 3 German Broca's aphasics. The two picture description tasks differed in the extent to which they necessitated the production of grammatical morphology. Various task-dependent changes in the character of agrammatic speech were observed. Overall, omission rates of grammatical morphemes went down and substitution rates tended to go up in picture description as compared to free conversation. Results are discussed by contrasting two explanatory concepts, task interaction and strategic variation.

Adult↗

Is "nonpropositional" speech preserved in aphasia?

Twenty-eight aphasic patients were tested on six tasks designed to assess "nonpropositional" speech and six matched tasks designed to assess propositional speech. The most reliable differentiation between nonpropositional and propositional speech type behaviors within a group of aphasics was found for the Numbers and Picture naming tasks. A smaller effect was observed on the Days of the week and Phrase repetition tasks. Only 6 of the 28 patients consistently showed a nonpropositional advantage across the range of task comparisons. However, a principal components analysis extracted two factors which differentiated propositional from nonpropositional tasks reasonably well. We conclude that nonpropositional speech, to the extent that it is measured by these tasks, is not preserved in aphasics as a group, though some patients may show a fairly consistent nonpropositional advantage. The distinction between propositional and nonpropositional speech does appear to have some application to aphasia, however. An error analysis supports the conjecture that propositional tasks involve semantic processing while nonpropositional tasks may place a greater load on phonological processing.

Aged↗

Patterns of recovery and change in verbal and nonverbal functions in a case of crossed aphasia: implications for models of functional brain lateralization and localization.

We present a 2-year verbal and nonverbal follow-up of a crossed aphasic patient. The patient had suffered from widespread ischemic damage in the area of right middle cerebral artery, with a parieto-temporal lesion. Three months postonset he showed classical Wernicke's aphasia associated with oral, limb and constructional apraxia and left hemineglect. However, follow-up findings showed a complex, dynamic pattern entirely consistent with cognitive models of language and nonlanguage abilities. Current models of functional brain lateralizations could not satisfactorily account for such longitudinal, fine-grain observations.

Agraphia↗

Influence of size and site of cerebral lesions on spontaneous recovery of aphasia and on success of language therapy.

Changes in linguistic competence were assessed with the Aachen Aphasia Test in 18 aphasic patients across 8 weeks of spontaneous recovery, 8 weeks of intensive language therapy, and after a follow-up period of 8 weeks without therapy. CT scans were obtained from all patients and were evaluated for size of lesion and for affection of Wernicke's area, adjacent regions of superior and middle temporal gyrus, inferior parietal lesions, and temporobasal lesions. Size of lesion had a negative influence on recovery in all phases. Patients with lesions to temporobasal regions showed less improvement during therapy and less total recovery, but a similar amount of spontaneous recovery than patients without such lesions. Lesions that affected the temporobasal regions were on average larger than those which spared them, but the dissociation between reduced therapy success and unaffected spontaneous recovery became even more conspicuous when the concurrent effect of lesion size was minimized by appropriate selection of patients. Possibly, temporobasal lesions cause a disconnection between the hippocampal formation and perisylvian language areas and hinder explicit learning of linguistic knowledge and compensatory strategies.

Adult↗

Broca's aphasia: a syntactic and/or a morphological disorder? A case study.

The patient described here suffers from Broca's aphasia without a comprehension disorder. She is unique, since she has two speech styles available and she shifts between them spontaneously. One style is characterized by a mild syntactic disorder and the other by a quite severe morphological and syntactic disorder. None of the current theories about the underlying disorder in Broca patients without comprehension deficits can account for the fact that two linguistically different speech styles can occur in one patient, except the adaptation theory. It will be argued that both styles can be explained by the assumption of an impairment to the grammatical encoder (a processor which transforms the preverbal message into a surface structure, Levelt, 1989). This view suggests that the distinction between syntactic and morphological agrammatism could be a matter of strategy, rather than of impairment.

Adult↗

An examination of impaired acoustic-phonetic processing in aphasia.

This research examines the nature of acoustic-phonetic impairments found in aphasia, and the reliability of patient performance on phoneme discrimination and identification tasks. Aphasic patients were tested on three phoneme discrimination tasks examining their ability to discriminate items on the basis of contrasts in sonorance, manner, place, or voicing using both spoken and synthetic stimuli. One of these tests involving the discrimination of spoken, one-syllable items was given to patients on three occasions over the course of 1 year to examine short- and long-term test reliability. In addition to these measures, patients were tested on three phoneme identification tasks using stimulus items drawn from the discrimination tests. The results of these tests show that aphasic patients may display stable patterns of performance on these measures over time and between tests. Furthermore, these findings suggest that discriminations of synthetic speech do not necessarily reflect patients' ability to discriminate spoken speech tokens. These results are interpreted as evidence that aphasics display stable, discrete impairments in acoustic-phonetic processing, and that these deficits may be measured reliably using phoneme discrimination and identification tasks using natural speech.

Adult↗

Optic aphasia: a case with spared action naming and associated disorders.

AG, a pure case of optic anomia (object naming impaired; action naming good) is described. We consider the fit of experimental data from AG to different theoretical accounts of optic aphasia. Overall, we find no evidence for impairments intrinsic to semantic representations, but we note a number of problems that we interpret as indicating a slight, and specific, weakness in semantic access from vision. We also note a mild problem in generating names to a cue (verbal fluency). The main aim of the report was to provide a full description of tests of visual, semantic, and speech output skills in this patient in relation to the processing of visually presented objects and scenes, within a sequential information processing account which enables some theoretical implications to be drawn, albeit not conclusively.

Adult↗

The effects of age-induced changes in communicative abilities on the type of aphasia.

The purpose of this study was to explore the possibility that some normal age-related changes in aspects of conversational discourse could account for the recurrent finding in aphasiology that fluent aphasics are approximately a decade older than their nonfluent counterparts. Normal subjects taken from two age groups representative of the mean age of fluent and nonfluent aphasics were submitted to an interview similar to the one used with aphasic patients in order to evaluate fluency. Results showed that, in answer to both closed and open questions, older subjects' discourse was made up of more than three times as many morphemes as younger subjects' discourse. The increase in fluency was more marked for men than it was for women. Thus, discourse-expressed changes in communicative strategies among the elderly could contribute to the semiology of language impairments in such a way that nonfluent aphasias would be more frequent. The reasons for this change in normal controls are still unknown but have been proposed to range from biological to sociological age-related characteristics of the individual.

Age Factors↗

Landau-Kleffner syndrome: much more than aphasia and epilepsy.

Landau-Kleffner is currently considered a rare syndrome that combines acquired aphasia and epilepsy in children. We present two cases and discuss evidence in the literature that suggest how this syndrome is actually only part of a more generalized situation in which epilepsy (a paroxysmal disorder) produces or is accompanied by a prolonged or permanent derangement of neuronal circuits responsible for higher cortical functions, including language. This reappraisal of the identity of the syndrome could have interesting consequences not only for the study of epilepsy but also for the study of higher cortical functions.

Aphasia↗