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PubMed2002

Progressive aphasia with Lewy bodies.

Dementia with Lewy bodies (DLB) may include both Alzheimer and Lewy body pathology, but has never been reported to cause primary progressive aphasia. We report a 69-year-old woman who died 11 years after presenting with the syndrome of progressive aphasia. Six years after aphasia onset she developed visual hallucinations, and subsequently parkinsonism. Autopsy examination revealed Alzheimer's disease (AD), cortical Lewy bodies, and depigmentation and Lewy bodies in the substantia nigra and locus ceruleus. The aphasia most likely reflected the initial onset of AD, and the psychosis and parkinsonism most likely reflected the subsequent onset of Lewy body pathology. This first reported case of progressive aphasia occurring within the context of AD and Lewy body pathology uniquely illustrates the clinical and pathological nosological relationships between these two disease processes, and demonstrates a limitation of the general term, 'DLB'.

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PubMed2002

Maurice Ravel and right-hemisphere musical creativity: influence of disease on his last musical works?

The problem of finding correspondence between a particular neuronal organization and a specific function of the human brain remains a central question of neuroscience. It is sometimes thought that language and music are two sides of the same intellectual coin, but research on brain-damaged patients has shown that the loss of verbal functions (aphasia) is not necessarily accompanied by a loss of musical abilities (amusia). Amusia without aphasia has also been described. This double dissociation indicates functional autonomy in these mental processes. Yet verbal and musical impairments often occur together. The global picture that emerges from studies of music and its neural substrate is by no means clear and much depends on which subjects and which aspect of musical abilities are investigated. An illustration of these concepts is provided by the case of the French composer Maurice Ravel, who suffered from a progressive cerebral disease of uncertain aetiology, with prominent involvement of the left hemisphere. As a result, Ravel experienced aphasia and apraxia and became unable to compose. The available facts favour a clinical diagnosis of primary progressive aphasia (PPA), with the possibility of an overlap with corticobasal degeneration (CBD). In view of Ravel's clinical history, we propose that two of his final compositions, the Bolero and the Concerto for the Left Hand, include certain patterns characteristic of right-hemisphere musical abilities and may show the influence of disease on the creative process.

Aphasia↗

PubMed1992

Progressive cognitive disorders due to focal cortical atrophy.

Aphasia is a common feature of dementia of the Alzheimer type (DAT) in association with behavioural change and other cognitive abnormalities. The clinical syndrome of primary progressive aphasia corresponds to the gradual onset and worsening of language dysfunction in patients who otherwise remained independent for many years in daily living activities, insight, judgement and behaviour. Characteristically, memory and other neuropsychological functions are relatively spared. Brain imaging studies indicate cortical atrophy of the left temporal region, and measures of regional cerebral metabolism show localized deficit in the corresponding region. Other progressive focal disorders due to degenerative cortical diseases have been also reported. In each case, localized atrophy and hypometabolism were observed, depending on the nature of the neuropsychological deficit. The underlying histology of focal cortical degeneration is heterogeneous, suggesting that these slowly progressive disorders represent either atypical variants of DAT or Pick's disease, or distinct neurological entities characterized by spongiform change and astrocytic gliosis.

Alzheimer Disease↗

PubMedDate not supplied

[Classification of the aphasic syndrome].

INTRODUCTION: The clinical examination of language should form part of the general examination of the mental state in patients with diffuse cognitive disorders, intellectual disorders, disorders of memory, etc. OBJECTIVE: To focus the examination of language towards understanding and classification of linguistic activity, through study of aphasia. DEVELOPMENT: We analyze the semiological diversity of aphasia and most relevant clinical forms using a diagnostic decision tree to consider the clinical, neuropsychological and neuroanatomical characteristics. The more detailed the study of the aphasic disorder, the more difficult it is to fit the case into a particular clinical type. In fact, as may be seen from current publications, there are more and more studies of aphasic persons considered to have unclassifiable aphasia. The more and more detailed studies of neuropsychological disorders mean that we now find terms such as primary progressive aphasia, semantic dementia and anarthria or progressive aphemia. This groups of abnormal linguistic features appearing in patients with focal degenerative illness may lead to some confusion, not only in the clinicosemiological sphere but also with regard to diagnosis, classification and evolution of the disorder. CONCLUSIONS: By studying aphasia we may study the anatomofunctional basis of language more fully, investigate the different systems for processing information, study cases with particular dysfunctions and specifically evaluate, case by case, the numerous, complex components of linguistic activity.

Aphasia↗

PubMed2004

A case of cortical deafness and anarthria.

Generally, cortical deafness is not complicated by anarthria and cortical anarthria does not affect auditory perception. We report a case of simultaneous progressive cortical deafness and anarthria. At the age of 70 years, the patient, a woman, noticed hearing problems when using the telephone, which worsened rapidly over the next 2 years. She was then referred to our hospital for further examinations of her hearing problems. Auditory tests revealed threshold elevation in the low and middle frequencies on pure-tone audiometry, a maximum speech discrimination of 25% and normal otoacoustic emissions and auditory brainstem, middle- and long-latency responses. An articulation test revealed abnormal pronunciation. Because of these problems only written and not verbal communication was possible; her ability to read and write was unimpaired. She showed no other neurological problems. Brain MRI demonstrated atrophic changes of the auditory cortex and Wernicke's language center and PET suggested low uptake of (18F) 2-fluoro-2-deoxy-d-glucose around the Sylvian fissures in both hemispheres. Neurologically, the patient was suspected of having progressive aphasia or frontotemporal dementia. Her cortical deafness and anarthria are believed to be early signs of this entity.

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PubMed2005

Apraxia of speech: an overview.

Apraxia of speech (AOS) is a motor speech disorder that can occur in the absence of aphasia or dysarthria. AOS has been the subject of some controversy since the disorder was first named and described by Darley and his Mayo Clinic colleagues in the 1960s. A recent revival of interest in AOS is due in part to the fact that it is often the first symptom of neurodegenerative diseases, such as primary progressive aphasia and corticobasal degeneration. This article will provide a brief review of terminology associated with AOS, its clinical hallmarks and neuroanatomical correlates. Current models of motor programming will also be addressed as they relate to AOS and finally, typical treatment strategies used in rehabilitating the articulation and prosody deficits associated with AOS will be summarized.

Aphasia↗

PubMed1996

Clinical, neuroimaging, and pathologic features of progressive nonfluent aphasia.

We report the clinical, neuroimaging, and neuropathologic features of progressive nonfluent aphasia (PNFA), a rare neurodegenerative syndrome most notable for its distinct language disturbance. Longitudinal observations of 3 patients revealed progressively telegraphic speech and writing, followed by gradual deterioration of sentence comprehension, and finally, preterminal mutism and dementia. Magnetic resonance imaging revealed cortical atrophy most pronounced in anterior regions of the left hemisphere. Functional neuroimaging demonstrated reduced cerebral activity most prominently in left frontal and temporal regions. At necropsy, microscopic pathology of brain was most consistent with the diagnosis of "dementia lacking distinctive histology" (DLDH). A review of published primary progressive aphasia cases with adequate clinical and histopathological descriptions reveals that the most common pathology underlying PNFA is DLDH. PNFA is one example of a family of clinical syndromes with similar underlying histopathology that affects different regions of the frontal lobe.

Adult↗

PubMedDate not supplied

Frontotemporal dementia: one disease, or many?: probably one, possibly two.

Accumulating evidence suggest that frontotemporal dementia is best viewed as a clinical syndrome even though there are distinct presentations of the behavioral variety, progressive aphasia, semantic dementia, corticobasal degeneration and progressive supranuclear palsy. Similarly the pathology should be regarded as a spectrum even though histological varieties are distinguished. More than half of FTD pathology is associated with ubiquitin positive and tau negative inclusions that are common in ALS. However the majority of FTD cases do not have ALS clinically and relatively few ALS cases develop FTD. The pathological and biochemical varieties can be dichotomized as tau positive and tau negative pathology and biochemistry. The genetics of the tau positive variety is associated with tau mutations and so far the tau negative variety is not, although some are linked to chromosome-17 also. There is a corresponding clinical dichotomy combining the behavioral variety of FTD presentation with semantic dementia and usually ubiquitin positive tau negative pathology on one hand and the association of primary progressive aphasia and cortical basal degeneration/PSP syndrome with tau positive pathology on the other. The overlap between them is too great to establish two separate diseases.

Aphasia↗

PubMed2000

Atypical and typical presentations of Alzheimer's disease: a clinical, neuropsychological, neuroimaging and pathological study of 13 cases.

There has been increasing awareness that some slowly progressive focal cortical syndromes can be the presenting features of Alzheimer's disease, but pathological evidence has been sparse. This clinico-pathological series presents our experience with pathologically proven atypical as well as typical Alzheimer's disease presentations. We report and compare four patterns of presentation: a typical pattern with initial amnesic syndrome (n = 4 cases), progressive visual dysfunction (n = 1), progressive biparietal syndrome (n = 2) and progressive aphasia (n = 6). The aphasic presentations include both fluent and non-fluent aphasic syndromes. The neuropsychological profiles and neuroimaging clearly reflect the presenting clinical features, and show a close relationship to the distribution of pathology in these cases. Of note was the sparing of medial temporal structures (hippocampus and/or entorhinal cortex) in several aphasic cases and the severe occipito-parietal involvement in those with prominent visuospatial disorders at presentation. Our data demonstrate the wide spectrum of Alzheimer's disease presentations. The recognition of atypical presentations of Alzheimer's disease is important when attempting to make an early accurate pre-morbid diagnosis of neurodegenerative disease.

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PubMed1993

[A clinicopathological case of progressive aphasia].

We report the case of a 59-year old right-handed woman presenting with progressive aphasia without any other neurological deficit and characterized by anomia, agramatism and auditory comprehension difficulties. CT scan showed no abnormalities. Four years later, aphasia was complete but neurological examination was normal. CT scan disclosed a mild cortico-subcortical cerebral atrophy with slight widening of both sylvian fissures. The results obtained with the Wisconsin Card Sorting Test and the Raven's Matrices showed only minor deficits. The IQ (WAIS) was 90. During the following 6 years the patient remained fully self-sufficient and carried out her homework normally. At that time MRI showed progression of cerebral atrophy more pronounced on the left side. Besides a rapid deterioration, twelve months later she developed severe dementia and died 13 years after the onset of the illness. Brain examination disclosed a severe atrophy (brain weight: 880 g) prominent in both frontal lobes and in the anterior perisylvian structures, more pronounced on the left side. There were no vascular lesions. Microscopy revealed widespread neuronal loss and astrocytic fibrillary gliosis confined to cortical areas and vacuolation in the superficial layers. Neurofibrillary tangles and neuritic plaques were found in the most atrophic areas but not in sufficient number to fulfill the histological criteria for Alzheimer's disease. There were no neuropathological changes of Pick's disease or subcortical degeneration. Previous microscopical studies of primary progressive aphasia showed non specific, mostly lobar atrophy similar to that observed in our case, although Alzheimer, Pick and Creutzfeldt-Jakob diseases have been reported. This neuropathological heterogeneity confirms that progressive aphasia is a non-specific language disorder mostly observed in lobar forms of brain degeneration.

Aphasia↗

PubMed1993

[Two cases of primary progressive non-fluent aphasia].

Two patients were described with a five to seven-year history of primary progressive non-fluent aphasia. One patient developed atypical trascortical motor aphasia with marked anarthria, which has led to mutism. Magnetic resonance (MR) imaging showed lobar atrophy of the frontal lobe accentuated in the bilateral superior frontal gyri, the left middle frontal gyrus, the left anterior cingulate gyrus and the left operculum with some extension into the left temporal lobe. Single photon emission computed tomography (SPECT) scans demonstrated a decrease of regional cerebral blood flow (rCBF) in the atrophic site. The patient was clinically and neuroradiologically diagnosed as having Pick's disease. Another patient presented with atypical Broca's aphasia, which has worsened with slowly progressive right hemiparesis. Mitigated, sometimes complete, echolalia was also observed. MR imaging and SPECT scans showed mild atrophy and a decrease of rCBF in the left perisylvian region involving the frontal operculum, while a positron emission tomographic study disclosed diffuse hypometabolism in the left hemisphere. We pointed out that the features of primary progressive aphasia were frequently atypical in the light of classical classification of aphasia and that non-fluent aphasia might be observed even in the early stage of cortical degenerative processes.

Aphasia, Broca↗

PubMed2003

Pick's complex and FTDP-17.

This essay advances the thesis with a review of the evidence that Pick's disease, frontotemporal dementia, primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy should be regarded as a clinically and biologically cohesive spectrum. The historically correct eponymic term Pick's complex, for both the clinical and pathological varieties is preferred. The discovery of tau mutations in frontotemporal dementia and parkinsonism linked to specific mutations in chromosome 17 and their resemblance to the sporadic cases validates the concept of Pick's complex. There are recently discovered overlaps between the three-repeat and four-repeat tauopathies, and the tau-negative varieties with or without motor neuron disease-type inclusions may be deficient in normal tau, therefore may be tauopathies also. Although clinical, pathological, and biochemical distinctions continue to be important, integration promises to be productive in this family of not so rare diseases.

Chromosomes, Human, Pair 17↗

PubMed2001

Association of REM sleep behavior disorder and neurodegenerative disease may reflect an underlying synucleinopathy.

Our objective was to examine whether rapid eye movement (REM) sleep behavior disorder occurs in disproportionally greater frequency in multiple system atrophy (MSA), Parkinson's disease (PD), and dementia with Lewy bodies (DLB), collectively known as the synucleinopathies, compared to other nonsynucleinopathy neurodegenerative disorders. In study 1, we reviewed the clinical records of 398 consecutive patients evaluated at Mayo Clinic Rochester for parkinsonism and/or cognitive impairment. The frequency of suspected and polysomnogram (PSG)-confirmed REM sleep behavior disorder (RBD) among subjects with the synucleinopathies MSA, PD, or DLB was compared to the frequency among subjects with the nonsynucleinopathies Alzheimer's disease (AD), frontotemporal dementia (FTD), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), mild cognitive impairment (MCI), primary progressive aphasia (PPA), and posterior cortical atrophy (PCA). In study 2, we reviewed the clinical records of 360 consecutive patients evaluated at Mayo Clinic Jacksonville for parkinsonism and/or cognitive impairment. The frequency of probable RBD among patients with PD and DLB was compared to the frequency among patients with AD and MCI. In study 3, we reviewed the brain biopsy or postmortem autopsy diagnoses of 23 Mayo Clinic Rochester patients who had been clinically examined for possible RBD and a neurodegenerative disorder. In study 1, patients with MSA, PD, or DLB were more likely to have probable and PSG-confirmed RBD compared to subjects with the nonsynucleinopathies (probable RBD 77/120=64% vs. 7/278=3%, p < 0.01; PSG-confirmed RBD 47/120=39% vs. 1/278=0%, p < 0.01). In study 2, patients with PD and DLB were more likely to have probable RBD compared to those with AD and MCI (56% vs. 2%, p < 0.01). In study 3, of the 23 autopsied patients who had been questioned about possible RBD, 10 were clinically diagnosed with RBD. The neuropathologic diagnoses in these 10 included Lewy body disease in nine, and MSA in one. Of the other 13 cases, 12 did not have a history suggesting RBD, and the one case who did had normal electromyographic atonia during REM sleep on PSG and autopsy findings of PSP. Only one of these 13 had a synucleinopathy. The positive predictive values for RBD indicating a synucleinopathy for studies 1-3 were 91.7%, 94.3%, and 100.0%, respectively. Clinically suspected and PSG-proven RBD occurs with disproportionally greater frequency in MSA, PD, and DLB compared to other neurodegenerative disorders. In the setting of degenerative dementia and/or parkinsonism, we hypothesize that RBD is a manifestation of an evolving synucleinopathy.

Adult↗

PubMed1993

Music and language in degenerative disease of the brain.

Music and language functions were studied in two musicians with degenerative disease. Both patients were tested on a standardized language battery and a series of music tasks. In the first case with left cortical atrophy and primary progressive aphasia, expressive music functions were spared with impaired reception of rhythm. The second case with posterior cortical atrophy, greater on the right, was nonaphasic, had spatial agraphia, a visuopractic deficit, and severe expressive music deficits, but intact rhythm repetition. The aphasic patient showed dissociations between music and language in fluency and content; continuous, organized, although reiterative music production was contrasted with nonfluent language. The nonaphasic patient showed the opposite pattern of deficits; unmusical production with impaired melody and rhythm organization that was contrasted with fluent, intelligible language. The double dissociation between language and music functions supports the existence of independent cognitive systems, one consistent with conventional left lateralization models of language, temporal sequence, and analytic music processing and another with a right lateralization model of implicit music cognition.

Agraphia↗

PubMed1996

Focal cortical atrophy syndromes.

The topography of Alzheimer's disease (AD) and its effects on language, perception, and praxis are briefly reviewed as background to the focal cortical atrophy syndromes, including primary progressive aphasia (PPA), posterior cortical atrophy (PCA), and corticobasal degeneration (CBD). Simplistically speaking, there are two main pathological and neuroimaging phenotypes associated with these syndromes. One is frontotemporal degeneration (FTD), a nonspecific spongiform degeneration, with gliosis and neuronal loss, sometimes with Pick cells and bodies, which is usually selective for frontal and anterior temporal cortex. The other is Alzheimer's disease, in which amyloid plaques and neurofibrillary tangles initially develop in the hippocampal region, and spread to the lateral temporal and parietal neocortex and then to the frontal cortex. In the case of PPA, left perisylvian dysfunction is usually evident at presentation clinically and on neuroimaging. Nonfluent progressive aphasia tends to progress anteriorly and is usually associated with FTD. In fluent progressive aphasia, the pathology may progress anteriorly due to FTD or posteriorly, reflecting AD. In PCA, the visual association cortex is targeted bilaterally, often more so on the right, and the pathology is usually indicative of AD. CBD is an asymmetric akinetic-rigid syndrome associated with apraxia, in which swollen achromatic neurons are present in the frontoparietal cortex and substantia nigra on light microscopy, suggesting to some that it may fall within the spectrum of FTD and Pick's disease. The application of new molecular biological techniques, however, suggest that CBD, FTD, and Pick's Disease may be pathologically distinct. The clinico-pathological features of each syndrome are reviewed and compared to those typical for AD, and single photon emission computerized tomography scans representative for each are illustrated.

Alzheimer Disease↗

PubMed1995

Achromatic neurons in the cortex of progressive supranuclear palsy.

Achromatic neurons (AN) in the cerebral cortex are a characteristic feature of several neurodegenerative conditions including Pick's disease, corticobasal degeneration and some cases of primary progressive aphasia. Although AN are occasionally seen in some other diseases, their presence in progressive supranuclear palsy (PSP) has not been previously documented. We found significant numbers of AN in the cerebral cortex of five out of seven cases which had been diagnosed pathologically as PSP. The identification of AN was greatly facilitated by the use of neurofilament immunohistochemistry. The entorhinal and transentorhinal cortices were most frequently involved, but in several cases AN were also seen throughout the neocortex. The presence and number of AN roughly correlated with a history of clinical dementia. This suggests that cortical AN may be a common and important pathological finding in PSP.

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PubMed1996

Non-Alzheimer forms of cortical degeneration: frequency in clinical practice and clinical report of six cases.

Recent clinical and neuropathological studies suggest the possibility of distinguishing some forms of cortical degeneration from Alzheimer's disease. We report data on the frequency of non-Alzheimer forms of cerebral atrophy that were diagnosed on the basis of clinical criteria. Six examples of these neurological disorders are described: two patients with Lewy body disease; two patients with frontal lobe type dementia, one of whom had associated features of motor neuron disease; a patient with primary progressive aphasia; and a patient with a familial dementia that was probably an atypical form of Pick's disease.

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PubMed2005

Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation.

We have investigated the pathological correlates of dementia in the brains from a consecutive series of 70 patients dying with a clinical diagnosis of frontotemporal lobar degeneration (FTLD). Clinical misdiagnosis rate was low with only 3 patients (4%) failing to show pathological changes consistent with this diagnosis; 1 patient had Alzheimer's disease and 2 had cerebrovascular disease (CVD). In the remaining 67 patients, the most common underlying histological cause was ubiquitin pathology with 24 (36%) cases so affected. In these, ubiquitin-positive inclusions were present in the cerebral cortex as small, rounded or crescent-shaped structures within the cytoplasm of neurones of layer II, together with coiled or curvilinear bodies within neurites, and in the hippocampus as small, solid and more spherical-shaped inclusion bodies within the cytoplasm of dentate gyrus granule cells. In one patient, "cat's eye" or "lentiform" intranuclear ubiquitin inclusions were also present. The second most common histological type was dementia lacking distinctive histology (DLDH), in which neither tau nor ubiquitin inclusions were present, with 16 cases (24%) being affected. Pick-type histology was seen in 14 cases (21%) and tau histological changes associated with frontotemporal dementia (FTD) linked to chromosome 17 (FTDP-17) were present in 11 cases (16%). One case (1%) showed an unusual tau pathology that could not be allocated to any of the other tau groups. Only 1 case (1%) had neuronal intermediate filament inclusion dementia. No cases with ubiquitinated, valosin-containing protein-immunoreactive intranuclear inclusion bodies of the type seen in inclusion body myopathy with Paget's disease of bone and frontotemporal dementia were seen. Clinicopathological correlation showed that any of these histological subtypes can be associated with FTD. However, for FTD with motor neurone disease (FTD+MND), semantic dementia or primary progressive aphasia (PA), the histological profile was either ubiquitin type or DLDH type; Pick-type histology was seen in only 1 case of PA. None of these latter three clinical subtypes was associated with a mutation in tau gene and FTDP-17 type of tau pathology. All cases of progressive apraxia were associated with Pick-type histology. Present data therefore indicate that, although ubiquitin pathology is the most common histological form associated with FTLD, this pathology is not tightly linked with, nor is pathologically diagnostic for, any particular clinical form of the disease, including FTD+MND.

Adenosine Triphosphatases↗