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At least 19 recordsLinked to original sources
PubMed1997

Imaging in primary progressive aphasia.

Primary progressive aphasia (PPA) presents with aphasia, with or without other minor cognitive dysfunction. We report five patients with PPA to show the correlation between their clinical signs and imaging findings. The patients can be divided into those with nonfluent (group 1) and those with fluent (group 2) aphasia. The characteristic speech impairment was bradylalia in group 1 and word amnesia in group 2. Impairment of comprehension was common but mild in both groups. On MRI, patients in group 1 showed predominantly left frontal and perisylvian atrophy with reduced uptake in the same region on single photon emission computed tomography (SPECT) using technetium-99m hexamethyl propyleneamine oxime (99mTc HMPAO). Patients in group 2 showed left temporal atrophy involving the superior, middle and inferior temporal gyri, hippocampus and parahippocampal gyrus on MRI and reduced uptake in the same region on SPECT. These findings correlated well with the functional anatomy of speech impairment.

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PubMed2006

Does this patient have primary progressive aphasia?

Primary progressive aphasia (PPA) is a distinct clinical entity in which the patient develops language deficits while other cognitive domains remain relatively preserved until late in the course of the illness. The diagnosis can be relatively clear through an appropriate diagnostic approach based on the history and physical examination. There is no cure, but speech therapy is beneficial in this illness.

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PubMed2004

[Primary progressive aphasia].

Primary progressive aphasia is a rare disease characterised by slow deterioration of language, which remains the leading symptom whereas other cognitive functions such as memory, orientation, judgement, and visual-spatial skills are relatively spared and mostly not affected until 5 to 7 years after disease onset. Structural imaging displays atrophy of the left temporal region which can also affect the frontal region or both hemispheres. Reduced neuronal activity in the left temporal lobe can be seen using functional imaging even before structural anomalies are detected. Neuropathological examination reveals neuronal cell loss and gliosis in the regions of atrophy which are sometimes accompanied by spongiform changes. T-positive neurons or plaques and neurofibrillary tangles are present in rare cases. A specific therapy is not known. Logopaedic therapy focussed on the main symptoms seems to stabilise linguistic abilities.

Aphasia, Primary Progressive↗
PubMedDate not supplied

Familial primary progressive aphasia.

Primary progressive aphasia (PPA) is a neurodegenerative disease presenting with isolated, progressive, language dysfunction. After at least 2 years, dementia may develop, but the aphasia predominates. Few families with hereditary PPA have been reported; some have autosomal dominance. A chromosome 17 mutation in tau exon 13 has been found in one family, and a few have linkage to chromosome 17. However, early appearance of prominent memory, behavior, and motor impairments differentiates these patients from typical PPA. The objective was to report clinical features, pathology, and genetic analysis of a family with typical PPA. We report three siblings with the typical clinical syndrome of PPA. Each presented with word-finding difficulties and early anomia. Ages at onset were 60, 61, and 65 years. Aphasia was the only symptom for at least 2 years. A nonaffected brother is 75 years of age. Family history review found no other affected relatives. Neuropathology in one patient demonstrated "dementia lacking distinctive histopathology" with ubiquitin-positive cortical neurons. DNA analysis of the proband did not detect any known mutation in tau exons 1-5, 7, or 9-13. To our knowledge, this is the first family presenting with hereditary aphasia in which typical PPA occurs in all affected members.

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PubMed2001

Primary progressive aphasia.

Primary progressive aphasia (PPA) is a focal dementia characterized by an isolated and gradual dissolution of language function. The disease starts with word-finding disturbances (anomia) and frequently proceeds to impair the grammatical structure (syntax) and comprehension (semantics) of language. The speech output in PPA can be fluent or nonfluent. Memory, visual processing, and personality remain relatively well-preserved until the advanced stages and help to distiguish PPA from frontal lobe dementia and the typical forms of Alzheimer's disease. The term "semantic dementia" was originally introduced to designate a different group of patients with a combination of verbal and visual processing deficits. In practice, however, this diagnosis is also being used in a variant sense to denote a subtype of PPA with fluent speech and impaired comprehension, even in the absence of visual processing deficits. Insofar as the diagnosis of semantic dementia can have these two different meanings, it is important to specify whether it is being used in the original sense or to denote a subtype of PPA. Structural and physiological neuroimaging confirms the selective predilection of PPA for the left hemisphere, especially for its language-related cortices. A few patients with PPA display the neuropathological markers of Alzheimer's disease, but in an unusual distribution. The majority of the autopsies in PPA have shown either Pick's disease or lobar atrophy without distinctive histopathology. The suggestion has been made that PPA and frontal lobe dementia constitute phenotypical variations of a unitary disease process within the "Pick-lobar atrophy" spectrum. Recent advances in chromosome 17-linked dementias justify a rigorous search for tau polymorphisms and tauopathy in sporadic PPA. An informed approach to this syndrome will increase the effectiveness with which clinicians can address the unique challenges associated with the diagnosis and care of PPA.

Aphasia, Primary Progressive↗
PubMed2002

Prominent hypometabolism of the right temporoparietal and frontal cortex in two left-handed patients with primary progressive aphasia.

Primary progressive aphasia (PPA) is characterized by progressive deterioration of language function with relative preservation of other cognitive functions. Previous studies based on neuroimaging and histology point to predominantly left temporal pathology in PPA patients. Here we report two left-handed subjects with typical symptoms of the nonfluent form of PPA in whom 18F-FDG PET revealed an asymmetric right-hemispheric pattern of reduced glucose metabolism in the frontal and temporoparietal cortex. These findings support the hypothesis that PPA can be considered as a symptom complex rather than a disease entity.

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PubMed1997

Primary progressive aphasia.

Primary progressive aphasia is an important recently emphasized clinical syndrome that is a common early manifestation of Pick's disease and Pick complex pathology. It is defined clinically as slowly progressive language disturbance that remains relatively isolated from other cognitive or behavioral deficits for at least 2 years. Subsequently, it may become associated with behavioral changes similar to those in frontal lobe dementia (FLD), extrapyramidal manifestations, and apraxia similar to corticobasal degeneration (CBD) and with motor neuron disease. Both the common nonfluent or logopenic variety leading to mutism (frontal involvement) and fluent or semantic varieties (temporal involvement) are recognized. Neuroimaging with CT, MRI, and SPECT are useful adjuncts confirming the diagnosis. PPA is a manifestation of frontotemporal degeneration or Pick complex, which is probably the second most common degenerative dementia after AD.

Aphasia, Primary Progressive↗
PubMed2002

Abeta peptide 1-42, Tau protein and S-100B protein level in cerebrospinal fluid of three patients with primary progressive aphasia.

Primary progressive aphasia (PPA) is a clinical syndrome characterized by a slowly progressive aphasia in the absence of accompanying signs of generalized dementia. While non-fluent PPA tends to progress frontally and is usually linked to frontotemporal degeneration, fluent PPA might be associated with both, frontotemporal degeneration or Alzheimer's disease. Although recent reports suggest that PPA belongs neuropathologically to the group of tauopathias, cerebrospinal fluid analysis has not been established as a means of diagnosis in PPA so far. In this paper we investigated Abeta peptide(1-42) (Abeta(1-42)), Tau protein and S-100B protein level in the cerebrospinal fluid of three patients with PPA. In all patients Tau protein and S-100B level were slightly elevated, however, Abeta(1-42) was found to be in normal range. Thus, our first results point to PPA being neurochemically linked to frontotemporal degeneration.

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PubMed2003

Axonal injury within language network in primary progressive aphasia.

Primary progressive aphasia (PPA) is characterized by an isolated progressive impairment of word use and comprehension reflecting the distribution of pathological processes within the left hemisphere. We used proton magnetic resonance spectroscopy (1H-MRS) to study in vivo the integrity of axonal fibers connecting perisylvian language areas in 11 patients with PPA, 11 subjects with Alzheimer's disease, and 22 controls. Brain metabolites (N-acetylaspartate, myoinositol, choline, creatine) were measured bilaterally within a volume of interest located in the central portion of the superior longitudinal fasciculus, a long associative bundle connecting Broca's area with Wernicke's area, and other language regions of the temporal lobe. In the PPA group, there was an asymmetrical N-acetylaspartate to creatine ratio reduction compared with Alzheimer's disease and controls, with greater changes on the left side. The myoinositol to creatine ratio was increased in the PPA group bilaterally compared with controls. The choline to creatine ratio did not differ among the three groups. These results indicate an asymmetrical focal axonal injury within the language network in PPA. The marked difference in the distribution of N-acetylaspartate to creatine between PPA and Alzheimer's disease suggests that proton magnetic resonance spectroscopy may help to differentiate between these two conditions.

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PubMed2003

Patterns of limb apraxia in primary progressive aphasia.

Primary progressive aphasia (PPA) is a syndrome characterized by a progressive language deficit without other dementia features for at least two years (). Other deficits that are likely to co-exist with aphasia, such as apraxia, have only been investigated in a few case studies and only at a rudimentary level for the most part. In this study we investigate the frequency and severity of apraxic deficits in PPA patients. Ten PPA patients and twelve aged-matched healthy adults performed eight transitive gestures to pantomime and to imitation. Gesture performance was measured along five movement dimensions and a composite score based on the arithmetic mean of the five dimensions was calculated. Overall, PPA patients performed worse than controls with both pantomime and imitation. Furthermore, individual comparisons revealed that out of the three apraxia patterns described by (pantomime alone, imitation alone, or apraxia in both conditions), the most frequent pattern in PPA patients was apraxia in both conditions. This result corresponds with previous findings in populations of stroke and Alzheimer's patients. Considering the occurrence of apraxia in this population, this study supports the idea that a comprehensive apraxia assessment should be administered in cases of PPA.

Aphasia↗
PubMed2004

Left hemicranial hypoplasia in 2 patients with primary progressive aphasia.

BACKGROUND: Primary progressive aphasia (PPA) leads to a gradual and relatively isolated dissolution of language function. The factors that determine the selectivity of the disease process remain unknown. We had speculated that PPA may occasionally arise as a tardive manifestation of genetic or acquired vulnerabilities involving the language network of the brain. OBJECTIVE: To explore predisposing factors for PPA. RESULTS: In 2 patients, PPA developed with a background of mild left hemicranial hypoplasia. CONCLUSION: In keeping with other observations of PPA in patients with dyslexia and childhood injury to the left temporal lobe, these 2 patients support the contention that some cases of PPA may arise in settings where the language network has become a locus of least resistance.

Activities of Daily Living↗
PubMed2000

Frontotemporal decreases in rCBF correlate with degree of dysnomia in primary progressive aphasia.

UNLABELLED: Primary progressive aphasia (PPA) is an uncommon degenerative dementia characterized by gradual impairment of language function with initial sparing of the memory domain. Using semiquantitative 99mTc-hexamethyl propyleneamine oxime (HMPAO) brain SPECT as a measure of regional cerebral blood flow (rCBF), we investigated the relationship between reduced 99mTc-HMPAO uptake and the severity of dysnomia in PPA. METHODS: Seven right-handed patients with PPA had their dysnomia assessed by the Boston Naming Test (BNT), a subtest of the Boston Diagnostic Aphasia Examination. Neuroimaging studies, including 99mTc-HMPAO brain SPECT, CT, and MRI, were performed. Correlational analysis between reduced rCBF and BNT was performed. RESULTS: Brain SPECT showed a reduction in 99mTc-HMPAO uptake involving the frontal and temporal lobes in all 7 patients. CT and MRI showed mild to moderate cerebral atrophy in 4 patients. Low scores on the BNT correlated with low frontotemporal 99mTc-HMPAO (Spearman r = 0.97, P = 0.004) in the 5 patients with left-hemisphere involvement. CONCLUSION: Decreased rCBF to the frontotemporal region characterized the cerebral abnormalities associated with PPA. The finding of focal rCBF abnormalities in the right hemisphere of 2 right-handed women corroborates that PPA symptoms may arise from a "non-left-dominant"-hemisphere degenerative process. Our results support the usefulness of rCBF SPECT imaging as a diagnostic aid in PPA.

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PubMedDate not supplied

[Primary progressive aphasia (Mesulam syndrome)].

Primary progressive aphasia is defined as a gradually appeared and gradually worsening disorder of speech without any major alteration of the other cognitive functions. It is regarded by some authors as a syndrome which may be due to various degenerative diseases of the cerebral cortex (notably Alzheimer's disease, owing to its frequency), while others see in it an autonomous disease related to a neuropathological process that is distinct from the main degenerative dementias. The principal clinical particularity of primary progressive aphasia is that it spares the patient's autonomy for a long time, but ultimately turns into global dementia. Despite the diversity of aphasic aspects accompanied or not, in neuroimaging, by morphological and metabolic asymmetry to the expense of the left hemisphere, a review of the autopsy cases published shows that the vast majority corresponded to a neuropathological pattern devoid of the characteristic features of Alzheimer's disease and nearer to Pick's disease. In practice, the diagnosis of Alzheimer's disease can be excluded in all patients whose clinical presentation and cerebral neuroimaging results are compatible with primary progressive aphasia. Moreover, the fact that this new clinicoanatomical entity has been individualized constitutes a definite step towards a better comprehension of degenerative dementias.

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PubMed2006

[Primary progressive aphasia: clinical aspects].

Primary progressive aphasia (PPA), initially described by Mesulam, is a syndrome of progressive deterioration of language, occurring in the presenium. Several classifications have been proposed, but the most useful one distinguishes non fluent and fluent forms of PPA. Both begin by anomia. In non fluent PPA, there is a progressive reduction of language, sometimes with aggramatism and articulatory impairment, but without impairment of comprehension. Fluent PPA is characterized by preserved fluency with severe impairment of single word comprehension. It is frequently confounded with semantic dementia (SD), but some observations can be differentiated from SD. After several years, all patients become mute, and most of them develop dementia, usually of frontal lobe type. The syndrome of PPA is due to neurodegenerative brain pathology affecting mostly the perisylvian regions of the left hemisphere. In most cases, no Alzheimer type pathology was found in the brain, but tauopathy (mainly in non fluent PPA) or motor neuron disease type pathology - tau negative ubiquitin inclusions (mainly in fluent PPA). However, Alzheimer type pathology was found in a substantial number of cases.

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PubMed2005

A longitudinal study of sentence comprehension difficulty in primary progressive aphasia.

CONTEXT: Patients with primary progressive aphasia have sentence comprehension difficulty, but the longitudinal course of this deficit has not been investigated. OBJECTIVE: To determine how grammatical, single word meaning, and working memory factors contribute to longitudinal decline of sentence comprehension in primary progressive aphasia. We hypothesised partially distinct patterns of sentence comprehension difficulty in subgroups of patients with progressive non-fluent aphasia (PNFA) and semantic dementia (SD). DESIGN: Cohort. SETTING: Institutional out patient referral centre. PATIENTS: PNFA (n = 14), SD (n = 10). MAIN OUTCOME MEASURE: Sentence comprehension accuracy. RESULTS: PNFA patients were significantly impaired at understanding grammatically complex sentences when first seen, and this was more evident than impairment of their comprehension of grammatically simple sentences (p<0.05). Comprehension of grammatically complex sentences correlated with their working memory deficit at presentation (p<0.05). PNFA patients showed modest decline over time in grammatical comprehension. In SD, comprehension of grammatically complex sentences was not more impaired than comprehension of grammatically simple sentences when first seen, but these patients demonstrated a significant longitudinal decline in understanding grammatically complex sentences (p<0.05). Cox regression analyses showed that a deficit in single word meaning contributes to the progressive impairment for grammatically complex sentences in SD (p<0.05), but working memory does not contribute to longitudinal decline in PNFA. CONCLUSION: Patients with PNFA and SD have sentence comprehension difficulty, but distinct factors contribute to this impairment during the course of their disease.

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PubMed1999

Primary progressive aphasia : a case report.

Primary progressive aphasia is due to focal left perisylvian degeneration and manifests with progressive decline in language function for two or more years. There is preservation of cognitive functions and activities of daily living continue to be normal. We report a case of progressive aphasia in a 65 year old lady.

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PubMed2005

Primary progressive aphasia: a comparative study of progressive nonfluent aphasia and semantic dementia.

Primary progressive aphasia (PPA), a degenerative disorder, is often misdiagnosed as Alzheimer's disease. Its subtypes, semantic dementia (SD), and progressive nonfluent aphasia (PNFA), are often difficult to differentiate from each other. Our objective was to highlight the differences in the language profiles of patients with SD and PNFA. To bring out these differences, we report two patients with PPA, one with SD and the other with PNFA. They were administered the Western aphasia battery (WAB) and a semantic battery, which assesses semantic memory. The profiles of language impairment on the WAB indicated that the patient with PNFA had syntactic errors in expressive speech but relatively preserved semantics and comprehension, whereas the patient with SD had preserved syntax but made semantic errors in expressive speech, and had impaired comprehension. There were differences in their performance on the semantic battery too. The patient with SD made relatively less errors on confrontation naming, although on the pointing task he failed to point to those line drawings, which he was unable to name on confrontation. In contrast, the finding of the PNFA patient was the reverse of this. Supplementing conventional neuropsychological tests with formal tests for assessment of language functions is useful in the early diagnosis of PPA. The performance of PPA patients on a detailed assessment of language that includes use of formal tests such as the semantic battery helps to differentiate PNFA from SD.

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