Week 1. 5 Dementia and PPA-Q.pdf

Aphasia due to progressive disease/dementia What is dementia? Acquired, progressive impairment of intellectual function Chronic, rather than temporary Affects memory and at least one other cognitive function Language Calculation Processing of visual information May affect personality Dementia is a s...

Aphasia due to progressive disease/dementia What is dementia? Acquired, progressive impairment of intellectual function Chronic, rather than temporary Affects memory and at least one other cognitive function Language Calculation Processing of visual information May affect personality Dementia is a syndrome caused by an underlying disease process Date Your Footer Here 1 Dementia Caused by diffuse, rather than focal damage Diffuse = widespread Focal = restricted to a specific brain region Normal Date Focal damage (T1 & T2) Your Footer Here Diffuse damage 2 Primary progressive aphasia (PPA) (Mesulam, 1982) A type of dementia where language processes affected first At least two years of isolated language impairment Memory problems, visuospatial deficits, and behavioral/personality change must NOT be present during this time Activities of daily living are limited ONLY by the language problem There must be no focal lesion (e.g., stroke) that could have caused the language problem Wilson, et al., 2012 Date 3 Underlying disease Progressive aphasia may result from a number of degenerative neurological diseases 60-70% fronto-temporal lobar degeneration (FTLD) 30-40% Alzheimer Disease Disease progression: First complaint is often anomia: “I know what it is, but I just can’t say the name of it...” Impaired object naming Word-finding difficulty in conversation From there, fluent or nonfluent aphasia may develop End stage is mutism Date Your Footer Here 4 Example of how people with PPA describe the problem Date Your Footer Here 5 How is progressive aphasia different from stroke-induced aphasia? (Mesulam et al. 2014) Progressive aphasia: Neuronal destruction can be selectively targeted; rarely complete destruction Gradual neuronal loss allows for reorganization PPA can be associated with selective damage to brain regions that often are not vulnerable to CVAs (e.g., anterior temporal lobes) → “For these reasons, the language disturbances in PPA do not fit syndromic patterns identified in stroke-induced aphasia, thus necessitating the development of a novel nomenclature and classification system” (p. 555) Date Your Footer Here 6 Diagnosis of PPA is a 2-step process (Gorno-Tempini et al., 2011) Basic PPA criteria (Mesulam, 2003) Classification into PPA variants based on: Speech production features (grammar, motor speech, sound errors, word-finding pauses) Repetition Single word/syntax comprehension Reading/spelling Naming Semantic knowledge 3 levels of diagnosis: Clinical Imaging-supported Definite pathologic Symptoms may overlap as disease progresses Date 7 Nonfluent/agrammatic variant AKA “progressive nonfluent aphasia” (PNFA); “nonfluent/agrammatic progressive aphasia” (NavPPA) Characteristics: Effortful, halting speech Hesitations, speech-sound errors Reduced length of utterances Poor sentence construction Comprehension may be spared early on Motor speech symptoms may also be present Dysarthria, apraxia of speech Atrophic regions primarily in anterior perisylvian distribution Date “left posterior fronto-insular atrophy” Gorno Tempini et al., 2011 8 Progressive Nonfluent aphasia (PNFA) Date 9 Semantic variant (semantic dementia) Selective impairment of semantic memory Naming & single-word comprehension severely impaired reduced category fluency (e.g. name as many animals as you can) reduced general knowledge Semantic impairment cuts across modalities Surface dyslexia/dysgraphia Relative sparing of grammatical and phonological aspects of language Atrophic regions primarily in bilateral (but L>R) anterior temporal lobes Semantic dementia Synonym judgment: SHIP-TOMB “do these words mean the same thing?” Logopenic variant Characteristics Anomia – word-finding difficulties Phonologic errors → impaired repetition beyond single words Speech rate may be slow Due to word-finding, as opposed to agrammatism & motor speech (as in PNFA) Atrophic regions primarily in left temporoparietal area Posterior temporal Supramarginal, angular gyri Gorno Tempini et al., 2011 Logopenic variant - PPA “Tell me about your occupation.”