ABSTRACT

References 766

In 1-5 per cent of patients with dementia, the underlying disorders are uncommon. Many of these patients develop dementia as part of a more prominent neurological syndrome (e.g. progressive supranuclear palsy (PSP)). However, awareness of these disorders may lead to effective treatments, or to better care from the knowledge of evolution of the disease and its attendant symptoms and signs. Those conditions with an underlying genetic basis may benefit from rapid scientific advances and/or require genetic counselling. This chapter attempts to summarize the clinical, diagnostic and therapeutic aspects of these disorders.