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PubMed1997

[A case of corticobasal degeneration presenting with primary progressive aphasia].

We report a 64-year-old right-handed woman whose initial symptom was slowly progressive aphasia without generalized dementia and who was subsequently diagnosed as having corticobasal degeneration (CBD). Neurological examination revealed disturbed vertical gaze, dysarthria, rigidity of the right upper extremity, and bilateral instinctive grasp reaction. Neuropsychological assessment disclosed Broca's aphasia, buccofacial apraxia, and memory disturbance. MRI of the brain showed atrophy of the frontotemporal lobes, which was more severe on the left than on the right, especially the left inferior frontal gyrus. In most reported cases of CBD, the initial symptom is motor dysfunction of the unilateral upper or lower extremity. However, we should be cautious that among cases with CBD, there have been rare cases that begin with progressive aphasia alone. In our case, the atrophied region of the cerebral cortex was most severe around the left inferior frontal gyrus. In a few reported cases with the initial symptom of aphasia, the atrophied region corresponded considerably to the type of the aphasia. On the other hand, in those whose initial symptom was mainly motor dysfunction of the unilateral extremity, the atrophied region was remarkable in the posterior part of the frontal lobe and parietal lobe. Therefore, we suggest that in CBD the distribution of the cerebral cortical lesions differs in accordance with whether the initial symptom is motor disturbance or aphasia, and that the type of aphasia corresponds to the location of the cortical lesion.

Aphasia, Broca↗
PubMed2003

Primary progressive aphasia: diagnosis, varieties, evolution.

A referred cohort of 67 clinically defined PPA patients were compared to 99 AD patients with formal language and nonverbal cognitive tests in a case control design. Language fluency was determined at the first and last follow up visits. Quantitation of sulcal and ventricular atrophy on MRI was carried out in 46 PPA and 53 AD patients. Most PPA patients (57%) are relatively fluent when first examined. Visuospatial and memory functions are initially preserved. Aphemic, stuttering, "pure motor" presentation, or agrammatic aphasia are seen less frequently. Later most PPAs become logopenic and nonfluent, even those with semantic aphasia (dementia). In contrast, AD patients were more fluent and had relatively lower comprehension, but better overall language performance. MRI showed significant left sided atrophy in most PPA patients. Subsequent to PPA, 25 patients developed behavioral manifestations of frontotemporal dementia and 15 the corticobasal degeneration syndrome, indicating the substantial clinical overlap of these conditions. Language testing, particularly fluency scores supported by neuroimaging are helpful differentiating PPA from AD. The fluent-nonfluent dichotomy in PPA is mostly stage related. The aphemic-logopenic-agrammatic and semantic distinction is useful, but the outcomes converge.

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PubMed2002

Modality-specific deterioration in naming verbs in nonfluent primary progressive aphasia.

A longitudinal study of oral and written naming and comprehension of nouns and verbs in an individual (M. M. L.) with nonfluent primary progressive aphasia (PPA) is reported. M. M. L. showed progressive deterioration of oral naming of verbs well before deterioration of written naming of verbs and before deterioration of oral or written naming of nouns. Her comprehension of both nouns and verbs remained intact, at least relative to oral naming of verbs. Her performance is compared to that of two other individuals with nonfluent PPA, who were tested at two time points. These patients showed similar patterns with respect to grammatical word class (verbs more impaired than nouns) and modality (spoken production more impaired than written production), but somewhat different courses of deterioration. The modality-specific nature of the observed verb production deficits rules out a semantic locus for the grammatical class effects. The results provide a new source of evidence for the hypothesis that there are distinct neural mechanisms for accessing lexical representations of nouns and verbs in language production.

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PubMedDate not supplied

Primary progressive aphasia as the initial manifestation of corticobasal degeneration. A "three in one " syndrome?

In 1994, the term "Pick complex" was proposed to indicate significant clinical and pathological overlapping between primary progressive aphasia, frontal lobe dementia and corticobasal degeneration. We report the case of a 60-year-old man, who initially presented progressive non-fluent aphasia with orofacial apraxia, and subsequently, over a period of 3 years, developed mutism, pathological laughter, extrapyramidal rigidity, dystonia, alien hand syndrome and bulbar signs. An extensive haematological, immunological and biochemical work up was normal. The results of neuroimaging studies and neuropsychological tests, along with the clinical evolution, finally led us to the ?three in one? diagnosis, supporting the concept of Pick complex.

Aphasia, Primary Progressive↗
PubMed2006

Primary progressive aphasia in a bilingual woman.

Multilingual aphasias are common because most people in the world know more than one language, but little is known of these syndromes except in patients who have had a stroke. We present a 76-year-old right-handed woman, fluent in English and Chinese, who developed anomia at age 70 and then progressed to aphasia. Functional neuroimaging disclosed mild left temporoparietal hypometabolism. Neurolinguistic testing was performed in both English and Chinese, representing a unique contribution to the literature. Results revealed conduction-like aphasia that was comparable in the two languages, although English was slightly better preserved. Primary progressive aphasia has disrupted 2 languages in a similar manner, suggesting their close neuroanatomic relationship in this case.

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PubMed2006

A case study of Primary Progressive Aphasia: improvement on verbs after rTMS treatment.

This case-report shows that high frequency repetitive Transcranial Magnetic Stimulation (hf-rTMS), applied to the left prefrontal cortex, may improve the linguistic skills in Primary Progressive Aphasia (PPA). The patient's performance was evaluated on a battery of language production and memory span tasks, before and after two hf-rTMS treatments and one SHAM treatment. We observed a significant and lasting improvement of the patient's performance on verb production following the application of hf-rTMS versus Baseline and SHAM conditions. This finding suggests that hf-rTMS may directly strengthen the neural connections within an area of metabolic dysfunction and encourages the use of rTMS as an alternative therapeutic tool for neurodegenerative forms of aphasia.

Aphasia, Primary Progressive↗
PubMed2006

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 of primary progressive aphasia (fPPA), a neurodegenerative disease primarily affecting language function. We present data on seven patients who met the diagnostic criteria for fPPA. All seven showed deficits relative to matched controls on both verbal and non-verbal measures of semantic memory, and these deficits were modulated by degree of anomia, concept familiarity and item typicality. Voxel-based morphometry revealed reduced grey matter density in the temporal lobes bilaterally (more widespread on the left), with the severity of atrophy in the left inferior temporal lobe being significantly related to performance on both the verbal and non-verbal measures. Together these findings suggest that patients who meet the diagnostic criteria for fPPA, can also meet the diagnostic criteria for early-stage SD provided that the impact of concept familiarity and typicality is taken into account. In addition, these findings support a claim that the patients' deficits on both verbal and non-verbal tasks reflect progressive deterioration of an amodal integrative semantic memory system critically involving the rostral temporal lobes, rather than a combination of atrophy in the left language network and a separate bilateral ventrotemporal-fusiform network.

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PubMed1997

Primary progressive aphasia, left anterior atrophy, and neurofibrillary hippocampal pathology: observations in an unusual case.

A 71-year-old, right-handed woman experienced onset of a slowly progressive, nonfluent language disorder. She maintained normal cognitive abilities until age 80 and developed a mild spastic right hemiparesis the following year. By age 82, she had become severely demented, mute, and akinetic. Postmortem brain examination showed moderate asymmetric atrophy (wt = 1000 g), which was most prominent in the left perisylvian and posterior frontal regions, with disproportionate enlargement of the left lateral ventricle. Microscopically, a high density of neurofibrillary tangles was present in the hippocampal pyramidal cell layer and entorhinal cortex (up to 45/400 x field, R > L). Gliosis and patchy neuronal loss were symmetrically present in the frontal and parietal lobes including speech areas, but neurofibrillary tangles and neuritic plaques were present in only small numbers (0-1/400 x field). In this case of primary progressive aphasia, pathologic changes associated with Alzheimer's disease occurred in allocortex but not in neocortex. These clinical and neuropathologic findings expand on those previously reported in primary progressive aphasia and suggest a need for further study of this syndrome.

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PubMed1996

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↗
PubMed2005

Anterior temporal laterality in primary progressive aphasia shifts to the right.

In aphasia due to stroke, language-related activity shifts not only to undamaged cortex within the dominant hemisphere but also toward right-sided areas homotopical to the left-sided lesion. We examined whether a rightward shift takes place in primary progressive aphasia (PPA). Nineteen PPA patients participated, 19 healthy subjects and 14 patients with amnestic mild cognitive impairment who served as controls. Subjects underwent neuropsychological assessment, structural magnetic resonance imaging (MRI), and a functional MRI with a factorial design: words versus pictures and associative-semantic versus visuoperceptual task. Measures of neuropsychological performance were entered as regressors into a multiple linear regression analysis, with response amplitude during the associative-semantic versus control conditions as outcome variable. Language competence correlated negatively with responses in the right anterior temporal cortex and positively with volume and responses in the left-sided homotope. In normal subjects, anterior temporal activation was more extensive to the left than the right (laterality index [LI], +0.64; standard error [SE], 0.11). Laterality was inverted in PPA with word comprehension deficit (LI, - 0.34; SE, 0.19), with an intermediate pattern in PPA without comprehension deficit (LI, +0.23; SE, 0.14). The rightward laterality shift previously reported in aphasic stroke extends to PPA, in particular, when comprehension is deficient.

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PubMed2005

Fluent versus nonfluent primary progressive aphasia: a comparison of clinical and functional neuroimaging features.

To better characterize fluent and nonfluent variants of primary progressive aphasia (PPA). Although investigators have recognized both fluent and nonfluent patients with PPA, the clinical and neuroimaging features of these variants have not been fully defined. We present clinical and neuropsychological data on 47 PPA patients comparing the fluent (n=21) and nonfluent (n=26) subjects. We further compared language features with PET/SPECT data available on 39 of these patients. Compared to the nonfluent PPA patients, those with fluent PPA had greater impairment of confrontational naming and loss of single word comprehension. They also exhibited semantic paraphasic errors and loss of single word comprehension. Patients with nonfluent PPA were more likely to be female, were more often dysarthric, and exhibited phonological speech errors in the absence of semantic errors. No significant differences were seen with regard to left hemisphere abnormalities, suggesting that both variants result from mechanisms that overlap frontal, temporal, and parietal regions. Of the language measures, only semantic paraphasias were strongly localized, in this case to the left temporal lobe. Fluent and nonfluent forms of PPA are clinically distinguishable by letter fluency, single word comprehension, object naming, and types of paraphasic errors. Nevertheless, there is a large amount of overlap between dysfunctional anatomic regions associated with these syndromes.

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PubMed2005

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.

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PubMed2006

Primary progressive aphasia accompanied by environmental sound agnosia: a neuropsychological, MRI and PET study.

As part of the frontotemporal dementias, primary progressive aphasia (PPA) is typically characterized by nonfluent speech with paraphasias, but there is growing evidence that also a fluent variant of PPA exists. We describe a patient suffering from PPA who adds to the broad clinical spectrum of this disorder. Moreover, we report for the first time that PPA may be associated with severe impairment in meaningful nonverbal sound recognition (environmental sound agnosia). These neuropsychological findings were found to be associated with distinct focal alterations in functional and structural neuroimaging.

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PubMed2004

A longitudinal study of behavior in frontotemporal dementia and primary progressive aphasia.

OBJECTIVE: To evaluate the construct validity of the Frontal Behavioral Inventory (FBI) and to describe the evolution of the behavioral abnormalities of the behavioral and aphasic presentations of frontotemporal dementia (FTD) by means of a 3-year longitudinal study. BACKGROUND: The FBI is a standardized behavioral questionnaire useful in the diagnosis and quantification of the personality and behavior disorder FTD. METHOD: Patients who had three consecutive yearly assessments with the FBI were selected, 12 with the behavioral variant of FTD (FTD-bv) and 14 with Primary Progressive Aphasia (PPA). RESULTS: FBI scores rose as the disease progressed in both the FTD-bv and PPA groups over the 3 years of testing. Initial mean FBI scores of the FTD-bv group were above the cutoff for FTD as established for this diagnosis with previous standardization. By the third year, the mean FBI score of PPA patients was also above the established cutoff for FTD. CONCLUSIONS: The outcome of the study demonstrates that the FBI is sensitive to changes in behavior and personality in both variants of FTD. The FBI can be used to describe the evolution of symptoms and the course of the illness of Pick complex patients who present initially with FTD-bv or who present with PPA and subsequently develop the behavioral disorder.

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PubMed1999

The neuropsychological signature of primary progressive aphasia.

An effect size analysis incorporating meta-analytic principles was used to review neuropsychological findings in patients with primary progressive aphasia (PPA). Studies dating back to 1982 were gathered and the neuropsychological test results from a total of 55 patients with PPA and 162 healthy controls were synthesized using effect size analyses. The results indicate that patients with PPA are most deficient on tests of verbal skill, followed by performance on measures of delayed recall, cognitive flexibility and abstraction, memory acquisition, attention/concentration, and, finally, performance skill. A rank-order list of specific neuropsychological tasks and test variables in order of sensitivity to PPA is also provided to aid in the interpretation of the quantitative results and in the differentiation of PPA from other disorders with prominent features of dementia such as Alzheimer's disease.

Aphasia↗
PubMed1996

Primary progressive aphasia: description of a clinical case with nine years of follow-up.

We describe the case of a woman with primary progressive aphasia who, over a period of nine years, has shown no signs of clinical deterioration and has only a symptomatic language disturbance. Neuropsychological follow-up has revealed progressive language impairment, with the integrity of praxis, visuoperceptive gnosia and short term visuospatial memory remaining intact; the only impairment was that revealed by long-term memory tests.

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PubMedDate not supplied

Distinct neurolinguistic symptom clusters in Alzheimer's-type dementia and primary progressive aphasia.

Mesulam's (1982) report describing six patients with a slowly progressive aphasia without accompanying signs of dementia led to the recognition of a syndrome now known as Primary Progressive Aphasia (PPA). Many more patients have been described since Mesulam's description was published (see Westbury & Bub, 1997, for a review). However, the published literature is both unsystematic and incomplete, making it difficult to place the findings into a coherent theoretical framework. In addition, little previous work (see Mesulam, 1987) has specifically attempted to specify the difference between PPA and dementia of Alzheimer's type (DAT), although the two disorders are easily confused since many language deficits can masquerade on early presentation as memory or cognitive deficits. In this paper, the linguistic deficits of 11 PPA patients are analyzed, and contrasted with the linguistic deficits of a group of 11 DAT patients. Patients in both groups were tested using an extensive battery of language tests, the Psycholinguistic Assessment of Language Battery (Caplan & Bub, 1990; Caplan, 1992). We consider seven linguistic symptom clusters that differentiate the two groups.

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