Semantic defi cits in amyotrophic lateral sclerosis

Abstract Our objective was to investigate, and establish neuroanatomical correlates of, semantic defi cits in amyotrophic lateral sclerosis (ALS) and amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD), compared to semantic dementia (SD) and controls. Semantic defi cits were evaluated using a naming and semantic knowledge composite score, comprising verbal and non-verbal neuropsychological measures of single-word processing (confrontational naming, comprehension, and semantic association) from the Sydney Language Battery (SYDBAT) and Addenbrooke ’ s Cognitive Examination – Revised (ACE-R). Voxel based morphometry (VBM) analysis was conducted using the region of interest approach. In total, 84 participants were recruited from a multidisciplinary research clinic in Sydney. Participants included 17 patients with ALS, 19 with ALS-FTD, 22 with SD and 26 age- and education-matched healthy controls. Signifi cant semantic defi cits were observed in ALS and ALS-FTD compared to controls. The severity of semantic defi cits varied across the clinical phenotypes: ALS patients were less impaired than ALS-FTD patients, who in turn were not as impaired as SD patients. Anterior temporal lobe atrophy signifi cantly correlated with semantic defi cits. In conclusion, semantic impairment is a feature of ALS and ALS-FTD, and refl ects the severity of temporal lobe pathology.

[1]  J. Hodges,et al.  A visual MRI atrophy rating scale for the amyotrophic lateral sclerosis-frontotemporal dementia continuum , 2014, Amyotrophic lateral sclerosis & frontotemporal degeneration.

[2]  J. Hodges,et al.  Cortical atrophy in ALS is critically associated with neuropsychiatric and cognitive changes , 2013, Neurology.

[3]  L. Goldstein,et al.  Is language impairment more common than executive dysfunction in amyotrophic lateral sclerosis? , 2012, Journal of Neurology, Neurosurgery & Psychiatry.

[4]  J. Hodges,et al.  Grey and White Matter Changes across the Amyotrophic Lateral Sclerosis-Frontotemporal Dementia Continuum , 2012, PloS one.

[5]  T. Ferman,et al.  Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72 , 2011, Acta Neuropathologica.

[6]  David Heckerman,et al.  A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD , 2011, Neuron.

[7]  C. Lomen‐Hoerth,et al.  Clinical Phenomenology and Neuroimaging Correlates in ALS-FTD , 2011, Journal of Molecular Neuroscience.

[8]  Nick C Fox,et al.  Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. , 2011, Brain : a journal of neurology.

[9]  Michael Hornberger,et al.  Neural basis of music knowledge: evidence from the dementias. , 2011, Brain : a journal of neurology.

[10]  O. Hardiman,et al.  The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study , 2011, Journal of Neurology, Neurosurgery & Psychiatry.

[11]  D. Knopman,et al.  Predicting survival in frontotemporal dementia with motor neuron disease , 2011, Neurology.

[12]  B. Miller,et al.  Classification of primary progressive aphasia and its variants , 2011, Neurology.

[13]  K. Sleegers,et al.  Genetic variability in progranulin contributes to risk for clinically diagnosed Alzheimer disease , 2008, Neurology.

[14]  M. Swash,et al.  Electrodiagnostic criteria for diagnosis of ALS , 2008, Clinical Neurophysiology.

[15]  John R Hodges,et al.  The Addenbrooke's Cognitive Examination Revised (ACE‐R): a brief cognitive test battery for dementia screening , 2006, International journal of geriatric psychiatry.

[16]  Ronald Peeters,et al.  Knowledge of visual attributes in the right hemisphere , 2006, Nature Neuroscience.

[17]  S H Appel,et al.  Prevalence and patterns of cognitive impairment in sporadic ALS , 2005, Neurology.

[18]  Nick C Fox,et al.  Sporadic and familial dementia with ubiquitin-positive tau-negative inclusions: clinical features of one histopathological abnormality underlying frontotemporal lobar degeneration. , 2005, Archives of neurology.

[19]  John Suckling,et al.  Frontotemporal white matter changes in amyotrophic lateral sclerosis , 2005, Journal of Neurology.

[20]  Jan Kassubek,et al.  Global brain atrophy and corticospinal tract alterations in ALS, as investigated by voxel‐based morphometry of 3‐D MRI , 2005, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[21]  Stephen M Smith,et al.  Fast robust automated brain extraction , 2002, Human brain mapping.

[22]  Thomas E. Nichols,et al.  Nonparametric permutation tests for functional neuroimaging: A primer with examples , 2002, Human brain mapping.

[23]  J. Hodges,et al.  Motor neurone disease, dementia and aphasia: coincidence, co-occurrence or continuum? , 2001, Journal of Neurology.

[24]  James L. McClelland,et al.  No Right to Speak? The Relationship between Object Naming and Semantic Impairment:Neuropsychological Evidence and a Computational Model , 2001, Journal of Cognitive Neuroscience.

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

[26]  L. Goldstein,et al.  Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis (ALS) , 2000, Neuropsychologia.

[27]  M. Swash,et al.  El Escorial revisited: Revised criteria for the diagnosis of amyotrophic lateral sclerosis , 2000, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[28]  J. Cedarbaum,et al.  The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function , 1999, Journal of the Neurological Sciences.

[29]  Julie S. Snowden,et al.  Semantic Dysfunction in Frontotemporal Lobar Degeneration , 1999, Dementia and Geriatric Cognitive Disorders.

[30]  J R Hodges,et al.  Dementia and aphasia in motor neuron disease: an underrecognised association? , 1998, Journal of neurology, neurosurgery, and psychiatry.

[31]  M. Freedman,et al.  Frontotemporal lobar degeneration , 1998, Neurology.

[32]  P N Leigh,et al.  Frontal lobe dysfunction in amyotrophic lateral sclerosis. A PET study. , 1996, Brain : a journal of neurology.

[33]  J. Hodges,et al.  Generating ‘tiger’ as an animal name or a word beginning with T: differences in brain activation , 1996, Proceedings of the Royal Society of London. Series B: Biological Sciences.

[34]  P. Lovibond,et al.  The structure of negative emotional states: comparison of the Depression Anxiety Stress Scales (DASS) with the Beck Depression and Anxiety Inventories. , 1995, Behaviour research and therapy.

[35]  James L. McClelland,et al.  Structure and deterioration of semantic memory: a neuropsychological and computational investigation. , 2004, Psychological review.

[36]  R. Caselli,et al.  Cognitive impairment, frontotemporal dementia, and the motor neuron diseases , 2003, Annals of neurology.