Progressive apraxia of speech (PAOS) is a 4R tauopathy characterized by difficulties with motor speech planning. Neurodegeneration in PAOS targets the premotor cortex, particularly the supplementary motor area (SMA), with degeneration of white matter (WM) tracts connecting premotor and motor cortices and Broca's area observed on diffusion tensor imaging (DTI). We aimed to assess flortaucipir uptake across speech-language-related WM tracts identified using DTI tractography in PAOS. Twenty-two patients with PAOS and 26 matched healthy controls were recruited by the Neurodegenerative Research Group (NRG) and underwent MRI and flortaucipir-PET. The patient population included patients with primary progressive apraxia of speech (PPAOS) and non-fluent variant/agrammatic primary progressive aphasia (agPPA). Flortaucipir PET scans and DTI were coregistered using rigid registration with a mutual information cost function in subject space. Alignments between DTI and flortaucipir PET were inspected in all cases. Whole-brain tractography was calculated using deterministic algorithms by a tractography reconstruction tool (DSI-studio) and specific tracts were identified using an automatic fiber tracking atlas-based method. Fractional anisotropy (FA) and flortaucipir standardized uptake value ratios (SUVRs) were averaged across the frontal aslant tract, arcuate fasciculi, inferior frontal-occipital fasciculus, inferior and middle longitudinal fasciculi, as well as the SMA commissural fibers. Reduced FA (p < .0001) and elevated flortaucipir SUVR (p = .0012) were observed in PAOS cases compared to controls across all combined WM tracts. For flortaucipir SUVR, the greatest differentiation of PAOS from controls was achieved with the SMA commissural fibers (area under the receiver operator characteristic curve [AUROC] = 0.83), followed by the left arcuate fasciculus (AUROC = 0.75) and left frontal aslant tract (AUROC = 0.71). Our findings demonstrate that flortaucipir uptake is increased across WM tracts related to speech/language difficulties in PAOS.
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Introduction: Apraxia of speech (AOS) is a motor speech disorder characterized by sound distortions, substitutions, deletions, and additions; slow speech rate; abnormal prosody; and/or segmentation between words and syllables. AOS can result from neurodegeneration, in which case it can be accompanied by the primary agrammatic aphasia (PAA), which when presenting together are called AOS+PAA. AOS can also be the sole manifestation of neurodegeneration, termed primary progressive AOS (PPAOS).
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December 2024
Department of Behavioral Neurology and Cognitive Neuroscience, Tohoku University Graduate School of Medicine, Japan.
The applause sign (AS) is a recognized phenomenon observed in progressive supranuclear palsy (PSP) and other neurological conditions where individuals produce over three claps following a request to clap only thrice after a demonstration. In this study, we introduced a novel linguistic phenomenon termed the oral applause sign (OAS) associated with the AS. The OAS is characterized by increased repetition counts of Japanese repetitive onomatopoeic words, such as uttering "pata-pata-pata" instead of the expected "pata-pata.
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December 2024
Department of Speech, Language and Hearing Sciences, University of Texas at Austin, United States; Department of Neurology, Dell Medical School, University of Texas at Austin, United States.
Script training is a speech-language intervention designed to promote fluent connected speech via repeated rehearsal of functional content. This type of treatment has proven beneficial for individuals with aphasia and apraxia of speech caused by stroke and, more recently, for individuals with primary progressive aphasia (PPA). In the largest study to-date evaluating the efficacy of script training in individuals with nonfluent/agrammatic primary progressive aphasia (nfvPPA; Henry et al.
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December 2024
Rare Diseases and Medical Genetics Unit, Bambino Gesù Children's Hospital, IRCSS, Rome, Italy.
O'Donnell-Luria-Rodan (ODLURO) syndrome is an autosomal dominant neurodevelopmental disorder mainly characterized by global development delay/intellectual disability, white matter abnormalities, and behavioral manifestations. It is caused by pathogenic variants in the KMT2E gene. Here we report seven new patients with loss-of-function KMT2E variants, six harboring frameshift/nonsense changes, and one with a 7q22.
View Article and Find Full Text PDFIntroduction: Non-fluent variant of primary progressive aphasia (nfvPPA) is a neurodegenerative disorder with a predominantly speech and language impairment. Apraxia of speech and expressive agrammatisms along with decreased speech fluency and impaired grammar comprehension are the most typical disorder manifestations but with the course of the disease other language disturbances may also arise. Most studies have investigated these symptoms individually, and there is still no consensus on whether they have similar or different neuroanatomical foundations in nfvPPA.
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