1/73. Relearning and subsequent forgetting of semantic category exemplars in a case of semantic dementia.Over 15 months of longitudinal assessment, a patient with semantic dementia, D.M., improved on tests of naming and category fluency for a specific set of items (Experiment 1). The authors attribute this to his home drill with the names of these concepts plus pictures and descriptions of them. In Experiment 2, D.M. produced significantly more exemplars on category fluency for semantic categories that he had been practicing at home than for nonpracticed categories, an effect that cannot be attributed to an inherent difference between the 2 sets because the fluency performance of control participants revealed no significant difference between the 2 sets. In Experiment 3, D.M. rehearsed some of his previously nonpracticed categories daily for a period of 2 weeks: His fluency scores on the experimental categories improved substantially, but they declined once he ceased the daily drill. The results are discussed with respect to current views of long-term memory, particularly new word learning and forgetting, and to current techniques for facilitating word finding in aphasia.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
2/73. Familial dementia with lewy bodies (DLB).We report a rare familial case of dementia with lewy bodies (DLB). The patient was a man who died at the age of 51. His parents were first cousins. Among three siblings, two were diagnosed as probable cases of DLB, and one was a possible case, according to the clinical diagnostic criteria of the consortium on DLB. Following the patient's autopsy, he was found to have had DLB without neurofibrillary tangles or senile plaques (pure form of diffuse lewy body disease). His other siblings have been followed for more than ten years. Although these patients with familial DLB displayed clinical variability, all three siblings showed progressive dementia of early onset and progressive language disorder with paraphasia and difficulty in finding words. Psychotic features were also seen in the three siblings. The patient's sister showed compulsive behavior, and the other two siblings showed symptoms of parkinsonism. Neuropathologically, in addition to the usual neuropathology of DLB, the autopsy findings showed numerous small spheroids in the stratum pyramidale from the subiculum to CA1 of the hippocampus. Significant neuronal loss in CA2-3 of the hippocampus was detected. Axonal flow disturbance may be involved in the hippocampal formations of this incidence of familial DLB.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
3/73. 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."- - - - - - - - - - ranking = 13.081778285812keywords = primary progressive, aphasia (Clic here for more details about this article) |
4/73. Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome.We report six patients with clinically diagnosed and electrophysiologically confirmed motor neurone disease (MND), in whom communication problems were an early and dominant feature. All patients developed a progressive non-fluent aphasia culminating in some cases in complete mutism. In five cases, formal testing revealed deficits in syntactic comprehension. comprehension and production of verbs were consistently more affected those that of nouns and this effect remained stable upon subsequent testing, despite overall deterioration. The classical signs of MND, including wasting, fasciculations and severe bulbar symptoms, occurred over the following 6-12 months. The behavioural symptoms ranged from mild anosognosia to personality change implicating frontal-lobe dementia. In three cases, post-mortem examination has confirmed the clinical diagnosis of MND-dementia. In addition to the typical involvement of motor and premotor cortex, particularly pronounced pathological changes were observed in the Brodmann areas 44 (Broca's area) and 45. The finding of a selective impairment of verb/action processing in association with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb representation is strongly connected to anterior cortical motor systems.- - - - - - - - - - ranking = 6keywords = aphasia (Clic here for more details about this article) |
5/73. 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.- - - - - - - - - - ranking = 4.0408891429062keywords = primary progressive, aphasia (Clic here for more details about this article) |
6/73. frontotemporal dementia: report of a familial case.The authors describe a 49-year-old woman (R.K.) who presented with one year of progressive frontal lobe dysfunction, including signs of expressive aphasia. Signs of parkinsonism were absent until late in the clinical course. Neuropsychologic testing and neuroimaging studies are described. The patient died at age 55, after 7 years of symptoms. family history was remarkable for a mother who died at the age of 45, after experiencing 7 years of progressive aphasia. R.K.'s brain showed asymmetric frontotemporal atrophy, which was more severe on the left side. Histopathologic analysis was remarkable for numerous tau-positive neurons with some classic-appearing Pick bodies and many ballooned neurons. Tau-positive glial cells were also present. The authors suggest that the abnormal tau aggregates are related to the symptoms experienced by affected members of this family.- - - - - - - - - - ranking = 2keywords = aphasia (Clic here for more details about this article) |
7/73. June 2001: 61 year old woman with confusion and obtundation.The June COM. A 61 year old female presents with a three week history of increasing confusion, lethargy and headache. A neurological exam revealed disorientation, mild expressive aphasia, bilateral papilledema, and a right pronator drift. She had a craniotomy and resection of tumor. The tumor histologically was consistent with a solitary fibrous tumor displaying malignant features of hypercellularity, marked nuclear atypia, high mitotic activity, and a high proliferation index. This case is unique as the first malignant variant of solitary fibrous tumor to be reported intracranially.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
8/73. frontotemporal lobar degeneration: a study in japan.frontotemporal lobar degeneration is the most common form of cortical dementia occurring in the presenium after Alzheimer's disease. We analyzed two types of frontotemporal dementia (FTD) and semantic dementia (SD) selected from a consecutive series of outpatients based on neuropsychological symptoms, psychiatric symptoms and abnormal behavior. In our series of 134 patients with primary degenerative dementia, there were 16 cases of FTD and 6 cases of SD. Patients with subgroups of FTD and patients with SD were distinguishable only by the presence of aphasia in the latter group. They were not distinguishable from one another by other neuropsychological examinations, behavioral abnormalities or psychiatric symptoms assessed with the Neuropsychiatric Inventory.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
9/73. Neuropsychological features of rapidly progressive dementia in a patient with an atypical presentation of Creutzfeldt-Jakob Disease.BACKGROUND: Creutzfeldt-Jakob Disease (CJD) is a degenerative disease of the brain, characterized by rapid and irreversible decline, with dementia, ataxia, myoclonus, and other neurological and neurobehavioral disorders associated with rapidly progressive spongiform encephalopathy. The mode of transmission and basic pathomechanism remain unclear. The clinical picture of CJD is highly diverse, producing a number of variants. MATERIAL AND methods: The patient to be described is a 68-year-old Polish female, JR, clinically diagnosed with CJD. The article presents the case history in detail, with particular emphasis on neuropsychological testing, which was initiated when the patient was still lucid and capable of cooperation. The first presenting symptom was agraphia, followed by hemianopsia and other vision disorders, culminating in visual hallucinations. As the progress of the disease accelerated there was rapidly progressive dementia, aphasia developing to organic mutism, myoclonus, hyperkinesia, ultimately loss of all verbal contact or voluntary movement. RESULTS: JR's neuropsychological parameters declined in a period of less than 3 months from near normal to levels characteristic of severe dementia. CONCLUSIONS: The clinical picture here presented is consistent with that of the Heidenhain variant of CJD, with spongiform encephalopathy beginning in the right occipital lobe. Several features of the case remain atypical, however, including the absence of the most common genetic mutation and the patient's long survival after onset.- - - - - - - - - - ranking = 1keywords = aphasia (Clic here for more details about this article) |
10/73. Jargonagraphia in a case of frontotemporal dementia.Jargonagraphia is known to occur after discrete brain lesions but not in primary degenerative dementia. We report a patient with frontotemporal dementia who developed jargonagraphia and nonfluent aphasia. Written output was graphically preserved but consisted of short words intermingled with abstruse neologisms. MRI showed predominant right frontotemporal cortical atrophy accompanied by white matter hyperintensities in the right anterior subcallosal periventricular region. diagnosis and MRI were corroborated by extensive neuropathological findings obtained 8 months later. The agraphia in this case is discussed with reference to both specific macroscopic and microscopic pathoanatomical lesions. We suggest that jargonagraphia can appear in frontotemporal dementia depending on the localization of lesions.- - - - - - - - - - ranking = 24.319136181969keywords = nonfluent aphasia, nonfluent, aphasia (Clic here for more details about this article) |
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