1/54. Primary progressive aphasia: a case report.We report a 69-year-old male patient whose motor aphasia started at the age of 61. The language disability remained isolated and progressed over a period of eight years without any additional cognitive deficits. Computed tomography (CT) and magnetic resonance imaging (MRI) showed moderate cortical atrophy with frontal dominance. Single photon emission tomography (SPECT) showed hypoperfusion in the frontotemporoparietal region, positron emission tomography (PET) demonstrated a global cortical reduction of glucose utilization with a lesser decrement in the occipital lobes. The clinical symptoms and the neuropsychological findings fit the diagnosis of primary progressive aphasia.- - - - - - - - - - ranking = 1keywords = primary progressive, aphasia (Clic here for more details about this article) |
2/54. Late acquired words in childhood are lost earlier in primary progressive aphasia.The psycholinguistic nature of the dissolution of the mental lexicon in a primary progressive aphasic patient was investigated in light of the general regression hypothesis that language dissolution is the inverse of language acquisition. Four years after onset of the symptoms, the patient scored 60% correct on a picture naming test, exhibiting some effects of lexical and sublexical age-of-acquisition, but 7 years after onset, he scored only 15% correct. While even a weak form of the regression hypothesis is not fully supported, age-of-acquisition effects seem to be preserved throughout the course of the lexical dissolution. Some implications are briefly discussed for future research.- - - - - - - - - - ranking = 3.0995354946636keywords = primary progressive, aphasia (Clic here for more details about this article) |
3/54. Forced hyperphasia and environmental dependency syndrome.A distinctive, language related fragment of the environmental dependency syndrome is described: compulsive, involuntary, environmentally dependent speaking. Because this syndrome represents the observe of aphasia, it is named forced hyperphasia. An 84 year old woman with acute left frontal infarction was admitted to hospital with gait disturbance, forced grasp reflex, and striking imitation behaviour. After 2 weeks her imitation behaviour disappeared, but an equally striking new behaviour emerged. In the presence of others she would call out the names of objects in the room, and also call out the actions and gestures of people in the room, even though she was not asked to do so, and even though she was asked to stop. For example, if the doctor scratched his nose, she said, "The doctor is scratching his nose." brain CT, MRI, and SPECT showed cerebral atrophy and a left superior frontal subcortical infarct. It is suggested that "forced hyperphasia" is a clinical fragment of the environmental dependency syndrome and that her compulsive, impulsive, involuntary release of spoken language resulted from the release of frontal inhibition of the complex reflex linking environmental cues to the set of motor, limbic, spatial, and linguistic associations underlying spoken language.- - - - - - - - - - ranking = 0.073094788666783keywords = aphasia (Clic here for more details about this article) |
4/54. Focal cortical dysplasia of the temporal lobe with late-onset partial epilepsy: serial quantitative MRI.We describe serial studies of focal cortical dysplasia causing temporal lobe seizures and progressive aphasia in a 54-year-old woman. Initially, MRI volumetry of the temporal lobes showed significant left cortical thickening corresponding to an elevated amino-acid uptake in the left temporoparietal and inferior frontal cortex on SPECT using 3-[123I]iodo-alpha-methyl-L-tyrosine (IMT). After 1 year there was severe shrinkage of the left temporal lobe, possibly the result of recurrent complex partial seizures.- - - - - - - - - - ranking = 0.073094788666783keywords = aphasia (Clic here for more details about this article) |
5/54. Primary progressive aphasia: a patient with stress assignment impairment in reading aloud.Surface dyslexia is a pattern of reading impairment which has been seldom described in Italian native speakers. We report the case of a female Italian patient, RM, suffering from primary progressive aphasia (PPA) of the fluent type, who presented stress assignment errors in reading aloud. In Italian these errors are considered to be strongly suggestive of surface dyslexia. We studied RM's reading performance in light of existing cognitive models on reading. Since the first assessment, she presented multi-level impairment involving pre-semantic, lexical-semantic and post-semantic stages. Her stress assignment errors have been interpreted as a generalisation of the most frequent tendency in Italian language: namely to assign stress to the penultimate syllable. In agreement with previous studies, our case suggests that surface dyslexia in PPA is not a monolithic entity but, on the contrary, that it may arise from impairment at various stages of the reading process.- - - - - - - - - - ranking = 0.92690521133322keywords = primary progressive, aphasia (Clic here for more details about this article) |
6/54. The role of the dynamic body schema in praxis: evidence from primary progressive apraxia.On an influential model of limb praxis, ideomotor apraxia results from damage to stored gesture representations or disconnection of representations from sensory input or motor output (Heilman & Gonzalez Rothi, 1993; Gonzalez Rothi et al., 1991). We report data from a patient with progressive ideomotor limb apraxia which cannot be readily accommodated by this model. The patient, BG, is profoundly impaired in gesturing to command, to sight of object, and to imitation, but gestures nearly normally with tool in hand and recognizes gestures relatively well. In addition, performance is profoundly impaired on imitation of meaningless gestures and on tasks requiring spatiomotor transformations of body-position information. We provide evidence that BG's apraxia is largely attributable to impairments external to the stored gesture system in procedures coding the dynamic positions of the body parts of self and others; that is, the body schema. We propose a model of a dynamic, interactive praxis system subserved by posterior parietal cortex in which stored representational elements, when present, provide "top-down" support to spatiomotor procedures computed on-line. In addition to accounting for BG's performance, this model accommodates a common pattern of ideomotor apraxia more readily than competing accounts.- - - - - - - - - - ranking = 2.2457250719972keywords = primary progressive (Clic here for more details about this article) |
7/54. Classic Pick's disease type with ubiquitin-positive and tau-negative inclusions: case report.We report on a patient presenting Pick's disease similar to the one reported by Pick in 1892, with ubiquitin-positive and tau-negative inclusions. His diagnosis was made on the basis of clinical (language disturbance and behavioural disorders), neuropsychological (progressive aphasia of the expression type and late mutism), neuroimaging with magnetic resonance (bilateral frontal and temporal lobes atrophy) and brain single photon emission computed tomography (frontal and temporal lobes hypoperfusion) studies. Macroscopic examination showed atrophy on the frontal and temporal lobes. The left hippocampus displayed a major circumscribed atrophy. The diagnostic confirmation was made by the neuropathological findings of the autopsy that showed neuronal loss with gliosis of the adjacent white matter and apearance of status spongiosus in the middle frontal and especially in the upper temporal lobes. There were also neuronal swelling (ballooned cell) and argyrophilic inclusions (Pick's bodies) in the left and right hippocampi. Anti-ubiquitin reaction tested positive and anti-tau tested negative.- - - - - - - - - - ranking = 0.073094788666783keywords = aphasia (Clic here for more details about this article) |
8/54. Argyrophilic grain disease mimicking temporal Pick's disease: a clinical, radiological, and pathological study of an autopsy case with a clinical course of 15 years.This report concerns an autopsy case of argyrophilic grain disease (AGD) mimicking temporal Pick's disease. The patient was a Japanese woman without hereditary burden who was 89 years old at the time of death. She developed memory impairment and began wandering at the age of 74, followed by prominent character changes about 6 years after disease onset. A neurological examination 5 months before her death revealed poor rapport, unconcern, severe dementia, and double incontinence, without aphasia or muscle rigidity. Serial neuroradiological examination revealed progressive enlargement of the bilateral inferior horns of the lateral ventricle, reflecting progressive atrophy of the medial temporal lobes. Macroscopically, neuropathological examination showed circumscribed atrophy of the bilateral amygdalae, hippocampi, parahippocampal gyri, and lateral occipitotemporal gyri. Histologically, there was neuronal loss in the areas mentioned above, the caudate nucleus, putamen, thalamus, substantia nigra, and locus ceruleus, with ballooned neurons in the cerebral cortex and amygdala. Numerous argyrophilic grains with coiled bodies were present not only in the limbic system, but also in the affected cerebrum. Rare neurofibrillary changes were present in the limbic areas, consistent with Braak stage II, with no senile plaques. Based on these findings and a review of the literature, we note that AGD is clinicopathologically similar not only to mesolimbocortical dementia, but also to atypical senile dementia of Alzheimer type. This report may contribute to the elucidation of the clinicopathological hallmarks of AGD.- - - - - - - - - - ranking = 0.073094788666783keywords = aphasia (Clic here for more details about this article) |
9/54. 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 = 0.073094788666783keywords = aphasia (Clic here for more details about this article) |
10/54. 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 = 0.17668932212483keywords = nonfluent, aphasia (Clic here for more details about this article) |
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