1/10. An autopsy case of Alzheimer's disease presenting with primary progressive aphasia: a clinicopathological and immunohistochemical study.This report describes an autopsied Alzheimer's disease (AD) patient with primary progressive aphasia (PPA) as an early symptom. The patient developed a progressive speech disturbance at the age of 70 years, and difficulty in comprehension became apparent 2 years later. magnetic resonance imaging scan disclosed asymmetrical brain atrophy, predominantly on the left temporal lobe. At the age of 74 years, the patient's dementia rapidly progressed with parkinsonism and he died after a disease duration of 6 years. At autopsy, the brain showed a marked temporo-frontal lobe atrophy, predominantly on the left side. There was severe neuronal loss with gliosis and tissue rarefaction in the atrophied cerebral cortex and amygdala. Many neurofibrillary tangles with neuropil threads were found in the cerebral cortex. Numerous amyloid deposits were distributed throughout the cerebral cortex, accompanied by amyloid angiopathies. This patient was clinically diagnosed with temporal lobe-dominant Pick's disease, although the possibility of corticobasal degeneration was made. The neuropathological diagnosis was AD with asymmetrical brain atrophy and widespread amyloid angiopathies.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/10. 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.- - - - - - - - - - ranking = 1.6666666666667keywords = brain (Clic here for more details about this article) |
3/10. Progressive supranuclear palsy presenting with primary progressive aphasia--clinicopathological report of an autopsy case.We report a Japanese autopsy case of progressive supranuclear palsy (PSP). The male patient was 74 years old at the time of death. At age 64, he developed non-fluent aphasia that progressed slowly over 8 years, eventually associated with behavioral abnormality, postural instability, and dysphagia at 2 years prior to his death. magnetic resonance imaging of the brain at age 73 demonstrated marked atrophy of the frontal lobes, particularly on the left side. Neuropathological examination revealed the typical pathology of PSP: loss of neurons, gliosis, occurrence of neurofibrillary tangles, oligodendroglial coiled bodies, and tuft-shaped astrocytes in the frontal cortex, associated with argyrophilic threads in the underlying white matter, in the basal ganglia, including the thalamus, globus pallidus, and subthalamic nucleus, and in the brainstem nuclei, including the substantia nigra, pontine nucleus, and inferior olivary nucleus. No astrocytic plaques or ballooned neurons were observed. Protein analysis revealed accumulation of hyperphosphorylated tau of 68 and 64 kDa consisting of the four repeat tau isoforms. We conclude that the present case represented PSP with an 8-year history of primary progressive aphasia (PPA). Although focal cortical symptoms in PSP are rare or absent, we should keep in mind the possibility of atypical PSP in which cortical pathology is predominant, particularly in the frontal lobe, and could result in PPA.- - - - - - - - - - ranking = 0.66666666666667keywords = brain (Clic here for more details about this article) |
4/10. Nothing to say, something to sing: primary progressive dynamic aphasia.We describe a 76-year-old man (ADY) with dynamic aphasia in the setting of a degenerative frontal lobe dementia: primary progressive dynamic aphasia. He displayed a striking paucity of propositional speech despite intact speech production, and preserved singing and prosody. Vocal expression in the verbal and musical domains was investigated in a series of neuropsychological experiments based on novel language and musical tasks that were designed to establish the nature and specificity of the verbal output deficit. The features of the language disorder indicated that the speech output pathway was disrupted at the early stage of generation of a new pre-verbal message. In contrast, tests of musical output demonstrated that the generation of new musical ideas was unimpaired. The domain-specificity of dynamic aphasia may result from the disruption of specific cognitive processes necessary for the creation of verbal messages, as well as selective damage of brain regions involved in language production.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
5/10. 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.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
6/10. frontotemporal dementia--Part I. history, prevalence, clinical forms.The authors report a comprehensive publication consisting of three parts going into the details of history, prevalence, clinical forms, differential diagnosis, genetics, molecular pathomechanism, pathology, clinical diagnosis and treatment of frontotemporal dementia (FTD). The first part of the present review deals with history, prevalence and clinical forms of FTD. The prototypical FTD with circumscribed atrophy was first described by Arnold Pick; Alois Alzheimer found the intraneural inclusions in the patients' brain. Later it was recognised that many patients had neither the atrophy nor the cellular changes, but genetic mutations have been identified. frontotemporal dementia is a degenerative condition with unknown etiology in the frontal and anterior temporal lobes of the brain. It is a progressive neurobehavioral syndrome characterized by early decline in social interpersonal conduct, early impairment in the regulation of personal conduct, early emotional blunting, and early loss of insight. There are no reliable epidemiological studies on the prevalence of FTD, but it is well-accepted that FTD is a common cause for dementia before the age of 65 (it constitutes approximately five percent of all irreversible dementias). The nomenclature of the FTD has been confusing and continues to be. Three major clinical syndromes can be identified: 1 frontal variant FTD (dementia of frontal type) in which changes in social behavior and personality predominate, 2. in semantic dementia (progressive fluent aphasia) there is a breakdown in the conceptual database which underlies language production and comprehension, 3. in progressive nonfluent aphasia the phonologic and syntactic components of language are affected. The authors report two cases, which can point to clinical symptoms and forms, and mention the problems of the differential diagnosis and therapy.- - - - - - - - - - ranking = 0.66666666666667keywords = brain (Clic here for more details about this article) |
7/10. alzheimer disease and nonfluent progressive aphasia.OBJECTIVE: To describe a patient with pathologically proven alzheimer disease (AD) who presented with a non-fluent progressive aphasic syndrome. DESIGN: Longitudinal neuropsychological assessment, structural (magnetic resonance imaging) and functional (single photon emission computed tomography) imaging, and postmortem brain examination. SETTING: memory and cognitive disorders clinic in a tertiary referral hospital. PATIENT: A 66-year-old man presented with a 5-year history of progressive nonfluent aphasia characterized by marked deficits in phonology and syntax with preservation of everyday abilities. His condition deteriorated rapidly and he died suddenly of a myocardial infarction 12 months later. RESULTS: Neuropsychological testing revealed mild global intellectual impairment with marked impairment of auditory verbal short-term memory, syntactic, and phonological abilities. His naming errors were predominantly phonological paraphasias. magnetic resonance imaging scans showed left perisylvian atrophy and results of a Tc 99m hexamethyl-propyleneamine-oxime single photon emission computed tomographic scan were normal. Postmortem pathological examination revealed typical AD pathological features with atypical distribution, involving predominantly perisylvian language areas, but sparing the medial temporal lobe. CONCLUSIONS: The language deficits in AD, which have received considerable attention, are thought to involve predominantly lexicosemantic processes. When AD presents as a relatively isolated language disturbance, the aphasia is usually of the fluent anomic type. To our knowledge, our patient represents the first fully documented case of progressive nonfluent aphasia with pathologically verified AD.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
8/10. Autosomal dominant progressive syndrome of motor-speech loss without dementia.This patient report describes a 68-year-old man with progressive dissolution in motor-speech without concomitant language or cognitive decline, with presumed autosomal dominant inheritance. Motor-speech impairments included marked difficulty in articulating words and in coordinating articulation, phonation, and respiration. brain imaging results revealed severe focal atrophy of the posterior frontal region extending to the anterior parietal and superior temporal regions bilaterally on structural (MRI) and functional (single photon emission computed tomography) brain imaging studies. The involved neural substrate represented the primary motor cortex, premotor cortex (supplementary motor area), and the postcentral gyrus. Familial history included similar difficulties in his mother, her sister, and his own sister. The isolated involvement of the motor-speech processes alone indicated that this syndrome was distinguishable from progressive aphasia associated with prominent loss of language and from Alzheimer's disease.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
9/10. A case of primary progressive aphasia. A 14-year follow-up study with neuropathological findings.Primary progressive aphasia has been clinically defined as a progressive language deficit leading to the dissolution of almost all language functions with relative preservation of other cognitive functions until late in the course of the disease. Two types of language impairment have been described for primary progressive aphasia, which differ with respect to the degree of fluency of spontaneous speech. Whereas some authors have emphasized non-fluency as a defining characteristic of primary progressive aphasia, others have proposed that phonemic rather than semantic paraphasias in naming, both in the fluent and the non-fluent subtype, should be used as a criterion to distinguish primary progressive aphasia from slowly progressive aphasia in other forms of degenerative brain disease. patients with fluent speech and semantic dementia, as typically seen in Alzheimer's disease, produce semantic paraphasias and circumlocutions rather than phonemic errors in naming. This paper reports the long-term follow-up of a patient with fluent aphasic speech, whose language profile over a decade was similar to that of patients with semantic dementia. Neuropathological examination revealed no evidence of Alzheimer's disease. Pick's disease or Pick variant, but showed spongiform changes of cortical layers (II and III) in temporal and, less severely, in frontal gyri. The present case indicates that semantic dementia is not a reliable indicator of probable Alzheimer's disease and supports the notion that there are different subtypes of primary progressive aphasia which cannot be defined by fluency or by the presence of phonemic paraphasia. Progress in identifying the neuropathological correlates of these subtypes in cases with lobar atrophy and spongiform changes should be expected from hereditary variants of progressive disorder.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
10/10. Slowly progressive pure word deafness.Among the reports of primary progressive aphasia, there are few about patients who exhibited progressive pure word deafness with detailed auditory and radiological examination as well as neuropsychological assessment. We describe a 67-year-old right-handed man who exhibited slowly progressive pure word deafness over a period of 9 years without exhibiting any other cognitive or mental deterioration. magnetic resonance imaging of his brain revealed generalized cortical atrophy, particularly in the left superior temporal region. Auditory examination revealed severe disability in discriminating each syllable or mora of Japanese words despite adequate auditory acuity. He also showed impairment in temporal auditory discrimination assessed by the click fusion test and the click counting test. His ability to discriminate meaningful environmental sounds was mildly impaired. We discuss the pathophysiology of slowly progressive pure word deafness over a period of many years which was not complicated by other language or cognitive dysfunctions.- - - - - - - - - - ranking = 0.33333333333333keywords = brain (Clic here for more details about this article) |
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