Synopsis

Childhood dementia is a collective term for over 100 neurodegenerative disorders that begin in childhood or adolescence, each marked by progressive cognitive decline. Most are rare or ultra-rare monogenic disorders with a combined birth frequency estimated at greater than 1 in 2,900 births. Despite their significant impact, fewer than 5% of these conditions have effective treatments and Australian health systems face significant challenges in meeting the complex and critical needs of affected patients and their families.

This presentation highlights Australian-led initiatives aimed at improving awareness, recognition, and clinical service provision for childhood dementia; drawing inspiration from the more established and successful frameworks used for adult-onset dementia, where increased community understanding and robust clinical support are driving better outcomes for patients and their families.

Speaker

Dr Nicholas Smith is a Consultant Neurologist and Department Head of the Neurology and Clinical Neurophysiology Service at The Women’s and Children’s Health Network, South Australia, where he maintains a subspecialist practice in the management of neurometabolic disease and paediatric cerebral neurodegenerative disease (Childhood Dementia). He holds a conjoint appointment with the Discipline of Paediatric Medicine, Adelaide University where his research group pursues a pre-clinical and translational programme focusing on the development of targeted pharmacological and genetic therapies for these disorders; he collaborates closely with national and international colleagues and multidisciplinary stakeholders to advocate for improved services for patients with childhood Dementia and their families.