Senior Research Officer
+612 9399 1823
Dr Burrell graduated from medicine at the University of New South Wales in 2000. He later completed Neurology training at Royal Prince Alfred and Concord Hospitals. In 2008, Dr Burrell completed a fellowship in neuromuscular disease and neurophysiology at Concord Hospital. Dr Burrell was awarded a scholarship from the Motor Neurone Disease Research Institute of Australia and the National Health and Medical Research Council and has recently completed his PhD with Professor John Hodges and Professor Matthew Kiernan. He now holds a position as Senior Research Officer at Neuroscience Research Australia and works as a Staff Specialist Neurologist at Concord General Hospital. Dr Burrell’s research focuses on the overlaps between frontotemporal dementia and motor neuron disease, as well as using neurophysiological and imaging biomarkers to link clinical symptoms and pathology in dementia syndromes.
DR CRISTIAN LEYTON Honorary Research Officer
DR EMMA DEVENNEY PhD student
To establish the frequency, severity, relationship to bulbar symptoms, and neural correlates of syntactic comprehension deficits across the frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) disease spectrum. In total, 85 participants were included in the study; 20 amyotrophic lateral sclerosis (ALS), 15 FTD-ALS, 27 progressive nonfluent aphasia (PNFA), and 23 controls. Syntactic comprehension was evaluated in ALS, FTD-ALS, PNFA, and controls using the Test for Reception of Grammar. Voxel-based morphometry examined neuroanatomical correlates of performance. Syntactic comprehension deficits were detected in 25% of ALS (p = 0.011), 92.9% of FTD-ALS (p < 0.001), and 81.5% of PNFA (p < 0.001) patients. FTD-ALS was disproportionately impaired compared to PNFA. Impaired Test for Reception of Grammar performance was frequent in ALS with early bulbar involvement but did not correlate with bulbar impairment overall. Left peri-insular atrophy correlated with syntactic comprehension deficits. Syntactic comprehension deficits are frequent in FTD-ALS, more severe than in PNFA, and related to left peri-insular atrophy. A significant minority of ALS patients are impaired, but the relationship between bulbar symptoms and syntactic impairment is not understood.
Motor disturbance is the primary marker of disease severity in ALS, but behavioural and functional impairment are common, and may decline independently of motor function. As such, the FTDFRS may provide valuable information in the assessment and monitoring of ALS.