‘Childhood dementia’ is, by its very name, confronting, complex and heartbreaking. Comprised of a group of rare, inherited, fatal neurodegenerative disorders, childhood dementias are characterized by unexpected and progressive memory loss and cognitive decline during early years of life, generally following a period of normal, healthy development. The prevalence of childhood dementia in Australia has been estimated at 5.6 per 100,000 children. Indeed, like all dementias, treatments are severely limited and there are no cures.
Batten disease, or neuronal ceroid lipofuscinosis (NCL), is one of the most common forms of childhood dementia and along with memory and cognitive decline, children and adolescents typically suffer vision loss, seizures, language and motor decline and, ultimately, premature death. Like other childhood dementias, managing the heterogeneity of Batten disease symptoms poses a significantly complex medical challenge and requires a coordinated multidisciplinary approach.
This paper examines the causes, signs and behavioural symptoms of childhood dementias, with a focus on Batten disease, and discusses the new education and training collaboration between Batten Disease Support & Research Association Australia and Dementia Support Australia, a joint-initiative that aims to provide ongoing behaviour support and advice to families and carers living with the devastating effects of Batten disease.