Making sense of progressive non-fluent aphasia: an analysis of conversational speech.

The speech of patients with progressive non-fluent aphasia (PNFA) has often been described clinically, but these descriptions lack support from quantitative data. The clinical classification of the progressive aphasic syndromes is also debated. This study selected 15 patients with progressive aphasia on broad criteria, excluding only those with clear semantic dementia. It aimed to provide a detailed quantitative description of their conversational speech, along with cognitive testing and visual rating of structural brain imaging, and to examine which, if any features were consistently present throughout the group; as well as looking for sub-syndromic associations between these features. A consistent increase in grammatical and speech sound errors and a simplification of spoken syntax relative to age-matched controls were observed, though telegraphic speech was rare; slow speech was common but not universal. Almost all patients showed impairments in picture naming, syntactic comprehension and executive function. The degree to which speech was affected was independent of the severity of the other cognitive deficits. A partial dissociation was also observed between slow speech with simplified grammar on the one hand, and grammatical and speech sound errors on the other. Overlap between these sets of impairments was however, the rule rather than the exception, producing continuous variation within a single consistent syndrome. The distribution of atrophy was remarkably variable, with frontal, temporal and medial temporal areas affected, either symmetrically or asymmetrically. The study suggests that PNFA is a coherent, well-defined syndrome and that varieties such as logopaenic progressive aphasia and progressive apraxia of speech may be seen as points in a space of continuous variation within progressive non-fluent aphasia.

[1]  M. L. Lambon Ralph,et al.  Generalization and Differentiation in Semantic Memory , 2008, Annals of the New York Academy of Sciences.

[2]  Guy B. Williams,et al.  The human perirhinal cortex and semantic memory , 2004, The European journal of neuroscience.

[3]  Guy B. Williams,et al.  Progressive non-fluent aphasia is associated with hypometabolism centred on the left anterior insula. , 2003, Brain : a journal of neurology.

[4]  J. Hodges,et al.  Semantic dementia. Progressive fluent aphasia with temporal lobe atrophy. , 1992, Brain : a journal of neurology.

[5]  A. Kertesz,et al.  Pragmatics in frontal lobe dementia and primary progressive aphasia , 1998, Journal of Neurolinguistics.

[6]  Bernard Laurent,et al.  Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes , 1996, Journal of the Neurological Sciences.

[7]  Jennifer L. Whitwell,et al.  Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech. , 2006, Brain : a journal of neurology.

[8]  Andrew Kertesz,et al.  The evolution and pathology of frontotemporal dementia. , 2005, Brain : a journal of neurology.

[9]  Eleanor M. Saffran,et al.  Immediate memory for word lists and sentences in a patient with deficient auditory short-term memory , 1975, Brain and Language.

[10]  E K Warrington,et al.  The anatomical localisation of selective impairment of auditory verbal short-term memory. , 1971, Neuropsychologia.

[11]  T. Shallice,et al.  Short-term memory impairment and spontaneous speech , 1977, Neuropsychologia.

[12]  Murray Grossman,et al.  The core and halo of primary progressive aphasia and semantic dementia , 2003, Annals of neurology.

[13]  R. Faber,et al.  Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. , 1999, Neurology.

[14]  M. Rogers,et al.  Dissolution of spoken language in primary progressive aphasia , 1998 .

[15]  F. Carella,et al.  Progressive dysarthria: definition and clinical follow-up , 2003, Neurological Sciences.

[16]  J. Hodges,et al.  Non-verbal semantic impairment in semantic dementia , 2000, Neuropsychologia.

[17]  Raymond Scott Turner,et al.  Clinical, neuroimaging, and pathologic features of progressive nonfluent aphasia , 1996, Annals of neurology.

[18]  Guy B. Williams,et al.  Development of an MRI rating scale for multiple brain regions: comparison with volumetrics and with voxel-based morphometry , 2009, Neuroradiology.

[19]  A Danek,et al.  Frontotemporale Lobärdegenerationen , 2009, Fortschritte der Neurologie-Psychiatrie.

[20]  Andrew Kertesz,et al.  The Diagnosis and Course of Frontotemporal Dementia , 2007, Alzheimer disease and associated disorders.

[21]  Kirrie J. Ballard,et al.  Patterns of language decline in non-fluent primary progressive aphasia , 1997 .

[22]  Andrew Kertesz,et al.  Primary progressive aphasia: Diagnosis, varieties, evolution , 2003, Journal of the International Neuropsychological Society.

[23]  M. Mesulam,et al.  Primary progressive aphasia: PPA and the language network , 2003, Annals of neurology.

[24]  M. Weiner,et al.  Cognition and anatomy in three variants of primary progressive aphasia , 2004, Annals of neurology.

[25]  K. Rankin,et al.  Anatomical correlates of early mutism in progressive nonfluent aphasia , 2006, Neurology.

[26]  Matthew A. Lambon Ralph,et al.  Progressive non-fluent aphasia is not a progressive form of non-fluent (post-stroke) aphasia , 2006 .

[27]  J. Gee,et al.  Confrontation Naming and Morphometric Analyses of Structural MRI in Frontotemporal Dementia , 2004, Dementia and Geriatric Cognitive Disorders.

[28]  J R Hodges,et al.  Nonfluent progressive aphasia and semantic dementia: A comparative neuropsychological study , 1996, Journal of the International Neuropsychological Society.

[29]  F Barkhof,et al.  Hippocampal atrophy on MRI in frontotemporal lobar degeneration and Alzheimer’s disease , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[30]  Karalyn Patterson,et al.  Clinical and pathological characterization of progressive aphasia , 2006, Annals of neurology.

[31]  Nancy Johnson,et al.  Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia , 2008, Annals of neurology.

[32]  Martin Rossor,et al.  Measurements of the amygdala and hippocampus in pathologically confirmed Alzheimer disease and frontotemporal lobar degeneration. , 2006, Archives of neurology.

[33]  P. Vitali,et al.  Right hemispheric dysfunction in a case of pure progressive aphemia: fusion of multimodal neuroimaging , 2004, Psychiatry Research: Neuroimaging.

[34]  T. Grabowski Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? , 2008 .

[35]  Sally Andrews,et al.  From inkmarks to ideas : current issues in lexical processing , 2006 .

[36]  J. Hodges,et al.  When More Yields Less: Speaking and Writing Deficits in Nonfluent Progressive Aphasia , 2004, Neurocase.

[37]  J. Hodges,et al.  Progressive supranuclear palsy presenting with dynamic aphasia. , 1996, Journal of neurology, neurosurgery, and psychiatry.

[38]  J. Hodges,et al.  Semantic dementia: a unique clinicopathological syndrome , 2007, The Lancet Neurology.

[39]  M. MacDonald,et al.  Sweet nothings: Narrative speech in semantic dementia , 2010 .

[40]  M. Grossman,et al.  A longitudinal study of sentence comprehension difficulty in primary progressive aphasia , 2005, Journal of Neurology, Neurosurgery & Psychiatry.

[41]  Bruce L. Miller,et al.  Progressive Nonfluent Aphasia and Its Characteristic Motor Speech Deficits , 2007, Alzheimer disease and associated disorders.

[42]  B. Miller,et al.  The logopenic/phonological variant of primary progressive aphasia , 2008, Neurology.

[43]  M. Grossman,et al.  Primary Progressive Aphasia: A Review , 2004, Neurocase.

[44]  J. Duffy Apraxia of speech in degenerative neurologic disease , 2006 .

[45]  T. Rogers,et al.  Semantic dementia and fluent primary progressive aphasia: two sides of the same coin? , 2006, Brain : a journal of neurology.