Citations

Background and purposeA subset of patients with Alzheimer's disease (AD) present with early and prominent language deficits. It is unclear whether the burden of underlying β‐amyloid pathology is associated with language or general cognitive impairment in these subjects.MethodsThe relationship between cortical β‐amyloid burden on [11C]Pittsburgh compound B (PiB) positron emission tomography (PET) and performance on the Montreal Cognitive Assessment (MoCA), the Wechsler Memory Scale − Third Edition (WMS‐III), the Boston Naming Test (BNT) and the Western Aphasia Battery (WAB) was assessed using regression and correlation analyses in subjects presenting with aphasia who showed β‐amyloid deposition on PiB PET.ResultsThe global PiB ratio was inversely correlated with MoCA (P = 0.02) and the WMS‐III Visual Reproduction (VR) subtest (VR I, P = 0.02; VR II, P = 0.04). However, the correlations between PiB ratio, BNT (P = 0.13), WAB aphasia quotient (P = 0.11) and WAB repetition scores (P = 0.34) were not significant.ConclusionThis study demonstrates that an increased cortical β‐amyloid burden is associated with cognitive impairment, but not language deficits, in AD subjects presenting with aphasia. The results suggest that β‐amyloid deposition could be partly contributing to impaired cognition in such patients whilst language dysfunction may be more influenced by other pathological mechanisms, perhaps downstream pathways of β‐amyloid deposition.

This study seeks to examine the effect of level of presentation on scaling of speech intelligibility of speakers with dysarthria. Previous data [Kim et al. (2007)] reported the somewhat surprising result that different presentation levels do not cause a significant change in speech intelligibility scores of speakers with dysarthria, but do for healthy speakers. The current study reports further data using the same speech stimuli, however, with a main focus on the across-conditions effect of presentation level, since the previous data were limited to within-conditions effects. Four conditions of level of presentation were generated by varying absolute level of presentation (high and low) and normalizing each utterance for peak vowel intensity (adjusted and nonadjusted). In this presentation, we will report how scaled speech intelligibility is affected by the level of presentation of speech samples produced by speakers with various types and severity of dysarthria and by healthy speakers. The results of this study will be helpful to understand the rationale of speech therapy for dysarthria, especially considering the frequent use of speech intelligibility and speech therapy techniques designed based on intensity variation of speech. [Work supported by NIH DC00319 and internal fund from Louisiana State University.]

Background and purposeTo describe speech, neurological and imaging characteristics of a series of patients presenting with progressive spastic dysarthria as the first and predominant sign of a presumed neurodegenerative disease.MethodsParticipants were 25 patients with spastic dysarthria as the only or predominant speech disorder. Clinical features, pattern of MRI volume loss on voxel‐based morphometry and pattern of hypometabolism on F18‐fluorodeoxyglucose positron emission tomography (FDG‐PET) scan are described.ResultsAll patients demonstrated speech characteristics consistent with spastic dysarthria, including strained voice quality, slow speaking rate, monopitch and monoloudness, and slow and regular speech alternating motion rates. Eight patients did not have additional neurological findings on examination. Pseudobulbar affect, upper motor neuron pattern limb weakness, spasticity, Hoffman sign and positive Babinski reflexes were noted in some of the remaining patients. Twenty‐three patients had electromyographic assessment and none had diffuse motor neuron disease or met El Escorial criteria for amyotrophic lateral sclerosis. Voxel‐based morphometry revealed striking bilateral white matter volume loss affecting the motor cortex (BA 4), including the frontoparietal operculum (BA 43) with extension into the middle cerebral peduncle. FDG‐PET showed subtle hypometabolism affecting the premotor and motor cortices in some patients, particularly in those who had a disease duration longer than 2 years.ConclusionsA neurodegenerative disorder that begins focally with spastic dysarthria due to involvement of the motor and premotor cortex and descending corticospinal and corticobulbar pathways is characterized. The descriptive label ‘progressive spastic dysarthria’ to best capture the dominant presenting feature of the syndrome is proposed.

We aimed to determine whether there is an association between teaching and the development of progressive speech and language disorders (SLDs). Occupation was compared between 100 patients with a progressive SLD, 404 patients with Alzheimer’s dementia, and the 2008 US census. In SLDs, the most common occupation was teacher (22%) versus 8% in Alzheimer’s dementia. The odds ratio (OR) of being a teacher in SLDs compared to Alzheimer’s dementia was 3.4 (95% confidence interval [CI] = 1.87-6.17). No differences were observed in the frequency of other occupations. The frequency of teachers was higher in SLDs compared to the US census, OR of 6.9 (95% CI = 4.3-11.1). Farming, forestry, and fishing occupations were more frequent in SLDs compared to the US census. We identified an association between progressive SLDs and the occupation of teaching. Since teaching is a communication demanding occupation, teachers may be more sensitive to the development of speech and language impairments.