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PubMed2004

Language, ageing and neurodegenerative disease.

UNLABELLED: Until recently brain-behavior relationships in healthy ageing and neurodegenerative disease were based on studies of the state of the brain after its final outcome, death. Direct in vivo imaging of dynamic cognitive systems at different disease stages offers an unprecedented opportunity to gain insight into evolving cognitive dysfunctions and changing brain systems. In this talk we will present new data on how this semantic system is affected by healthy ageing, lesions and neurodegenerative disease. 1. Age: One of the principal cognitive consequences of normal ageing is a decline of episodic memory while semantic memory is preserved. Despite the fact that semantic task performance remains at equal levels, the underlying semantic brain network shows clear alterations: The gradient between left and right prefrontal cortex is significantly decreased, principally due to a decrease of left prefrontal activation. 2. Focal brain lesions: In a patient who suffered from an ischemic lesion that is confined to the right anterior fusiform gyrus, associative semantics were still performed at a high level while drawing from memory and pseudo-object decision task were severely impaired. The right anterior fusiform group activation coincided with the lesion. In this patient the mirror left fusiform region was significantly more active during picture semantics than under normal circumstances. A case with a lesion in the mirror left fusiform region will also be discussed. 3. Neurodegenerative disease: We will contrast two patient groups: Patients with a progressive word finding and semantic memory deficit (primary progressive aphasia, most often due to frontotemporal degeneration) and patients with an isolated episodic memory deficit (incipient Alzheimer's disease, mild cognitive impairment). In patients with primary progressive aphasia different components of the semantic network, i.e. inferior frontal sulcus, superior temporal sulcus and anterior temporal pole, show less activity than in healthy controls. The activity levels correlate with performance on off-line picture naming tasks. Interestingly, the right medial temporal lobe, classically implicated in episodic memory functions, shows higher activity in primary progressive aphasics than in healthy controls, suggesting that patients make use of non-verbal episodic memory strategies to compensate for their semantic deficit. In contrast, patients with mild cognitive impairment clinically have only an isolated episodic memory deficit and perform within the normal range on semantic tasks. Despite normal performance the semantic system for words and pictures show profound alterations, including among other differences decreased activity in Wernicke's area (posterior middle temporal gyrus) on the left compared to age-matched controls. CONCLUSION: cognitive brain systems for semantic memory change with increasing age. These changes are qualitatively different from those found in incipient Alzheimer's disease or in primary progressive aphasia. The degree to which brain systems adapt in a plastic way to pathogenetic processes at multiple levels, is a determinant of disease manifestation and an important target for current and future therapies.

Aging↗
PubMed2000

The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia.

OBJECTIVE: To provide evidence for the hypothesis that the corticobasal degeneration syndrome (CBDs) overlaps significantly with primary progressive aphasia and frontotemporal dementia, and that CBDs is part of the Pick complex. BACKGROUND: Corticobasal degeneration has been mainly described as a movement disorder, but cognitive impairment is also increasingly noted. METHODS: Thirty-five cases of clinically diagnosed CBDs were followed-up with clinical, neuropsychological, and neuroimaging investigations. Twenty-nine patients were seen prospectively in movement disorder and cognitive neurology clinics; five of these came to autopsy. Six other autopsied cases that fulfilled the clinical criteria of CBDs were added with retrospective review of records. RESULTS: All 15 patients presenting with movement disorders developed behavioral, cognitive, or language deficits shortly after onset or after several years. Patients presenting with cognitive problems (n = 20), progressive aphasia (n = 13), or frontotemporal dementia (n = 7) developed the movement disorder subsequently. Eleven cases with autopsy had CBD or other forms of the Pick complex. CONCLUSIONS: There is a clinical overlap between CBD, frontotemporal dementia, and primary progressive aphasia. There is also a pathologic overlap between these clinical syndromes. The recognition of this overlap will facilitate the diagnosis and avoid consideration of CBD as "heterogenous."

Aphasia↗
PubMedDate not supplied

Intensive training of phonological skills in progressive aphasia: a model of brain plasticity in neurodegenerative disease.

Three patients with a typical syndrome of nonfluent primary progressive aphasia (Mesulam's syndrome) were trained daily with a remediation protocol including auditory exercises specifically designed to involve several aspects of phonological processing, a domain known to be specifically affected in the condition. The speech content of the exercises was based on the temporal theory of phonological processes according to which increasing the duration of formant transition should facilitate phoneme discrimination and phoneomic awareness. Significantly improved performance on the trained tasks was demonstrated in the three patients. Improvement further generalized to other tasks such as nonword repetition and reading. We conclude that such results (1) argue for using intensive focused therapy of language impairment in neurodegenerative disorders, (2) may constitute a good model of brain plasticity in neurodegenerative disorders in general, and (3) support theories of phonological processing emphasizing temporal features of the auditory signal.

Aged↗
PubMed2004

[Corticobasal degeneration and atypical progressive supranuclear palsy: their symptomatology, laboratory examination and differential diagnosis].

Corticobasal degeneration (CBD) and atypical progressive supranuclear palsy (PSP) were reviewed with special reference to their symptomatology, laboratory examination and differential diagnosis. In our survey of the autopsy cases of CBD in Japan, only about 60% of the pathologically confirmed CBD cases were correctly diagnosed clinically, meaning that atypical (non-classical) clinical forms are common in CBD. Concerning the autopsy cases of PSP in Japan, 75% of the PSP cases had correct clinical diagnosis. In literatures, the clinically atypical CBD includes (1) frontotemporal dementia, also with primary progressive aphasia and frontal lobe dementia as subforms, (2) PSP-like form, and (3) others. The clinically atypical PSP comprises (1) pure akinesia, (2) pure easy falling syndrome (Yuasa), (3) no postural instability, (4) no gaze palsy, (5) asymmetric parkinsonism, (6) no or severe dementia, etc.. PSP with cortical manifestations such as primary progressive aphasia and CBD-like features were also reported. The atypical CBD and PSP probably reflect the distribution of tau pathology different from that in typical forms. Except for the report that phosphorylated tau is increased in CSF in CBD, but not in PSP (Urakami et al), no reliable laboratory data have been available on clinical differentiation between atypical CBD and PSP.

Basal Ganglia↗
PubMed1997

Automatic analysis of cerebral atrophy.

3D MR data obtained for 10 healthy control subjects have been used to build a brain atlas. The atlas is built in four stages. First, a set of features that are unambiguously definable and anatomically relevant need to be computed for each item in the database. The chosen features are crest lines along which the maximal principal curvature of the surface of the brain is maximal in its associated principal direction. Second, a nonrigid registration algorithm is used to determine the common crest lines among the subjects in the database. These crest lines form the structure of the atlas. Third, a set of crest lines is taken as a reference set and a modal analysis is performed to determine the fundamental deformations that are necessary to bring the individual data in line with the reference set. The deformations are averaged and the set of mean crest lines becomes the atlas. Finally, the standard deviation of the deformations between the atlas and the items in the database defines the normal variation in the relative positions of the crest lines in a healthy population. In a fully automatic procedure, the crest lines on the surface of the brain adjacent to the cerebral ventricles in a patient with primary progressive aphasia were compared to the atlas; confirmation that the brain of this patient demonstrates atrophy was provided by stereological analysis that showed that the volume of the left cerebral hemisphere is 48.8 ml (CE 2.8%) less than the volume of the right cerebral hemisphere in the region of the temporal and frontal lobes. When the amplitude of the deformations necessary to register the crest lines obtained for the patient with the atlas were greater than three standard deviations beyond the variability inherent in the atlas, the deformation was considered significant. Four of the main deformation modes of the longest crest line of the surface of the brain adjacent to the cerebral ventricles were significantly different in the patient with primary progressive aphasia compared to the atlas. The ventricles are preferentially enlarged in the left cerebral hemisphere. Furthermore, they are closer together posteriorly and further apart anteriorly than in the atlas. These observations may be indicative of the atrophy of the temporal and frontal lobes of the left cerebral hemisphere noted in the patient. Ultimately, the approach may provide a useful screening technique for identifying brain diseases involving cerebral atrophy. Serial studies of individual patients may provide insights into the processes controlling or affected by particular disease.

Aged↗
PubMed1999

[Progressive focal cortical atrophies].

Progressive focal cortical atrophies are degenerative conditions characterised by the insidious onset and gradual exacerbation of an impairment in a single cognitive domain related to circumscribed cerebral atrophy. Several focal cortical syndromes with deficits in the realm of cognition are reviewed: progressive impairment of language (primary progressive aphasia), speech (progressive anarthria), semantic memory (semantic dementia), episodic memory (pure progressive amnesia), vision (progressive perceptual or visuo-spatial deficits) and gesture (progressive apraxia). These conditions are histologically heterogeneous and can be associated with focal non-specific neuronal loss and gliosis with some spongiform changes (non-specific lesions), pathological features of Pick's disease (inclusion bodies and swollen neurones) or Alzheimer's disease (AD) (senile plaques and neurofibrillary tangles). A relationship between neuropsychological profiles and lesional types emerges from this review of the literature. Non-fluent primary progressive aphasia, semantic dementia and progressive anarthria are usually associated with non-specific lesions and Pick-type pathology. Progressive disorders of episodic memory and progressive visuo-spatial deficits are more often related to AD. If adequate clinical characterisation can determine the underlying disorder, it appears even more important to establish the neuropsychological profile in patients with cortical degenerative disease. Progressive deficits of only one domain of cognition may well be due to preferential involvement of anatomically and functionally defined neural systems and could therefore be considered as "system atrophies". There remains no doubt that these syndromes are particularly well suited models for studies on the relationship between cerebral functions and their neural substrate.

Aged↗
PubMed2001

Slowly progressive aphasia: a four-year follow-up study.

This paper reports the long-term follow-up of GC, a patient with primary progressive aphasia of the fluent type. GC presented at onset with an anomia characterized by sparing of first letter knowledge, that applied mainly to proper names and living categories. No semantic deficits were observed in the first stage of the disease, and MRI showed a left temporal lobe atrophy with a gradient from the pole to the posterior regions, the latter being less involved. We now report the clinical evolution of GC from the 2nd to the 4th year of disease. As the disease progressed, the anomia became more severe and the phenomenon of first letter sparing was no longer detectable. Also semantic knowledge was gradually affected and, eventually, was dramatically lost. However, no other cognitive deficits were seen at the last examination. By that time, the temporal atrophy shown by MRI was bilateral, although still more evident on the left side.

Aphasia, Primary Progressive↗
PubMed1997

[Progressive degenerative aphasia: clinical and neuroradiological observations in 18 cases].

INTRODUCTION: In some types of degenerative dementia aphasia is the main disorder. In primary progressive aphasia. (PPA) atrophy is limited to the dominant peri-sylvan region. We present 18 cases of progressive aphasia of degenerative origin, with or without dementia. MATERIAL AND METHODS: We describe the clinical and neuro-radiological findings in 3 patients with 'aphasic dementia and motor neuron disease (ADMND)', 7 with 'semantic dementia' (DS), and 4 with 'fronto-temporal dementia' with 'marked non-fluent aphasia' (AFTD). Criteria published in recent years were used. RESULTS: In patients with ADMND non-fluent aphasia progressed to global aphasia, with dementia occurring after 2-9 months, and death after an average of 17 months. In cases with SD, initial anomic aphasia progressed to transcortical sensory or global aphasia, and in patients with AFTD, Broca's aphasia or motor transcortical aphasia progressed to global aphasia. Seven of these patients had been initially diagnosed as having PPA and became demented after two years or more. In most of the cases the cognitive disorder had the characteristics of fronto-temporal dementia. All cases had cortical atrophy or asymmetrical cortical or cortico-subcortical atrophy. The 4 cases of non-fluent PPA were not demented after 21 months-6 years of illness, and showed perisylvan and left fronto-temporopolar atrophy. CONCLUSIONS: The PPA may correspond to the initial form of at least three varieties of dementia, usually the fronto-temporal type. Dementia occurs after two years or more, except in patients with motor neurone disease, when there is a latent period of less than one year.

Age of Onset↗
PubMed2006

Corticobasal degeneration as cause of progressive non-fluent aphasia: clinical, radiological and pathological study of an autopsy case.

A Japanese male developed gradual loss of spontaneous speech at age 60. Three years later meaningful speech had deteriorated to the point that it had become restricted to monotonous utterances. Neuropsychological examination at age 62 showed that he had severe non-fluent aphasia. A brain MRI demonstrated mild cortical atrophy with ischemic lesions in the cerebral white matter. He was diagnosed as having primary progressive aphasia. At age 63, he was admitted to the hospital to reevaluate the neurological condition. Neurologic examination showed severe non-fluent aphasia, hyperreflexia, snout and sucking reflexes. No alien hand was observed. He was able to walk, dress, wash himself and use chopsticks as well as name real objects. At age 65, 99Tc-hexamethylpropyleneamine oxime single photon emission computed tomography (HMPAO-SPECT) revealed diffuse cerebral hypoperfusion that was particularly prominent in the left frontal lobe. An MRI showed progressive cortical atrophy with the definite atrophy of the left paracentral gyrus. The hippocampal formation and putamen were also atrophic. He died of pneumonia at age 67. The brain weighed 810 g with atrophy of the frontal lobe, globus pallidus, enlargement of the lateral ventricles and depigmentation of the substantia nigra. Microscopic examination showed severe neuronal loss and gliosis in the cerebral cortex, globus pallidus interna and substantia nigra. Ballooned neurons were observed in the cerebral cortex. Gallyas-Braak method revealed numerous astrocytic plaques and argentophilic threads in the cerebrum. Clinical diagnosis of corticobasal degeneration sometimes is difficult in individuals with atypical clinical presentations. More exact clinical and radiological criteria may warrant a diagnosis of corticobasal degeneration.

Aphasia, Primary Progressive↗
PubMedDate not supplied

[Differential diagnosis between Mesulam's aphasia and aphasia secondary to ischemic disorder by brain perfusion SPECT].

Two cases with aphasic dysfunction with similar clinical features and structural neuroimaging studies results are presented. In these cases, brain perfusion SPECT was the most useful complementary study in the differential diagnosis of two pathologies with different etiologies: primary progressive aphasia or Mesulam's aphasia and aphasia secondary to a stroke.

Aged↗
PubMed2003

[Routine clinical diagnosis of primary progressive non-fluent aphasia].

It may be difficult to distinguish between a primary progressive aphasia at a very mild stage from the beginning of Alzheimer's disease (AD). However, this may be achieved by carrying out simple neuro-psychological tests. Nine non-fluent PPA (NFPPA) and 76 AD patients with comparable MMSE as well as 58 control subjects were evaluated using simple tests: MMSE, fluency, apraxia, naming, digital span, story memory, 5 words memory test. NFPPA patients had significantly impaired functions during the semantic category fluency and naming tests as compared to AD patients, whereas they showed a better delayed recall of the 5 words and story memory tests. As compared to AD, MMSE of NFPPA patients was also better in the time orientation and word recall sub-tests, although inferior in words repetition and language items. Thus, with comparable MMSE, NFPPA patients have more lexico-semantic difficulties, but a better delayed verbal memory than AD patients. These simple tests easily confirm the language impairment of NFPPA patients as opposed to the mnestic difficulties of AD, even at very early stages of these pathologies.

Adult↗
PubMed1997

Surface dyslexia in nonfluent progressive aphasia.

This article presents the case of a 59-year-old male, JH, with a 6-year history of primary progressive aphasia (PPA), a disorder characterized by isolated language deterioration with relative preservation of other cognitive abilities. JH also shows typical features of surface dyslexia, a reading disorder exemplified by the selective preservation of phonological reading. One recent theory is that surface dyslexia in individuals with PPA results from a loss of semantic knowledge. In this paper we consider an additional possibility and present data supporting the notion that surface dyslexia may also arise from the malfunction in the links between semantic representations and phonology. JH has remarkably preserved lexical semantic knowledge when assessed on tasks that do not require verbal output. Further, item-by-item comparisons of his oral reading and comprehension ability show no significant correspondence between his reading and semantic knowledge. These findings lead us to conclude that, in JH's case, the surface dyslexia is attributable not to a semantic deficit per se but rather to the inability to access phonological information from semantics. JH's language profile is considered in relation to potential sources of surface dyslexia and other cases of progressive aphasia.

Aphasia, Broca↗
PubMed2006

[Primary progressive non-fluente aphasia].

When a subject shows changes in his/her ability to express himself/herself verbally or to understand the messages from the people he/she relates with, it is customary to say that such a person has a language disorder, nosologically classified in the field of aphasias. In this article, we show the case of a patient diagnosed with progressive aphasia, where such a diagnosis describes the data resulting from the neuropsychological assessment process. Finally, we provide a few reflections with regard to the case.

Aged↗
PubMed1993

[Heterogeneity of Alzheimer's disease].

Despite the original distinction between Alzheimer's disease (AD) and senile dementia, both are currently considered to be one single disease based on their common neuropathological findings. We have reviewed the literature to investigate whether there is proof to assume the existence of subgroups of AD. Historically, there is a division based on the age at onset. Although typical cortical symptoms may be more prominent in early onset cases they may be encountered in senile cases too. Cortical neuronal loss may be more severe in early onset patients, but hippocampal neuronal loss is equally severe in both groups. In older patients cerebral reserve is reduced by age-related neuronal loss and a small amount of AD-type lesions might be sufficient to cause dementia. Aphasia has been proposed as indicator of a subgroup. However, it probably occurs in all AD patients when the cortical degenerative process progresses. AD cases presenting with aphasia are rare and more often prove to be Pick's disease or primary progressive aphasia. Extrapyramidal signs are present in 25% of patients without neuroleptics and in 90% of all patients. They are usually of mild intensity, appear during the course of the disease and are found to be of extranigral origin. Myoclonus may also be encountered at any stage, although more frequent in early onset AD. Its presence is often associated with a more progressive course. Most AD cases are sporadic and some are familial, suggesting an autosomal dominant transmission. Molecular genetics reveals that some patients with familial early-onset AD have a mutation on chromosome 21. Other genes, probably on chromosome 19, may be associated with late onset familial cases, suggesting heterogeneity in familial AD. White matter involvement on computerized tomography or magnetic resonance imaging has been reported to be more prominent in a subgroup of AD patients, with later onset and confusional symptoms. The typical bilateral temporo-parietal hypometabolism on positron emission tomography and hypoperfusion on single photon emission computed tomography, is not found in all AD cases but may be indicative of a subgroup. Based on this review of the literature one homogeneous subgroup emerges: "probable" AD patients displaying memory disturbances with predominant cortical signs (especially aphasia), with a low prevalence of confusion and white matter involvement, exhibiting symptoms at a relatively early age but not exclusively below 65 years, and with a higher prevalence of genetic predisposition and more widespread neuropathological lesions at postmortem examination.

Age Factors↗
PubMed1990

Cerebral blood flow in progressive aphasia without dementia. Case report, using 133xenon inhalation, technetium 99m hexamethylpropyleneamine oxime and single photon emission computerized tomography.

We report a case of progressive aphasia without clinical signs of intellectual or behavioral impairment, satisfying Mesulam's clinical criteria of primary progressive aphasia, as 4 yrs of extensive psychometric testing and radiological imaging, comprising CT and MRI, failed to detect evidence of relevant involvement outside the left perisylvian regions. Cranial CT was normal but MRI showed multiple bilateral lesions in the deep white matter. Cerebral blood flow (CBF) studies by single photon emission computerized tomography, however, showed an initial frontotemporal focus of hypoperfusion that progressively extended to include most of the ipsilateral hemisphere and the contralateral frontal lobe. This suggests that CBF imaging may yet be the most sensitive technique in revealing subclinical injury in the degenerative brain diseases of focal onset.

Administration, Inhalation↗
PubMed1997

Bilateral temporal lobe volume reduction parallels cognitive impairment in progressive aphasia.

BACKGROUND: Patients with isolated aphasia in the absence of other cognitive abnormalities have been the focus of several studies during the past decade. It has been called primary progressive aphasia (PPA), and the typical features of this syndrome are marked atrophy of the left temporal lobe according to the radiological examination and a language disorder as the initial symptom. In previous studies of PPA, the selection of the patients was based mainly on linguistic symptoms. Now, when computed tomography or magnetic resonance imaging scans are part of the routine investigation of cognitive impairment and suspected dementia, the patients with lobar atrophy will be found at an earlier stage. In the present study, we used a new approach and defined the study group by selecting patients with obvious left temporal lobe atrophy, assessed by MRI, and we referred to them as patients with temporal lobe atrophy (TLA). OBJECTIVE: To identify the features that distinguish TLA from other primary neurodegenerative disorders. PATIENTS: Six patients with TLA were compared with patients with Alzheimer disease (AD), patients with frontal lobe dementia (FLD), and healthy control subjects. METHODS: The investigations included magnetic resonance imaging volumetry, single photon emission computed tomography, and neuropsychologic and linguistic evaluations. RESULTS: In the TLA group, the mean volume of the left temporal lobe was 35% smaller than the right, while in the AD and FLD groups, the atrophy was symmetrical and bilateral. In the TLA group, the absolute volumes of the temporal lobes were significantly smaller on the left side compared with the AD and FLD groups, whereas there was no difference on the right side. The cerebral blood flow pattern in TLA was asymmetric and differed from that in the other study groups. All patients with TLA had a history of progressive Wernicke-type aphasia, ranging from 2 to 6 years. They showed primary verbal memory impairment but had preserved visuospatial functions. The clinical condition of all patients with TLA deteriorated during the study period; severe aphasia developed, and the patients exhibited signs of frontal lobe dysfunction. Serial volumetric measurements in 4 of 6 patients showed an annual 8% to 9% decrease of both left and right temporal lobes. CONCLUSIONS: The initial marked asymmetry in cognitive function found in patients with TLA contrasts with the general decline found in patients with AD. The bilateral degenerative process evident in patients with TLA paralleled the clinical deterioration, indicating TLA to be a non-AD lobar atrophy that develops into generalized cognitive dysfunction and dementia.

Aged↗
PubMedDate not supplied

Progressive nonfluent aphasia with dementia: a case report.

We report a patient with progressive nonfluent aphasia and mild dementia. On 9-month follow-up evaluation, there was significant deterioration in both language and cognition. Unlike in Alzheimer's disease, the aphasia was of the Broca's type. In contrast with primary progressive aphasia, a dementia was present and the cognitive and language decline showed a rather rapid course.

Alzheimer Disease↗