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Aphasia

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3741. "Diffusion-clinical mismatch" is associated with potential for early recovery of aphasia. (Abstract)

"Diffusion-clinical mismatch" is associated with potential for early recovery of aphasia. Diffusion-perfusion mismatch (perfusion-weighted imaging [PWI] abnormality minus diffusion-weighted imaging [DWI] abnormality) can identify candidates for acute stroke intervention, but PWI is often not obtainable. The authors hypothesized that language tests can predict volume of hypoperfusion, and thus mismatch, in acute left hemisphere stroke, and that the estimated mismatch can predict potential

2005 Neurology

3742. Acute aphasia in multiple sclerosis: A multicenter study of 22 patients. (Abstract)

Acute aphasia in multiple sclerosis: A multicenter study of 22 patients. Aphasia is usually considered to be rare in multiple sclerosis (MS). To determine the clinical and radiologic characteristics of MS patients with acute aphasia, the authors investigated data from 2,700 patients from three MS centers and found 22 patients with acute aphasia (0.81%). Aphasia was the first clinical manifestation of MS in eight patients (36%). Brain MRI showed giant plaques in eight cases (40%). A full (...) recovery was observed in 14 patients (64%). Furthermore, acute aphasia did not appear to be a criterion for poor prognosis.

2004 Neurology

3743. Natural history of primary progressive aphasia. (Abstract)

Natural history of primary progressive aphasia. To characterize the natural history of primary progressive aphasia (PPA).Forty-nine patients (28 women) with newly diagnosed with PPA presenting to a memory disorders clinic between 1992 and 2001 were prospectively evaluated.Median age at onset was 62 years (range 49 to 73 years) and at first visit was 66 years (52 to 80 years). The median duration of follow-up was 4 years (1 to 11 years). Impairments in activities of daily living developed (...) a median of 6 to 7 (2 to 12) years post onset. Seventy-five percent of patients eventually met clinical diagnostic criteria for frontotemporal dementia (FTD), 14% met diagnostic criteria for dementia with Lewy bodies, and 8% developed signs of corticobasal degeneration; 60% of the patients died after a median course of 7 years (3 to 17 years) at a median age of 71 years (56 to 81 years). Patients showing high Mini-Mental State Examination scores, moderate aphasia, and fluent language at first visit

2005 Neurology

3744. Behavioral features in semantic dementia vs other forms of progressive aphasias. Full Text available with Trip Pro

Behavioral features in semantic dementia vs other forms of progressive aphasias. To compare the behavioral profiles in different variants of primary progressive aphasia (PPA).We classified 67 patients with PPA into three clinical variants: semantic dementia (SEMD), progressive nonfluent aphasia (PNFA), and logopenic progressive aphasia (LPA), and we compared the severity of behavioral dysfunction, as measured by the Neuropsychiatric Inventory, in these groups and patients with frontotemporal (...) was not detected in PNFA or LPA.Semantic dementia is associated with significantly more behavioral dysfunction than other variants of primary progressive aphasia, specifically behavioral features typical of frontotemporal dementia.

2006 Neurology

3745. Corticobasal degeneration and progressive aphasia. (Abstract)

Corticobasal degeneration and progressive aphasia. To describe language impairment in the corticobasal degeneration syndrome (CBDS) presenting as either a cognitive or motor disorder, to compare the evolution of aphasia in CBDS with primary progressive aphasia (PPA), and to examine whether the side of maximal cerebral atrophy or akinesia reflects the severity of aphasia.We divided 40 patients with CBDS according to motor or cognitive onsets and conducted detailed language assessments (...) with the Western Aphasia Battery (WAB). We analyzed scores according to the side of atrophy and motor rigidity. Longitudinal performance over three annual assessments was compared against matched patients with PPA and Alzheimer disease.Language at baseline was more impaired in cognitive than motor-onset CBDS but there was no correlation between the side of atrophy or motor impairment and the WAB. Serial assessment (n = 19) showed a similar evolution of aphasia in cognitive-onset CBDS and PPA and delayed

2006 Neurology

3746. Paradoxical features of word finding difficulty in primary progressive aphasia. Full Text available with Trip Pro

Paradoxical features of word finding difficulty in primary progressive aphasia. Impaired word retrieval is a main symptom of primary progressive aphasia (PPA). The cognitive features of this impairment in PPA are poorly understood. We studied 12 patients with PPA (6 English-speaking and 6 Dutch-speaking), 7 patients with early-stage clinically probable Alzheimer's disease (PRAD), 5 patients with mild cognitive impairment (MCI), and 15 age-matched, cognitively intact, control subjects. Subjects

2005 Annals of Neurology

3747. Prion protein codon 129 genotype prevalence is altered in primary progressive aphasia. (Abstract)

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

2005 Annals of Neurology

3748. Clinical and pathological characterization of progressive aphasia. (Abstract)

Clinical and pathological characterization of progressive aphasia. 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.Hierarchical cluster analysis of the cases according to their clinical language deficits was used to establish an unbiased, data-driven (...) classification.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

2006 Annals of Neurology

3749. Foreign accent syndrome as the initial sign of primary progressive aphasia. (Abstract)

Foreign accent syndrome as the initial sign of primary progressive aphasia. Foreign accent syndrome (FAS) is a rare speech disorder characterised by the emergence of a new accent, perceived by listeners as foreign. FAS has usually been described following focal brain insults, such as stroke. We describe the unusual case of a woman presenting with FAS as the earliest symptom of progressive degenerative brain disease. At presentation, she showed no language or other cognitive impairment (...) , and functional and structural brain imaging were normal. Follow-up 1 year later revealed the emergence of mild expressive language problems. Repeat functional neuroimaging showed mild hypoperfusion of the perisylvian speech area of the left hemisphere, and structural imaging showed mild left perisylvian atrophy. We interpret the case as an unusual presentation of primary progressive non-fluent aphasia. The case provides further evidence of the variable and circumscribed nature of the clinical presentation

2007 Neurosurgery and Psychiatry

3750. Trying to tell a tale: discourse impairments in progressive aphasia and frontotemporal dementia. Full Text available with Trip Pro

Trying to tell a tale: discourse impairments in progressive aphasia and frontotemporal dementia. To assess discourse in patients with frontotemporal dementia (FTD).The authors asked patients with progressive nonfluent aphasia (PNFA), patients with semantic dementia (SemD), and nonaphasic patients with a disorder of social comportment and executive functioning (SOC/EXEC) to narrate the story of a wordless children's picture book.The authors found significant discourse impairments in all three (...) in progressive aphasia is due largely to the language impairments of these patients.

2006 Neurology

3751. Progranulin mutations in primary progressive aphasia: the PPA1 and PPA3 families. (Abstract)

Progranulin mutations in primary progressive aphasia: the PPA1 and PPA3 families. Primary progressive aphasia (PPA) is a language-based dementia characterized by fluent or nonfluent language disorder as its principal feature.To describe progranulin gene mutations in 2 families with PPA.Report of affected families.Academic research.Two families, PPA1 and PPA3, were studied. Genomic DNA was isolated from 3 of 4 siblings in PPA1, from all 3 siblings in PPA3, and from more than 200 control

2007 Archives of Neurology

3752. Semantic impairment in stroke aphasia versus semantic dementia: a case-series comparison. Full Text available with Trip Pro

Semantic impairment in stroke aphasia versus semantic dementia: a case-series comparison. Different neuropsychological populations implicate diverse cortical regions in semantic memory: semantic dementia (SD) is characterized by atrophy of the anterior temporal lobes whilst poor comprehension in stroke aphasia is associated with prefrontal or temporal-parietal infarcts. This study employed a case-series design to compare SD and comprehension-impaired stroke aphasic patients directly on the same (...) semantic representations degrade in SD. The stroke aphasia group also showed multimodal deficits and consistency across different input modalities, but inconsistent performance on tasks requiring different types of semantic processing. They were insensitive to familiarity/frequency--instead, tests of semantic association were influenced by the ease with which relevant semantic relationships could be identified and distractors rejected. In addition, the aphasic patients made associative semantic errors

2006 Brain

3753. Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Full Text available with Trip Pro

Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. Apraxia of speech (AOS) is a motor speech disorder characterized by slow speaking rate, abnormal prosody and distorted sound substitutions, additions, repetitions and prolongations, sometimes accompanied by groping, and trial and error articulatory movements. Although AOS is frequently subsumed under the heading of aphasia, and indeed most often co-occurs with aphasia, it can be the predominant or even (...) the sole manifestation of a degenerative neurological disease. In this study we determine whether the clinical classifications of aphasia and AOS correlated with pathological diagnoses and specific biochemical and anatomical structural abnormalities. Seventeen cases with initial diagnoses of a degenerative aphasia or AOS were re-classified independently by two speech-language pathologists--blinded to pathological and biochemical findings--into one of five operationally defined categories of aphasia

2006 Brain

3754. Aphasia after hemispherectomy in an adult with early onset epilepsy and hemiplegia. Full Text available with Trip Pro

Aphasia after hemispherectomy in an adult with early onset epilepsy and hemiplegia. A 55 year old left handed man with left hemisphere subcortical encephalomalacia, seizures, language impairment, and right hemiparesis from a motor vehicle accident at age five was evaluated for epilepsy surgery. The patient continued to speak and followed commands during a left intracarotid amobarbital test (IAT). Left functional hemispherectomy resulted in expressive aphasia. Based on postoperative outcome (...) , language was bilateral. The injury after primary development of language function, the predominantly subcortical lesion, and the late timing of surgical intervention well past development and plasticity may have been factors in the emergence of postoperative aphasia.

2004 Neurosurgery and Psychiatry

3755. Aphasia: progress in the last quarter of a century. Full Text available with Trip Pro

Aphasia: progress in the last quarter of a century. In the last 25 years, characterization of aphasia has shifted from descriptions of the language tasks that are impaired by brain damage to identification of the disrupted cognitive processes underlying language. At the same time advances in technology, including functional imaging, electrophysiologic studies, perfusion imaging, diffusion tensor imaging, and transcranial magnetic stimulation, have led to new insights regarding the relationships (...) between language and the brain. These insights, together with computational models of language processes, converge on the view that a given language task relies on a complex set of cognitive processes and representations carried out by an intricate network of neural regions working together. Recovery from aphasia depends on restoration of tissue function or reorganization of the cognitive/neural network underlying language, which can be facilitated by a number of diverse interventions. The original

2007 Neurology

3756. Deterioration of naming nouns versus verbs in primary progressive aphasia. (Abstract)

Deterioration of naming nouns versus verbs in primary progressive aphasia. Disproportionate impairment of naming nouns versus verbs and the opposite pattern have been reported in cases of focal brain damage or degenerative disease, indicating that processing of nouns and verbs may rely on different brain regions. However, it has not been clear whether it is the spoken word forms or the meanings (or both) of nouns and verbs that depend on separate neural regions. We tested oral and written (...) naming of nouns and verbs, matched in difficulty, in patients with nonfluent primary progressive aphasia (nonfluent PPA; n = 15), fluent primary progressive aphasia (fluent PPA; n = 7), and amyotrophic lateral sclerosis with frontotemporal dementia (ALS-FTD; n = 6). Patients with nonfluent PPA and ALS-FTD, both individually and as groups, were significantly more impaired on verb naming than on noun naming and significantly more impaired on oral naming than written naming. Patients with fluent PPA

2004 Annals of Neurology

3757. Cognition and anatomy in three variants of primary progressive aphasia. Full Text available with Trip Pro

Cognition and anatomy in three variants of primary progressive aphasia. We performed a comprehensive cognitive, neuroimaging, and genetic study of 31 patients with primary progressive aphasia (PPA), a decline in language functions that remains isolated for at least 2 years. Detailed speech and language evaluation was used to identify three different clinical variants: nonfluent progressive aphasia (NFPA; n = 11), semantic dementia (SD; n = 10), and a third variant termed logopenic progressive (...) aphasia (LPA; n = 10). Voxel-based morphometry (VBM) on MRIs showed that, when all 31 PPA patients were analyzed together, the left perisylvian region and the anterior temporal lobes were atrophied. However, when each clinical variant was considered separately, distinctive patterns emerged: (1) NFPA, characterized by apraxia of speech and deficits in processing complex syntax, was associated with left inferior frontal and insular atrophy; (2) SD, characterized by fluent speech and semantic memory

2004 Annals of Neurology

3758. Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? Full Text available with Trip Pro

Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? Considerable controversy exists regarding the relationship between semantic dementia (SD) and progressive aphasia. SD patients present with anomia and impaired word comprehension. The widely used consensus criteria also include the need for patients to exhibit associative agnosia and/or prosopagnosia: many authors have used the label SD for patients with non-verbal, as well as verbal, semantic deficits (...) on formal testing even if they recognize the objects and people encountered in everyday life; others interpret the criterion of agnosia to require pervasive recognition impairments affecting daily life. According to this latter view, SD patients have pathology that disrupts both a bilateral ventrotemporal-fusiform network (resulting in agnosia) and the left hemisphere language network (resulting in profound aphasia). These authors suggest that this profile is different to that seen in the fluent form

2006 Brain

3759. Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia. Full Text available with Trip Pro

Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia. Frontotemporal lobar degeneration (FTLD) refers to a focal, non-Alzheimer form of cerebral degeneration that encompasses the distinct clinical syndromes of frontotemporal dementia (FTD), progressive non-fluent aphasia (PNFA) and semantic dementia. Some patients show tau-based pathological changes and in familial cases mutations have been identified in the microtubule-associated protein tau

2006 Brain

3760. Proxy and self-report agreement on the Stroke and Aphasia Quality of Life Scale-39. Full Text available with Trip Pro

Proxy and self-report agreement on the Stroke and Aphasia Quality of Life Scale-39. Health related quality of life outcomes are increasingly used to measure the effectiveness of stroke interventions. People with severe aphasia after stroke may be unable to self-report on such measures, necessitating the use of proxy respondents. We explored the level of agreement between people with aphasia (PWA) and their proxies on the Stroke and Aphasia Quality of Life Scale (SAQOL-39) and whether (...) this agreement is influenced by demographic variables and proxy levels of depression and carer strain.People with chronic aphasia (> or = 6 months post stroke) were recruited through the UK national charity for PWA. They were interviewed on the SAQOL-39 and their nominated proxies were interviewed on the SAQOL-39, the General Health Questionnaire and the Caregiver Strain Index. Proxy respondents had to be > or = 18 years of age, see the person with aphasia at least twice a week and have no known severe

2007 Neurosurgery and Psychiatry

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