Chapter 13 Dementia in Young Adults

Abstract

Publisher Summary The chapter presents a discussion on dementia in young adults. The presentation of dementia in a young adult, defined here as a person who had achieved normal intellectual and emotional integrity by the age of 18 years and subsequently developed cognitive and behavioral deficit, is always a striking clinical phenomenon. The long list of conditions resulting in new onset of cognitive impairment among young adults can be grouped into three major categories: (1) Conditions in which the cognitive deficit is a sequel to a completed insult to the central nervous system (CNS). (2) Disorders in which the cognitive deficit is a result of a known, pre-existing, previously diagnosed chronic disease, which in advanced stages, at least in a portion of cases, is typically associated with dementia. (3) Disorders in which the cognitive deficit, with or without associated neurologic findings, is the chief presenting complaint. This chapter focuses on storage and metabolic diseases during early adulthood. During the early phase of diagnostic considerations, magnetic resonance imaging (MRI) and especially T2 and fluid attenuation inversion recovery images (FLAIR) images appear to be very helpful in suggesting a leukodystrophy. Adrenoleukodystrophy (ALD) is inherited in an X-linked recessive pattern. It is because of a defect in the ALD peroxisomal membrane protein (Xq28), which impairs the transport of very long chain fatty acids (VLCFA) or their derivatives into peroxisomes. Adult polyglucosan body disease (APGBD) is a rare condition among the leukodystrophies.