SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Aphemia, also called anarthria or severe apraxia of speech, is a rare disorder of speech production usually resulting from vascular lesions affecting the inferior premotor cortex of the left hemisphere. A patient presenting with aphemia as the sole manifestation of primary progressive aphasia (PPA) is reported.
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) were described as separate entities, but prior to that an extrapyramidal variety of Pick's disease was recognized. Subsequently a pathological overlap between these conditions and clinical overlap between frontotemporal dementia, primary progressive aphasia, corticobasal degeneration syndrome and more recently PSP was recognized. Initially only the movement disorder had been emphasized, but now the behavioral and language symptoms are considered common. The syndromes of frontotemporal dementia/Pick's disease can be produced by underlying CBD, PSP or Pick pathology as well as with neural inclusions of the motor neuron disease type. The concept of this overlap has been confirmed genetically finding a similar spectrum of pathology with different tau mutations and even with tau negative pathology, which could be a deficiency of normal tau. The overlap of CBD with PSP and both with PPA and FTD allows to consider these relatively rare conditions as part of a more commonly occurring degenerative disease than previously recognized.
There is growing evidence that AD consists of different subtypes, and that language is a pertinent factor to identify a subgroup with a fast rate of cognitive decline. We report the first results of a longitudinal study in which we compared two groups of patients with probable AD. The main result showed that a subgroup with stable MMSE scores during a 1-year follow-up period had an impairment in language domains which are usually preserved until an advanced stage of the disease. It is proposed that this group may correspond to a variety of AD who, in addition to symptoms of AD, present characteristics of primary progressive aphasia. In other respects, we underscore that the high loading in language-mediated tasks of the MMSE makes it a poor index to accurately measure the rate of cognitive decline.
The right frontal lobe is important for the expression of emotional prosody, emotional faces, and automatic speech. The authors describe a woman who presented with progressive expressive affective aprosodia, affective prosoplegia, amusia, and loss of automatic speech but with an intact ability to understand emotional prosody and faces as well as express and understand syntactic prosody. MRI showed predominant right frontal cortical atrophy. The authors suggest that this patient has a form of frontotemporal dementia, analogous to primary progressive aphasia but disrupting right frontal lobe-mediated functions.
OBJECTIVE: To characterize the presenting clinical features for frontotemporal dementia (FTD) and contrast them with the degree of frontal and temporal hypoperfusion on SPECT imaging. METHODS: The authors evaluated 74 patients who eventually met Consensus Criteria for the FTD form of frontotemporal lobar degeneration (excluding primary progressive aphasia and semantic dementia) on 2-year follow-up. On first presentation, these patients had undergone both an FTD Inventory for 12 features based on core and supportive Consensus Criteria and SPECT imaging. The initial clinical diagnostic features were contrasted with variations in regional SPECT hypoperfusion. RESULTS: The patients with FTD had more hypoperfusion in the right frontal lobe than in other regions; the subgroup of 25 patients who met Consensus Criteria from the first presentation had the most right frontal hypoperfusion. Frontal lobe involvement was associated with significant apathy, whereas temporal lobe involvement was associated with hypomania-like behavior. Right frontal lobe hypoperfusion further predicted loss of insight, environmental dependency, and stereotyped behaviors. Other associations included left frontal hypoperfusion with a decline in personal hygiene and left temporal hypoperfusion with compulsions and mental rigidity. CONCLUSIONS: On first presentation, frontotemporal dementia (FTD) is disproportionately a right frontal disease evident on behavioral measures and on SPECT. Nonetheless, patients with FTD can initially present with further regional differences in clinical diagnostic features, such as apathy with bifrontal hypoperfusion and hypomania-like behaviors with anterior temporal involvement.
Loss of cognitive function in the elderly population is a common condition encountered in general medical practice. Diagnostic criteria and approaches have become more refined and explicit in the past several years. Precise diagnosis is feasible clinically. In this article, the precursor state and major subtypes of dementia are considered. Mild cognitive impairment is the term given to patients with cognitive impairment that is detectable by clinical criteria but does not produce impairment in daily functioning. When daily functioning is impaired as a result of cognitive decline, dementia is the appropriate syndromic label. Specific causes of dementia tend to have distinctive clinical presentations: the anterograde amnesic syndrome of Alzheimer disease; the syndrome of dementia with cerebrovascular disease; the syndrome of Lewy body dementia with its distinctive constellation of extrapyramidal features, disordered arousal, and dementia; the behavioral-cognitive syndrome of frontotemporal dementia; the primary progressive aphasias; and the rapidly progressive dementias. Because dementia syndromes have distinctive natural histories, precise diagnosis leads to a better understanding of prognosis. As new treatments become available for Alzheimer disease, the most common of the dementias, accurate diagnosis allows the appropriate patients to receive treatment.
The syndrome of progressive fronto-temporal dementia represents the clinical expression of several pathological processes, mostly degenerative, preferentially involving the frontal and temporal lobes. These processes include dementia with Pick bodies, cortico-basal degeneration, dementia with ITSNU (Inclusions Tau and Synuclein Negative, Ubiquitinated), also known as dementia of motorneuron disease type, dementia with basophilic inclusion bodies, and dementia lacking specific histopathology, and in addition all variants linked to mutations in the tau gene, located in chromosome 17. The term "Pick complex" encompasses these processes and their clinical manifestations, which in addition to fronto-temporal dementia include primary progressive aphasia and apraxic-motor syndromes. The pathologic processes are better discriminated by histopathology than distribution of atrophy, but the latter is the main determinant of the clinical presentation.
BACKGROUND AND PURPOSE: Pick complex (PC) is the name given to a group of diseases comprising Pick disease and its variations, all of which have a large degree of pathologic and clinical overlap. Because of this overlap, the observation of neuropathologic changes in vivo is difficult, although these changes play important roles in the criteria used for classification. The purpose of this study was to evaluate changes in brain metabolism in PC with proton chemical shift imaging ((1)H-CSI). METHODS: Nine patients with PC (three each with frontotemporal dementia, corticobasal degeneration [CBD], and primary progressive aphasia [PPA]) and five healthy subjects underwent (1)H-CSI. Volumes of interest were selected at the level of the basal ganglia by using a spin-echo sequence (TR/TE, 2000/13). Peak areas and ratios of N-acetylaspartate (NAA), creatine (Cr), and choline (Cho) were calculated in voxels in the basal ganglia and perisylvian regions. RESULTS: Reduced NAA/Cho ratios were observed in the right basal ganglia of the patients with PC. In patients with CBD or PPA, low NAA/Cr values were detected in the right perisylvian region. CONCLUSION: In PC, (1)H-CSI decreased NAA values in a wide area. Significantly reduced NAA levels in the right hemisphere in patients with PC suggests a neurodegenerative change and may reflect cases in which the right hemisphere is dominantly affected, compared with the left hemisphere. (1)H-CSI provided information that could not be obtained with other imaging techniques. Thus, (1)H-CSI may provide useful information for understanding the pathologic process underlying PC.
INTRODUCTION: This is the first part of a research work into primary and secondary language disorders (LD) in the stages of minimum to mild pathological deterioration in the degenerative process of cortical dementias. AIMS. In this first part, in the light of recent models of symbolic computation of language processing, we review the most significant publications on the subject matter. DEVELOPMENT: We begin with an introduction to the historical development of the approaches used to deal with the subject. Following that, we briefly describe the components of the language processing system (LPS) in the light of symbolic computation models. We then analyse the publications dealing with those models. Our aim was to determine whether the LD reported in those publications are the result of a primary involvement of any of the LPS components, or whether they are only the result of disorders affecting other components of the cognitive system. In this case, the supposed LD would be secondary and thus a communication, but not language, disorder. CONCLUSIONS: Despite the important variations from one individual to another that have been systematically observed by different authors, it seems that only LD belonging to so called primary progressive aphasia are actually cases of this disorder. In all the other dementias in the above mentioned stages of the development process these disorders are secondary to the involvement of other subsystems of the LPS.
This historical review describes the brain disease which afflicted the great impressionist-classicist composer Maurice Ravel (1875-1937). The usual interpretation of the symptoms Ravel exhibited during his disease is primary progressive aphasia / Pick's disease. Some authors see this as the cause for his lost musical creativity during the last years of his life. By contrast, in our review it is presented why a car accident in 1932, with the probable consequence of a mild to moderate traumatic brain injury, could be the key event in his life, triggering the loss of his ability to compose. In addition, the influence of Ravel's disease on his musical style is evaluated. Although some authors see a link, we try to explain why there is no clear evidence for this.
Explore the source record for details and available documents.
Clinical diagnosis of fronto-temporal dementia (FTD) and its rare variants (semantic dementia and primary progressive aphasia) represents a special challenge bearing in mind its difficulties and is important due to therapeutic differences with the most common Alzheimer's dementia. Of note, cholinesterase inhibitors commonly prescribed in Alzheimer's disease are not effective in FTD and a novel drug, memantine, has not been evaluated as yet. Neuropsychological evaluation is an important add-on to neurological, psychiatric and neuroimaging assessments in the clinical diagnosis of FTD. In this paper, we have described characteristics of neuropsychological deficits observed in FTD (such as attention, language, visuospatial and memory impairments) as well as tools used (tests) in clinical practice. Special attention is paid to the utility of neuropsychological examination in the differential diagnosis of FTD versus Alzheimer's disease, vascular dementia and other, less common forms of dementia.
Less-common causes of dementia are briefly discussed. Disorders covered include Pick's disease, primary progressive aphasia, progressive supranuclear palsy, Huntington's disease, olivopontocerebellar atrophies, closed head injury, dementia pugilistica, and Creutzfeldt-Jakob disease. Pseudodementia is also discussed. These disorders are compared and contrasted with the more common etiologies of dementia that are reviewed in other articles in the issue. Topics covered include treatment, differential diagnosis, clinical features, and pathogenesis.
PURPOSE: To determine whether magnetic susceptibility functional MR imaging of cerebral blood volumes provides information similar to fludeoxyglucose F 18 positron emission tomography (PET) brain images in patients undergoing evaluation for dementia. METHODS: Ten subjects were studied with both PET and functional MR. Clinical diagnoses included probable Alzheimer disease (n = 5), possible Alzheimer disease (n = 1), Pick disease (n = 2), and primary progressive aphasia (n = 2). The studies were quantitatively evaluated by coregistration of PET and functional MR images followed by regression analyses of corresponding regions of interest. Qualitatively, each brain was categorized into eight regions, and each was classified as normal or abnormal by visual inspection. RESULTS: Correlation coefficients between registered functional MR and PET images were excellent (mean, r = 0.58) in most of the cerebrum. Significant correlations were observed in 72 of 74 brain sections. Qualitatively, 16 brain regions were judged to be abnormal by both MR imaging and PET; 46 regions were normal by both; 10 regions were abnormal by PET only; and 8 regions were abnormal only by functional MR. The concordance between functional MR and PET was 78%, which was highly significant. CONCLUSION: Cerebral blood volumes images derived from magnetic susceptibility (functional MR) provide information similar to fludeoxyglucose F 18 PET images in demented patients undergoing evaluation for dementia.
Primary progressive aphasia is a rare disorder of unknown cause. We report a patient with progressive loss of speech output, a clinical variant of PPA, characterized by festinating speech. A 60-year-old right handed woman was admitted to our hospital, because of progressive deterioration of her speech. On admission, she was alert and orientated without dementia. A severe impairment of her articulation was observed: her speech rate was so fast that her speech became almost intelligible. The orofacial apraxia and difficulty in tapping were also present. The other neurological findings were normal. Neuroradiological studies showed the left perisylvian atrophy. Festinating speech has not been previously reported in patients with PPA; patient with PPA usually show a slow speech rate with effortful expression. Since festinating speech is occasionally present in the extrapyramidal disorders, such as Parkinson's disease, progressive supranuclear palsy, or pure akinesia, it appears likely that the combined lesions of the perisylvian region and the basal ganglia are responsible for her characteristic speech disorder with festinating speech.
A case study of a 65 year old man is described with an eight-year history of progressive primary non-fluent aphasia accompanied by agrammatism, phonemic paraphasias and mild spelling dysgraphia. His naming ability, however, has remained at an exceptionally high level and there has been no evidence of impairment of word or sentence comprehension. Non-verbal skills and memory functions have also been preserved within the range of his very high premorbid level of abilities. Single photon emission computed tomography was consistent with bifrontal hypoperfusion. We argue that the selective language deficits in this patient are characteristic of dynamic aphasia and of other speech disturbances which are also known to be associated with left frontal lesions. The possible underlying pathology is discussed in the context of known degenerative disorders.
Explore the source record for details and available documents.
Explore the source record for details and available documents.