Young-Onset Dementia and Neurodegenerative Disorders of the Young With an Emphasis on Clinical Manifestations

Young-onset dementia (YOD) refers to a neurological ailment primarily affecting people below 65 years of age in roughly about 8% of cases found through various researches. The high rate of prevalence of secondary dementias among older patients proves that younger people show a better prognosis of the conditions causing dementia than older people. However, effective interventions have to be usually provided early in the course of cognitive decline to help facilitate cognitive improvement. The risk of development of prodromal dementia is high if there is a development of psychoses in middle-aged or older people. When there is a development of psychoses in middle to late life, the likelihood of this indicates prodromal dementia is high. The clinical presentation is quite variable and often subtle in frontotemporal dementia (FTD) but may be dominated by personality change, behavioral disturbances, motivation, or the loss of empathy. There is great heterogeneity in the probable causes of dementia in young age as compared to dementia in old age, and some observed differences also exist in the course and characteristics of the disease. These causes may range from the most probable cause such as Alzheimer's disease (AD) to causes with low probability, such as metabolic disorders and prion diseases. The symptoms of young-onset dementia include a gradual development of personality and behavioral changes over a period of years. However, in the initial stages of young-onset dementia, this change can be attributed to various issues, such as depression, marital problems, and menopause. Other neurodegenerative diseases such as Huntington's disease show presentations such as changes in personality, chorea, and depression that can be observed in patients in their early adulthood. A few other neurodegenerative disorders are myoclonic epilepsy with ragged red fibers (MERRF) and mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with presentations such as characterized muscle weakness, poor growth, problems with vision and hearing, and the involvement of the multi-organ system, including the central nervous system to name a few. There is also the prevalence of juvenile parkinsonism in the community, which represents a group of clinicopathological entities present before the age of 21. Young-onset Parkinson's disease (PD) (YOPD) appears to have the same pathological presentation as late-onset Parkinson's disease (LOPD). Recent researches have proved that "gene therapy" can be useful in the treatment and in preventing the progression of symptoms in cases of neurodegenerative diseases.