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11/32. Abeta peptide 1-42, Tau protein and S-100B protein level in cerebrospinal fluid of three patients with primary progressive aphasia.

    Primary progressive aphasia (PPA) is a clinical syndrome characterized by a slowly progressive aphasia in the absence of accompanying signs of generalized dementia. While non-fluent PPA tends to progress frontally and is usually linked to frontotemporal degeneration, fluent PPA might be associated with both, frontotemporal degeneration or Alzheimer's disease. Although recent reports suggest that PPA belongs neuropathologically to the group of tauopathias, cerebrospinal fluid analysis has not been established as a means of diagnosis in PPA so far. In this paper we investigated Abeta peptide(1-42) (Abeta(1-42)), Tau protein and S-100B protein level in the cerebrospinal fluid of three patients with PPA. In all patients Tau protein and S-100B level were slightly elevated, however, Abeta(1-42) was found to be in normal range. Thus, our first results point to PPA being neurochemically linked to frontotemporal degeneration. ( info)

12/32. Modality-specific deterioration in naming verbs in nonfluent primary progressive aphasia.

    A longitudinal study of oral and written naming and comprehension of nouns and verbs in an individual (M. M. L.) with nonfluent primary progressive aphasia (PPA) is reported. M. M. L. showed progressive deterioration of oral naming of verbs well before deterioration of written naming of verbs and before deterioration of oral or written naming of nouns. Her comprehension of both nouns and verbs remained intact, at least relative to oral naming of verbs. Her performance is compared to that of two other individuals with nonfluent PPA, who were tested at two time points. These patients showed similar patterns with respect to grammatical word class (verbs more impaired than nouns) and modality (spoken production more impaired than written production), but somewhat different courses of deterioration. The modality-specific nature of the observed verb production deficits rules out a semantic locus for the grammatical class effects. The results provide a new source of evidence for the hypothesis that there are distinct neural mechanisms for accessing lexical representations of nouns and verbs in language production. ( info)

13/32. 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. ( info)

14/32. 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. ( info)

15/32. 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. ( info)

16/32. Primary progressive aphasia: reversed asymmetry of atrophy and right hemisphere language dominance.

    Primary progressive aphasia (PPA) is almost always associated with asymmetrical left hemisphere degeneration. The authors report that right hemisphere atrophy in a patient with PPA was associated with atypical right hemisphere dominance for language. This suggests that neuronal damage in PPA is therefore tightly linked to the underlying anatomy of the language network. ( info)

17/32. Is slowly progressive anarthria a "pure" motor-speech disorder? Evidence from writing performance.

    It is usually assumed that writing is normal in patients with anarthria, but a careful examination of the literature shows that they produce deletions, transpositions and insertions. Indeed, a matter of debate concerns the distinction between primary progressive aphasia (PPA) and slowly progressive anarthria (SPA). If writing deficits were purely linguistic errors, then there would be no reason to consider slowly progressive anarthria as distinct from non-fluent PPA. We report the case of a patient with SPA in whom writing abilities were specifically assessed. No lexical-semantic deficits were detected, but errors were deletions, substitutions or transpositions, with no frequency, length or lexicality effect; moreover, controls produced the same kind of errors during articulatory suppression. It is suggested that subvocal rehearsal plays a role in writing, allowing the conversion/assembly of the phonological string in a graphemic representation. Therefore, writing deficits do not appear to have a linguistic basis and SPA seems distinguishable from nonfluent forms of aphasia. ( info)

18/32. Primary progressive aphasia: a comparative study of progressive nonfluent aphasia and semantic dementia.

    Primary progressive aphasia (PPA), a degenerative disorder, is often misdiagnosed as Alzheimer's disease. Its subtypes, semantic dementia (SD), and progressive nonfluent aphasia (PNFA), are often difficult to differentiate from each other. Our objective was to highlight the differences in the language profiles of patients with SD and PNFA. To bring out these differences, we report two patients with PPA, one with SD and the other with PNFA. They were administered the Western aphasia battery (WAB) and a semantic battery, which assesses semantic memory. The profiles of language impairment on the WAB indicated that the patient with PNFA had syntactic errors in expressive speech but relatively preserved semantics and comprehension, whereas the patient with SD had preserved syntax but made semantic errors in expressive speech, and had impaired comprehension. There were differences in their performance on the semantic battery too. The patient with SD made relatively less errors on confrontation naming, although on the pointing task he failed to point to those line drawings, which he was unable to name on confrontation. In contrast, the finding of the PNFA patient was the reverse of this. Supplementing conventional neuropsychological tests with formal tests for assessment of language functions is useful in the early diagnosis of PPA. The performance of PPA patients on a detailed assessment of language that includes use of formal tests such as the semantic battery helps to differentiate PNFA from SD. ( info)

19/32. 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. ( info)

20/32. Progressive anomia with preserved oral spelling and automatic speech.

    We report a patient, Newton, with a progressive classical anomia resulting from focal degeneration of the left hemisphere. In naming tasks Newton spelt aloud picture names that he could not retrieve, indicating a dissociation between orthography and phonology. Unusually, his writing and letter-pointing performance were impaired and spelling was achieved only through alphabet recitation. A study of automatic speech tasks demonstrated strikingly preserved naming performance on automatic compared to nominative tasks. We argue that automatic tasks provide phonological cues that facilitate phonological activation. With progression of disease Newton has shown increasing difficulty reading and repeating words, which we interpret in terms of a progressive elevation in the threshold for activation of phonology. Phonological cueing of picture names has yielded superior naming than word reading and even repetition, a finding consistent with the notion that task characteristics influence likelihood of phonological activation and naming success, but contrary to the notion that there exist separate task-specific output systems. We conclude that Newton exhibits a unique pattern of deficits, which have theoretical relevance for the debate on the relationship between phonology and orthography, the role of automatic speech and the relationship between naming, reading and repetition. ( info)
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