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PubMed2003

Nothing to say, something to sing: primary progressive dynamic aphasia.

We describe a 76-year-old man (ADY) with dynamic aphasia in the setting of a degenerative frontal lobe dementia: primary progressive dynamic aphasia. He displayed a striking paucity of propositional speech despite intact speech production, and preserved singing and prosody. Vocal expression in the verbal and musical domains was investigated in a series of neuropsychological experiments based on novel language and musical tasks that were designed to establish the nature and specificity of the verbal output deficit. The features of the language disorder indicated that the speech output pathway was disrupted at the early stage of generation of a new pre-verbal message. In contrast, tests of musical output demonstrated that the generation of new musical ideas was unimpaired. The domain-specificity of dynamic aphasia may result from the disruption of specific cognitive processes necessary for the creation of verbal messages, as well as selective damage of brain regions involved in language production.

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PubMed1989

Creutzfeldt-Jakob disease presenting as isolated aphasia.

Progressive aphasia without dementia (primary progressive aphasia) is increasingly recognized as an important neurobehavioral syndrome. Clinical diagnosis of progressive aphasia is difficult early in its course, and the differential diagnosis is usually said to include Alzheimer's and Pick's diseases. We report a 61-year-old man with autopsy-proven Creutzfeldt-Jakob disease (CJD) whose major initial manifestation was a progressive, fluent aphasia. Myoclonus was absent, and characteristic EEG abnormalities appeared relatively late. We believe that this case of CJD is unique in its presentation of profound and isolated aphasia. CJD should be considered in the differential diagnosis of the progressive aphasia syndrome.

Aphasia↗

PubMed2000

Clinical concept of frontotemporal dementia.

The clinical concept of frontotemporal dementia is reviewed by discussing its relationships to several related concepts. These include dementia of the frontal lobe type, slowly progressive aphasia without dementia or primary progressive aphasia, semantic dementia and frontotemporal lobar degeneration. A number of examples of confusion in the terminology are also examined.

Aphasia, Primary Progressive↗

PubMed2006

Behavioral features in semantic dementia vs other forms of progressive aphasias.

OBJECTIVE: To compare the behavioral profiles in different variants of primary progressive aphasia (PPA). METHODS: 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 dementia (FTD) and Alzheimer disease (AD). RESULTS: SEMD was associated with significantly more socioemotional behavioral dysfunction than the other two variants of PPA and than AD, specifically more disinhibition, aberrant motor behavior, and eating disorders-behaviors that are typical of FTD. In contrast, PNFA and LPA did not differ from each other or from AD in the type or severity of behavioral dysfunction. Behavioral abnormalities increased in severity with disease duration in SEMD, but this association was not detected in PNFA or LPA. CONCLUSIONS: Semantic dementia is associated with significantly more behavioral dysfunction than other variants of primary progressive aphasia, specifically behavioral features typical of frontotemporal dementia.

Affective Symptoms↗

PubMed2001

[Aphasia and dementia].

Primary progressive aphasia (PPA) is an uncommon neurodegenerative syndrome characterized by a relatively isolated dissolution of language function at the beginning, followed by deterioration of general cognitive function and of activities of daily living after 2 or more years. On account of neuropathological and clinical findings, PPA is supposed to form part of the spectrum of frontotemporal lobar degeneration. We present a case study of a 66-year-old woman with a probable fluent progressive aphasia. She initially experienced word amnesia and developed after 2 - 3 years gradual regression of word comprehension, over-fluent speech with semantic paraphasias, and at last generalized dementia. In addition to minor bilateral cortical volume reduction on CCT, MRI showed left temporal lobe atrophy involving hippocampus, SPECT revealed reduced uptake left frontal and temporal.

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PubMed2006

Effects of alternative communication on the communicative effectiveness of an individual with a progressive language disorder.

This study was designed to investigate the effects of two different modes of communication on the communicative output of an individual who is no longer able to communicate verbally, presenting with a primary progressive aphasia and apraxia of speech. The two treatment approaches included training the patient with a text-to-speech alternative communication device and with American sign language. An alternating treatment design was used to compare two communicative approaches (an alternative communication device and American sign language) on the subject's communicative effectiveness. Communicative effectiveness was measured in terms of number of words, correct information units and percentage correct information units, using a protocol that was adapted to quantify the output generated by the alternative communication device and American sign language. Increases across all three measures resulted for both the alternative communication device and American sign language. The clinical implications are explored, and the results add to existing studies regarding treatment possibilities using alternative communication for individuals who present with a progressive speech and language disorder, without concomitant cognitive deficits.

Aphasia, Primary Progressive↗

PubMed2000

Automatic measurement of changes in brain volume on consecutive 3D MR images by segmentation propagation.

This article presents a technique to automatically measure changes in the volume of a structure of interest in successive 3D magnetic resonance (MR) images and its application in the study of the brain and lateral cerebral ventricles. The only manual step is a segmentation of the structure of interest in the first image. The analysis comprises, first, precise rigid co-registration of the time series of images; second, computation of residual deformations between pairs of images; third, automatic quantification of the volume change, obtained by propagation of the segmentation of the structure of interest through the series of MR images. This approach has been applied to monitor changes in the volume of the brain and lateral cerebral ventricles in a healthy subject and a patient with primary progressive aphasia (PPA). Results are consistent with those obtained by application of the boundary shift integral (BSI) and by stereology in the same subjects.

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PubMed1998

Slowly progressive pure word deafness.

Among the reports of primary progressive aphasia, there are few about patients who exhibited progressive pure word deafness with detailed auditory and radiological examination as well as neuropsychological assessment. We describe a 67-year-old right-handed man who exhibited slowly progressive pure word deafness over a period of 9 years without exhibiting any other cognitive or mental deterioration. Magnetic resonance imaging of his brain revealed generalized cortical atrophy, particularly in the left superior temporal region. Auditory examination revealed severe disability in discriminating each syllable or mora of Japanese words despite adequate auditory acuity. He also showed impairment in temporal auditory discrimination assessed by the click fusion test and the click counting test. His ability to discriminate meaningful environmental sounds was mildly impaired. We discuss the pathophysiology of slowly progressive pure word deafness over a period of many years which was not complicated by other language or cognitive dysfunctions.

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PubMed2005

Progressive, isolated language disturbance: its significance in a 65-year-old-man. A case report with implications for treatment and review of literature.

Language disturbances are common features occurring in different neurodegenerative diseases, including Alzheimer's disease (AD) and the Frontotemporal Lobar Degeneration (FTLD) variants Primary Progressive Aphasia (PPA) and Semantic Dementia (SD). Despite AD and FTLD are supposed to have a different pathophysiology, PPA has been demonstrated to have in some cases an AD pathological component. The syndromic and etiological heterogeneity is crucial for the differential diagnosis and consequently for a therapeutical approach. Here, the case of a patient with progressive isolated language disturbances is presented, and further discussed on the basis of current diagnostic criteria and available guidelines for treatment.

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PubMed2001

Focal degenerative dementia syndromes.

Focal degenerative dementia syndromes are associated with a characteristic clinical picture, such as frontotemporal dementia, primary progressive aphasia, semantic dementia, corticobasal degeneration, and the Balint syndrome. A lobar approach may be used to classify the degenerative dementias. The underlying pathology of these various syndromes seems to be less heterogeneous than previously thought.

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PubMed2003

Progressive dysarthria: definition and clinical follow-up.

Progressive dysarthria is a common sign of several degenerative disorders of the central nervous system; it may also be a distinct nosographic entity. We identified nine patients in which progressive dysarthria remained the sole neurological sign for at least 2 years after onset. At least a year after hospital admission, the following diagnoses were made: two cases of corticobasal degeneration, one of frontotemporal dementia, one of primary progressive aphasia, one of motor neuron disease (MND)-dementia, one of ALS, and one of ALS-aphasia. In the remaining two patients progressive dysarthria remained the only neurological sign at latest examination. We conclude that in most cases progressive dysarthria is the presenting sign of an established neurodegenerative disease (generally degenerative dementia or motor neuron disease), although the possibility that progressive dysarthria is a distinct entity cannot be excluded. To clarify this issue, studies (probably multicenter) on more patients with longer clinical follow-up and pathological confirmation are required.

Amyotrophic Lateral Sclerosis↗

PubMed2005

Is slowly progressive anarthria a "pure" motor-speech disorder? Evidence from writing performance.

It is usually assumed that writing is normal in patients with anarthria, but a careful examination of the literature shows that they produce deletions, transpositions and insertions. Indeed, a matter of debate concerns the distinction between primary progressive aphasia (PPA) and slowly progressive anarthria (SPA). If writing deficits were purely linguistic errors, then there would be no reason to consider slowly progressive anarthria as distinct from non-fluent PPA. We report the case of a patient with SPA in whom writing abilities were specifically assessed. No lexical-semantic deficits were detected, but errors were deletions, substitutions or transpositions, with no frequency, length or lexicality effect; moreover, controls produced the same kind of errors during articulatory suppression. It is suggested that subvocal rehearsal plays a role in writing, allowing the conversion/assembly of the phonological string in a graphemic representation. Therefore, writing deficits do not appear to have a linguistic basis and SPA seems distinguishable from nonfluent forms of aphasia.

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PubMed2004

Contrasting metabolic impairment in frontotemporal degeneration and early onset Alzheimer's disease.

[18F]FDG positron emission tomography (PET) scans of 14 patients comprising the clinical prototypes of dementias that are considered to be associated with frontotemporal lobar degeneration (FTLD) were compared to a population of 15 patients with early onset Alzheimer's disease (EOAD). The FTLD group included patients with frontotemporal dementia (FTD), semantic dementia (SD), and primary progressive aphasia (PPA). A voxel to voxel group comparison identified metabolic impairment in the bilateral ventromedial frontal area, the left anterior insula, and inferior frontal cortex, and indicated the right middle temporal gyrus to exhibit increased activity in FTLD compared to EOAD patients. All identified cortical structures are considered to be critically involved in neuropsychological features associated with FTLD (altered social behavior, aphasia) and EOAD (impaired linguistic and visuo-constructive abilities). In conjunction with recent insights from neuropathologic investigations, these results implicate that the a priori heterogeneous prototypes of FTLD (FTD, SD, PPA) may share more common ground than previously assumed, and therefore would become distinguishable as an entire group from EOAD.

Age of Onset↗

PubMed2005

The evolution and pathology of frontotemporal dementia.

This is a clinicopathologic study of a prospective, clinic-based cohort of patients with frontotemporal dementia (FTD)/Pick complex, who were followed to autopsy. A total of 60 patients with the clinical syndromes of the behavioural variant of FTD (FTD-bv) (n = 32), primary progressive aphasia (PPA) (n = 22), corticobasal degeneration syndrome (CBDS) (n = 4) and progressive supranuclear palsy (PSP) (n = 2) at onset, referred to a cognitive neurology clinic who had subsequent post-mortem examination were included. The most common histological variety was motor neurone disease type inclusion (MNDI) (n = 18), followed by corticobasal degeneration (CBD) (n = 12), then Pick's disease (n = 6), dementia lacking distinctive histology (DLDH) (n = 6) and PSP (n = 3). Others fulfilled the histological criteria for Alzheimer's disease combined with glial pathology (n = 6), Alzheimer's disease only (n = 4), Lewy body variant (n = 2), prion disease (n = 1), vascular dementia (n = 1) and undetermined (n = 1). The most common first syndrome among the MNDI and DLDH (tau negative) pathologies was FTD-bv, but subsequently progressive aphasia (PA), occasionally CBDS and semantic dementia also developed. Tau positive histologies of CBD, PSP and Pick bodies were most frequently associated with PPA onset or CBDS/PSP, but behavioural symptoms were also common. Age of onset was earlier in tau negative cases, but the duration of illness and gender distribution were about the same in all histological variants. Although the tau negative and positive histologies are predicted to some extent by the clinical onset, the extent of the overlap and the convergence of the syndromes in the course of the disease argue in favour of maintaining the clinical and pathological varieties under a single umbrella.

Adult↗

PubMedDate not supplied

[Visual art, creativity and dementia].

BACKGROUND: Visual art is an expression of neurological function and how it organizes and interprets perception. The art is predominantly in the right hemisphere, in contrast, the left side, have inhibitory effects on artistic expression. In normal subjects, inhibitory and excitatory mechanisms could interact in a complex harmony, reflecting a paradoxical functional facilitation. Brain diseases such as dementia could change this harmony and then, alter the artistic abilities. OBJECTIVE: Evaluate the art expression in the degenerative diseases. PATIENTS AND METHODS: Artistic abilities of 3 painters with degenerative diseases were assessment. RESULTS: Patient 1: A 83 - year old right handed female, diagnosis: Alzheimer's disease. Artistic description: low productivity, simplified versions of earlier and alteration of the visuospatial organization. Patient 2: A 78-year-old right handed female, diagnosis: Primary Progressive Aphasia (PPA); Artistic description: oversimplified drawings which maintaining overall spatial organization, without impair artistic skills. Patient 3: A 68 year-old right handed woman, diagnosis: Fronto-Temporal Dementia (FTD). Artistic description: Increased artistic activity, originality, freedom, utilization of intense colours with perseverative and repetitive copying of similar paintings of her own work. CONCLUSIONS: Visual art in Alzheimer's disease is a consequence of visuospatial and constructive disabilities. In contrast, the conservation of this cognitive functions and left asymmetrical involved, in FTD and PPA respectively, suggest artistic preservation, independently of the language injury. The disproportionate functional prevalence of the right over the left could lead to a release of novelty - seeking in art and can contribute to emergent creativity. These observations suggest an organization for art in the brain and proposed bases for further investigations in dementias.

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PubMed2001

The Apolipoprotein E genotype in patients affected by syndromes with focal cortical atrophy.

The role of the Apolipoprotein E (APOE) alleles in syndromes associated with focal cerebral atrophy (fronto-temporal dementia, primary progressive aphasia, corticobasal degeneration) is still controversial. We studied the APOE allele distribution in 39 patients with clinically diagnosed syndromes associated with focal cerebral atrophy (FCA), in 50 patients with early-onset probable Alzheimer's disease (EOAD), and in 60 patients with late-onset probable AD (LOAD). The APOE genotype was determined from a blood sample, using polymerase chain reaction and restriction enzyme digestion. The APOE epsilon4 allele frequency was significantly higher in the EOAD (21.0%) and LOAD (33.3%) groups, but not in the FCA group (5.1%), as compared with controls. In our population, the epsilon2 allele frequency was significantly higher in patients with FCA (12.8%) than in controls (4.8%). These results show that the APOE epsilon4 allele is not a risk factor for syndromes associated with FCA. The potential role of the epsilon2 allele in these syndromes needs further investigation.

Age of Onset↗

PubMed2000

Volumetric study of lobar atrophy in Pick complex and Alzheimer's disease.

BACKGROUND: Lobar atrophy is an important neuroimaging feature of Pick complex (PiC). However, differences in patterns of focal brain atrophy between PiC and Alzheimer's disease (AD), and among PiC subgroups, have not been studied quantitatively. OBJECTIVE: To compare volumetric measures among primary progressive aphasia (PPA), frontotemporal dementia (FTD) and AD; to assess association between brain atrophy and cognition. PATIENTS: Seventeen patients with PPA, 11 with FTD and 24 with probable AD were studied. METHODS: We measured total and regional volume quantitatively using MRI and computerized volumetry. Contributing factors were controlled statistically or by adopting brain volume ratios. We investigated the classifying power of volumetry and correlated regional brain volume with cognitive and language test scores. RESULTS: The ratio for fronto-temporo-central region was smaller on the left in PPA and on the right in FTD. AD and some PPA patients had smaller parietal lobes. The frontal ratios correctly classified 93% of PPA and FTD patients, but only 50% of the entire PiC and AD patients. Language-dependent examinations correlated with the left fronto-temporal volume. CONCLUSIONS: Brain atrophy differs in PPA, FTD and AD, but there is some morphological overlap between PiC and AD in parietal volumes. Focal brain atrophy is most consistently associated with language impairments.

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PubMed1995

Syndromes of language dissolution in aging and dementia.

This article has reviewed the language deterioration of aging, dementia, and the syndrome of primary progressive aphasia. Language deterioration is generally mild in normal aging but is a universal accompaniment of dementing diseases. Isolated, progressive language disturbance, especially nonfluent aphasia, is the hallmark of the syndrome called ¿Primary Progressive Aphasia¿ or PPA. The language findings in these patients illustrate that distinctions between focal and generalized brain disease are difficult. Much remains to be learned about the spectrum of diseases that can produce progressive aphasia. The discovery of biological or genetic markers for these diseases is likely to lead to a better understanding of their behavioral characteristics.

Aging↗