Abstract

Professor John Hodges MD, FRCP, FRACP, F Med Sci John is Professor of Cognitive Neurology at the University of New South Wales based at the Neuroscience Research Australia where he co-directs the Frontotemporal Dementia Research Group (FRONTIER www.ftdrg.org).

John qualified in Medicine from London University with honours (1975) and undertook periods of psychiatric and neurological raining in Southampton, Oxford and San Diego and obtained his MD in 1988. From 1997 to 2007 he was the MRC Professor of Behavioural Neurology with a joint appointments in the Department of Clinical Neuroscience at Addenbrooke's Hospital and the MRC Cognition and Brain Sciences Unit Cambridge where he lead a multidisciplinary research group. He has a longstanding interest in many aspects of cognition particularly in the context of neurodegenerative disorders. His current research focuses on aspects of frontotemporal dementia. He is the author of over 400 journal articles and five books including Cognitive Assessment for Clinicians (OUP 2007), Early Onset Dementia (OUP) Frontotemporal Dementia Syndromes (CUP, 2007). His real passions remain jazz, cricket, ceramics and his family.

Frontotemporal Dementias (FTD) is a complex disorder with various presentations and a range of underlying pathologies. The symptomatology of FTD depends on the initial distribution of pathological changes in the brain. Those with orbitomesial frontal changes present changes in social cognition and behaviour (behavioural variant FTD) while those with anterior temporal lobe involvement manifest the syndrome of Semantic Dementia. Others with perisylvian pathology have Progressive Nonfluent Aphasia. A recently recognised variant is termed Logopenic Progressive Aphasia reflects pathology at the angular gyrus region.

There is also considerable overlap at a clinical and pathological level between FTD and both motor neuron disease and the Parkinsonian disorders. Up to a quarter of cases are inherited and unlike Alzheimer's disease, the pathology of FTD is heterogeneous involving a number of protein abnormalities including tau and TDP-43. The last decade has witnessed an explosion of knowledge concerning the pathology and genetics especially since the discovery of the C9orf72 mutation associated with both familial FTD and MND. The later is also associated with a high rate of psychosis.

Advances in neuropsychology and in brain imaging have facilitated the early diagnosis and differentiation of these disorders. The assessment of patients with potential FTD, and related disorders, depends upon a comprehensive evaluation of behavioural symptoms, cognition and language changes as well as brain imaging and ancillary investigations.

The talk will present an overview of FTD stressing the genesis of symptoms, such as impaired emotion and recognition and theory of mind, and will place this knowledge in the context of the genetics and biology of FTD.