A 56-year-old right-handed man suffered from progressive apraxia of speech (AOS), characterized by agrammatism and buccofacial apraxia. He also became mute at the later stages of the disease progression. At autopsy, the left precentral gyrus, pars opercularis, and hippocampus showed severe atrophy. Pick bodies and Pick cells were observed. In this report, we also review previous case reports of AOS. Pick's disease is among the most commonly associated of the major diseases. Brain lesions associated with AOS may be found in regions such as the precentral gyrus and the pars opercularis in the left hemisphere.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3556793 | PMC |
| http://dx.doi.org/10.1080/13554794.2011.654210 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
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).
View Article and Find Full Text PDFOral applause sign in progressive supranuclear palsy.
Cortex
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.
View Article and Find Full Text PDFInvestigating changes in connected speech in nonfluent/agrammatic primary progressive aphasia following script training.
Cortex
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.
View Article and Find Full Text PDFMolecular and clinical Insights into KMT2E-Related O'Donnell-Luria-Rodan syndrome in a novel patient cohort.
Eur J Med Genet
February 2025
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.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!