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A talented artist developed a progressive aphasia syndrome associated with frontotemporal dementia (FTD). As her disease progressed, language and executive skills declined, but her paintings became freer and more original. She demonstrates that artistic development can occur in the setting of language-dominant types of FTD. The study of artistic development(More)
OBJECTIVE To characterize dementia-induced changes in visual art production. BACKGROUND Although case studies show altered visual artistic production in some patients with neurodegenerative disease, no case-controlled studies have quantified this phenomenon across groups of patients. METHOD Forty-nine subjects [18 Alzheimer disease, 9 frontotemporal(More)
The Wnt/β-catenin signalling pathway shares a key component, β-catenin, with the cadherin-based adhesion system. The signalling function of β-catenin is conferred by a soluble cytoplasmic pool that is unstable in the absence of a Wnt signal, whilst the adhesion function is based on a cadherin-bound, stable pool at the membrane. The cadherin complex is(More)
Grammatical performance of individuals with Williams syndrome (WS) has been reported as being unimpaired, despite their comparatively low IQ and poor general cognitive ability. Specific language impairment (SLI) is often seen as the converse of WS, showing poor linguistic ability relative to level of cognitive functioning. Detailed profiles of language(More)
BACKGROUND While the body axis is largely patterned along the anterior-posterior (A-P) axis during gastrulation, the central nervous system (CNS) shows dynamic changes in the expression pattern of Hox genes during neurulation, suggesting that the CNS refines the A-P pattern continuously after neural tube formation. This study aims at clarifying the role of(More)
Williams syndrome (WS) is a rare genetic disorder resulting from a deletion on chromosome 7. A number of studies have shown that individuals with WS have a superior linguistic profile compared to their non-verbal abilities, however the evidence has been inconclusive, as many studies have disputed such a profile. The vast majority of studies on WS have(More)
This paper uses a combination of perceptual and electropalatographic (EPG) analysis to explore the presence and characteristics of connected speech processes in the speech output of five older children with developmental speech impairments. Each of the children is shown to use some processes typical of normal speech production but also to use a range of(More)
This paper investigates whether people with Williams syndrome (WS) have prosodic impairments affecting their expression and comprehension of four main uses of intonation. Two adolescent males with WS were assessed using the PEPS-C battery, which considers prosodic abilities within a psycholinguistic framework, assessing prosodic form and function in both(More)
A combination of perceptual and electropalatographic (EPG) analysis is used to investigate speech production in three adolescent speakers with a history of cleft palate. All the subjects still sound markedly atypical. Their speech output is analysed in three conditions: diadochokinetic tasks; single word production; connected speech. Comparison of the EPG(More)
While most cranial ganglia contain neurons of either neural crest or placodal origin, neurons of the trigeminal ganglion derive from both populations. The Wnt signaling pathway is known to be required for the development of neural crest cells and for trigeminal ganglion formation, however, migrating neural crest cells do not express any known Wnt ligands.(More)