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PubMed1994

Corticonigral degeneration with neuronal achromasia presenting with primary progressive aphasia: ultrastructural and immunocytochemical studies.

We describe a clinico-pathological variant of a degenerative disorder involving Broca's, Wernicke's, and supplementary motor areas, which presented as primary progressive aphasia, dysarthria, bucco-facial apraxia, and hearing loss as initial symptoms, followed by organic personality changes. Postmortem examination revealed severe focal atrophy of the cerebral convolutions in the frontal operculum, superior frontal gyrus, and superior and transverse temporal gyri in addition to diffuse atrophy of the frontal and temporal lobes in both hemispheres. Microscopical examination revealed argyrophilic neuronal inclusions (ANIs) in the neuronal perikarya and presynaptic terminal throughout the central nervous system, as well as neuronal loss and swollen chromatolytic neurons in the affected cortices. Neocortical ANIs showed a positive immunoreaction with an anti-tau antibody but only a weak reaction with an anti-ubiquitin antibody immunohistochemically. Ultrastructurally, neocortical ANIs consisted of 15-nm thick smooth-surfaced tubules and tubules with constrictions at 120-150-nm intervals; thus they were different from the typical paired helical filaments of the 80-nm interval constrictions observed in the subiculum. ANIs were also found in the basal ganglia, brain stem nuclei, and cervical cord. Accordingly, ANIs appear distinct from neurofibrillary tangles (NFTs) of progressive supranuclear palsy, NFTs of Alzheimer-type dementia, and Pick bodies. The authors consider that this case fits the histopathological criteria of corticonigral degeneration with neuronal achromasia except for the unusual extension to the temporal lobes.

Aphasia↗
PubMed2005

Category and letter fluency in semantic dementia, primary progressive aphasia, and Alzheimer's disease.

This study examined the impact of various degenerative dementias on access to semantic knowledge and the status of semantic representations. Patients with semantic dementia, primary progressive aphasia, and Alzheimer's disease were compared with elderly controls on tasks of category and letter fluency, with number of words generated, mean lexical frequency and errors recorded. The findings are consistent with the view that category and letter fluency rely on both common and unique cognitive processes. Fluency tasks, with the richness of data obtained, are valuable in distinguishing different dementia syndromes from one another.

Age of Onset↗
PubMedDate not supplied

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.

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PubMed2000

Primary progressive aphasia: a patient with stress assignment impairment in reading aloud.

Surface dyslexia is a pattern of reading impairment which has been seldom described in Italian native speakers. We report the case of a female Italian patient, RM, suffering from primary progressive aphasia (PPA) of the fluent type, who presented stress assignment errors in reading aloud. In Italian these errors are considered to be strongly suggestive of surface dyslexia. We studied RM's reading performance in light of existing cognitive models on reading. Since the first assessment, she presented multi-level impairment involving pre-semantic, lexical-semantic and post-semantic stages. Her stress assignment errors have been interpreted as a generalisation of the most frequent tendency in Italian language: namely to assign stress to the penultimate syllable. In agreement with previous studies, our case suggests that surface dyslexia in PPA is not a monolithic entity but, on the contrary, that it may arise from impairment at various stages of the reading process.

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PubMed1994

Focal abnormalities of P3 ERPs unveiled in patients with cortical lesions and primary progressive aphasia by average reference recordings.

Event related potentials (ERPs) to an auditory odd-ball paradigm were recorded with the linked earlobe reference (LER) and with a computer calculated average reference (AR), excluding the two linked earlobe derivations. The study was performed in 30 patients with lesions of frontal, parietal, occipital cortex, unilateral and bilateral lesions of the medial temporal lobe (MTL), in four patients affected by Primary Progressive Aphasia (PPA) and in 56 age matched controls. Latencies, amplitudes and scalp distribution of the earlier ERP components (P1, N1, P2, N2) were within normal limits for both LER and AR recordings. P3 scalp distribution in patients was normal when LER was used, with the exception of two patients affected by bilateral MTL lesions. When P3 was recorded using AR, the scalp distribution was statistically different from normal distributions in all patients. A negativity, instead of the positive P3 observed in controls, was recorded in patients from leads corresponding to the affected areas. This finding might have clinical applications, and confirms earlier studies suggesting that P3 is generated simultaneously from different cortical areas.

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PubMed2001

Corticobasal degeneration presenting with nonfluent primary progressive aphasia: a clinicopathological study.

A 62-year-old woman initially presented with slowly progressive nonfluent aphasia with minimal intellectual involvement. Echolalia and personality change were prominent whereas parkinsonian features and signs suggesting parietal lobe dysfunctions were not present. The patient's language deficit was consistent with transcortical motor aphasia. She did not manifest extrapyramidal signs. The patient was diagnosed as having Pick's disease or frontal lobe dementia. She died at age 65, 2 years and 9 months following disease onset. Neuropathological findings including cytoskeletal abnormalities, however, were clearly distinct from those of classical Pick's disease and were consistent with those reported in corticobasal degeneration (CBD). The distribution of her cortical lesions was accentuated in the frontal language-related area. The clinical manifestations in CBD are diverse, and primary progressive nonfluent aphasia should be considered as an initial symptom of CBD. Neuropathological examination of such patients should include cytoskeletal abnormality studies.

Aphasia, Primary Progressive↗
PubMed1991

Primary progressive aphasia with focal neuronal achromasia.

We describe the clinical, radiologic, neuropsychological, and neuropathologic features of a 69-year-old man with a 3-year history of progressive transcortical expressive aphasia. Neuropsychological testing showed progressive dysfunction of expressive language. Neuropathologic examination demonstrated focal cortical degeneration involving the left superior frontal gyrus, with swollen achromasic neurons and no evidence of Alzheimer's disease, Pick's disease, Creutzfeldt-Jakob disease, Lewybody disease, or other dementing disorders. This case adds to the known heterogeneity of the underlying pathology of patients with primary progressive aphasia.

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PubMed1994

Swallowing difficulty in primary progressive aphasia: a case report.

A 71-year-old right handed man showed insidious and progressive decline of language skills, in contrast with relatively preserved performance on tests and ability to be independent in daily living. Three years later, he developed swallowing difficulties. MRI scans showed widening of the left sylvian fissure. A SPECT scan demonstrated focal hypometabolism restricted to the left temporal lobe. Videofluoroesophagography revealed marked hesitation of oral preparation and oral phases, while the reflex phases were unimpaired. The patient failed to perform oral tasks on command and imitation without motor and sensory deficit. We believe that his swallowing difficulty was due to oral apraxia. This case adds to the heterogeneity of patients with primary progressive aphasia.

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PubMed1999

Late acquired words in childhood are lost earlier in primary progressive aphasia.

The psycholinguistic nature of the dissolution of the mental lexicon in a primary progressive aphasic patient was investigated in light of the general regression hypothesis that language dissolution is the inverse of language acquisition. Four years after onset of the symptoms, the patient scored 60% correct on a picture naming test, exhibiting some effects of lexical and sublexical age-of-acquisition, but 7 years after onset, he scored only 15% correct. While even a weak form of the regression hypothesis is not fully supported, age-of-acquisition effects seem to be preserved throughout the course of the lexical dissolution. Some implications are briefly discussed for future research.

Age Factors↗
PubMed1998

Primary progressive semantic aphasia: a case study.

A longitudinal case study of a patient with a progressive loss of meaning of objects with preserved phonology and syntax is presented. Repeated measures of language, praxis, visual cognition, and semantic processing were carried out. The patient still has preserved conversational speech, social skills, and orientation in her 8th year of her illness, but shows severe anomia and comprehension deficit in all modalities of stimulus presentation. In addition to standardized tests of language, cognition, and memory, specific experiments of categorization, modalities of word access, item consistency, category specificity, and definition of words were carried out. Results indicate a frequency dependent loss of meaning that was consistent in all modalities and throughout all object categories. However, the relative preservation of visual categorization of all categories tested and the language based categorization of animals suggested some fractionation of semantic memory. Relative preservation of autobiographical and personal memories versus semantic memory was a striking observation. Evidence for selective impairment of central semantic processing was obtained from experiments indicating item consistency of loss and the lack of semantic cuing. Neuroimaging evidence of left temporal lobe atrophy and the classical picture is compatible with similar cases published under the term semantic dementia or "transcortical sensory aphasia with visual agnosia" and suggest the diagnosis of Pick's disease.

Anomia↗
PubMed2002

Progressive aphasic syndromes: clinical and theoretical advances.

PURPOSE OF REVIEW: Knowledge of the neural basis for language and related aspects of cognition has been advanced through detailed studies of patients with primary progressive aphasia. This brief review highlights some recent work. RECENT FINDINGS: The impairment of semantic knowledge in patients with semantic dementia appears to influence performance in a wide variety of linguistic and cognitive domains, including morphological agreements such as the irregular past tense. Computational studies modeling the deficits of these patients have advanced interpretations of the impairments in semantic dementia. Imaging analyses have confirmed the presence of temporal atrophy cross-sectionally and longitudinally in these patients. In patients with semantic dementia, it appears that both the left temporal and right temporal regions contribute in different proportions to naming and comprehension, although the nature of the process underlying the consolidation of knowledge in semantic memory continues to be actively debated. In patients with progressive non-fluent aphasia, recent work has emphasized an impairment with verbs. Functional neuroimaging work with progressive non-fluent aphasics, compared directly to non-aphasic patients with frontotemporal dementia, has demonstrated a dissociation for grammatical and working memory aspects of sentence processing within the left frontal cortex. SUMMARY: These findings will improve diagnostic accuracy, prognostic ability, and therapeutic potential in patients with progressive aphasia.

Aphasia, Primary Progressive↗
PubMed2006

Corticobasal degeneration and progressive aphasia.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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 aphasia in motor-onset CBDS. CONCLUSION: Aphasia is common in the corticobasal degeneration syndrome but there is little correlation with the laterality of clinical deficits. Cognitive-onset corticobasal degeneration syndrome and primary progressive aphasia are similar such that their aphasia appears identical.

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