Very Early Semantic Dementia With Progressive Temporal Lobe Atrophy

Czarnecki, Kathrin; Duffy, Joseph R.; Nehl, Carissa R.; Cross, Shelley A.; Molano, Jennifer Rose V.; Jack, Clifford R.; Shiung, Maria M.; Josephs, Keith A.; Boeve, Bradley F.

doi:10.1001/archneurol.2008.507

Cited by 29 publications

(20 citation statements)

References 20 publications

(19 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, few patients with C9ORF72-associated slowly progressive bvFTD have been reported (Khan et al, 2012), but in this study the longest follow-up period was 7 years. Czarnecki et al (2008) describe a patient with a slowly progressive course of semantic variant PPA, but the possible genetic cause of the disease is not known in this case. We had a unique opportunity to follow up a patient with C9ORF72-associated FTLD for two decades and were able to obtain diverse longitudinal information of the neurological and neuropsychological progression of his disease.…”

Section: Discussionmentioning

confidence: 97%

Slowly progressive frontotemporal lobar degeneration caused by theC9ORF72repeat expansion: a 20-year follow-up study

et al. 2014

View full text Add to dashboard Cite

A hexanucleotide expansion in chromosome 9 open-reading frame 72 (C9ORF72) has been found to be a major cause of frontotemporal lobar degeneration (FTLD). We describe a 20-year follow-up of a unique case with very slowly progressive FTLD caused by the C9ORF72 repeat expansion. In serial neuropsychological examinations, the patient's cognitive decline was exceptionally slow and after 20 years the patient still was mainly independent in activities of daily living. Our case indicates that there is great individual variation in the progression and duration of C9ORF72-associated FTLD, and also language variants or mixed phenotypes may be present.

show abstract

Section: Discussionmentioning

confidence: 97%

Slowly progressive frontotemporal lobar degeneration caused by theC9ORF72repeat expansion: a 20-year follow-up study

et al. 2014

View full text Add to dashboard Cite

show abstract

“…Naturally, the relationship between progressive fluent aphasia and semantic dementia will be questioned. Some, but not all patients with similar presentations have gone on to develop semantic dementia (Czarnecki et al, 2008; Mesulam et al, 2012). Others may point to the tests we used to assess for breakdown of semantic knowledge, which were not as comprehensive as our assessment of language function.…”

Section: Discussionmentioning

confidence: 99%

Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech

Botha

Duffy

Whitwell

et al. 2015

Cortex

Self Cite

132

128

View full text Add to dashboard Cite

The consensus criteria for the diagnosis and classification of primary progressive aphasia (PPA) have served as an important tool in studying this group of disorders. However, a large proportion of patients remain unclassifiable whilst others simultaneously meet criteria for multiple subtypes. We prospectively evaluated a large cohort of patients with degenerative aphasia and/or apraxia of speech using multidisciplinary clinical assessments and multimodal imaging. Blinded diagnoses were made using operational definitions with important differences compared to the consensus criteria. Of the 130 included patients, 40 were diagnosed with progressive apraxia of speech (PAOS), 12 with progressive agrammatic aphasia, 9 with semantic dementia, 52 with logopenic progressive aphasia, and 4 with progressive fluent aphasia, while 13 were unclassified. The PAOS and progressive fluent aphasia groups were least impaired. Performance on repetition and sentence comprehension was especially poor in the logopenic group. The semantic and progressive fluent aphasia groups had prominent anomia, but only semantic subjects had loss of word meaning and object knowledge. Distinct patterns of grey matter loss and white matter changes were found in all groups compared to controls. PAOS subjects had bilateral frontal grey matter loss, including the premotor and supplementary motor areas, and bilateral frontal white matter involvement. The agrammatic group had more widespread, predominantly left sided grey matter loss and white matter abnormalities. Semantic subjects had bitemporal grey matter loss and white matter changes, including the uncinate and inferior occipitofrontal fasciculi, whereas progressive fluent subjects only had left sided temporal involvement. Logopenic subjects had diffuse and bilateral grey matter loss and diffusion tensor abnormalities, maximal in the posterior temporal region. A diagnosis of logopenic aphasia was strongly associated with being amyloid positive, (46/52 positive). Our findings support consideration of an alternative way of identifying and categorizing subtypes of degenerative speech and language disorders.

show abstract

“…32 Patients can become obsessive and have passionate changes in political or religious views that are antithetical to previous beliefs. Their personality is often described as cold and they have little empathy for others.…”

Section: Primary Progressive Aphasia Syndromesmentioning

confidence: 99%

Primary progressive aphasia: clinicopathological correlations

2010

View full text Add to dashboard Cite

Primary progressive aphasia (PPA) is a disorder of declining language that is a frequent presentation of neurodegenerative diseases such as frontotemporal lobar degeneration. Three variants of PPA are recognized: progressive nonfluent aphasia, semantic dementia, and logopenic progressive aphasia. In an era of etiology-specific treatments for neurodegenerative conditions, determining the histopathological basis of PPA is crucial. Clinicopathological correlations in PPA emphasize the contributory role of dementia with Pick bodies and other tauopathies, TDP-43 proteinopathies, and Alzheimer disease. These data suggest an association between a specific PPA variant and an underlying pathology, although many cases of PPA are associated with an unexpected pathology. Neuroimaging and biofluid biomarkers are now emerging as important adjuncts to clinical diagnosis. There is great hope that the addition of biomarker assessments to careful clinical examination will enable accurate diagnosis of the pathology associated with PPA during a patient’s life, and that such findings will serve as the basis for clinical trials in this spectrum of disease.

show abstract

Very Early Semantic Dementia With Progressive Temporal Lobe Atrophy

Cited by 29 publications

References 20 publications

Slowly progressive frontotemporal lobar degeneration caused by theC9ORF72repeat expansion: a 20-year follow-up study

Slowly progressive frontotemporal lobar degeneration caused by theC9ORF72repeat expansion: a 20-year follow-up study

Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech

Primary progressive aphasia: clinicopathological correlations

Contact Info

Product

Resources

About